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)

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.
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PMID:Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma. 159 14

Spindle cell rhabdomyosarcoma, a recently described variant of embryonal rhabdomyosarcoma in children, carries a favorable prognosis when compared with other types of rhabdomyosarcoma. This tumor is rare in adults, and only four cases have been documented previously. The clinicopathological study of such a case occurring in the retroperitoneal space of a 53-year-old man is herein reported. The patient died of uncontrolled local recurrence and hepatic metastases seven months after diagnosis. Based on the analysis of the data of the five cases reported, including the present one, it can be stated that spindle cell rhabdomyosarcoma in adults is not associated with the favorable outcome observed in the pediatric population.
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PMID:Spindle cell rhabdomyosarcoma in adults. A case report and literature review. 1067 75

Spindle cell rhabdomyosarcoma is an uncommon subtype of embryonal rhabdomyosarcoma. Found almost exclusively in children, these tumors are classically located in the paratesticular and head and neck regions. Morphologically these lesions can resemble several other benign or malignant soft-tissue spindle cell lesions, especially smooth muscle or myofibroblastic tumors, and thus immunohistochemical staining is often needed to prove skeletal muscle differentiation. Although there is extensive literature reporting the genetics of embryonal rhabdomyosarcoma, little is reported specific to the spindle cell subtype. Below we present the case of a 7-month-old male presenting with a large posterior neck mass that was diagnosed as spindle cell rhabdomyosarcoma. Karyotype evaluation revealed a t(6;8) (p12;q11.2) chromosomal translocation within the lesion. We review the histologic and immunohistochemical diagnosis of these tumors and discuss the genetics of rhabdomyoscarcomas.
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PMID:Spindle cell rhabdomyosarcoma of the neck with t(6;8) translocation: report of a case and literature review. 2311 71

Spindle cell rhabdomyosarcoma is a rare subtype of rhabdomyosarcoma mainly seen in children. Occasional aberrant staining with a variety of immunohistochemical markers has been noted. The aberrantly expressed markers include alpha-smooth muscle actin, cytokeratin, S100, neurofilaments, CD20, immunoglobins, and CD117. We report herein two pediatric cases displaying strong CD34 positivity and one with additional focal CD117 positivity, causing considerable difficulty in distinction from solitary fibrous tumor and extra-gastrointestinal stromal tumor. To our knowledge, CD34 staining has been merely reported in rhabdomyosarcoma. Spindle cell rhabdomyosarcoma has to be considered in the differential diagnosis of childhood spindle cell tumors. Post-chemotherapy specimens should be evaluated in caution, since chemotherapy can cause considerable changes in tumor antigen expression. Since CD117 and CD34 are stem cell markers, their positivity in pediatric tumors should be interpreted with caution. Even if the morphology is not supportive, a wide immunohistochemical panel should be applied in childhood malignant solid tumors.
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PMID:Spindle cell rhabdomyosarcoma displaying CD34 positivity: a potential diagnostic pitfall; report of two pediatric cases. 2402 13

Spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma that affects young males and most commonly involves the paratesticular region. We report a case of paratesticular spindle cell rhabdomyosarcoma in a 14-year-old boy, who presented with a painless scrotal mass. Left inguinal orchidectomy was performed. Histopathological and immunohistochemical examination of the mass revealed spindle cell rhabdomyosarcoma of the paratesticular region.
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PMID:Paratesticular Spindle Cell Rhabdomyosarcoma. 2650 Jul 26

We report a case of a 3-year-old North African child, initially assessed for nonspecific urinary symptoms such as haematuria and burning urination. The ultrasound evaluation showed a vegetating mass occupying the lumen with weak vascular signs at the Colour-Doppler evaluation. An explorative cystoscopy was performed and it revealed a nonbleeding lesion, white in colour, pedunculated, projecting into the lumen, and associated with a brown satellite formation. Histological examination showed a mixed Botryoid and Spindle Cell Rhabdomyosarcoma. This mixed histology has not been described before and no statistical data are reported in literature so far. Despite the Embryonal Rhabdomyosarcoma variant being the most common, the association characterized by two histological Rhabdomyosarcoma subtypes such as Botryoid and Spindle Cell is rarely observed and it is important to get an accurate histological diagnosis in order to immediately start the correct treatment protocol.
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PMID:Mixed Botryoid and Spindle Cell Bladder Rhabdomyosarcoma: An Outstanding Pediatric Case. 2811 2