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1.  Pregnancy related symptomatic vertebral hemangioma 
Vertebral hemangiomas are benign vascular tumors of the spine that remain asymptomatic in most cases and incidentally encountered on imaging. Rarely, altered hemodynamic and hormonal changes during pregnancy may expand these benign lesions resulting in severe cord compression. The management of symptomatic vertebral hemangioma during pregnancy is controversial as modalities like radiotherapy and embolization are not suitable and surgery during pregnancy has a risk of preterm labor. Few cases of pregnancy related symptomatic vertebral hemangioma with marked epidural component have been reported in the literature. We report a case of 23-year-old primigravida who developed rapidly progressive paraparesis at 28 weeks of gestation and spine magnetic resonance imaging (MRI) revealed upper thoracic vertebral hemangioma with extensive extra-osseous extension and spinal cord compression. Laminectomy and surgical decompression of the cord was performed at 32 weeks of the pregnancy. There was significant improvement in muscle power after a week of surgery. Six weeks postoperatively she delivered a full term normal baby with subsequent improvement of neurologic deficit. Repeat MRI of dorsal spine performed at 3 months postoperatively showed reduced posterior and anterior epidural components of vertebral hemangioma.
PMCID: PMC3992751  PMID: 24753678
Epidural extension; laminectomy; pregnancy; spinal cord compression; vertebral hemangioma
2.  An intradural cervical chordoma mimicking schwannoma 
Journal of Injury and Violence Research  2012;4(3 Suppl 1): Paper No. 83.
Chordoma is a relatively rare tumor originating from the embryonic remnants of the notochord. This is an aggressive, slow growing and invasive tumor. It occurs mostly at the two ends of neuroaxis which is more frequent in the sacrococcygeal region. Chordoma in vertebral column is very rare. This tumor is extradural in origin and compresses neural tissues and makes the patient symptomatic. This tumor found extremely rare in the spinal region as an intradural tumor.
The present study reports a rare case of intradural chordoma tumor as well as its clinical manifestations and treatment options.
The patient was a 50-year-old female presented with 9 months history of progressively worsening neck pain, cervical spine chordoma resembling neurinoma and right arm numbness. Physical examination showed no weakness in her limbs, but she had upward plantar reflex and mild hyperreflexia. In a magnetic resonance imaging (MRI) scan of the cervical spine there was an ill-defined enhancing mass in the posterior aspect of C2-C3 body caused cord compression more severe in right side as well as foraminal scalloping. The patient underwent surgery and after midline posterior cervical incision and paravertebral muscle stripping a laminectomy was performed from C1 through C4 using a high speed drill. Needle biopsy revealed chordoma on frozen section and all of accessible parts of tumor were excised. The gross and microscopic histopathological appearance was consistent with chordoma.
Chordomas are malignant tumors that arise from remains of embryonic notochord. These ectopic rests of notochord termed “ecchordosis physaliphora “can be found in approximately 2% of autopsies. These are aggressive, slow growing, locally invasive and destructive tumors those occur in the midline of neuroaxis. They generally thought to account for 2% to 4% of all primary bone neoplasms and 1% to 4% malignant bone neoplasms. They are the most frequent primary malignant spinal tumors after plasmacytomas. The incidence has been estimated to be 0.51 cases per million. The most common location is sacrococcygeal region followed by the clivus. These two locations account for approximately 90% of chordomas. Of the tumors that do not arise in the sacrum or clivus, half occur in the cervical region, with the remainder found in the lumbar or thoracic region, in descending order of frequency. Cervical spine chordomas account for 6% of all cases. Distal metastasis most often occurs in young patients, those with sacrococcygeal or vertebral tumors, and those with atypical histological features. These tumors usually spread to contiguous anatomical structures, but they may be found in distant sites (skin, musculoskeletal system, brain, and other internal organs). Seeding of the tumor has also been reported, and the likely mechanism seems to be tumor cell of contamination during the surgical procedures. The usual radiological findings in chordomas of spine are destructive or lytic lesions with occasional sclerotic changes. They tend to lie anterolateral, rather than dorsal towards the cord, and reportedly known to invade the dura. The midline location, destructive nature, soft tissue mass formation and calcification are the radiological hallmarks of chordomas. Computed Tomography (CT) scan is the best imaging modality to delineate areas of osteolytic, osteosclerotic, or mixed areas of bone destruction.Chordoma is usually known as a hypovascular tumor which grows in a lobulated manner. Septal enhancement which reflects a lobulated growth pattern is seen in both CT and MRI and even in gross examination. Other epidural tumors include neurinoma, neurofibroma, meningioma, neuroblastoma, hemangioma, lymphoma and metastases. Their differentiation from chordoma may be difficult due to the same enhancement pattern on CT and MRI.
A dumbbell-shaped chordoma is a rare pathogenic condition. The dumbbell shape is a characteristic finding of neurinomas in spine but in spinal neurinomas extention to transverse foramina has not yet been reported. Although our case mimicked a dumbbell shaped neurogenic tumor, its midline location and destructive pattern were characteristic feature indicating a clue to the diagnosis of chordoma that was already confirmed with histopathology.
This unusual behavior of tumor extension can be explained by the soft and gelatinous nature of the tumor enabling the mass to extend or creep into the existing adjacent anatomical structures.
Cervical Chordoma, Intradural, Computed tomography
PMCID: PMC3571609
3.  Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature 
Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.
PMCID: PMC3243847  PMID: 22200026
Idiopathic hypertrophic spinal pachymeningitis; Spinal cord compression; Chronic nonspecific inflammation; Dural thickening
4.  Testicular seminomatous mixed germ cell tumor with choriocarcinoma and teratoma with secondary somatic malignancy: a case report 
Testicular tumors are a heterogeneous group of neoplasms exhibiting diverse histopathology and can be classified as seminomatous and non-seminomatous germ cell tumor types. Mixed germ cell tumors contain more than one germ cell component and various combinations have been reported. Here, we present a rare case of a mixed germ cell tumor composed of seminoma, choriocarcinoma and teratoma with a secondary somatic malignancy.
