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1.  Rhabdomyosarcoma of the breast – a rare malignancy 
Patient: Female, 60
Final Diagnosis: Rhabdomyosarcoma of the breast
Symptoms: Lump in axilla
Medication: —
Clinical Procedure: Mastectomy
Specialty: Oncology
Objective:
Rare disease
Background:
Primary nonepithelial malignancies of the breast include primary breast sarcomas, therapy-related breast sarcomas, the phyllodes tumors, and primary breast lymphomas. They account for less than 5% of all breast neoplasms.
Case Report:
We report the case of a 60-year-old postmenopausal female diagnosed with rhabdomyosarcoma with infiltrating duct carcinoma. She was treated with modified radical mastectomy with axillary clearance and postoperative chemotherapy.
Conclusions:
Primary rhabdomyosarcoma of the breast in adults is extremely rare. Rhabdomyosarcomas in adults account for less than 3% of all adult primary soft-tissue sarcomas.
Primary breast sarcomas usually present as large painless breast lumps with no associated skin and nipple changes or axillary lymphadenopathy; they are more aggressive and have more rapid growth than epithelial malignancies or benign breast lesions. The tumor can grow to large size, around 5.8 cm. Affected patients are typically women in their 50 s (ranging from 17 to 89 years), but it is also seen in men.
The treatment of primary breast sarcomas requires a multidisciplinary approach. Surgery remains the mainstay of therapy. Chemotherapy has no clearly defined role in primary breast or soft-tissue sarcomas.
The prognosis of primary breast sarcomas depends on the histologic grade and size of the tumor. They spread locally and hematogenously, but they are not usually associated with axillary lymphadenopathy.
doi:10.12659/AJCR.883976
PMCID: PMC3715332  PMID: 23869249
rhabdomyosarcoma; breast; rhabdomyosarcoma breast
2.  Poorly differentiated synovial sarcoma of the vagina: a case report and a clinical literature review 
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.
doi:10.3332/ecancer.2008.99
PMCID: PMC3234049  PMID: 22275979
3.  Signature-Based Small Molecule Screening Identifies Cytosine Arabinoside as an EWS/FLI Modulator in Ewing Sarcoma 
PLoS Medicine  2007;4(4):e122.
Background
The presence of tumor-specific mutations in the cancer genome represents a potential opportunity for pharmacologic intervention to therapeutic benefit. Unfortunately, many classes of oncoproteins (e.g., transcription factors) are not amenable to conventional small-molecule screening. Despite the identification of tumor-specific somatic mutations, most cancer therapy still utilizes nonspecific, cytotoxic drugs. One illustrative example is the treatment of Ewing sarcoma. Although the EWS/FLI oncoprotein, present in the vast majority of Ewing tumors, was characterized over ten years ago, it has never been exploited as a target of therapy. Previously, this target has been intractable to modulation with traditional small-molecule library screening approaches. Here we describe a gene expression–based approach to identify compounds that induce a signature of EWS/FLI attenuation. We hypothesize that screening small-molecule libraries highly enriched for FDA-approved drugs will provide a more rapid path to clinical application.
Methods and Findings
A gene expression signature for the EWS/FLI off state was determined with microarray expression profiling of Ewing sarcoma cell lines with EWS/FLI-directed RNA interference. A small-molecule library enriched for FDA-approved drugs was screened with a high-throughput, ligation-mediated amplification assay with a fluorescent, bead-based detection. Screening identified cytosine arabinoside (ARA-C) as a modulator of EWS/FLI. ARA-C reduced EWS/FLI protein abundance and accordingly diminished cell viability and transformation and abrogated tumor growth in a xenograft model. Given the poor outcomes of many patients with Ewing sarcoma and the well-established ARA-C safety profile, clinical trials testing ARA-C are warranted.
Conclusions
We demonstrate that a gene expression–based approach to small-molecule library screening can identify, for rapid clinical testing, candidate drugs that modulate previously intractable targets. Furthermore, this is a generic approach that can, in principle, be applied to the identification of modulators of any tumor-associated oncoprotein in the rare pediatric malignancies, but also in the more common adult cancers.
Todd Golub and colleagues show that a gene expression-based screen of small-molecule libraries can identify candidate drugs that modulate cancer-associated oncoproteins.
Editors' Summary
Background.
