Ewing's sarcoma is a malignant tumor of bones that primarily affects children and young adults. The true origin of this small round cell lesion still remains controversial. It was originally described by James Ewing in 1921 as arising from undifferentiated osseous mesenchymal cells; however, recent studies suggest that Ewing's tumor might be neuroectodermally derived from various degrees of differentiation of the primitive neural tissues. This paper reports a rare case of ES of the mandible in an 11-year-old girl, which had been previously misdiagnosed and treated as a dental abscess. In the clinical examination, a hard immobile expansive mass of 2 cm diameter was observed on the left side of the mandible. Radiographic examination revealed a diffuse radiolucent lesion with ill-defined borders and wide vestibular bone plate destruction. Microscopically, the tumor was composed by monotonous small round cells that exhibited immunoreactivity for CD99, vimentin and desmin. Surgical resection of mandible followed by mandibular reconstruction was adopted. The patient was subjected to multiagent chemotherapy with Vincristine [VC], Dactinomycin [AC], Cyclophosphamide [CP] and Doxorubicin [AD]).
Child; diagnosis; Ewing's sarcoma; immunohistochemistry; mandible
Gastrointestinal stromal tumors are rare tumors. They commonly metastasize within the abdominal cavity, particularly to the liver. Less commonly, metastases can be found in the bone.
We here present a case of metastasis to the scapula in a 54-year-old Caucasian male patient with an advanced gastrointestinal stromal tumor, which was subsequently successfully treated with resection and sunitinib.
The present study is, to the best of our knowledge, the second to describe scapular metastasis of a gastrointestinal stromal tumor. Our patient was treated by scapulectomy. The overwhelming majority of scapular tumors are metastases that arise from soft tissue, hepatocellular and thyroid tumors. Gastrointestinal stromal tumor metastasis occurs rarely. Scapular surgery can successfully provide local control of the disease. After the surgery, patients should continue with medical treatment.
Synovial sarcomas (SS) account for 5–10% of soft-tissue sarcomas and typically arise in the para-articular regions of adolescents and young adults. Nonetheless, SS can occasionally occur in other regions of the body. Here, we present a first clinical literature report of a patient with an SS arising from the vaginal wall. A 40-year-old patient who presented a necrotic polypoid lesion, measuring 50 mm and extending from the external urethral meatus to the middle part of the anterior vaginal wall. The biopsy showed a poorly differentiated SS with abundant necrosis and a SYT-SSX1 mutation. A staging CT scan was negative for distant metastases. The patient, prior to the radical surgery, received neoadjuvant chemotherapy (ifosfamide and epirubicin) for three cycles. She underwent post-operative external radiotherapy and brachytherapy (50 Gy) due to close margins (<1 mm) in the pathologic specimen. She relapsed 11 and 16 months later with lung metastases, which, both times, were successfully removed by surgical resection. At 24 months from diagnosis, the patient is alive without further evidence of disease. In summary, in the presence of unfavourable prognostic factors, neoadjuvant chemotherapy could be the primary approach to reduce the tumour size and the risk of distant micro-metastases allowing a less aggressive radical surgery if the tumour is located in a non-extremity site. Hence, a multidisciplinary approach, if not influencing overall survival and disease-free survival, may improve the quality of life. In fact, in our patient we obtained a complete clinical control in the pelvis, avoiding pelvic exenteration with neoadjuvant chemotherapy.
Sarcomas of the broad ligament are very uncommon. To our knowledge, there are no cases published of undifferentiated round cell sarcoma of the broad ligament. Round cell sarcomas are a rare and very aggressive variant, which due to their sensitivity to chemotherapy, have an acceptable prognosis. We report the case of a 27-year-old woman who presented with a pelvic mass with a 7-cm diameter placed on the right broad ligament. After surgery, she was diagnosed with undifferentiated round cell sarcoma of the broad ligament. The patient received adjuvant chemotherapy and radiotherapy, and after 12 years of follow-up, she still remains asymptomatic. Proper differential diagnoses as well as an appropriate adjuvant therapy after surgical treatment seem to be essential to obtain good oncological outcomes in this rare entity.