Case presentation
A 31-year-old Caucasian man presented with splenic rupture to our hospital. A right-sided testicular swelling had been present for 6 months and his alpha-fetoprotein, beta-human chorionic gonadotropin, and lactose dehydrogenase were increased. An ultrasound of his scrotum revealed an enlarged right testis with heterogeneous echogenicity. Multiple hypervascular lesions were noted in his liver and spleen. He underwent transcatheter embolization therapy of his splenic artery followed by splenectomy and right-sided orchiectomy. A computed tomography scan also showed metastasis to both lungs. During his last follow up after four cycles of cisplatin-based chemotherapy, the level of tumor markers had decreased, decreases in the size of his liver and pulmonary lesions were noted but new sclerotic lesions were evident in his thoracolumbar region raising concern for bony metastasis.
Prognosis of testicular tumor depends mainly on the clinical stage, but emergence of a sarcomatous component presents a challenge in the treatment of germ cell tumors and the histological subtype of this component can be used as a guide to specific chemotherapy in these patients.
PMCID: PMC3917416  PMID: 24380446
Mixed germ cell tumors; Rhabdomyosarcoma; Sarcomatous component
5.  Multilevel vertebral hemangiomas: two episodes of spinal cord compression at separate levels 10 years apart 
European Spine Journal  2005;14(7):706-710.
This case report presents a 66-year-old woman with multiple vertebral hemangiomas causing spinal cord compression at different levels with a long symptom-free interval between episodes of compression. She presented with back pain and progressive weakness and numbness in her lower limbs for 3 months. Ten years earlier, she had had a symptomatic T4 vertebral hemangioma operated successfully, and had made a full recovery. Magnetic resonance imaging (MRI) of the thoracic and lumbar spine revealed multiple thoracic and lumbar vertebral hemangiomas. Extraosseous extension of a hemangioma at T9 was causing spinal cord compression. Selective embolization was performed preoperatively, and cord decompression was achieved via anterior T9 corpectomy. The patient’s neurological status improved rapidly after surgery. After a course of radiotherapy, she was neurologically intact and could walk independently. One year later, MRI showed complete resolution of the cord edema at T9, and showed regression of the high signal intensity that had been observed at unoperated levels. These findings indicated diminished vascularity and reduced aggression of the tumor.
PMCID: PMC3489226  PMID: 15856339
Vertebral hemangioma; Cord compression; Embolization; Corpectomy
6.  Posterior Reversible Encephalopathy Syndrome in a Case of Postoperative Spinal Extradural Haematoma: Case Report and Review of Literature 
Asian Spine Journal  2011;5(1):64-67.
A 14-year-old girl presented with progressive paraparesis and paresthesia of one-year duration. Magnetic resonance imaging revealed a T6 vertebral hemangioma with epidural compression on the spinal cord. Following angiography and embolization, she underwent dorsal laminectomy and excision of the soft tissue component compressing the cord. In the postoperative period she had rapid worsening of lower limb power and imaging demonstrated an epidural haematoma at the operative site. The patient was taken up for urgent re-exploration and evacuation of haematoma. Postoperatively the patient complained of visual failure, headache and had multiple episodes of seizures. An magnetic resonance imaging brain showed characteristic features of posterior reversible encephalopathy syndrome (PRES) and the patient improved gradually after control of hypertension. This is the first documented case of PRES following spinal cord compression in a patient without any known risk factors. We postulate the possible mechanism involved in its pathogenesis.
PMCID: PMC3047900  PMID: 21386948
Posterior reversible encephalopathy syndrome; Magnetic resonance imaging; Hematoma; epidural; spinal
7.  Intramedullary Spinal Cord Metastasis of Choriocarcinoma 
The authors describe a case of choriocarcinoma that metastasized to the cerebral cortex, vertebral body, and intramedullary spinal cord. A 21-year-old woman presented with sudden headache, vomiting and a visual field defect. Brain computed tomography and magnetic resonance examinations revealed an intracranial hemorrhage in the left temporo-parietal lobe and two enhancing nodules in the left temporal and right frontal lobe. After several days, the size of the hemorrhage increased, and a new hemorrhage was identified in the right frontal lobe. The hematoma and enhancing mass in the left temporo-parietal lobe were surgically removed. Choriocarcinoma was diagnosed after histological examination. At 6 days after the operation, her consciousness had worsened and she was in a state of stupor. The size of the hematoma in the right frontal lobe was enlarged. We performed an emergency operation to remove the hematoma and enhancing mass. Her mental status recovered slowly. Two months thereafter, she complained of paraplegia with sensory loss below the nipples. Whole spine magnetic resonance imaging revealed a well-enhancing mass in the thoracic intramedullary spinal cord and L2 vertebral body. Despite chemotherapy and radiotherapy, the patient died 13 months after the diagnosis.
PMCID: PMC3358599  PMID: 22639709
Choriocarcinoma; Intracerebral hematoma; Intramedullary spinal cord metastasis; Spinal metastasis
8.  Management of Refractory Metastatic Anal Squamous Cell Carcinoma Following Disease Progression on Traditional Chemoradiation Therapy 
Case Study
Ms. S.G., a 56-year-old woman with a poorly differentiated squamous cell carcinoma of the anal canal, American Joint Committee on Cancer stage III (T2, N1, M0), was initially diagnosed in December, 2007 at an outside institution after she had noted blood in her stool for approximately 6 months. Her medical history was unremarkable. She had no known history of HIV or other sexually transmitted diseases. At the time of presentation, Ms. S.G. had an Eastern Cooperative Oncology Group performance status of 1 related to cancer-related pain. Her appetite and weight were both stable.
A complete colonoscopy demonstrated a large, immobile, ulcerated, firm, 4-cm lesion in the distal rectum, arising from the anal canal. Initial staging positron emission tomography/computed tomography (PET/CT) scan revealed a hypermetabolic inferior anorectal mass with left perirectal and presacral nodal metastases. There was no definite evidence of distant metastatic disease.
Ms. S.G. received chemoradiation treatment following her diagnostic studies, with a total dose of 45 Gy over 26 fractions to the pelvis with concurrent infusional fluorouracil (5-FU; 2, 450 mg over 7 days) and mitomycin C (12 mg/m2 on day 1) at an outside institution. However, during her chemoradiation therapy, Ms. S.G. experienced a 3-week treatment break due to severe radiation dermatitis, as recommended by her outside treating oncologist.
Upon treatment completion, Ms. S.G. underwent a biopsy of the anal canal, which revealed no evidence of residual malignancy. As recommended by her treating oncologist, she received four additional cycles of adjuvant infusional 5-FU in combination with leucovorin. Shortly thereafter, Ms. S.G. developed progressive pelvic pain. She underwent a second PET/CT scan, revealing mixed findings: interval resolution of abnormal standardized uptake value (SUV) activity at the primary tumor in the anal canal, but an increase in the size and SUV of nodal disease within the left perirectal and presacral regions. A CT-guided biopsy noted a perirectal abscess requiring drainage but was inconclusive for disease recurrence; Ms. S.G. was treated with IV antibiotics.
Six weeks later, repeat radiographic imaging noted additional changes suspicious for regional recurrence, which was biopsy-confirmed. Ms. S.G. was subsequently referred to MD Anderson Cancer Center for consideration of salvage pelvic exenteration.
On physical exam a mass was palpated in the left lower quadrant, but there was no evidence of inguinal adenopathy. On digital rectal exam there was notable external erythema with a fixed mass and moderate sphincter tone. A chest CT scan showed no definite evidence of metastatic disease, but an MRI of the abdomen/pelvis indicated the presence of a complex partially necrotic mass (7.6 × 4.9 × 7.3 cm3) extending to the rectosigmoid junction, inseparable from the left lateral bowel wall, with partial encasement of the bowel. In addition, there was infiltration of the left piriformis muscle and cervix consistent with local recurrence. She was referred to medical oncology and radiation oncology for consideration of reirradiation with concurrent neoadjuvant chemotherapy for palliation and possible surgical resection.
In early December 2008, Ms. S.G. received intensity-modulated radiation therapy (IMRT), with a total dose of 27 Gy over 18 fractions. She received concurrent infusional 5-FU at 300 mg/m2/day, from Monday to Friday, on the days of radiation. She also received a weekly bolus dose of cisplatin at 20 mg/m2. The intent was to treat to 30 Gy, but the patient deferred further treatment early due to anorectal irritation. She then underwent restaging with a PET/CT scan and a pelvic MRI in February 2009, revealing radiographic partial response of the known pelvic recurrence and reduced pelvic pain (Figures 1A and 1B). Figure 1 Figure 1. Contrast-enhanced axial MRI image of the lower pelvis. (A) Pretreatment, complex mass at the rectosigmoid junction measuring approximately 7.6 × 4.9 × 7.3 cm3. (B) Posttreatment, large necrotic mass measuring 3–4 cm in greatest dimension.
Unfortunately, in the interim, she developed multiple bilateral liver lesions and punctate pulmonary nodules consistent with distant disease (Figures 2A, 2B, and 3A). Figure 2 Figure 2. Contrast-enhanced axial CT images of the lung. (A) Subcentimeter nodular opacity in the left upper lobe. (B) Subcentimeter opacity in the right upper lung lobe. Figure 3 Figure 3. Contrast-enhanced axial CT image of the liver. (A) Pretreatment, multiple bilateral liver lesions. (B) Posttreatment, near-complete resolution of liver lesions.
Ms. S.G. proceeded to undergo systemic chemotherapy with carboplatin at an area under the concentration-time curve of 5 and paclitaxel at 175 mg/m2 day 1, every 21 days. She tolerated the treatment well. After three cycles of chemotherapy, radiographic imaging indicated a mixed response to treatment: interval resolution of the pulmonary nodules, stability of disease in the pelvic mass, but progression of the hepatic metastases.
Given Ms. S.G.'s continuing excellent performance status, further treatment was recommended. Based on recent published literature, a regimen of cisplatin at 80 mg/m2 day 1, vinorelbine at 25 mg/m2 day 1 (repeated every 28 days), and weekly cetuximab (VCC) at 250 mg/m2 was initiated. Remarkably, following three cycles of treatment, despite receiving multiple prior lines of chemotherapy, her restaging CT scan demonstrated complete radiographic response of the intrathoracic disease, stable response of the anorectal mass, and near-complete resolution of the hepatic lesions (Figures 3A and 3B).
Overall, Ms. S.G. had tolerated her treatment very well. Given her response and tolerability, she was evaluated again for curative surgical resection. However, she opted to receive the VCC regimen closer to home and was lost to follow-up. Unfortunately, we were unable to obtain medical records confirming if she indeed received additional treatment as recommended. Ms. S.G. was noted to have passed away due to progression of her disease approximately 6 months later.
PMCID: PMC4093318
9.  Preoperative Sclerotherapy Using Sodium Tetradecyl Sulphate (Fibro-Vein™) Can Assist in the Management of Vertebral Hemangiomas 
Global Spine Journal  2012;2(3):169-174.
Vertebral hemangiomas are benign lesions accounting for 2 to 3% of all spinal tumors. They are usually asymptomatic and found incidentally on imaging. Uncommonly, vertebral hemangiomas with significant epidural extension can result in radiculopathy or spinal cord compression. Decompressive surgery with or without stabilization is often required when neurological deficits are present. However, surgery can be associated with massive hemorrhage as these tumors are hypervascular. Preoperative embolization and sclerotherapy are well-known management strategies used to minimize intraoperative bleeding and improve symptoms. Recently, the use of sclerosants such as ethanol has decreased, due to reported complications such as Brown–Sequard syndrome. We describe the use of sodium tetradecyl sulfate (Fibro-Vein™, STD Pharmaceutical, Hereford, UK) as an effective alternative to ethanol in the preoperative management of vertebral hemangiomas. To our knowledge, this has not been previously reported. In three patients, we demonstrated minimal intraoperative blood loss using a combination of preoperative embolization of arterial feeders and sclerotherapy with sodium tetradecyl sulfate to control and secure venous drainage. No patients developed complications related to the procedure. In addition to minimal blood loss, a clear dissection plane was also noted intraoperatively.
PMCID: PMC3864480  PMID: 24353964
vertebral hemangioma; sclerotherapy; Fibro-Vein™
10.  Lumbar Spinal Extradural Angiolipoma: Case Report and Review of the Literature 
Angiolipomas in the lumbar spinal region are extremely rare. The present report describes the identification of such a tumor and its removal, and discusses the tumor characteristics and prognosis. A 74-year-old woman was presented with a 5-month history of lower back pain. Severe radiculopathy was experienced in the left leg for 5 days prior to the presentation, and there were no neurological deficits. Magnetic resonance (MR) images showed an approximately 3.5 cm heterogeneously enhanced and elongated mass at the left L5-S1 level. A portion of the mass appeared with high signal intensity on T2-weighted MR images, with low signal intensity on T1-weighted images, and with high signal intensity on T1 fat suppression enhancement images. Resection of the tumor was approached via an L5 and S1 laminectomy. A fibrous sticky yellowish hypervascular tumor was identified. Histological study revealed the tumor as an angiolipoma. Symptoms were relieved after tumor excision, and there were no neurological sequelae. Although extremely rare, lumbar epidural angiolipoma should be considered in the differential diagnosis of lumbar spinal epidural lesions. The prognosis after surgical management of this lesion is favorable.
PMCID: PMC2588319  PMID: 19096690
Angiolipoma; Spinal neoplasm; Lumbar epidural tumor
11.  Unusual presentation of giant cell tumor originating from a facet joint of the thoracic spine in a child: a case report and review of the literature 
Giant cell tumor of the synovium is a common benign lesion that frequently occurs at the tendon sheaths in the hand; it is usually found in adults over 30 years old. It is related to pigmented villonodular synovitis. Giant cell tumor of the synovium or pigmented villonodular synovitis has been described rarely in the axial skeleton especially in the thoracic vertebrae of a child.
Case presentation
A previously healthy 7-year-old Thai girl presented with back pain and progressive paraparesis and was unable to walk for 1 month. She had weakness and hyperreflexia of both lower extremities. Magnetic resonance imaging showed a well-defined homogeneously and intensely enhanced extradural mass with cord compression at T4 to T7 levels. The patient underwent laminectomy at T4 through to T7 and total tumor removal. Permanent histopathologic sections and immunostains revealed a giant cell tumor of the synovium. Postoperative neurological status recovered to grade V. Magnetic resonance imaging at the 1-year follow-up showed no recurrence and there was no clinical recurrence at the 2-year follow-up.
We report an extremely rare case of giant cell tumor in the epidural space that extended from a thoracic facet joint. The tumor was removed successfully through laminectomies. Although giant cell tumor of a facet joint of the thoracic spine is very rare, it must be considered in the differential diagnosis for masses occurring in the epidural space in a child. Total tumor removal is the best treatment. Careful monitoring of recurrence can achieve a good clinical outcome.
PMCID: PMC3711725  PMID: 23830026
Giant cell tumor; Spine; Synovium
12.  Non-Hodgkin's lymphoma “masquerading” as Pott's disease in a 13-year old boy 
Lymphomas are malignant neoplasms of the lymphoid lineage. They are broadly classified as either Hodgkin disease or as non-Hodgkin lymphoma (NHL). Burkitt's lymphoma, a variety of NHL, is significantly most common in sub-Saharan Africa, where it accounts for approximately one half of childhood cancers. Lymphoblastic lymphoma is less common. A case of paravertebral high grade non-Hodgkin's lymphoma (lymphoblastic lymphoma) “masquerading” as Pott's disease in a 13-year-old child is reported. The present report was informed by the unusual presentation of this case and the intent of increasing the index of diagnostic suspicion. A brief appraisal is provided of the clinical parameters, management strategies and challenges. AT was a 13-year boy that presented on account of a slowly evolving and progressively increasing hunch on the back and inability to walk over 4 and 8 months duration, respectively. There was subsequent inability to control defecation and urination. There was no history of cough. He and his twin brother lived with their paternal grandfather who had chronic cough with associated weight loss. The grandfather died shortly before the child's admission. The child had no BCG immunization. The essential findings on examination were in keeping with lower motor neurons (LMN) paralysis of the lower limbs. The upper limbs appeared normal. There was loss of cutaneous sensation from the umbilicus (T10) downward. There was a firm, (rather tense), non-tender non-pulsatile, smooth swelling over the mid-third of the back (T6-L1) the mass had no differential warmth. It measures about 20×12 cm. Chest radiograph showed no active focal lung lesion, but the thoraco-lumbar spine showed a vertebral planner at L1 and a wedged collapse of T11-T12 vertebrae. There was sclerosis of the end plates of all the vertebral bodies with associated reduction in the bone density. He had an excision biopsy on the 90th day on admission, following which his clinical state rapidly deteriorated. He died within 48 h post surgery. This report aims at raising the local index of clinical suspicion by highlighting the reality of rarities, even in the presence of inadequate diagnostic facilities!
PMCID: PMC3237173  PMID: 22174499
Non-Hodgkin's lymphoma; lyphoblastic lymphoma; Pott's disease
13.  Mediastinal Choriocarcinoma Masquerading as Relapsed Hodgkin Lymphoma 
Case Reports in Oncology  2011;4(3):512-516.
Primary mediastinal choriocarcinoma is a rare extragonadal germ cell malignancy. We describe the first case of a patient who developed mediastinal choriocarcinoma after treatment for Hodgkin lymphoma (HL). A 25-year-old man with classic HL, nodular sclerosis subtype, underwent treatment with splenectomy followed by radiation therapy. Unfortunately, his disease relapsed with a paraspinal mass, and he was subsequently treated with MOPP (mechlorethamine, Oncovin, procarbazine, and prednisone) alternating with ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine). He achieved a complete remission after 6 cycles. Ten years after treatment, the patient presented with a persistent cough, haemoptysis, right supraclavicular lymphadenopathy, and weight loss. His chest X-ray showed opacification of the lower right hemithorax with a widened mediastinum. Given unresponsiveness to several antibiotics and lack of evidence for lung volume loss, there were concerns over lung infiltration with relapsed lymphoma. Transbronchial fine needle aspiration biopsy suggested recurrence of his HL. MOPP alternating with ABVD was again given. Due to disease progression, brachytherapy as well as a cocktail of dexamethasone, cytarabine, and cisplatin were also tried. However, on a subsequent excisional lymph node biopsy, it turned out that the tumour was in fact choriocarcinoma and not relapsed HL. Unfortunately, despite aggressive therapy, the patient's disease rapidly progressed, and he died within 2 weeks.
PMCID: PMC3220912  PMID: 22114578
Germ cell tumour; Choriocarcinoma; Hodgkin lymphoma; Mediastinal tumour
14.  Breathlessness With Pulmonary Metastases: A Multimodal Approach 
Case Study 
Sarah is a 58-year-old breast cancer survivor, social worker, and health-care administrator at a long-term care facility. She lives with her husband and enjoys gardening and reading. She has two grown children and three grandchildren who live approximately 180 miles away.
One morning while showering, Sarah detected a painless quarter-sized lump on her inner thigh. While she thought it was unusual, she felt it would probably go away. One month later, she felt the lump again; she thought that it had grown, so she scheduled a visit with her primary care physician. A CT scan revealed a 6.2-cm soft-tissue mass in the left groin. She was referred to an oncologic surgeon and underwent an excision of the groin mass. Pathology revealed a grade 3 malignant melanoma. She was later tested and found to have BRAF-negative status. Following her recovery from surgery, Sarah was further evaluated with an MRI scan of the brain, which was negative, and a PET scan, which revealed two nodules in the left lung.
As Sarah had attended a cancer support group during her breast cancer treatment in the past, she decided to go back to the group when she learned of her melanoma diagnosis. While the treatment options for her lung lesions included interleukin-2, ipilimumab (Yervoy), temozolomide, dacarbazine, a clinical trial, or radiosurgery, Sarah's oncologist felt that ipilimumab or radiosurgery would be the best course of action. She shared with her support group that she was ambivalent about this decision, as she had experienced profound fatigue and nausea with chemotherapy during her past treatment for breast cancer. She eventually opted to undergo stereotactic radiosurgery.
After the radiosurgery, Sarah was followed every 2 months. She complained of shortness of breath about 2 weeks prior to each follow-up visit. Each time her chest x-ray was normal, and she eventually believed that her breathlessness was anxiety-related. Unfortunately, Sarah’s 1-year follow-up exam revealed a 2 cm × 3 cm mass in her left lung, for which she had a surgical wedge resection. Her complaints of shortness of breath increased following the surgery and occurred most often with anxiety, heat, and gardening activities, especially when she needed to bend over. Sarah also complained of a burning "pins and needles" sensation at the surgical chest wall site that was bothersome and would wake her up at night.
Sarah met with the nurse practitioner in the symptom management clinic to discuss her concerns. Upon physical examination, observable signs of breathlessness were lacking, and oxygen saturation remained stable at 94%, but Sarah rated her breathlessness as 7 on the 0 to 10 Borg scale. The nurse practitioner prescribed duloxetine to help manage the surgical site neuropathic pain and to assist with anxiety, which in turn could possibly improve Sarah’s breathlessness. Several nonpharmacologic modalities for breathlessness were also recommended: using a fan directed toward her face, working in the garden in the early morning when the weather is cooler, gardening in containers that are at eye level to avoid the need to bend down, and performing relaxation exercises with pursed lip breathing to relieve anxiety-provoked breathlessness. One month later, Sarah reported relief of her anxiety; she stated that the fan directed toward her face helped most when she started to feel "air hungry." She rated her breathlessness at 4/10 on the Borg scale.
Sarah’s chest x-rays remained clear for 6 months, but she developed a chronic cough shortly before the 9-month exam. An x-ray revealed several bilateral lung lesions and growth in the area of the previously resected lung nodule. Systemic therapy was recommended, and she underwent two cycles of ipilimumab. Sarah’s cough and breathlessness worsened, she developed colitis, and she decided to stop therapy after the third cycle. In addition, her coughing spells triggered bronchospasms that resulted in severe anxiety, panic attacks, and air hunger. She rated her breathlessness at 10/10 on the Borg scale during these episodes. She found communication difficult due to the cough and began to isolate herself. She continued to attend the support group weekly but had difficulty participating in conversation due to her cough.
Sarah was seen in the symptom management clinic every 2 weeks or more often as needed. No acute distress was present at the beginning of each visit, but when Sarah began to talk about her symptoms and fear of dying, her shortness of breath and anxiety increased. The symptom management nurse practitioner treated the suspected underlying cause of the breathlessness and prescribed oral lorazepam (0.5 to 1 mg every 6 hours) for anxiety and codeine cough syrup for the cough. Opioids were initiated for chest wall pain and to control the breathlessness. Controlled-release oxycodone was started at 10 mg every 12 hours with a breakthrough pain (BTP) dose of 5 mg every 2 hours as needed for breathlessness or pain. Sarah noted improvement in her symptoms and reported a Borg scale rating of 5/10. Oxygen therapy was attempted, but subjective improvement in Sarah’s breathlessness was lacking.
Sarah’s disease progressed to the liver, and she began experiencing more notable signs of breathlessness: nasal flaring, tachycardia, and restlessness. Opioid doses were titrated over the course of 3 months to oxycodone (40 mg every 12 hours) with a BTP dose of 10 to 15 mg every 2 hours as needed, but her breathlessness caused significant distress, which she rated 8/10. The oxycodone was rotated to IV morphine continuous infusion with patient-controlled analgesia (PCA) that was delivered through her implantable port. This combination allowed Sarah to depress the PCA as needed and achieve immediate control of her dyspneic episodes. Oral lorazepam was also continued as needed.
Sarah’s daughter moved home to take care of her mother, and hospice became involved for end-of-life care. As Sarah became less responsive, nurses maintained doses of morphine for control of pain and breathlessness and used a respiratory distress observation scale to assess for breathlessness since Sarah could no longer self-report. A bolus PCA dose of morphine was administered by Sarah’s daughter if her mother appeared to be in distress. Sarah died peacefully in her home without signs of distress.
PMCID: PMC4093448  PMID: 25032021
15.  Intradural Migration of a Sequestrated Lumbar Disc Fragment Masquerading as a Spinal Intradural Tumor 
Intervertebral intradural lumbar disc herniation (ILDH) is a quite rare pathology, and isolated intradural lumbar disc herniation is even more rare. Magnetic resonance imaging (MRI) may not be able to reveal ILDHs, especially if MRI findings show an intact lumbar disc annulus and posterior longitudinal ligament. Here, we present an exceedingly rare case of an isolated IDLH that we initially misidentified as a spinal intradural tumor, in a 54-year-old man hospitalized with a 2-month history of back pain and right sciatica. Neurologic examination revealed a positive straight leg raise test on the right side, but he presented no other sensory, motor, or sphincter disturbances. A gadolinium-enhanced MRI revealed what we believed to be an intradural extramedullary tumor compressing the cauda equina leftward in the thecal sac, at the L2 vertebral level. The patient underwent total L2 laminectomy, and we extirpated the intradural mass under microscopic guidance. Histologic examination of the mass revealed a degenerated nucleus pulposus.
PMCID: PMC3467376  PMID: 23091677
Intradural disc herniation; Spinal intradural tumor; Magnetic resonance imaging
16.  Aneurysmal bone cysts of the spine 
European Spine Journal  2012;22(3):593-601.
Aneurysmal bone cyst is a benign, relatively uncommon lesion, representing 1.4 % of primary bone tumors. The vertebral column is involved in 3–30 % of cases. This report describes clinical characteristics and treatment results of 18 patients with aneurysmal bone cyst of the spine.
Between 1991 and 2008, 18 patients with aneurysmal bone cyst of the spine were surgically treated in our department. The clinical records, radiographs, histologic sections, and operative reports were analyzed.
There were 11 male and 7 female patients; mean age was 22.1 years (range 7–46 years). Localizations were cervical (3), cervicothoracic (2), thoracic (3), lumbar (4), and sacrum (6). Tumor was localized on the left side in 11 cases, on the right side in 2 and at midline in 5 patients. The two most common clinical features were axial pain (14 patients) and radicular pain (8 patients). Neurological signs were paraparesis in 3, monoparesis in 6. Mean duration of symptoms was 9 months (range 3 months–3 years). All patients underwent surgery: total removal was performed in 13 patients and subtotal resection in 5. Posterior (11), anterolateral (1), or combined anterior-posterior (6) approaches were used. Mean follow-up duration was 112.3 months (range 4–21 years). We detected four recurrences in subtotal excision group (4/5), and one recurrence in total excision group (1/13).
Treatment options for aneurysmal bone cysts are simple curettage with or without bone grafting, complete excision, embolization, radiation therapy, or a combination of these modalities. Radical surgical excision should be the goal of surgery to decrease the recurrence rate. Recurrence rate is significantly lower in case of total excision.
PMCID: PMC3585636  PMID: 23053752
Aneurysmal bone cyst; Spine tumor; Spinal fusion; Tumor recurrence
17.  Demyelinating disease masquerading as a surgical problem: a case series 
We report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease.
Case presentation
The first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale.
The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes.
The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent.
These cases illustrate the importance of considering a demyelinating disease in the differential diagnosis of a mass lesion. Critical analyses of clinical presentations coupled with good physical examination are vital in assisting clinicians to reach the correct diagnosis.
PMCID: PMC2737773  PMID: 19830201
18.  Thoracic extraosseous, epidural, cavernous hemangioma: Case report and review of literature 
A 40-year-old male presented with mid-thoracic backache and progressive, ascending, spastic, paraparesis for one year. Magnetic resonance imaging demonstrated an extraosseous, extradural mass, without any bone invasion at the T2-T4 vertebral levels, located dorsal to the thecal sac. The spinal cord was compressed ventrally. The lesion was totally excised after a T2-T4 laminectomy. Histopathological examination revealed a cavernous hemangioma. The authors reported this case and reviewed the literature, to explain why extraosseous, extradural, cavernous hemangiomas should be considered in the differential diagnosis of extradural thoracic compressive myelopathy.
PMCID: PMC3821420  PMID: 24250167
Cavernous hemangioma; epidural; magnetic resonance imaging; myelopathy; thoracic spine
19.  A case of poorly differentiated hepatocellular carcinoma with intriguing ultrasonography findings 
Oncology Letters  2012;4(3):393-397.
A 60-year-old female was referred to the Toho University Omori Medical Center due to ultrasonography findings revealing a notable hepatic mass. The 44×32 mm isoechoic mass had a high-echo band along the margin in the liver. Contrast-enhanced ultrasonography (CEUS) with Sonazoid detected an enhancement pattern extending from the outer periphery to the inside of the mass in the vascular phase and a pattern similar to that of surrounding hepatic tissue in the post-vascular phase. High-flow hepatic hemangioma was suspected due to the hyperechoic rim-like high-echo band, the oval shape and the CEUS findings. However, computed tomography revealed a hypervascular hepatocellular carcinoma (HCC) pattern and the patient underwent surgery. Histopathological findings revealed poorly differentiated HCC. As poorly and moderately differentiated HCC types are conventionally classified as ‘hypervascular HCC’, few detailed ultrasonography (US) studies of poorly differentiated HCC are available. US characteristics of hypervascular HCC include the presence of a halo (hypoechoic band) around the lesion and reduced signal intensity in the post-vascular phase of CEUS. US in the patient revealed a mass with a hyperechoic band and a signal intensity in the post-vascular phase of CEUS that was almost identical to that of the surrounding liver parenchyma. These findings suggest poorly differentiated HCC and indicate that further research on hypervascular HCC is required.
