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)

To our knowledge, this is the first fully documented case of an adult-type orbital rhabdomyoma in an 8-year-old white boy, which originated in the belly of the medial rectus muscle and grew into the anterior orbit to incorporate the trochlea. Incomplete excision resulted in recurrence; the lesion was misinterpreted as a rhabdomyosarcoma and exenteration was performed. The patient was free of disease 25 years later. Reexamination of original pathologic material showed the typical histology of an adult-type rhabdomyoma. Adult-type rhabdomyoma, the most differentiated tumor in a spectrum of benign and malignant striated muscle neoplasms, should be distinguished from fetal rhabdomyoma (also benign) and pleomorphic rhabdomyosarcoma, the malignant striated muscle tumor most likely to be confused with rhabdomyoma. An awareness of the benign nature of rhabdomyoma (rhabdomyoma never evolves into a sarcoma) should prevent unnecessary radical surgery. The tumor can be adequately managed by a local excision that includes all the lobulations of tumor within a margin of normal tissue.
 ...
PMID:Rhabdomyoma of the orbit. 120 93

Tumors arising in the parapharyngeal space are rare. Even more rare are those which originate from the pharyngeal and parapharyngeal musculature. Three examples of muscle tumors presenting as parapharyngeal masses are reported, ie, a rhabdomyoma, rhabdomyosarcoma, and a leiomyosarcoma, and the literature pertaining to these tumors is reviewed. To the best of our knowledge, this represents the first case of a parapharyngeal leiomyosarcoma reported in the literature.
 ...
PMID:Muscle tumors in the parapharyngeal space. 162 93

The diagnostic utility of several antibodies against desmin and their optimal staining conditions have not been systematically evaluated. Sections of paraffin-embedded tissues from 584 cases were stained with a monoclonal antibody against desmin (Clone DER 11 from DAKO), using an avidin-biotin-peroxidase technique. The results were tabulated and compared with those from previous reports. The following observations were made: (1) When pronase digestion was performed before staining, desmin was equally demonstrable in tissues fixed in formalin, Zenker's, Bouin's, or B5 fixative; however, desmin staining was lost or significantly diminished in tissues fixed in absolute ethyl alcohol. In contrast, when pronase was not used, a positive staining was demonstrated only in tissue fixed in absolute ethyl alcohol. (2) Positive staining was found in normal muscle (92 of 92 cases), leiomyoma (12 of 13), rhabdomyoma (6 of 6), rhabdomyosarcoma (31 of 31), leiomyosarcoma (18 of 26). (3) Desmin was never found in epithelia, normal mesenchymal tissue other than muscle, tumors stimulating rhabdomyosarcoma, and epithelial tumors. (4) A positive staining was documented in 1 tumor (a fibrous histiocytoma) of 42 benign predominantly spindle cell tumors and in 8 of 89 predominantly spindle cell sarcomas. (5) Desmin was never documented in myoepithelial cells but stained myofibroblasts in 2 of 12 examples of granulation tissue and in 29 of 67 samples containing tumor-associated desmoplasia. The authors' data on the diagnostic sensitivity and specificity of the evaluated antibody should improve the use of desmin in diagnostic pathology.
 ...
PMID:The diagnostic utility of desmin. A study of 584 cases and review of the literature. 168 48

A tumor of the tongue with features consistent with the diagnosis of fetal cellular rhabdomyoma was seen in an 18-month-old infant. The tumor recurred 10 and 22 months after initial resection. The histologic condition of the first recurrence was similar to the original tumor except for some increased nuclear irregularities and mitotic activity. No adjunctive therapy was administered initially or after the first recurrence. The second recurrence showed mixed embryonal/alveolar rhabdomyosarcoma. This case represented a unique model of a highly differentiated striated muscle tumor converting to a moderately differentiated rhabdomyosarcoma, and illustrated differential diagnostic difficulties in distinguishing between fetal cellular rhabdomyoma and differentiated rhabdomyosarcoma. The patient was compared with eight children and adolescents with primary sarcomas of the tongue who entered the Intergroup Rhabdomyosarcoma Studies (IRS) I, II, and III protocols. The tumors of all eight arose at the base of the tongue. There were five embryonal, one alveolar, and one mixed embryonal/alveolar rhabdomyosarcomas, and one undifferentiated myxoid sarcoma. Five rhabdomyosarcomas were poorly differentiated, and two had a moderate degree of myogenesis. These sarcomas of the tongue represent approximately 0.34% of all cases entered in IRS studies.
 ...
PMID:Is fetal cellular rhabdomyoma an entity or a differentiated rhabdomyosarcoma? A study of patients with rhabdomyoma of the tongue and sarcoma of the tongue enrolled in the intergroup rhabdomyosarcoma studies I, II, and III. 202 57

We report a case of paratesticular rhabdomyosarcoma in a 5-year-old boy who was submitted to radical orchidectomy via the inguinal approach and high ligation of the spermatic cord. Clinical and retroperitoneal staging classify this case under group I of the Intergroup Rhabdomyoma Study. Adjuvan chemotherapy with 12 courses of VAC have achieved a good clinical course to date (14 months after surgery) and a good outcome is predicted. We review the literature and discuss the features of this mesenchymal tumor.
 ...
PMID:[Paratesticular rhabdomyosarcoma]. 220 Mar 54

Tumors of the heart are a rare cause of embolism of the peripheral arterial system. A series of 26 patients operated on for heart tumor in the Institute of Cardiovascular Diseases in Bratislava over the years 1979-1988 was analyzed. Pseudomyxoma was histologically verified in 24 patients and rhabdomyoma and rhabdomyosarcoma in the other two patients. A total of 12 embolic events was recorded in 9 patients (34.6%). There were 8 instances of embolism in the central nervous system and 4 in the extremities. In all cases pseudomyxoma of the left parts of the heart was the source of embolism. In 21 cases diagnosis was established and operation indicated on the basis of ultrasonographic evidence. The authors recommend the biatrial transseptal approach. Early surgical removal of a heart tumor is the therapy of choice which prevents the development of potential complications from the heart and peripheral arteries.
 ...
PMID:[Tumors of the heart and arterial embolisms]. 276 62

The authors have recently developed a monoclonal antibody, HHF35, that recognizes the muscle-specific isoforms of actin. To determine its potential usefulness in the differential diagnosis of "small, round, blue cell" tumors of childhood, they immunolabeled formalinor B-5-fixed tissue sections from known cases of rhabdomyosarcoma or rhabdomyoma (30), neuroblastoma (9), retinoblastoma (2), and Ewing's sarcoma (9) with HHF35 and with antibodies to creatine kinase M, myoglobin, vimentin, and neuron-specific enolase. HHF35 reacted with 29 of 30 cases of rhabdomyosarcoma, whereas antibodies to creatine kinase M and myoglobin were positive on only 12 and 7 tumors, respectively. HHF35 did not react with any case of neuroblastoma, retinoblastoma, or Ewing's sarcoma when the antibody diluent contained 50 mM EDTA. These results indicate that HHF35 is a highly sensitive and specific marker for myogenic differentiation and that it will be useful in the differential diagnosis of rhabdomyosarcomas.
 ...
PMID:Diagnosis of rhabdomyosarcomas with HHF35, a monoclonal antibody directed against muscle actins. 335 41

A recurrent soft tissue tumor from the groin in a 37-year-old male is described. The tumor cells contain many leptomeric structures, both fibrils and complexes, but none of the other characteristic features of striated muscle: alternating parallel thick and thin myofilaments with Z-line material. A review of one rhabdomyoma and six rhabdomyosarcomas demonstrates that leptomeric structures are present in large amounts in the rhabdomyoma but are very infrequent in only three of the six rhabdomyosarcomas. A careful search for leptomeric structures is indicated when the ultrastructural diagnosis of either rhabdomyoma or rhabdomyosarcoma is being considered.
 ...
PMID:Soft tissue tumor with prominent leptomeric fibrils and complexes. 396 28

Fetal rhabdomyomas are rare benign tumors of skeletal muscle that must be distinguished histologically from various sarcomas, including rhabdomyosarcoma. They have recently been subdivided into two histologic variants myxoid and cellular fetal rhabdomyomas, respectively. This article describes the sixth fetal rhabdomyoma to be reported in the oral regions, illustrates the histologic features of the two types, and discusses the means of distinguishing these lesions from various other conditions with which they may be confused.
 ...
PMID:Fetal rhabdomyoma of the tongue, with a discussion of the two histologic variants of this tumor. 657 62

Seven cases of vaginal polyps with atypical stromal cells were investigated. Three of the cases were classified as vaginal rhabdomyoma and four as fibroepithelial polyps with atypical stromal cells. All of the patients were adult women with minor or no symptoms. None of the tumor recurred or metastasized. The recognition of the vaginal polyps with bizarre stromal cells is important in order to avoid misdiagnosis of sarcoma, particularly botryoid rhabdomyosarcoma.
 ...
PMID:Vaginal polyps with pseudosarcomatous features. A clinicopathologic study of seven cases. 668 96


1 2 3 4 5 Next >>