sarcoma; broad ligament tumour; round cells tumour; undifferentiated sarcoma
The term synovioma was coined by Smith in 1927, and later in 1936 Knox suggested the name synovial sarcoma. It occurs primarily in the paraarticular regions, usually in close association with tendon sheaths, bursae, and joint capsules. On rare occasions it may be seen in areas without any apparent relationship to synovial structures as in parapharyngeal region or the abdominal cavity. The first description of synovial sarcoma in the head and neck region was by Pack and Ariel in 1950. The majority of these tumors seem to take origin from paravertebral connective tissue spaces and manifest as solitary retropharyngeal or parapharyngeal masses near the carotid bifurcation. Synovial sarcoma has been reported in soft palate, tongue, maxillofacial region, angle of mandible, sternoclavicular region, scapular region, and the esophagus. We report a case of 28-year-old male patient with synovial sarcoma in mandibular region with biphasic pattern.
Biphasic pattern; head and neck; synovial sarcoma
Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma), adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor) and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma) in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma.
chemotherapy; pediatric sarcoma; rhabdomyosarcoma; osteosarcoma; Ewing sarcoma; synovial sarcoma
Pancreatoblastoma is a rare tumor and surgery with complete resection is the main
treatment approach. Prognosis for patients with residual disease after surgery is
usually dismal. A 14-year-old girl with pancreatoblastoma in the pancreatic body and
tail was submitted to preoperative chemotherapy. She underwent surgery and the tumor
was resected with microscopic margins. Postoperative chemotherapy was followed by
high dose chemotherapy and autologous hematopoietic stem cell transplantation. After
four years she remains very well with no evidence of disease. This is the first case
reported of pancreatoblastoma that was treated with autologous hematopoietic stem
cell transplantation as first line treatment without radiotherapy at the site of the
Hematopoietic stem cell transplantation; Pediatrics; Pancreatic neoplasms; Drug therapy, combination; Primary treatment
Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast.
A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy.
A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria
Malignant melanoma of the conjunctiva is an extremely rare non-cutaneous neoplasm with infrequent skeletal metastatic spread.
We present the case of a 54 year old female Caucasian patient with osseous metastases originating from a malignant melanoma of her right conjunctiva. Metastatic deposits were identified in the left humeral diaphysis and left tibial metaphysis. Clinical, radiological and scintigraphic evaluation necessitated prompt stabilisation of both long bones. Following reamed intramedullary nailing and post-operative radiotherapy she remains asymptomatic six months post-operatively.
This unusual pattern of metastatic spread to the appendicular skeleton of an extremely rare melanomatous lesion requires diagnostic vigilance as well as a multidisciplinary approach for accurate diagnosis, staging and management. Due to the poor prognosis, treatment goals should be directed to palliation of symptoms and prolongation of the quality of life.
A synovial sarcoma (SS) is an aggressive soft tissue tumor that classically occurs in the extremities near, but rarely within large joints, in young adults. Variable symptoms and clinical manifestations may be encountered and a definite diagnosis should depend on pathological results. This poses certain difficulties in arriving at a prompt diagnosis and appropriate treatment.
We report the case of a 68-year-old woman patient who presented an inguinal mass with swelling and pain in the right lower limb. She underwent surgery, and later received systematic intravenous chemotherapy. The pathological studies, especially the specific chromosomal translocation of a t(X;18) (p11.2;q11.2), confirmed the diagnosis as a synovial sarcoma. To the best of our knowledge, this is the first report of a monophasic synovial sarcoma in the inguinal region.
Besides making the readership aware of the rarity of location and age of this present case, this report distinctly highlights the great value of a molecular analysis of an SYT associated genetic alteration in the diagnosis of synovial sarcoma occurring at rare sites especially when immunochemical results are equivocal.
Primary Ewing’s sarcoma of the nasal bone has not been previously described. This case presented as a mass in the left ala of the nose in a five year old female child. The clinical, radiological, microscopic features are described and a review of literature is presented. The case was treated with neoadjuvant chemotherapy and local electron beam radiation therapy. The child was free of disease when she reported for follow up in July 1997. Although wide excision is part of the treatment approach in Ewing’s sarcoma, in sites where surgery is not suitable local radiotherapy and chemotherapy adequately controls primary disease.
Ewing's sarcoma is a neoplasm of the undifferenciated small round cells, which generally affects the bone and deep soft tissues of children and adolescents. We present a case of gastric Ewing's sarcoma; a 35-year-old female who had no symptoms. While she was at a routine medical checkup, a protruding mass in her gastric antrum was incidentally found on esophagogastroduodenoscopy. Endoscopic ultrasonogram showed a submucosal mass on the same lesion and a laparosopic wedge resection was done. Pathologic gross findings showed a granular grape appearance tissue and histoloigc examination revealed a small round cell tumor with CD 99 immunoexpression positive. In general, a combined modality therapy for Ewing's sarcoma such as surgical resection with chemotherapy, is accepted as an effective method. However, this patient had no adjuvant chemotherapy after surgery and she has no recurrence for eleven months.
Ewing's sarcoma; Combined modality therapy; Chemotherapy
Over the past decade, there has been an increased interest in understanding the operative indications and techniques in treating scapular fractures and tracking their outcomes. Multiple studies have documented poor functional outcomes following nonoperative management of displaced scapular fractures. There is a groundswell of recognition that severe deformity from scapular malunion is associated with functional consequences for patients. This has led to a growing recognition that scapular fractures should be held to the same standards as other bodily fractures with regard to fracture fixation principles, including anatomic articular reduction, proper alignment, and stable internal fixation. Through research, there has been an improved understanding of scapular fracture patterns and the relevant surgical approaches and exposures used for fracture fixation. As with many bones, however, there still remains the absence of a compelling study that defines thresholds for surgical indication based on degrees of deformity and amounts of displacement.
Trauma; Upper extremity trauma; Scapula fracture; Dynamic scapular function; Shoulder girdle injury; Scapular malunion; Current review; ORIF scapula; Proximal forequarter
Synovial sarcoma is a malignant soft tissue tumor that most commonly occurs in the extremities of young and middle-aged adults, in the vicinity of large joints. Although synovial sarcoma is frequently associated with joints, it may arise in unexpected sites, such as the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcoma of the pleura is rare. To date, nearly 36 cases of primary synovial sarcoma of the pleura have been reported since Gaertner et al. published the first case in 1996. The oncologic characteristics, treatment, and prognosis for pleural synovial sarcomas are not well defined because of a paucity of data. However, a multimodal approach, including surgical resection, chemotherapy, and radiotherapy, has generally been suggested. We report the outcome of one patient with primary pleural synovial sarcoma treated with radical resection and adjuvant treatment.
Primary synovial sarcoma; Pleura; Synovial sarcoma
Primary liposarcoma of the lung is extremely rare with only 11 cases reported worldwide. However, metastatic pulmonary liposarcoma was reported around 15% out of all metastatic pulmonary soft tissue sarcoma.
A case of 53 year-old man who presented with mass at left scapular region was referred to our unit in June 2006. He had history of thigh liposarcoma 14 years back which was operated and received a course of radiotherapy. However, he denied of having chest pain, shortness of breath or any other respiratory symptom. Biopsy of the mass came back as liposarcoma (round cell type). CT scan showed presence of left pleural base mass. He underwent left posterolateral thoracotomy and excision of infrascapular as well as intrathoracic mass 3 months later. Post operatively was complicated by hypovolemic shock and anemia. He was put under intensive care monitoring and discharged well at day 52 post-operatively. He was supposed to come for oncology appointment in December 2006 for radiotherapy.
Discussion & Conclusions:
Pulmonary liposarcoma can be devided into 4 main subtypes; myxoid, round cell, well differentiated and pleomorphic, which have different post operative survival rate. Five years survival rate of metastatic liposarcoma varies from as high as 77% in myxoid type as low as 18 % in round cell type. Even though metastatic pulmonary liposarcoma has the worst prognosis compared to other soft tissue sarcomas, some studies and centres believe that complete resection will give better prognostic value in term of survival rate and recurrences.
Sarcoma surgeons face unique challenges in younger patients with significant skeletal growth remaining. The heightened concerns regarding radiation in the very young and the drastic changes expected in the lengths and cross-sectional areas of bones affect the decision-making for both soft-tissue and bone sarcomas in this population. Nonetheless, there is sparse literature focused on sarcoma surgery in this age group. The records of one tertiary regional sarcoma treatment program were reviewed to identify all patients ten years old or younger at the time of local control surgery for limb or limb-girdle sarcomas. Demographic information, diagnosis, surgery performed, complications, and general outcomes were gleaned from the medical records. 43 patients were identified, including 15 with osteosarcomas, 11 Ewing’s sarcoma family tumors, five rhabdomyosarcomas, and two synovial sarcomas, among others. Location of tumors varied widely, but demonstrated a predilection for the upper extremity more than is typical in adolescents with the same tumor types. Survival was favorable overall, with only five patients dying from disease. Most patients continued to function well at latest follow-up, but 16 experienced additional surgical interventions following the index procedure. Sarcoma surgery in the younger growing child presents challenges for the surgeon, patient, and parents, but is usually successful in the long-term.
