Rhabdomyosarcomas (RMS) are malignant soft tissue tumors, exhibiting skeletal muscle differentiation.There is a bimodal age distribution, between 2-6 years and a second peak between 10-18 years; it is uncommon after 45 years.Common locations include head and neck (26%), genitourinary tract (17%) and extremities (15%). small round blue cell tumor ; skeletal muscle-like cross-striations can occur; based on the stage of neoplastic cell development; Alveolar UpToDate. In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). 1 Spindle cell rhabdomyosarcoma (SC-RMS), 1 of the 3 embryonal rhabdomyosarcoma … The spindle cell variant of rhabdomyosarcoma (RMS) is uncommon and is most often encountered in the paratesticular region of children in whom it has a good prognosis. We present four cases of ssRMS and 16 a … To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published. We reviewed 173 cases of paratesticular rhabdomyosarcoma (RMS) of Intergroup Rhabdomyosarcoma Studies (IRS)-I, -II, and -III for evaluation of possible histological factors that might account for the good prognosis of these patients. Spindle cells should make think: 1. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). 3.1. Spindle cell - may … Epidemiology, pathology, and molecular pathogenesis. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma first described by Cavazzana, Schmidt and Ninfo in 1992. Spindle cell variant of embryonal rhabdomyosarcoma in pediatric age group may be considered in differential diagnosis of prostatic sarcomas when the patient displaying urinary outlet obstruction and aggressive behaviour. Pathology - Research and Practice. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked … The spindle cell variant of rhabdomyosarcoma (spindle cell RMS) in adults has been defined as an entity, separated from embryonal rhabdomyosarcoma (ERMS), with unfavourable clinical outcome. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. © Copyright PathologyOutlines.com, Inc. Click, Primitive malignant round cell tumor with skeletal muscle differentiation by immunohistochemistry or ultrastructure, Relatively rare, 7% of all rhabdomyosarcomas, 6% of all paratesticular tumors but still the most common nongerminal malignant tumor in the paratesticular region, Paratesticular region is the most common site for rhabdomyosarcomas in teenagers, No preference for either side or race has been demonstrated, Embryonal RMS (including its variant spindle cell type) is the most common subtype in this region, although any subtype can occur, Embryonal RMS is also the most common childhood malignant tumor of spermatic cord, Occurs in all age groups but most common in children; peak age is 9 years, ~80% occur before age 21 years, 20% are equally distributed in older age groups, Alveolar RMS and pleomorphic RMS are less common, pleomorphic RMS is least common, Alveolar RMS occurs mainly in young adults and adolescents, Spindle cell RMS first described in 1992 by German-Italian Cooperative Sarcoma Study (, Most commonly found in paratesticular region of young boys (, Mean age is 6.6 years (median 5 years), compared to nonspindle cell variants of paratesticular RMS (mean age 5.9 years, median 4 years), Usually nontender scrotal mass, stage I / II at presentation, Less likely to metastasize (16%) than nonspindle cell variant (~31%) (, Better survival (88%) than patients with spindle cell RMS in other locations (58%) in the IRS I & II studies (, Most cases are centered around paratesticular soft tissue with variable testicular involvement, Commonly spreads through lymphatics to iliac lymph nodes but hematologic spread to lungs and liver also occurs, Short clinical history of painless swelling in scrotum of days to weeks duration is most common presentation, Pain or history of trauma is extremely uncommon (~7% cases for each), Tumor is usually large at presentation, often reaching the inguinoscrotal region, 1/3 to 1/2 have metastases at presentation, In the largest series of pediatric RMS, 18 of 216 patients presented with distant metastases (, Adults have a higher prevalence of metastases at presentation (, Suspected clinically or by imaging, confirmed by histology, Negative markers for germ cell and sex cord stromal tumors, Liver function tests may be affected by metastases, MRI reveals a heterogeneously enhancing, well defined soft tissue mass classically encasing or displacing the testis, Age of the patient ≥ 10 years or < 1 year, Site of origin: parameningeal, bladder, prostate, abdomen, trunk, extremities are associated with poor prognosis; orbital, paratesticular and vaginal locations are associated with better prognosis, