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

Rhabdomyosarcomas of the perianal and perineal regions are uncommon. This study was performed to clarify the clinical characteristics and guidelines of surgical treatment of patients with perianal and perineal rhabdomyosarcomas younger than 20 years of age. Twenty-nine patients, 26 patients identified in the Japanese literature and three of our own, were analyzed and the results were compared with the data reported from the Intergroup Rhabdomyosarcoma Study Group (IRSG). Female predominance and a twin-peak age distribution in infancy and adolescence were characteristic findings of the Japanese patients that were not observed in the IRSG studies. The demographic differences between the two groups were attributed to the differences in demographics of patients younger than 10 years of age. Of the 29 patients, 17 were categorized into clinical groups III/IV and 21 patients into stages 3/4. Alveolar histology was diagnosed in 18 patients. In patients more than 10 years of age, the female predominance was more prominent and the incidences of advanced clinical groups/stages and alveolar histology were significantly higher than those in patients younger than 10 years of age. Inguinal lymph nodes were always involved in patients with lymph node metastases and three patients developed metastases to the breast. Information regarding the survival time was available for 18 patients and the 5-year overall survival was 20%. Two patients with a group I/stage 2 tumor and one with a group II/stage 3 tumor survived for more than 2 years with no evidence of the disease. In these patients, the tumors were excised by primary surgery or primary reexcision and they were not accompanied by lymph node metastasis. Based on these data, complete tumor resection prior to chemotherapy should be pursued and the inguinal lymph nodes should be at least sampled because nodal involvement is closely associated with the patient's prognosis.
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PMID:Clinical characteristics and surgical treatment of perianal and perineal rhabdomyosarcoma: analysis of Japanese patients and comparison with IRSG reports. 1630 4

Spindle cell (sarcomatoid) carcinoma of the breast is a rare variant of breast cancer that has been classified under the broad rubric of metaplastic carcinoma. Because the term "metaplastic carcinoma" comprises a heterogeneous group of tumors, it has been difficult to reliably predict biologic potential or to determine optimal therapy. To better characterize the spindle cell subset of metaplastic breast carcinomas, we reviewed 29 cases. All patients were adult females ranging from 40 to 96 years of age (median, 68 years). Tumor size ranged from 1.5 to 15 cm (median, 4 cm). Treatment was by excision and/or mastectomy with axillary node evaluation in most cases, often combined with postoperative radiation and/or chemotherapy. All cases were clinically of breast origin, showed >or=80% spindled/sarcomatoid morphology, and demonstrated keratin positivity and/or close association with ductal carcinoma in situ. Immunohistochemical studies showed evidence suggesting myoepithelial differentiation as exhibited by immunoreactivity for smooth muscle actin, cytokeratin 14, and p63 in a subset of cases (39%). Twenty-seven cases exhibited pure spindled or sarcomatoid morphology of variable appearance and nuclear grade, whereas 2 contained high-grade invasive ductal carcinoma comprising <or=20% of the tumor mass. Two cases exhibited heterologous elements (1 rhabdomyosarcoma and 1 with both chondrosarcoma and osteosarcoma) and 4 were associated with ductal carcinoma in situ. Follow-up data were available on 24 of 29 patients (range, 1-120 months; median, 20 months). Of 20 cases in which axillary nodes were biopsied, definitive nodal metastases were identified in only 1 (5%), and this was in a case with a significant component of invasive ductal carcinoma. Three patients developed local recurrences. Extranodal metastases occurred in 11 of 24 patients (46%), most commonly to the lungs. Ten of 24 patients (42%) died of disease at a median interval of 11.5 months (range, 1-46 months) and 3 patients were alive with metastatic disease. Eight patients were alive with no evidence of recurrent or metastatic disease (median, 29.5 months). Based on this series, spindle cell/sarcomatoid carcinoma of the breast is a highly aggressive neoplasm with a high rate of extranodal metastases. Purely spindled/sarcomatoid tumors have a significantly lower rate of nodal metastases than conventional ductal and lobular breast carcinomas.
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PMID:Spindle cell (sarcomatoid) carcinoma of the breast: a clinicopathologic and immunohistochemical analysis of 29 cases. 1725 81

Alveolar rhabdomyosarcoma is a high-grade neoplasm, which forms about 30% of rhabdomyosarcomas. A rare solid variant has been described. A 14-year-old girl presented with inguinal lymph nodal mass and was treated with 6 cycles of CHOP chemotherapy and local radiation. After 3 months, she presented with generalized lymphadenopathy, pleural and pericardial effusions. A histopathologic diagnosis of solid variant of alveolar rhabdomyosarcoma was made. The skeletal muscle origin was confirmed by positive immunostaining for desmin. A primary site was identified in the lower limb muscles. The patient was treated with salvage chemotherapy but had progressive disease. The pediatric and adolescent cases of this rare tumor reported in English language literature are reviewed. In addition, the importance of biopsy in the diagnosis of suspected lymphomas and the pitfalls of needle aspirations are briefly discussed.
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PMID:Solid variant of alveolar rhabdomyosarcoma mimicking non-Hodgkin lymphoma: case report and review of literature. 1901 78