Cancer occurs when cells accumulate genetic changes (mutations) that allow them to divide uncontrollably and to travel throughout the body (metastasize). Chemotherapy, a mainstay of cancer treatments, works by killing rapidly dividing cells. Because some normal tissues also contain dividing cells and are therefore sensitive to chemotherapy drugs, it is hard to treat cancer without causing serious side effects. In recent years, however, researchers have identified some of the mutations that drive the growth of cancer cells. This raises the possibility of designing drugs that kill only cancer cells by specifically targeting “oncoproteins” (the abnormal proteins generated by mutations that transform normal cells into cancer cells). Some “targeted” drugs have already reached the clinic, but unfortunately medicinal chemists do not know how to inhibit the function of many classes of oncoproteins with the small organic molecules that make the best medicines. One oncoprotein in this category is EWS/FLI. This contains part of a protein called EWS fused to part of a transcription factor (a protein that controls cell behavior by telling the cell which proteins to make) called FLI. About 80% of patients with Ewing sarcoma (the second commonest childhood cancer of bone and soft tissue) have the mutation responsible for EWS/FLI expression. Localized Ewing sarcoma can be treated with nontargeted chemotherapy (often in combination with surgery and radiotherapy), but treatment for recurrent or metastatic disease remains very poor.
Why Was This Study Done?
Researchers have known for years that EWS/FLI expression drives the development of Ewing sarcoma by activating the expression of target genes needed for tumor formation. However, EWS/FLI has never been exploited as a target for therapy of this cancer—mainly because traditional approaches used to screen libraries of small molecules do not identify compounds that modulate the activity of transcription factors. In this study, the researchers have used a new gene expression–based, high-throughput screening (GE-HTS) approach to identify compounds that modulate the activity of EWS/FLI.
What Did the Researchers Do and Find?
The researchers used a molecular biology technique called microarray expression profiling to define a 14-gene expression signature that differentiates between Ewing sarcoma cells in which the EWS/FLI fusion protein is active and those in which it is inactive. They then used this signature to screen a library of about 1,000 chemicals (many already approved for other clinical uses) in a “ligation-mediated amplification assay.” For this, the researchers grew Ewing sarcoma cells with the test chemicals, extracted RNA from the cells, and generated a DNA copy of the RNA. They then added two short pieces of DNA (probes) specific for each signature gene to the samples. In samples that expressed a given signature gene, both probes bound and were then ligated (joined together) and amplified. Because one of each probe pair also contained a unique “capture sequence,” the signature genes expressed in each sample were finally identified by adding colored fluorescent beads, each linked to DNA complementary to a different capture sequence. The most active modulator of EWS/FLI activity identified by this GE-HTS approach was cytosine arabinoside (ARA-C). At levels achievable in people, this compound reduced the abundance of EWS/FLI protein in and the viability and cancer-like behavior of Ewing sarcoma cells growing in test tubes. ARA-C treatment also slowed the growth of Ewing sarcoma cells transplanted into mice.
What Do These Findings Mean?
These findings identify ARA-C, which is already used to treat children with some forms of leukemia, as a potent modulator of EWS/FLI activity. More laboratory experiments are needed to discover how ARA-C changes the behavior of Ewing sarcoma cells. Nevertheless, given the poor outcomes currently seen in many patients with Ewing sarcoma and the historical reluctance to test new drugs in children, these findings strongly support the initiation of clinical trials of ARA-C in children with Ewing sarcoma. These results also show that the GE-HTS approach is a powerful way to identify candidate drugs able to modulate the activity of some of the oncoproteins (including transcription factors and other previously intractable targets) that drive cancer development.
Additional Information.
Please access these Web sites via the online version of this summary at http://dx.doi.org/10.1371/journal.pmed.0040122.
Cancerquest from Emory University, provides information on cancer biology (also includes information in Spanish, Chinese and Russian)
The MedlinePlus encyclopedia has pages on Ewing sarcoma
Information for patients and health professionals on Ewing sarcoma is available from the US National Cancer Institute
Cancerbackup offers information for patients and their parents on Ewing sarcoma
Wikipedia has pages on DNA microarrays and expression profiling (note that Wikipedia is a free online encyclopedia that anyone can edit)
doi:10.1371/journal.pmed.0040122
PMCID: PMC1851624  PMID: 17425403
4.  Ewing's sarcoma of the mandible 
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]).
doi:10.4103/0975-5950.94479
PMCID: PMC3343390  PMID: 22639511
Child; diagnosis; Ewing's sarcoma; immunohistochemistry; mandible
5.  Embryonal rhabdomyosarcoma of the cervix presenting as a cervical polyp in a 16-year-old adolescent: a case report 
Introduction
Embryonal rhabdomyosarcoma of the female genital tract is rare in the cervix. It has been mainly discussed in the context of individual case studies. It tends to occur in children and young women. Treatment ranges from radical surgery to conservative surgery, followed by chemotherapy.