PMCID: PMC3673642  PMID: 23741241
poorly differentiated hepatocellular carcinoma; Sonazoid; contrast-enhanced ultrasonography; liver
20.  Cardiovascular changes after PMMA vertebroplasty in sheep: the effect of bone marrow removal using pulsed jet-lavage 
European Spine Journal  2010;19(11):1913-1920.
Clinically, the displacement of intravertebral fat into the circulation during vertebroplasty is reported to lead to problems in elderly patients and can represent a serious complication, especially when multiple levels have to be treated. An in vitro study has shown the feasibility of removing intravertebral fat by pulsed jet-lavage prior to vertebroplasty, potentially reducing the embolization of bone marrow fat from the vertebral bodies and alleviating the cardiovascular changes elicited by pulmonary fat embolism. In this in vivo study, percutaneous vertebroplasty using polymethylmethacrylate (PMMA) was performed in three lumbar vertebrae of 11 sheep. In six sheep (lavage group), pulsed jet-lavage was performed prior to injection of PMMA compared to the control group of five sheep receiving only PMMA vertebroplasty. Invasive recording of blood pressures was performed continuously until 60 min after the last injection. Cardiac output and arterial blood gas parameters were measured at selected time points. Post mortem, the injected cement volume was measured using CT and lung biopsies were processed for assessment of intravascular fat. Pulsed jet-lavage was feasible in the in vivo setting. In the control group, the injection of PMMA resulted in pulmonary fat embolism and a sudden and significant increase in mean pulmonary arterial pressure. Pulsed jet-lavage prevented any cardiovascular changes and significantly reduced the severity of bone marrow fat embolization. Even though significantly more cement had been injected into the lavaged vertebral bodies, significantly fewer intravascular fat emboli were identified in the lung tissue. Pulsed jet-lavage prevented the cardiovascular complications after PMMA vertebroplasty in sheep and alleviated the severity of pulmonary fat embolism.
PMCID: PMC2989274  PMID: 20725752
Vertebroplasty; Pulmonary fat embolism; Cardiovascular changes; Pulsed jet-lavage; In vivo study
21.  Unique presentation of a giant mediastinal tumor as kyphosis: a case report 
Although posture distortion is a common problem in elderly patients, spinal deformity caused by a thymoma has not been previously reported. Thymomas are slowly growing tumors that predominantly cause respiratory symptoms.
Case presentation
We report the case of an 83-year-old woman who was admitted with a giant mediastinal mass that had caused progressive spinal distortion and weight loss to our department. The clinical and laboratory investigations that followed revealed one of the largest thymomas ever reported in the medical literature, presenting as a mass lesion placed at the left hemithorax. She underwent complete surgical excision of the tumor via a median sternotomy. Two years after the operation, she showed significant improvement in her posture, no pulmonary discomfort, and a gain of 20 kg; she remains disease free based on radiographic investigations.
In this case, a chronic asymmetric load on the spine resulted in an abnormal vertebral curvature deformity that presented as kyphosis.
PMCID: PMC3348087  PMID: 22475440
22.  Posteriorly migrated thoracic disc herniation: a case report 
Posterior epidural migration of thoracic disc herniation is extremely rare but may occur in the same manner as in the lumbar spine.
Case presentation
A 53-year-old Japanese man experienced sudden onset of incomplete paraplegia after lifting a heavy object. Magnetic resonance imaging revealed a posterior epidural mass compressing the spinal cord at the T9-T10 level. The patient underwent emergency surgery consisting of laminectomy at T9-T10 with right medial facetectomy, removal of the mass lesion, and posterior instrumented fusion. Histological examination of the mass lesion yielded findings consistent with sequestered disc material. His symptoms resolved, and he was able to resume walking without a cane 4 weeks after surgery.
Pre-operative diagnosis of posterior epidural migration of herniated thoracic disc based on magnetic resonance imaging alone may be overlooked, given the rarity of this pathology. However, this entity should be considered among the differential diagnoses for an enhancing posterior thoracic extradural mass.
PMCID: PMC3582544  PMID: 23402642
Intervertebral disc herniation; Posterior migration; Thoracic spine
23.  The Fracture Unit Model. A Model for Implementation in Italy: “Multidisciplinary Approach for the Prevention and Treatment of Osteoporotic Vertebral Compression Fractures: VCF Unit” 
Reduced BMD is a risk factor for vertebral fractures (VFs). Every one SD increase in BMD is associated with a 2- to 2.5-fold increase in the risk of VFs. The presence of a previous fracture, vertebral or of other districts, is another important predictor of an increased risk of future fractures, independently of the association between BMD and fracture risk. Thus, the presence of both a low BMD and a previous fracture dramatically increases fracture risk. The definition of osteopo-rotic VFs has undergone considerable variations over the years, going from initial clinical sign of OP, through the now superseded definition of VFs as a disease, to a complication of OP resulting from bone fragility. The prevalence of VFs increases with age in both sexes, and it is calculated that at the age of 80 years, 37% of Caucasian women will have at least one radiographically evident VF. It has been estimated, again in Caucasian women, that the percentage incidence of fractures is 0.5% in those aged 50-55 years, 1.4% in those aged 65-69 years, and over 2% in women older than 75. However, two factors prevent an accurate assessment of the epidemiology of VFs. First, most VFs escape clinical diagnosis. Second, the absence of a “gold standard” radiographic definition of VFs has given rise to different ways of defining these lesions. VFs are rarely a cause of mortality, but they are associated with increased impairment of general conditions. Recurrent VFs have irreversible clinical consequences, such as reduction of height and chronic vertebral pain, which provoke an intensification of the pain and a greater degree of disability due to accentuation of kyphosis. The presence of VFs and kyphosis leads to a reduced thoracic volume and, consequently, to a loss of lung volume, in some cases severe enough to result in respiratory insufficiency. The consequences of the intense pain are: reduced range of motion, loss of balance, slowed gait and greater difficulty carrying out normal daily activities. In rare cases, lower limb pain and weakness may appear, caused by compression of the spinal medulla by the deformed vertebral body. The main aim of treatment is to restore the patient to his/her pre-trauma levels of functioning. This can be achieved through recourse to mini-invasive percutaneous techniques, vertebroplasty and kyphoplasty, with the aim of reducing the pain caused by osteo-porotic vertebral compression fractures, of preventing progression of the vertebral collapse and of rapidly re-establishing functional activity. Most fracture patients are discharged without undergoing a thorough bone metabolism assessment that could identify the causal factor of the fracture. In a high percentage (up to 95%) of patients with recent fractures, BMD is not measured and, therefore, a diagnosis of OP is not made. Consequently, these patients are not prescribed drugs capable of effectively reducing the risk of further fractures. Specialist orthopaedic centres need to introduce protocols designed to ensure application of the current procedures for diagnosing and treating OP.