Osteochondrolipoma is an extremely rare histological variant of lipoma with osseous and cartilaginous differentiation. The present study reports an unusual case of an osteochondrolipoma occurring in the left scapular region of a 49-year-old male. The physical examination revealed a 3-cm, hard, non-tender and minimally mobile mass. Plain radiography revealed a faintly ossified soft-tissue mass without evidence of bone erosion. Computed tomography (CT) confirmed the presence of a lesion and the normal appearance of the scapula. Magnetic resonance imaging (MRI) showed a well-circumscribed subcutaneous mass with an almost homogeneous high signal intensity on the T1- and T2-weighted sequences. Contrast-enhanced fat-suppressed T1-weighted sequences demonstrated a faint peripheral and septal enhancement of the mass. A marginal excision of the tumor was performed. Histologically, the tumor was predominantly composed of mature adipocytes mixed with thin trabeculae of mature bone. In addition, small amounts of mature hyaline cartilage and osteoid were identified in the periphery of the lesion. Based on these findings, the tumor was diagnosed as an osteochondrolipoma. The patient demonstrated no evidence of local recurrence within six months of follow-up. Although rare, osteochondrolipoma should be considered as a differential diagnosis of a well-defined, calcified/ossified, subcutaneous mass in the scapular region.
osteochondrolipoma; scapula; magnetic resonance imaging; pathology
Clear cell sarcoma of tendons and aponeuroses (CCSTA) appears usually in the extremities and rarely in the trunk.
We present an unusual case of CCSTA overlying the scapular region and with secondary osseous extension in the lower scapula. The patient underwent a wide local excision with removal of the tumor and the lower two thirds of the scapula. He had no local recurrences but he developed lung metastases after 5 months in spite of postoperative chemotherapy. He finally died ten months later.
The patients with CCSTA have a variable unpredictable course. Despite treatment the overall prognosis is poor.
Retinoblastoma is a highly malignant neoplasm. Most of the cases are usually advanced at the time of detection, requiring enucleation to salvage the child's life. Just treating the patient for cancer is not enough; the cosmetic rehabilitation of these patients is equally important and it should always be an integral part of their treatment, to help them re-integrate in the aesthetic conscious society. Rehabilitating such patients require a multidisciplinary approach involving the combined and timely efforts of an ophthalmologist, paediatric oncologist and a skilled maxillofacial prosthodontist. This paper presents a case of 3½-year-old girl who had enucleation of her right eye due to retinoblastoma along with chemotherapy and radiotherapy at the age of 3 years. The patient was recalled regularly for follow-up at 3 month intervals for ophthalmic examinations and she was rehabilitated cosmetically with customised ocular prosthesis during the various stages of her developmental growth.
Custom ocular prosthesis; enucleation; impression technique; ocular defect; retinoblastoma
Alveolar soft– part sarcoma (ASPS) is a rare form of soft tissue sarcoma and is most often seen in adolescents and young adults. Surgical excision of the primary tumor and pulmonary metastases has resulted in prolonged survival in some patients while the benefit of adjuvant chemotherapy and/or radiotherapy has been disputed. An 11– year–old boy with ASPS which presented with a markedly vascular tumor in the left thigh, and multiple bilateral pulmonary metastases 8 months after diagnosis is described. The patient has remained disease–free for over 5 years since the initial diagnosis.
alveolar soft – part sarcoma; children; pulmonary; metastases; long term survival
Tuberculosis of the flat bones is rare and only a small percentage involves the scapular bone.
We report a rare case of tuberculosis of the scapula in a 14-year-old. Diagnostic clues include lytic areas with low density seen in the body of the scapula involving a glenoid margin associated with typical clinical features. Treatment should include a regimen of four antitubercular drugs along with surgical debridement if required.
Although rare, tuberculosis should be suspected in patients presenting with a chronic sinus in the scapular region, particularly in the developing world.