Case presentation
A 16-year-old Moroccan adolescent girl presented to our center with a protruding mass from her vaginal introitus, as a polyp of 6cm. An examination revealed a polyp within her vagina, thought to be arising from her cervix and a polypectomy was performed. Microscopic findings are consistent with an embryonal rhabdomyosarcoma (botryoide type). A computed tomography of her thorax, abdomen and pelvis were performed and residual disease was found as a mass located at her cervix, which measured approximately 4.5cm in its widest dimensions, without evidence of metastatic disease. Due to the fact that she is young, after discussions in a multidisciplinary meeting, she was subsequently treated with four cycles of multi-agent chemotherapy. Two cycles of chemotherapy and radiotherapy were administered due to the lack of response, but she presented vaginal bleeding with persistence of the same mass in computed tomography. Hence a total interadnexal hysterectomy was made. A histologic examination found residual embryonal rhabdomyosarcoma (botryoide type) located in all her cervix and she is currently under chemotherapy.
Conclusions
The presence of a cervical polyp in an adolescent is a gynecologic oddity and must necessarily be examined histologically because it might be a rhabdomyosarcoma. This is extremely important because diagnosis at an early stage of the disease is a highly favorable prognostic factor that allows “fertility-sparing surgery” for these young patients.
doi:10.1186/1752-1947-8-241
PMCID: PMC4092352  PMID: 24986146
Cervix; Embryonal rhabdomyosarcoma; Management; Prognostic factors
6.  A 63-year-old woman presenting with a synovial sarcoma of the hand: a case report 
Introduction
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.
Case presentation
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.
Conclusion
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.
doi:10.1186/1752-1947-6-385
PMCID: PMC3514372  PMID: 23148739
Synovial sarcoma; Hand; Surgery; Malignant tumor
7.  An unusual case of metastasis of a pulmonary undifferentiated pleomorphic sarcoma to the right ventricle: a case report 
Introduction
Undifferentiated pleomorphic sarcoma is defined as a pleomorphic high-grade sarcoma whose line of differentiation cannot be determined. These tumors constitute less than 5% of all sarcomas in adults. Cardiac neoplasms are rare, and most are metastatic in origin. More than one-third of cardiac metastases originate from lung cancer. Symptoms of cardiac neoplasms usually appear late in the course of the disease and are often ignored because of the more severe effects of the primary malignancy or its therapy. We present the case of a patient with undifferentiated pleomorphic sarcoma of the lung presenting with symptomatic right-heart failure secondary to cardiac metastasis. The purpose of this report is to present this unusual case.
Case presentation
Our patient was a 59-year-old Chinese woman with symptomatic metastasis of an undifferentiated pleomorphic sarcoma of the lung to the right ventricle. She had a history of a stage IV, pulmonary, undifferentiated pleomorphic sarcoma that had been successfully treated with chemotherapy and radiotherapy 4 years ago. A complete response was obtained, and she was in remission until the cardiac metastasis. She underwent surgical excision of the cardiac mass because it caused dyspnea and posed a high risk of sudden death, pulmonary embolism or tricuspid obstruction. Histopathological and immunohistochemical examinations of the surgical specimen established the diagnosis of undifferentiated pleomorphic sarcoma and confirmed that the cardiac tumor was a metastasis from the lung.