Adult sclerosing rhabdomyosarcoma (ASRMS) is a rare variant of rhabdomyosarcoma with a characteristic histological appearance of small, round cells in a dense, hyalinized stroma. Although nodal metastases of soft-tissue sarcomas are considered uncommon, up to 5% overall are associated with lymph node metastases. Nonetheless, there is little literature on the cytologic characteristics of metastatic soft-tissue sarcomas in lymph nodes, and to our knowledge, there are no reports of nodal metastasis of ASRMS diagnosed by fine-needle aspiration (FNA) cytology. We report here a 55-year-old woman who presented with a right thigh mass and associated ipsilateral inguinal lymphadenopathy. Biopsy of the mass revealed a uniform population of small, round cells in a dense, sclerotic background. A diagnosis of ASRMS was rendered. Subsequently, the patient underwent FNA of an enlarged inguinal lymph node, which revealed an identical population of small, round cells in a dense, myxoid background. This case highlights the cytologic features of a rare form of rhabdomyosarcoma, and emphasizes the utility of FNA in the assessment of lymphadenopathy in the setting of a soft-tissue sarcoma.
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PMID:Fine-needle aspiration diagnosis of a metastatic adult sclerosing rhabdomyosarcoma in a lymph node. 2009 93

Paratesticular rhabdomyosarcoma is a rare but aggressive malignancy in children and adolescents. Prognosis is related to initial tumor resectability as well as staging of the disease based on tumor invasiveness, tumor bulk, nodal disease and metastases. We report the unusual presentation of paratesticular rhabdomyosarcoma with metastatic extension through the inguinal canal and encasement of the abdominal aorta. These features portend a poor prognosis given their association with a greater stage of disease and unresectable nature at presentation. Delayed surgical resection follows a regimen of chemotherapy and radiation therapy in such cases of extensive disease. Encasement of the abdominal aorta has been shown to increase presurgical risk for intraoperative vascular injury when related to other malignancies, but its role in relation to metastatic paratesticular rhabdomyosarcoma has not been investigated. Also, rhabdomyosarcoma should be considered in the differential diagnoses of tumors that demonstrate encasement of the abdominal aorta.
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PMID:Paratesticular rhabdomyosarcoma with metastatic encasement of the abdominal aorta. 2125 28

Identification of nodal involvement is important for treatment planning in patients with rhabdomyosarcoma, and is facilitated by sentinel node biopsy. Although it is employed primarily for extremity tumors, we report using sentinel node biopsy in a patient with parameningeal rhabdomyosarcoma arising in the ethmoid sinus. Lymphoscintigraphy with single photon emission computed tomography following injection of tracer at the tumor site helped identify contralateral cervical node involvement not previously recognized by physical exam, cross sectional imaging, or other functional imaging. This case demonstrates how information from sentinel node identification and biopsy can change therapy recommendations in patients with parameningeal rhabdomyosarcoma.
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PMID:Use of sentinel node biopsy for staging parameningeal rhabdomyosarcoma. 2174 75

Rhabdomyosarcoma (RMS), which affects mainly pediatric patients, is currently classified into 3 major categories: embryonal, alveolar, and pleomorphic. We have identified a distinct variant of RMS that is characterized by epithelioid morphology, reminiscent of poorly differentiated carcinoma or melanoma, for which we propose the designation "epithelioid RMS." We examined the clinicopathologic features of 16 such cases, which were received in consultation between 1991 and 2009. Ten patients were men, and 5 were women (gender was unknown in 1 case), with a median age of 70.5 years (range 14 to 78). Primary anatomic sites were upper extremity (4), lower extremity (2), head and neck (3), trunk (3), hypopharynx (1), and left atrium (1). Two patients presented with nodal metastases (neck and mediastinal) with unknown primary sites. Of the tumors in somatic locations, 10 were intramuscular, and 2 were subcutaneous. Tumor size ranged from 3 to 8.5 cm; the tumors had nodular fleshy cut surfaces, grossly appreciable necrosis, and infiltrative edges. Microscopically, tumors showed sheet-like growth of uniformly sized epithelioid cells with abundant amphophilic-to-eosinophilic cytoplasm, large vesicular nuclei, and frequently prominent nucleoli. Necrosis and infiltration into adjacent structures (such as skeletal muscle or fat) were present in all cases, and mitotic counts were high (median count 23 per 10 high-power fields) with frequent atypical forms. By immunohistochemistry, all tumors showed diffuse desmin expression, and myf-4 immunostaining was diffuse to multifocally positive. S100-protein was absent in all cases. Cytokeratin was negative in 12 cases and very focally positive in 4 cases. Clinical follow-up information was available for 11 cases (median duration 10 mo). Most patients underwent surgical resection with chemotherapy and/or radiation (none with RMS-specific protocols). Two patients had multiple recurrences, and 2 patients developed satellite nodules near the primary lesion. Six patients had regional lymph node metastases, and 6 patients developed distant metastases, most commonly to the lung. So far, 7 patients have died of disease, 5 within 1 year and 2 within 5 years. Epithelioid RMS is a novel morphologically distinct variant of RMS that closely mimics carcinoma or melanoma. It primarily affects older patients, with a male predilection. The clinical course as determined thus far is aggressive.
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PMID:Epithelioid rhabdomyosarcoma: clinicopathologic analysis of 16 cases of a morphologically distinct variant of rhabdomyosarcoma. 2192 82

