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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The fine needle aspirate in a case of pleomorphic giant-cell carcinoma of the pancreas, an unusual but highly malignant variant of ductal carcinoma of the pancreas, was characterized by bizarre tumor giant cells, "osteoclastlike" giant cells and abundant mitoses. The differential diagnostic possibilities include sarcoma (rhabdomyosarcoma, malignant fibrous histiocytoma and liposarcoma), melanoma, choriocarcinoma, metastatic giant-cell carcinoma of the lung and giant-cell tumor of the pancreas. A combination of clinical history, imaging findings and fine needle aspiration biopsy with transmission electron microscopy could lead to the appropriate diagnosis and help differentiate this entity from the other possible considerations.
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PMID:Fine needle aspiration of pleomorphic giant-cell carcinoma of the pancreas. Case report with ultrastructural observations. 346 52

Seven patients underwent resection of a sarcoma of the extremity requiring excision of a vital artery and revascularization. These included osteosarcoma of the pubis, osteosarcoma of the distal femur, undifferentiated sarcoma of the thigh, liposarcoma of the thigh, liposarcoma of the popliteal space, chondrosarcoma of the proximal tibia and rhabdomyosarcoma of the thumb. Preoperative evaluation included computed tomographic scan, magnetic resonance imaging and angiography. Operation involved excision of the iliac artery in one instance, femoral artery in three, popliteal artery in two instances and radial artery in one. Wide local resection including revascularization was undertaken when the tumor could be resected with the artery but separated from the nerve and it was a low grade malignant disease or the patient refused amputation for a high grade malignant disease. In five patients, the tumor margins were adequate. One patient with an osteosarcoma of the pubis with distant disease had a palliative resection and one patient had positive margins but refused amputation. Coverage of the soft tissue and vascular grafts was achieved using a distant pedicle flap in two patients. Amputation was avoided and each patient remained ambulatory. Five patients remained free of disease with patent grafts at six months to six years of follow-up study. One patient died of late myocardial infarction and one who underwent palliative resection died eight months later of metastatic disease. Involvement of the major arterial circulation does not preclude adequate resection of sarcomas of the extremity with limb salvage.
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PMID:Limb sparing operations for sarcomas of the extremities involving critical arterial circulation. 347 4

544 malignant soft tissue tumors have been collected at the Pediatric Tumor Registry in Kiel including 300 cases of rhabdomyosarcoma (55%). In 237 of the 300 cases the diagnosis is certain. Liposarcoma and malignant fibrous histiocytoma which are typical tumors of adult age are rare in our material. Among rhabdomyosarcomas embryonal rhabdomyosarcoma (eRMS) clearly predominates accounting for almost 72% of all rhabdomyosarcomas. Differentiation of tumor cells in eRMS may vary considerably. Therefore, three groups of eRMS were distinguished and analyzed for clinico-pathologic features: 1. Primitive eRMS with less than 10 rhabdomyoblasts. 2. Intermediate eRMS with 10-50% rhabdomyoblasts. 3. Well differentiated eRMS with greater than 50% rhabdomyoblasts. By immunohistochemistry, vimentin positive cells were found in all three groups. The number of desmin positive cells depended upon the grade of differentiation. Thus, there were more desmin positive cells in well differentiated eRMS. Primitive and well differentiated eRMS were predominantly located in the head and neck area, intermediate eRMS in the abdomen. Primitive eRMS were noted in higher stages than tumors of the other two groups. Response to chemotherapy as evaluated in the 7th week of treatment was better in well differentiated eRMS. Moreover, patients of this group achieved more often complete remission. It is concluded from the present study that differentiation in eRMS may have an influence on the clinical presentation and clinical course of the disease. Therefore, this question should be investigated in more detail in a larger prospective study.
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PMID:[Rhabdomyosarcoma: morphology and cellular differentiation]. 352 25

The differential diagnosis of malignant soft tissue tumors constitutes a major problem in surgical pathology. A distinction among sarcomas is sometimes difficult, but this is a situation in which histologic examination of representative tissue will give a diagnosis in the majority of cases. Electron microscopy sometimes contributes to the proper diagnosis of sarcomas. For electron microscopy, fresh samples from 110 cases of sarcoma filed at the Department of Pathology, Faculty of Medicine, Kyushu University, were examined. The distinguishing ultrastructural appearance of the following tumors are discussed: Spindle cell sarcomas (leiomyosarcoma 10 cases, malignant Schwannoma 5, fibrosarcoma 5, monophasic synovial sarcoma 7). Round cell sarcomas (rhabdomyosarcoma 10 cases, extraskeletal Ewing's sarcoma 1, malignant neuroepithelioma 1, malignant rhabdoid tumor of soft parts 3, monophasic synovial sarcoma 9). Pleomorphic sarcomas (malignant fibrous histiocytoma 24 cases, pleomorphic liposarcoma 1). Myxoid sarcomas (myxoid malignant fibrous histiocytoma 8 cases, myxoid liposarcoma 8, extraskeletal myxoid chondrosarcoma 4). Sarcomas with a specific pattern. Using electron microscopy, general analysis of cellular morphology and detection of specific structures are helpful for evaluating these sarcomas. Nevertheless, poorly differentiated sarcomas may still pose a diagnostic dilemma, since their specific features are poorly represented.
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PMID:[An ultrastructural analysis of malignant soft tissue tumors]. 359 2

This is a retrospective review of 67 patients with soft tissue or visceral sarcomas treated during 1973-1982. There were 40 males and 27 females. The mean age was 36 with a range of 1 to 77 years of age. The most common histologic subtypes were rhabdomyosarcoma (19%), malignant fibrohistiocytoma (13%), fibrosarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans (12% each), and liposarcoma (10%). Among the 42 soft tissue sarcomas 33% occurred in the trunk, 35% and 21% in the lower and upper extremities, and 10% in the head and neck region. Among the 25 visceral sarcomas, 16% were in the thorax, 56% involved abdominal or retroperitoneal organs, and 28% were in the head and neck region. Overall, 33 of the 54 patients with early sarcomas were treated by surgical resection alone while 21 others had postoperative adjuvant radiotherapy and/or chemotherapy. Factors influencing survival are presented and patterns of metastasis discussed. Overall, 45% of the patients showed metastatic disease either at diagnosis (15%) or during the follow-up period (30%). The lung was involved in two-thirds of the cases and was the most common site of spread. In patients with rhabdomyosarcoma and malignant fibrohistiocytoma, regional lymph node metastasis was noted in 36% and 17%, respectively.
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PMID:Nonosseous sarcomas in a military hospital. 369 35

Although the greatest incidence of testicular neoplasms is in the age group 20 to 35.9% of all intrascrotal tumors occur in males over 60. They may be classified into four major groups based on the tissue origin of the tumor. The most common group comprising 50% are lymphoreticular neoplasms or lymphomas. They occur in both blacks and whites, result in a diffuse enlargement of the testis and commonly involve the epididymis and cord. Prognosis is poor and survival is usually less than two years. Germ cell tumors comprise 25% and the tumors are usually large. Most of them are the classical seminoma which has a good prognosis following orchiectomy and retroperitoneal radiation. A few are the spermatocytic seminoma which is usually benign. Three percent are teratocarcinoma with embryonal elements which is highly malignant and survival less than two years. Ten percent are tumors of gonadal stromal origin. There are two types, the Leydig cell and the more rare Sertoli cell. About one-fourth of these patients develop gynecomastia and some a decrease in libido. The prognosis is good as less than 10% of these tumors are malignant. Neoplasms of supportive and paratesticular structures comprise a heterogeneous group of benign and malignant lesions and comprise about 15% of intrascrotal tumors. Most of them have the same features as tumors of similar tissues encountered throughout the body. They are the mesothelioma or adenomatoid tumor, fibroma, rhabdomyosarcoma, leiomyoma, lipoma, liposarcoma, mucinous cystadenocarcinoma, and leiomyosarcoma. As in all intrascrotal tumors, the diagnosis, treatment, and prognosis are based on the microscopic findings after removal of the tumor.
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PMID:Intrascrotal tumors in the older male. 372 56

The incidence of pleomorphic carcinoma of the pancreas is low. In 34 cases of non-endocrinic cancer of the pancreas detected pathologically by operation and autopsy in our hospital from 1961 to 1984, 3 were pleomorphic carcinoma, comprising 8.8%. In histology, the tumor was clearly characterized by the bizarre uninuclear and multinuclear giant cells with abundant eosinophilic cytoplasm and malignant spindle cells. In these 3 cases, the tumor was originated from the acinic epithelium in 2 and from the ductal epithelium in 1. This tumor should be differentiated from pleomorphic rhabdomyosarcoma, liposarcoma, fibrosarcoma and lymphosarcoma, etc.
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PMID:[Pleomorphic carcinoma of the pancreas--report of 3 cases]. 374 49

Histopathologic peer review of specimens from 216 consecutive patients with soft-tissue or bone sarcomas on Southeastern Cancer Study Group protocols was performed by a panel of three pathologists. Subtype of sarcoma, degree of confidence in diagnosis, and grade were compared with agreement or disagreement in pathologic opinion from the primary member institution v the pathology review panel. The most common soft-tissue sarcoma was leiomyosarcoma, followed by malignant fibrous histiocytoma, fibrosarcoma, liposarcoma, malignant schwannoma, and rhabdomyosarcoma. There was total agreement between the primary pathologist and reviewer in 66% of cases. However, 12 cases (6%) were considered on review to not be sarcomas. In 27% of cases the subtype of sarcoma was felt to be incorrect by reviewers. Discordant diagnoses were more common when the confidence of the pathologist was less and when the tumor was of higher grade. There was no improvement in frequency of agreement in the second half of the study, despite educational workshops. We conclude that histologic peer review is important in sarcoma studies and is essential in many patients with presumed sarcomas.
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PMID:Soft-tissue and bone sarcoma histopathology peer review: the frequency of disagreement in diagnosis and the need for second pathology opinions. The Southeastern Cancer Study Group experience. 377 18

The preoperative results of computed tomography (CT) (n = 59) and digital subtraction angiography (DSA) (n = 14) were compared with the intraoperative site and the pathological histological finding. The tumor was correctly estimated by CT in 69% (difference less than 20%), and overestimated in 31% (difference greater than 20%). The superiority of CT consisted in the visualization of intratumor alterations and the representation of the muscle compartments concerned and of the spatial relationship of the tumor to the bone and large vessels. Specific morphological CT criteria were found only in rhabdomyosarcoma and liposarcoma. Unequivocial preoperative appraisals with regard to the histological finding to be expected were possible only in the case of lipoma. DSA was helpful for evaluation of vascular involvement and vascularization. The technique contributed to differential diagnosis (malignant-benign) in only 70%. In peripheral soft-tissue tumors (lower leg, forearm), arterial DSA is preferable to venous DSA. Angiography should be employed after the CT investigation and reserved for specific cases.
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PMID:Comparison of computed tomography and digital subtraction angiography of preoperative evaluation of soft-tissue tumors of the limbs. 391 21

Multimodality therapy and limb salvage procedures constitute a significant advance in the treatment of soft tissue sarcoma of the extremity. The applicability of these procedures to soft tissue sarcoma of the distal extremities was evaluated in a retrospective study of 159 such cases treated during a 10-year period. Thirty-seven patients were treated by operation alone, 57 had operation and adjuvant chemotherapy, and 65 had multimodal therapy. The distal extremities are unusual sites for soft tissue sarcomas and accounted for only 20% of our patients with sarcoma. The majority of patients were younger than 50 years and the sex distribution was approximately equal. The major histologic types were synovial cell sarcoma (18.2%), fibrosarcoma (15%), liposarcoma (13.8%), and rhabdomyosarcoma (13.8%). The 5-year survival rate was 72% with multimodality therapy, 72% for operation and chemotherapy, and 51% for operation alone. The local recurrence rate was 13% with operation alone, 9% with operation and adjuvant chemotherapy, and 12% with multimodality therapy. Based on our review, wide local excision and adjuvant chemotherapy proved to be as effective as multimodal therapy.
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PMID:Soft tissue sarcomas of the distal extremities. 395 64


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