Conclusions
In patients who have known metastatic neoplasms and present with cardiac manifestations, whether detected during history taking or physical examination, the clinician should be alert to the possibility of cardiac metastases. In patients with cardiac metastases, the therapeutic alternatives are limited to palliative treatment of symptoms and chemotherapy. In some patients, surgery can be used to relieve symptoms. We have reported the first case of symptomatic cardiac metastases from an undifferentiated pleomorphic sarcoma of the lung. Our patient underwent surgical resection, and her symptoms improved significantly. This case is unique because it is the only reported case of undifferentiated pleomorphic sarcoma of the lung which metastasized to the heart, and in which symptomatic improvement was effectively obtained with surgical resection.
doi:10.1186/1752-1947-7-165
PMCID: PMC3750226  PMID: 23805953
Cardiac metastasis; Lung undifferentiated pleomorphic sarcoma; Surgical resection
8.  Delayed presentation of osteochondroma on the ventral surface of the scapula 
Osteochondroma is a cartilage-covered bony excrescence that arises from the surface of a bone. It is the most common benign bone tumor in the scapula and can also present as multiple masses in multiple hereditary exostosis. A solitary scapular lesion might lead to “snapping scapula” syndrome, which is characterized by a sometimes audible but usually palpable grinding sensation experienced with scapular abduction. Snapping scapula is usually painless without specific symptoms; however, discomfort may result from the mechanical effects of a ventral scapular mass projecting onto a normally smooth, gliding scapulothoracic joint. Furthermore, malignant transformation of the cartilaginous cap, by virtue of the increased mass, may precipitate symptoms. The visual deformities and interference with major joint function are the most frequent musculoskeletal complaints related by patients. Indications for operative intervention include painful mechanical dysfunction unresponsive to conservative measures and sarcomatous change. Ventral scapular osteochondromas have been reported to cause scapular asymmetry and should be ruled out as part of a differential diagnosis to a “winged” scapula. A solitary osteochondroma can be excised either by arthroscopic or by open means, with the size of the lesion dictating precise management. Scapular osteochondromas usually are detected early during maturation due to mechanical symptoms or gross deformity. Unfortunately, most require surgical excision. Most surgical excisions have been reported in adolescents and young adults. We report an unusual case of osteochondroma from the ventral surface of the scapula in 56-year-old woman who had experienced delayed onset mechanical symptoms which required surgical excision.
doi:10.4103/0973-6042.96996
PMCID: PMC3391786  PMID: 22787335
Osteochondroma; scapula; shoulder
9.  Pseudo-arthrosis of the spine of the scapula: a case report with a delayed diagnosis 
Scapular spine fractures are rare injuries. The aim of this study was to evaluate a late-diagnosed scapular spine pseudo-arthrotic patient. Because of the surrounding soft tissue mass and overlapping of the scapula with the thoracal bones on a roentgenogram, diagnosis may be missed or delayed for years. We present a case of scapular spine pseudo-arthrosis in a 50-year-old man, who sustained a traffic accident 2 years ago. He was treated as a soft tissue injury of the left shoulder and later as a rotator cuff tear. His scapular spine fracture was diagnosed as pseudo-arthrosis of the scapular spine with a diagnostic delay of 2 years. Isolated scapular spine fractures are rare, usually associated with other injuries and frequently treated non-operatively. Sagging of the acromion as a result of a scapular spine fracture may mimic supraspinatus outlet impingement. If a painful pseudo-arthrosis limits the function of a shoulder, fractured ends should be fixed until union occurs. Although scapular spine fractures are rarely seen, they must take place in the differential diagnosis of impingement syndromes of the shoulder.
doi:10.1007/s11751-014-0210-2
PMCID: PMC4278966  PMID: 25540121
Pseudo-arthrosis; Scapula; Late diagnosis
10.  Gastrointestinal stromal tumor of the rectum with scapular metastasis: a case report 
Introduction
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.
Case presentation
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.
Conclusion
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.
doi:10.1186/1752-1947-6-145
PMCID: PMC3407775  PMID: 22676893
11.  Epidemiology and therapies for metastatic sarcoma 
Clinical Epidemiology  2013;5:147-162.
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.
doi:10.2147/CLEP.S28390
PMCID: PMC3660127  PMID: 23700373
chemotherapy; pediatric sarcoma; rhabdomyosarcoma; osteosarcoma; Ewing sarcoma; synovial sarcoma
12.  A 46-year-old Chinese woman presenting with retroperitoneal follicular dendritic cell sarcoma: a case report 
Introduction
Follicular dendritic cells are non-phagocytic, non-lymphoid cells of the immune system that are necessary for antigen presentation and the regulation of reactions in the germinal centers of the lymph nodes. Follicular dendritic cell sarcoma is an unusual cancer, particularly in the intra-abdominal region. In the present report we describe an unusual case of retroperitoneal follicular dendritic cell sarcoma that emphasizes the difficulty of diagnosing and treating this tumor. Retroperitoneal follicular dendritic cell sarcoma has only been rarely reported in the literature to date.
