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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body, particularly the trunk, retroperitoneum, or the head and neck. Pretreatment radiologic imaging is critical for defining the local extent of a tumor, staging the disease, guiding biopsies, and aiding in diagnosis. Core-needle biopsy is the preferred biopsy technique for diagnosing soft tissue sarcomas. The American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcomas is based on histologic grade, the tumor size and depth, and the presence of distant or nodal metastases. Despite improvements in local control rates with wide local resections and radiation therapy, metastasis and death remain a significant problem in 50% of patients who present with high-risk soft tissue sarcomas. The most common site of metastasis is the lungs, and metastasis generally occurs within two to three years after the completion of therapy. Progress in the molecular characteristics of these tumors should in the near future translate into molecularly based therapies that can be incorporated into standard treatment strategies.
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PMID:Soft tissue sarcomas. 1506 99

For patients with soft tissue sarcoma in an extremity, the outcome is thought to be poor if lymph node metastasis develops. The purpose of this study was to examine the impact of lymphatic involvement from soft tissue sarcoma on patient survival. Thirty-nine (3.7%) of 1066 patients who had surgery for soft tissue sarcoma in an extremity had lymph node metastases develop. Three (20%) of 15 patients with epithelioid sarcoma, four (19%) of 21 patients with rhabdomyosarcoma, two (11.1%) of 18 patients with clear cell sarcoma, and two (11.1%) of 18 patients with angiosarcoma had lymphatic involvement. Thirty patients who had resection of involved lymph nodes had an estimated 5-year survival of 57%, whereas nine patients treated without surgery all died within 30 months. An estimated 4-year survival of 71% for patients with isolated lymph node metastases was significantly better than 21% for patients with synchronous systemic and lymph node involvement. There was no difference in outcome for patients with isolated lymphatic involvement compared with patients with American Joint Committee on Cancer Stage III extremity sarcomas. These results suggest that long-term survival is possible after surgical resection of lymphatic metastases from soft tissue sarcoma. The American Joint Committee on Cancer should consider separating isolated nodal metastases from systemic involvement in patients with Stage IV sarcoma.
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PMID:Lymph node metastasis in soft tissue sarcoma in an extremity. 1534 63

Malignant melanoma of soft tissue, also called clear cell sarcoma (CCS) of tendons and aponeuroses, presents as a soft tissue mass, usually in the lower extremity of young females. We report two cases of CCS of the upper extremity--in a Korean and a Saudi Arabian girl. Because of the presence of melanin, melanosomes, S-100 protein and the tendency for regional nodal metastases, it has been suggested that this entity be considered a melanoma rather than a soft tissue sarcoma. The evidence for a neuroectodermal origin of these neoplasms is compelling. Despite these features, CCS has been reported as showing a distinct chromosomal translocation pattern t(12;22)(q13;q12) in certain isolated cytogenetic or molecular diagnostic case reports; however, there is no information about the breakpoints at genomic level. Such genetic studies need to be performed on primary and metastatic cutaneous, mucosal or ocular melanomas prior to considering them specific for malignant melanoma of soft tissue/CCS. Microscopically, solid nests and fascicles of pale fusiform or cuboidal cells are divided by fine fibrous tissue septa. Characteristically, the cells possess a round to oval nucleus with a central prominent nucleolus. Pleomorphism may or may not be present; the cytoplasm is clear due to the presence of glycogen but, occasionally, the cytoplasm is more eosinophilic, resembling a melanoma. A tumour size (> or = 5 cm) and the presence of necrosis are statistically significant predictors of poor prognosis. A lesion on the proximal part of an extremity progresses slower than one that is more distal (i.e. acral). An early diagnosis and initial radical surgery are essential for a favourable clinical outcome. Once regional lymph node metastases or haematogenous dissemination has occurred, the prognosis becomes dismal.
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PMID:Lessons to be learned: a case study approach. Malignant melanoma of soft tissue. 1571 52

A 40-yr-old woman with an asymptomatic sinonasal rhabdomyosarcoma (RMS) initially presented with submental nodal metastasis. The fine-needle aspiration (FNA) and the subsequent biopsy of the nodal metastasis were misinterpreted as metastatic carcinoma because the primary tumor was occult, the tumor cells were exclusively round cells with a nested arrangement, and rhabdomyoblasts were absent. The correct diagnosis of metastatic RMS became apparent when the primary sinonasal tumor, detected in a CT, was biopsy proven to be an alveolar RMS. Retrospectively, there were helpful clues to the correct diagnosis in the nodal FNA and biopsy. When FNA cytology or biopsy histology of a lymph node suggests metastatic carcinoma but the tumor cells are nonimmunoreactive to carcinoma markers, the differential diagnosis should be expanded to include not only metastatic melanoma but also metastatic sarcoma and lymphoma. Cytologically, the presence of multinucleated giant tumor cells, including the rosette forms, in the FNA smears should alert the cytopathologist to the possibilities of sarcoma and anaplastic large cell lymphoma.
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PMID:Rhabdomyosarcoma in an adult presenting with nodal metastasis: a pitfall in fine-needle aspiration cytology of lymph nodes. 1583 Mar 59

