Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinicopathologic findings in 200 cases of malignant fibrous histiocytoma (MFH) with follow-up information are presented. This tumor occurred principally as a mass on an extremity (lower extremity 49%, upper extremity 19%) or in the abdominal cavity or retroperitoneum (16%) of adults (peak incidence 61-70 years of age). It typically involved deep fascia (19%) or skeletal muscle (59%) and only rarely was confined to the subcutis without fascial involvement (7%). The MFH had variable morphologic features and frequently showed transitions from areas having a highly ordered storiform pattern to less differentiated areas having a pleomorphic appearance. The rate of local recurrence of the tumor was 44%, and of metastasis, 42%. Metastasis was most frequently to the lung (82%) and lymph nodes (32%). Factors that influenced the rate of metastasis included depth, size, and inflammatory component of the tumor. Tumors that were small, superficially located, or had a prominent inflammatory component metastasized less frequently than larger, more deeply located tumors. In our experience the MFH is the most common soft tissue sarcoma of late adult life, and many tumors previously diagnosed as pleomorphic variants of liposarcoma, fibrosarcoma, or rhabdomyosarcoma are probably examples of MFH. Although the histogenesis of this neoplasm remains controversial, we feel it is best regarded as a primitive and pleomorphic sarcoma showing partial fibroblastic and histiocytic differentiation, as reflected by collagen production and occasional phagocytosis.
 ...
PMID:Malignant fibrous histiocytoma: an analysis of 200 cases. 20 8

An ultrastructural study of pleomorphic liposarcoma with an unusual clinical presentation is described. A 69-year-old Caucasian female presented with recurrent superficial phlebitis of left leg, which responded only partially to conventional therapy. Following investigations, a mass was excised. Diagnosis of pleomorphic liposarcoma was made on microscopic examination. Numerous lipid droplets in the cytoplasm and often within intranuclear extensions of cytoplasm were seen on oil red O stain of frozen sections. Ultrastructural features are quite distinctive. The cells varied from small to often large pleomorphic cells with numerous lipid spaces without limiting membrane, large pleomorphic nuclei, and an abundance of cytoplasmic organelles. Centrioles were very prominent, in close proximity to nuclei, and collections of autophagocytic inclusions. Intercellular collagen was immature and scant. These features are compared to ultrastructural features of malignant fibrous histiocytoma, pleomorphic rhabdomyosarcoma, and cardiac myxoma, indicating that ultrastructural features are distinctive and help differentiate similar soft tissue tumors.
 ...
PMID:Ultrastructural observations in pleomorphic liposarcoma. 21 74

The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.
 ...
PMID:A clinicopathological study on soft tissue tumors of the head and neck. 22 15

A patient with abdominal liposarcoma is described, and the treatment of this in children extremely uncommon tumor is discussed. After complete surgical excision, local irradion with 5000-6000 rad should be considered only if no vital organs are irradiated. Because of the high tendency for local recurrence and for distant metastases of this tumor a primary polychemotherapy beginning immediately after surgical excision is proposed as in cases of other solid tumors in children for instance rhabdomyosarcoma or fibrosarcoma. 18 months after the surgical excision and after the beginning of polychemotherapy our patient is clinically well without demonstrable tumor growth.
 ...
PMID:[Treatment of abdominal liposarcoma in children (author's transl)]. 65 96

Pleomorphic carcinoma of the pancreas is a well defined histopathological entity characterized by non-cohesive, sarcoma-like growth pattern, and bizarre mono- and multinucleated tumor giant cells with abundant eosinophilic cytoplasm. Fifteen cases are identified in autopsy files of the Department of Pathology, Washington University School of Medicine, which represent 7.1% of all the non-endocrine pancreatic malignancies found at autopsy. Pleomorphic carcinoma is comparable to pancreatic adenocarcinoma in clinical features such as age, sex, and presenting symptoms except that it is more likely to occur in the body and tail of the pancreas, metastases invariably develop, hematogenous spread is more common, and the median survival is worse. Pleomorphic carcinoma could be distinguished from the pancreatic tumors that resemble giant cell tumor of the bone. Differential diagnostic features between it and amelanotic melanoma, hepatocellular carcinoma, choriocarcinoma, pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma, fibroxanthosarcoma, poorly differentiated epidermoid carcinoma, and giant cell carcinomas of the lung and thyroid are discussed.
 ...
PMID:Pleomorphic carcinoma of the pancreas: an analysis of 15 cases. 87 Jan 68