Case presentation
A 46-year-old Chinese woman of Han ethnicity presented with chronic right lower quadrant abdominal pain over the preceding 4 weeks. The tumor was resected and submitted to histopathological examination. The case was verified as retroperitoneal follicular dendritic cell sarcoma by microscopic examination and immunohistochemical analysis. After diagnosis, she received postoperative radiotherapy and chemotherapy. She has survived 3 years postoperatively, although she has a pulmonary metastasis.
Conclusions
Retroperitoneal follicular dendritic cell sarcoma may demonstrate aggressive potential. This study indicated that postoperative adjuvant radiotherapy and chemotherapy could extend the survival of a patient with retroperitoneal follicular dendritic cell sarcoma.
doi:10.1186/1752-1947-8-113
PMCID: PMC3977978  PMID: 24708915
Follicular dendritic cell sarcoma; Immunohistochemistry; Pathology; Retroperitoneal; Therapy
13.  Primary osteogenic sarcoma of the breast 
Background
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.
Case presentation
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.
Conclusion
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
doi:10.1186/1477-7819-4-90
PMCID: PMC1702348  PMID: 17156481
14.  Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases 
Sarcoma  1999;3(1):11-15.
Introduction and purpose. Primary Ewing's sarcoma arising from the bones of the head and neck region is extremely rare representing only 1– 4% of all Ewing's sarcoma cases. Previous reports suggest a better prognosis for that particular anatomic site. The purpose of this study was to analyze the clinico-epidemiologic characteristics of that rare clinical presentation, as well as its patterns of failure and prognosis following treatment.
Materials and methods. This study included a retrospective review of the medical records of patients with the diagnosis of Ewing's sarcoma of the head and neck region treated at King Faisal Specialist Hospital and Research Center between 1975 and 1996.
Results. Out of a total number of 24 cases analyzed, there were 17 males and 7 females with a ratio of 2.4:1. The median age at diagnosis was 16.5 years. A painful swelling was the most common clinical presentation.The maxilla was the most common site of presentation (9/24 cases). There were 3/24 cases who presented with metastatic disease at diagnosis.The majority of patients (16/24 cases) had a tumor size >10 cm. Most patients were treated with systemic chemotherapy plus localized irradiation following an initial biopsy.With a mean follow up of 3.4 years, the 5-year actual overall survival (OS) for the whole group was 53%, while the 5-year actuarial disease-free survival (DFS) was 30%. These figures were higher than those repor ted from our institution for young patients (≤ 14 years treated for Ewing' s sarcoma in other anatomic locations (30% v 15%). The response to chemotherapy was the only prognostic factor that affected both the OS and DFS.
Conclusion. The prognosis of Ewing's sarcoma of the head and neck region is slightly better than that of other anatomic sites.The response to systemic chemotherapy is one of the most important prognostic factors affecting both DFS and OS of Ewing's sarcoma of the head and neck. Multimodality therapy consisting of an initial biopsy, aggressive combination chemotherapy and localized radiotherapy is the treatment of choice for Ewing's sarcoma of the head and neck region and may result in long-term survival.
doi:10.1080/13577149977811
PMCID: PMC2395405  PMID: 18521259
15.  Ewing sarcoma of the liver with multilocular cystic mass formation: a case report 
BMC Cancer  2015;15:16.
Background
Ewing sarcoma is a rare tumor that occurs commonly in the long bones of children or adolescents that can also arise in soft tissues including the extremities, retroperitoneum, chest wall, and rarely in the liver as primary sites. We report a case of Ewing sarcoma arising primarily in the liver and, to our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver.
Case presentation
A 27-year-old Japanese woman was admitted with sudden onset right upper abdominal pain. Clinical examination revealed a multilocular cystic mass consisting of thickened, irregular septa and nodal walls in the right hepatic lobe. Ultrasound-guided aspiration biopsy of the liver mass showed clusters of small atypical round cells and the clinical preoperative diagnosis was mucinous cystadenoma of the liver. The patient underwent an extended right hepatectomy and histopathological findings revealed sheet-like proliferation of small- to medium-sized round cells. Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1 was detected by reverse transcriptase polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma arising primarily in the liver and the patient received adjuvant systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. We found no evidence of recurrence 15 months after completing chemotherapy.