There is growing evidence that interconnections among molecular pathways governing tissue differentiation are nodal points for malignant transformation. In this scenario, microRNAs appear as crucial players. This class of non-coding small regulatory RNA molecules controls developmental programs by modulating gene expression through post-transcriptional silencing of target mRNAs. During myogenesis, muscle-specific and ubiquitously-expressed microRNAs tightly control muscle tissue differentiation. In recent years, microRNAs have emerged as prominent players in cancer as well. Rhabdomyosarcoma is a pediatric skeletal muscle-derived soft-tissue sarcoma that originates from myogenic precursors arrested at different stages of differentiation and that continue to proliferate indefinitely. MicroRNAs involved in muscle cell fate determination appear down-regulated in rhabdomyosarcoma primary tumors and cell lines compared to their normal counterparts. More importantly, they behave as tumor suppressors in this malignancy, as their re-expression is sufficient to restore the differentiation capability of tumor cells and to prevent tumor growth in vivo. In addition, up-regulation of pro-oncogenic microRNAs has also been recently detected in rhabdomyosarcoma.In this review, we provide an overview of current knowledge on microRNAs de-regulation in rhabdomyosarcoma. Additionally, we examine the potential of microRNAs as prognostic and diagnostic markers in this soft-tissue sarcoma, and discuss possible therapeutic applications and challenges of a "microRNA therapy".
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PMID:MicroRNAs in rhabdomyosarcoma: pathogenetic implications and translational potentiality. 2194 49

Adrenal cortical carcinosarcoma is a rare variant of adrenal cortical carcinoma. Sarcomatous change in adrenal cortical carcinomas is exceptionally rare, with only 9 cases previously described. Adrenal cortical carcinosarcomas tend to be aggressive tumors, with locoregional recurrence and rapid metastases from the sarcomatoid component. We describe what seems to be the first case of sarcoma arising in oncocytic adrenal cortical carcinoma. The sarcomatous component here was pleomorphic rhabdomyosarcoma. This occurred in a 45-year-old man who had nodal and pulmonary metastases of the rhabdomyosarcomatous component at presentation and who died of progressive disease 11 months later. Here, we discuss the clinical, radiologic, and pathologic findings and review the literature on adrenal cortical carcinosarcomas.
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PMID:Oncocytic adrenal cortical carcinosarcoma with pleomorphic rhabdomyosarcomatous metastases. 2234 39

Sentinel lymph node biopsy (SLNB) is an established staging technique in many malignancies, but reports describing this procedure for the evaluation of regional lymph nodes in childhood and adolescents are still scarce. SLNB has progressively gained a role in the staging of pediatric melanoma and some histotypes among soft tissue sarcomas (rhabdomyosarcoma, synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma). We report our experience with 29 patients who were evaluated with SLNB during the past 12 years: 9 alveolar rhabdomyosarcoma, 1 embryonal rhabdomyosarcoma, 7 Ewing Sarcoma/pPNET, 1 clear cells sarcoma, 2 epithelioid sarcoma, 1 epithelioid sclerosing fibrosarcoma, 3 myxoid liposarcoma, 1 adult fibrosarcoma, 1 malignant peripheral nerve sheath tumor, 1 undifferentiated sarcoma, 1 sarcoma not otherwise specified, and 1 melanoma. All, but one, patients underwent a lymphoscintigraphy, and in more than half of the patients, an intraoperative lymphatic mapping with a blue dye injection was performed. In our experience, SLNB was a feasible and reliable technique that allowed a targeted sampling of regional lymph nodes. This technique avoids aggressive operations or random biopsies, leading to an accurate staging without complications. Recently new imaging techniques, such as positron emission tomography-computed tomography scan, have obtained good results in identifying distant and lymphatic metastases, but they have been found to be less reliable than SLNB, especially for small volume metastatic nodal disease in sarcoma.
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PMID:Sentinel node biopsy in pediatric patients: the experience in a single institution. 2547 65


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