On the basis of these considerations, we undertook to develop, in collaboration with the Department of Specialist Surgical Sciences of the University of Florence, the Orthopaedics and Traumatology Units 1, 2 and 3, the Recovery and Functional Re-education Unit, the Neurosurgery Unit, and the third Radiodiagnostics Unit of the Careggi Hospital in Floren-ce, a protocol that involves a range of specialists in assessing the introduction of variable, outcome-targeted medical therapies for osteoporotic patients submitted to kyphoplasty following fragility fractures of the vertebra. To choose the appropriate medical therapy, and to monitor its effects, the patients will be submitted to a series of clinical investigations. The “appropriate” therapy could include calcium and vitamin D supplementation, biphosphonates, SERMs, bone anabolic agents and combinations of drugs. The safety of the medical therapy and any adverse effects will be monitored at each follow-up visit through an appropriate questionnaire. This study aims to compare the outcomes of the group following a traditional pathway with those following a modified pathway (prescription of a targeted medical therapy), by means of metabolic, instrumental and functional tests performed at 2 months, 6 months, 1 year and 2 years. The general aim of the study will be to evaluate the efficacy and safety of a modified versus a traditional pathway in the care of osteoporotic patients undergoing kyphoplasty for VFs. The primary endpoint of the study will be the percentage of successes in the modified compared with the traditional pathway group. Secondary endpoints will be: change in femoral and lumbar BMD, changes in biochemical markers of bone remodelling and quality of life, assessment of safety parameters: overall and symptomatic cement leakage, pulmonary embolism, spinal medulla compression, radicular pain, radiculopathies and assessment of total procedure-related, cement-related and access-related adverse events. The ultimate aim of the study will be to prepare guidelines for the management, in terms of metabolic diagnosis and relative medical therapy, of patients with OP complicated by VFs.
PMCID: PMC3213825
24.  Progression of Prostate Cancer Despite an Extremely Low Serum Level of Prostate-Specific Antigen 
Korean Journal of Urology  2010;51(5):358-361.
A 61-year-old man who had been diagnosed with prostate cancer 9 years ago and had been treated with pelvic irradiation and intermittent androgen deprivation therapy visited the emergency room because of back pain and weakness in both legs. Spine magnetic resonance imaging showed a lumbar epidural mass and spine metastasis. The whole-body workup revealed multiple metastases to the lymph nodes, bone, liver, and lung. The serum prostate-specific antigen was 0.02 ng/ml. He underwent laminectomy, posterior fixation, and epidural mass excision, and metastatic adenocarcinoma from the prostate was diagnosed. The patient underwent 1 cycle of docetaxel-based chemotherapy. More chemotherapy could not be done because of his general weakness. The patient died one month later of multiple organ failure.
PMCID: PMC2873892  PMID: 20495701
Disease progression; Multiple organ failure; Neoplasm metastasis; Prostate-specific antigen; Prostatic neoplasms
25.  Distinguishing benign notochordal cell tumors from vertebral chordoma 
Skeletal Radiology  2008;37(4):291-299.
The objective was to characterize imaging findings of benign notochordal cell tumors (BNCTs).
Design and patients
Clinical and imaging data for 9 benign notochordal cell tumors in 7 patients were reviewed retrospectively. Conventional radiographs (n = 9), bone scintigrams (n = 2), computed tomographic images (n = 7), and magnetic resonance images (n = 8) were reviewed. Eight of the 9 lesions were stained with hematoxylin-eosin and microscopically examined.
There were 3 male and 4 female patients with an age range of 22 to 55 years (average age, 44 years). Two patients had two lesions at different sites. The lesions involved the cervical spine in 4 patients, the lumbar spine in 2, the sacrum in 2, and the coccyx in 1. The most common symptom was mild pain. The lesions of 2 patients were found incidentally during imaging studies for unrelated conditions. Five patients underwent surgical procedures. One patient died of surgical complications. All other patients have been well without recurrent or progressive disease for 13 to 84 months. Radiographs usually did not reveal significant abnormality. Five lesions exhibited subtle sclerosis and 1 showed intense sclerosis. Technetium bone scan did not reveal any abnormal uptake. Computed tomography images had increased density within the vertebral bodies. The lesions had a homogeneous low signal intensity on T1-weighted magnetic resonance images and a high intensity on T2-weighted images without soft-tissue mass. Microscopically, lesions contained sheets of adipocyte-like vacuolated chordoid cells without a myxoid matrix.
Benign notochordal cell tumors may be found during routine clinical examinations and do not require surgical management unless they show extraosseous disease. These tumors should be recognized by radiologists, pathologists, and orthopedic surgeons to prevent operations, which usually are extensive.
PMCID: PMC2257990  PMID: 18188556
Spine; Vertebra; Benign notochordal cell tumor; Notochordal hamartoma; Notochordal rest; Chordoma; MRI

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