Conclusion
We present an extremely rare case of Ewing sarcoma arising primarily in the liver. To our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver, and the first case with a multilocular cystic liver mass. Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion. Clinicians should consider the possibility of Ewing sarcoma in young patients with a multilocular cystic mass with thick and/or irregular cyst walls in the liver.
doi:10.1186/s12885-015-1017-3
PMCID: PMC4307901  PMID: 25608963
Ewing sarcoma; Primitive neuroectodermal tumor; Cysts; Liver Neoplasms; Chemotherapy
16.  Primary Pulmonary Ewing’s Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children 
Ewing’s sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing’s sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.
doi:10.7860/JCDR/2014/8681.4713
PMCID: PMC4190773  PMID: 25302247
Children; Ewing’s sarcoma; Pulmonary; Superior vena cava (SVC) syndrome
17.  Synovial sarcoma of the infratemporal fossa: A case report 
Oncology Letters  2014;8(5):2165-2170.
Synovial sarcomas (SS) are high-grade soft-tissue sarcomas, predominantly found in the deep soft tissues of the lower extremities, with only 3–5% occurring in the head and neck region. Primary SS of the infratemporal fossa (ITF) is exceptionally uncommon. The present study reports the case of a 23-year-old female with an SS arising in the ITF. To the best of our knowledge, this case is only the second patient with intracranial involvement recorded in the literature. The patient was treated primarily with surgery, followed by a total of 60 Gy adjuvant radiotherapy and chemotherapy, consisting of cisplatin (25 mg/m2 intravenously on days one to three), epirubicin (25 mg/m2 intravenously on days one and two) and ifosfamide (1.8 g/m2 intravenously on days one to five) for three cycles. At present, two years after this multimodal therapy, the patient exhibits no signs of loco-regional recurrence or distant metastases. This study highlights the importance of a multidisciplinary approach in the diagnosis and treatment of this extremely rare entity with intracranial extension. In addition, the study reviews the English literature with regard to SS of ITF and discusses the clinicopathological features, management and outcome.
doi:10.3892/ol.2014.2436
PMCID: PMC4186634  PMID: 25295104
synovial sarcoma; infratemporal fossa
18.  Pediatric Soft Tissue Sarcomas 
Soft tissue sarcomas in children are relatively rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or one of the non-RMS soft tissue sarcomas (NRSTS). Of these, 350 are cases of RMS. RMS is the most common soft tissue sarcoma in children 14 years old and younger, and NRSTS is more common in adolescents and young adults. Infants also get NRSTS, but their tumors constitute a distinctive set of histologies, including infantile fibrosarcoma and malignant hemangiopericytoma, not seen in adolescents. Surgery is a major therapeutic modality for all pediatric soft tissue sarcomas, and radiation can play a role in the local therapy for these tumors. RMS is always treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the subset of NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas, including new approaches to therapy aimed at improving the dismal prognosis of patients who have recurrent and metastatic disease.
doi:10.1016/j.suc.2008.03.008
PMCID: PMC4273573  PMID: 18514702
19.  Two cases of acrometastasis to the hands and review of the literature 
Current Oncology  2008;15(5):51-58.
This paper reports two cases of acrometastasis to the hands. The first case involved a 78-year-old woman with a permeative osteolytic lesion in her proximal second metacarpal. A biopsy of this lesion suggested a diagnosis of non-small-cell lung carcinoma with secondary osseous metastasis. This was the first presentation of the woman’s primary diagnosis. A single 8-Gy fraction of palliative radiotherapy was delivered to the patient’s left hand. The treatment proved successful: the woman soon experienced pain relief and regained the use of her hand. The second case involved a 69-year-old woman with extensive lytic destruction involving the proximal two thirds of her third metacarpal. This patient had been diagnosed with carcinoma of the breast in 1990. She also received a single 8-Gy fraction of radiation, which improved both her pain and her hand mobility.
An extensive review of the literature uncovered 257 previously reported cases of acrometastasis. Articles were analyzed based on age and sex of the patient, site of the primary carcinoma, metastatic locations within the hand and affected appendage or appendages, the treatment given, and the patient’s length of survival. Men were almost twice as likely to experience acrometastasis as women, and the median age of the patients overall was 58 years (range: 18 months–91 years). Lung, kidney, and breast carcinoma were the three most prevalent primary diagnoses reported in the literature. Cancers of the colon, stomach, liver, prostate, and rectum affected the remainder of the population.