Clear cell sarcoma (CCS), also known as clear cell sarcoma of tendons and aponeuroses or malignant melanoma of soft tissue, is a rare malignant tumor and is histogenitically related to melanoma. The aim of this study was to describe our experience with the sentinel node (SN) procedure for CCS patients and to discuss the potential value of this technique for CCS patients. Five patients with a subcutaneous CCS, who underwent an SN procedure, are described. Two patients had positive SNs, with additional tumor positive nodes in both lymph node dissection specimens. Only the patients with tumor positive SNs developed recurrent disease during an average follow-up of 33 months. None of the negative SN patients developed recurrent disease and all were alive after an average follow-up of 39 months. SN status seems to predict additional nodal involvement and recurrent disease as well as survival. The SN procedure might be a useful and accurate staging procedure in CCS patients, comparable to the situation in melanoma.
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PMID:Sentinel node biopsy for clear cell sarcoma. 1667 85

Epithelioid sarcoma is a rare soft tissue sarcoma with a propensity for local aggressiveness, regional nodal spread, and pulmonary metastases. We report a case of epithelioid sarcoma in a neonate with bilateral optic nerve hypoplasia who developed liver, kidney, and bone metastases. The unusual presenting features and pattern of disease progression in this patient suggest that a different disease evaluation strategy should be considered for infants with epithelioid sarcoma.
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PMID:Neonatal epithelioid sarcoma: a distinct clinical entity? 1681 48

The procedure of sentinel node biopsy has been used previously in clear cell sarcoma. There are few studies reported. Due to the similar biological features with melanoma, this procedure can be effective. Sentinel biopsy is an option to detect nodal subclinical metastases. A 19-year-old man presented with ulcerated lesion in the fifth finger of the left hand. The biopsy reported clear cell sarcoma. Immunohistochemistry was positive for Vimentin and S-100. There was no evidence of regional disease. The sentinel lymph node biopsy, using patent blue and Tc-99 rhenium, was positive for metastases. Axillar dissection was carried out. The final report confirmed three metastasic nodes and the patient received adjuvant chemotherapy.
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PMID:[Clear cell sarcoma and sentinel lymph node biopsy. Case report and literature review]. 1688 85

Cutaneous angiosarcoma of the scalp is a rare highly aggressive malignant tumor that typically afflicts elderly patients and commonly presents with extensive local spread and distant metastasis. Distant metastases favor lung, liver, lymph nodes, and skin. Overall, the prognosis is poor. It differs from other soft tissue sarcomas in that the size of the lesion at presentation instead of tumor grade is the important prognostic factor. Optimal treatment is yet to be determined. Wide-margin complete excision with postoperative radiotherapy has been the most effective therapy. Chemotherapy and gene therapy have been used with some success. Local extent is critical in surgical planning, especially in the head and face, and is difficult to determine accurately with clinical examination and morphologic imaging tools. We report the case of a 70-year-old man diagnosed with multifocal angiosarcoma of the scalp. PET/CT imaging with F-18 2-fluoro-2-deoxyglucose (F-18 FDG) not only showed avid FDG uptake by an angiosarcoma (SUVmax = 10.7), but also simultaneously showed local extension of multifocal lesions with periosteal involvement and excluded metastatic abdominal nodal disease. PET/CT imaging after chemotherapy and before radiation therapy showed complete resolution of FDG uptake in the scalp and osseous lesions. Evaluation of more cases of this subset of soft tissue sarcoma with FDG PET/CT may suggest a possible role in not only staging angiosarcomas to determine the extent of local as well as distant disease, but also to potentially help determine response to therapy and early recognition of local or distant recurrence.
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PMID:F-18 fluorodeoxyglucose PET/CT as an imaging tool for staging and restaging cutaneous angiosarcoma of the scalp. 1692 Dec 76

A 12-year-old Persian cat was examined for a firm swelling of the right tarsal region and enlargement of the corresponding right popliteal lymph node. Cytologic evaluation demonstrated a population of malignant cells consistent with large cell lymphoma. Necropsy revealed a multi-lobulated subcutaneous mass involving the tarsus with some extension into adjacent deep muscular tissue. Histologically, the mass was composed of round cells with eosinophilic cytoplasm and pleomorphic anisokaryotic nuclei. Evidence of articular and nodal infiltration by these cells was observed. Differential diagnoses included synovial sarcoma and histiocytic sarcoma. Neoplastic cells were negative for cytokeratin, CD79a, and CD3 and positive for CD18, vimentin, lysozyme, and alpha-1-antitrypsin, most consistent with a diagnosis of histiocytic sarcoma. This is the first report of a histiocytic sarcoma involving a joint of a cat. The final diagnosis was based on the light microscopic appearance in combination with the immunohistochemical stains.
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PMID:Histiocytic sarcoma in the tarsus of a cat. 1709 63

Sinus histiocytosis with massive lymphadenopathy (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.
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PMID:Sinus histiocytosis with massive lymphadenopathy--a review of seven cases. 1718 39


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