A group of 79 patients with soft tissue sarcomas and 46 with primary malignant bone tumors underwent lymphangiography as part of the initial diagnostic workup. In the group of soft tissue sarcomas, the overall incidence of metastases was 22 of 79 (28%). According to the site of origin, the highest incidence was found in tumors originating from the buttocks (57%), followed by those from the inferior limbs (31%). Considering the histology, the incidence ranges from 50% in anaplastic sarcoma, to 43% in rhabdomyosarcoma, to 23% for liposarcoma and fibrosarcoma. The incidence of lymphographically proven metastases in bone tumors was 8 to 46 (17%), with 1 out of 4 in reticulum cell sarcoma, 1/1 in chordoma, and 22% in Ewing's sarcoma. A radiographic/histologic correlation on the lymph nodes was obtained in 19/79 (24%) soft tissue sarcomas and in 4/46 (9%) bone tumors. In the 12 radiographically negative and 11 radiographically positive cases, this correlation was always correct. The results of this study suggest a larger use of lymphography in these tumors.
 ...
PMID:Lymphographic evaluation in bone and soft tissue sarcomas. 89 95

Fifteen patients with surgically incurable, advanced, and metastatic sarcomas were treated with courses of adriamycin and vincristine-actinomycin D alternating within a 7-week cycle. Three patients had objective partial responses for more than 3 months (liposarcoma, 4 months; fibrous histiocytoma, 15+ months; desmoid tumor, 19+ months) while two other patients (liposarcoma, leiomyosarcoma) had lesser responses. No beneficial effect could be attributed to therapy in two patients with leiomyosarcoma, two patients each with synovial cell sarcoma, fibrosarcoma, and chondrosarcoma, and one patient each with rhabdomyosarcoma and mesenchymoma. No additive effect of alternating full doses of these agents could be demonstrated over the published data on response to adriamycin or actinomycin D alone.
 ...
PMID:Alternating administration of adriamycin (NSC-123127) and vincristine (NSC-67574)-actinomycin D (NSC-3053) in advanced sarcomas. 110 47

One hundred fifty-five adult patients with "operable" soft part sarcomas including rhabdomyosarcoma, liposarcoma, leiomyosarcoma and fibrosarcoma of the trunk and extremities are reviewed. Local recurrences of 93% and 60% occurred after local and wide excisions of the primary tumor. In this series of patients, amputation was the most efficient procedure for controlling the primary site. The absolute 5 and 10-year survival rates for all groups of tumors were 50% and 26%. Development of a second primary tumor of a different cell type occurred in 9% of the patients. Local recurrence, single distant metastasis, and/or second primary tumors should be considered potentially curable and appropriate surgical and/or radiation therapy carried out.
 ...
PMID:Soft tissue sarcomas. 113 30

A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).
 ...
PMID:Ganglioneuroblastoma associated with malignant mesenchymoma. 118 58

Synovial sarcoma is a clinically and morphologically well defined entity that has been described extensively in Literature. It occurs primarily in the para-articular regions, usually in close association with tendon sheaths, bursae and joint capsules. On rare occasions it is also encountered in areas without any apparent relationship to synovial structures, as in the parapharyngeal region or the abdominal wall. It is considered the fourth most common type of sarcoma (7-10%) after malignant fibrous histiocytoma, liposarcoma and rhabdomyosarcoma. There are three histological variants: the classical biphasic, the monophasic fibrous type and the monophasic epithelial type (the biphasic and monophasic fibrous type are equally common). Clinical sign complaints are subtle and at times noted 20 years before diagnosis. The course of the disease is slow and insidious. The most typical presentation is that of a palpable deep-seated swelling or mass associated with pain or tenderness. Patients with synovial sarcoma in the head and neck (10%) tend to have difficulties in swallowing and breathing and not infrequently have alteration or loss of voice. Head and neck synovial sarcoma seem to originate from the paravertebral connective tissue spaces and manifest themselves as solitary retropharyngeal or parapharyngeal masses near the forking of the carotid. Additional cases in this general area have been reported in the soft palate, tongue, maxillofacial region, mandible corner, sternoclavicular region, scapular region and the cervical oesophagus. As in other types of sarcoma, the principal sites of metastases are the lung, but many make their appearance many years after the initial diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Synovial sarcoma of the head and neck: a case report of parapharyngeal region and review of the literature]. 133 46


1 2 3 4 5 6 7 8 9 10 Next >>