Overall, the right hand was more often host to the metastatic lesions. In addition, almost 10% of the patients experienced lesions in both hands. The third finger was the digit most affected by osseous metastases reported in the literature. Lesions of the thumb, fourth finger, second finger, and fifth finger were less commonly reported. The region of the digit most often affected within the patient population was the distal phalanx. The metacarpal bones, proximal phalanges, and middle phalanges comprised the remainder of the four most frequent acrometastatic sites. In the literature, single lesions were more prevalent than multiple bony lesions.
Based on the reported cases, amputation appeared to be the preferred method of treatment. Radiation, excision, and systemic therapy were the next most frequently used treatments. Patient survival was not well documented within the literature. However, the median survival of patients in the reported cases was 6 months. Thus, our review suggested that a diagnosis of hand metastasis is an indication of poor prognosis.
This report serves to emphasize the importance of properly diagnosing acrometastases. Identifying and effectively treating these metastases in a timely manner can lead to a dramatic improvement in a patient’s quality of life.
PMCID: PMC2582515  PMID: 19008991
Acrometastasis; hand metastasis; bone metastases
20.  Clinical management of secondary angiosarcoma after breast conservation therapy 
Aim
The aim of this paper is to summarize the treatment outputs of secondary angiosarcoma after breast conservation therapy at St. Eizabeth Cancer Centre, Slovakia.
Background
Angiosarcoma of the breast is a rare but very aggressive malignant tumor of the vascular endothelium, characterized by rapidly proliferating and extensively infiltrating growth. Breast angiosarcoma may occur de novo, or as a complication of radiation therapy, or chronic lymphedema secondary to axillary lymph node dissection for mammary carcinoma. Radiotherapy in the treatment of breast cancer is associated with an increased risk of subsequent sarcoma.
Materials and methods
Retrospective study of medical records from the cancer databases was done in order to analyze the secondary breast angiosarcoma. This disease is an iatrogenic condition that warrants close follow-up and judicial use of radiotherapy in breast conserving therapy. Therefore, it is more prevalent in cases treated with radiotherapy, occurring especially in or adjacent to the radiation field. Clinical histories and follow-up data of identified patients after breast conservation therapy of invasive breast cancer were reviewed. In addition, a comprehensive literature review on diagnosis and treatment procedures was done in order to summarize state-of-the-art clinical approach.
Results and discussions
Three cases of secondary angiosarcoma after breast conservation therapy (BCT) were identified among 4600 patients treated at St. Elizabeth Cancer Institute during previous 16 years (1995–2011). Secondary breast angiosarcoma was diagnosed in a median period of 11 years following primary radiotherapy, median age at the time of diagnosis was 75 years. Surgical treatment consisted of radical mastectomy. The first patient, a 56-year-old woman received neoadjuvant chemotherapy (docetaxel + gemcitabin), second one (75 year) was treated by radiotherapy (TD 26 Gy, 2 Gy per fraction), since chemotherapy was not indicated. The last patient (80 year) got adjuvant chemotherapy (paclitaxel). Average follow up of the patients was 31 months. As of 31 July 2012, our patients were doing well without evidence of recurrent disease after treatment.
Conclusions
Angiosarcoma remains a difficult management problem with poor loco-regional and distal control. In our study, an overall incidence rate of secondary breast angiosarcoma is 0.065%. Although the prognosis for this disease is poor (typical survival period is 14.5–34 months with a 5-year survival rate of approximately 15%), all the three patients treated at our institute are alive and disease-free at the end of reported period. Finally, it is assumed that the use of breast conserving therapy will increase the incidence of post-irradiation angiosarcoma but the small difference in risk of subsequent sarcoma of the breast cancer patients receiving radiotherapy does not suppress its benefit.
doi:10.1016/j.rpor.2013.07.013
PMCID: PMC4056516  PMID: 24936318
Breast cancer; Sarcoma; External-beam radiotherapy; Multimodality therapy; Radiationinduced late effects
21.  EXTREMITY SARCOMA SURGERY IN YOUNGER CHILDREN: TEN YEARS OF PATIENTS TEN YEARS AND UNDER 
The Iowa Orthopaedic Journal  2011;31:145-153.