Undifferentiated uterine sarcoma (UUS) is a rare tumor with an aggressive growth pattern. They occur in women from 40 to 60 years and are generally characterized by poor prognosis, a high rate of local recurrence, and distant metastases. UUS accounts for 0.2% of all gynecological malignancies. Possible treatments include surgery, radiotherapy, and chemotherapy.
A 65-year-old female with postmenopausal bleeding was found to have a uterine mass for which she underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. The pathologic evaluation was consistent with undifferentiated endometrial sarcoma. She began experiencing headaches with associated visual disturbances. Magnetic resonance imaging (MRI) of the brain showed a homogenous enhancing occipital dural-based mass measuring 1.6 × 1.8 × 1.7 cm. Due to the rarity of metastatic uterine sarcoma to the brain, this was believed to represent a meningioma and subsequently observed. Interval MRI scan revealed a significant increase in size of the right occipital mass to 2.3 cm with increased edema and mass effect. She underwent right occipital image guided craniotomy for resection of the mass. Histopathology confirmed UUS metastases.
Randomized trials analyzing these treatment options are limited due to the rarity of this disease; therefore, a standard therapy is not established. Based on a review of the literature, this is only the fourth case reported of UUS metastatic to the brain.
Brain metastases; endometrial stromal sarcoma; uterine sarcoma; undifferentiated uterine sarcoma
Alveolar soft part sarcoma (ASPS) constitutes a rare soft tissue malignant neoplasm comprising less than 1 % of all soft tissue sarcomas. ASPS demonstrates a strong predilection for adolescents and young adults, with a female predominance reported. The head and neck region is the most commonly affected region in pediatric patients with the tongue and orbit affected most commonly. Herein we present the clinical, radiographic, histopathologic, immunohistochemical and molecular features of two examples of ASPS affecting the oral cavity of 4 and 13 year-old boys, along with a focused review of the literature on intraoral ASPS in pediatric patients.
Alveolar soft sarcoma; Children; Oral cavity; Tongue; Pathology; Immunohistochemistry; Molecular; ASPSCR1/TFE3 fusion; Management
Synovial sarcoma is a high-grade, soft-tissue sarcoma that most frequently is located in the vicinity of joints, tendons or bursae, although it can also be found in extra-articular locations. Most patients with synovial sarcoma of the hand are young and have a poor prognosis, as these tumors are locally aggressive and are associated with a relatively high metastasis rate. According to the literature, local recurrence and/or metastatic disease is found in nearly 80% of patients. Current therapy comprises surgery, systemic and limb perfusion chemotherapy, and radiotherapy. However, the 5-year survival rate is estimated to be only around 27% to 55%. Moreover, most authors agree that synovial sarcoma is one of the most commonly misdiagnosed malignancies of soft tissues because of their slow growing pattern, benign radiographic appearance, ability to change size, and the fact that they may elicit pain similar to that caused by common trauma.
We describe an unusual case of a large synovial sarcoma of the hand in a 63-year-old Caucasian woman followed for 12 years by a multidisciplinary team. In addition, a literature review of the most pertinent aspects of the epidemiology, diagnosis, treatment and prognosis of these patients is presented.
Awareness of this rare tumor by anyone dealing with hand pathology can hasten diagnosis, and this, in turn, can potentially increase survival. Therefore, a high index of suspicion for this disease should be kept in mind, particularly when evaluating young people, as they are the most commonly affected group.
Synovial sarcoma; Hand; Surgery; Malignant tumor
Aim of the study
To present a case of a patient with cervical carcinoma in stage IIA who was diagnosed with pelvic bone sarcoma 28 years after radiotherapy.
A 37-year-old woman with IIA cervix cancer was treated with external beam irradiation and brachytherapy. The patient had undergone conventionally fractionated external beam irradiation using the “box” technique, with the total dose of 50 Gy and brachytherapy with radium applicators (intrauterine tube and fornix applicator) with the dose of 60 Gy calculated at point A. After treatment she was followed up for 2 years. Twenty-six years later, inoperable pelvic bone sarcoma was diagnosed within the irradiated field. The clinical course was aggressive and rapid progression during chemotherapy was observed.
For patients receiving radiotherapy, long-term careful follow-up is mandatory due to second cancer risk. In the case of any suspicious symptoms, such patients need proper diagnosis to detect any disease as early as possible.
cervix cancer; radiotherapy; pelvic bones; radiation-induced sarcoma