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.
PMCID: PMC3215128  PMID: 22096434
22.  Scapular osteochondrolipoma: Imaging features with pathological correlation 
Oncology Letters  2013;6(3):817-820.
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.
doi:10.3892/ol.2013.1455
PMCID: PMC3789040  PMID: 24137417
osteochondrolipoma; scapula; magnetic resonance imaging; pathology
23.  Long-Term Results of Modified Green Method in Sprengel’s Deformity 
Aim
Congenital undescended scapula (Sprengel deformity) is a rare deformity that is reported in the literature mostly as small case series with short- or medium-term follow-up periods. Here, we aimed to present the long-term results of this deformity treated with modified Green procedure.
Method
The modified Green procedure was performed in 24 patients (28 shoulders) with Sprengel deformity. Clavicular osteotomy accompanied in all, and omovertebral bone excision in 13 shoulders. The mean age of the patients at the time of surgery was 4.5 years (range 1.5–17 years). Mean follow-up period was 11 years 4 months (4.3–17 years). Preoperative cosmetic appearance was noted as Cavendish III in 17 shoulders and as IV in 11 shoulders. In addition to the Cavendish scale, shoulder abduction, shoulder asymmetry, and scapular elevation and medialization were evaluated.
Results
The decrease in scapular elevation and Cavendish scale, and the improvement in shoulder abduction and scapular medialization postoperatively were statistically significant (P < 0.001). Cosmetic improvement of at least one Cavendish grade were attained in 88.9% of shoulders. One patient (unilateral) who was Cavendish grade IV preoperatively died in the early postoperative period from unrelated causes. Of the remaining 10 preoperatively grade IV shoulders, 2 remained at the same grade, 1 improved to grade III, and 7 shoulders to grade I. Of the 17 preoperatively grade III shoulders, 1 shoulder stayed the at same grade, 7 shoulders improved to II, and 9 shoulders to grade I. Postoperative winging in 2 shoulders and hypertrophic scarring in 6 shoulders were noted.
Conclusion
The modified Green procedure is a relatively safe and reliable method in the treatment of severe Sprengel deformity cases and provides highly constructive and aesthetic results in the long term.
doi:10.1007/s11832-010-0265-7
PMCID: PMC2908338  PMID: 21804892
Sprengel’s deformity; Congenital high scapula; Modified Green procedure
24.  Undifferentiated round cell sarcoma of the broad ligament 
ecancermedicalscience  2013;7:303.
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.
doi:10.3332/ecancer.2013.303
PMCID: PMC3622498  PMID: 23589732
sarcoma; broad ligament tumour; round cells tumour; undifferentiated sarcoma
25.  Peripheral primitive neuroectodermal tumor of the dura in a 51-year-old woman following intensive treatment for breast cancer 
Patient: Female, 51
Final Diagnosis: Ewing sarcoma
Symptoms: Visual disturbances
Medication: —
Clinical Procedure: —
Specialty: Oncology
Objective:
Rare disease
Background:
Primitive neuroectodermal tumor/Ewing sarcoma (PNET/EWS) is a round blue cell sarcoma that shows varying degrees of neuroectodermal differentiation. PNET/EWS as a primary intracranial tumor is extremely uncommon.
Case Report:
We report a unique case of peripheral PNET presenting as an intracranial mass in an adult following chemotherapy and radiotherapy for a solid tumor. A 51-year-old woman with previously treated left breast cancer was evaluated for a newly developed brain mass. She underwent craniotomy with resection. Surgical pathology was consistent with a peripheral PNET/EWS with Ewing sarcoma gene translocation. She was treated appropriately with vincristine, cyclophosphamide, and doxorubicin (later dactinomycin) alternating with ifosfamide and etoposide.
Conclusions:
Although development of PNET/EWS presenting along the CNS is exceedingly rare in adults, establishing the proper diagnosis of this “small blue cell tumor” is critical. The further distinction between central PNET and peripheral PNET can greatly impact both prognosis and treatment. Our case also highlights the importance of considering the impact of prior intensive therapies, including radiation and chemotherapy, on predisposing to future PNET/EWS.
doi:10.12659/AJCR.890656
PMCID: PMC4102603  PMID: 25045413
Central Nervous System; Neuroectodermal Tumors, Primitive, Peripheral; Sarcoma, Ewing

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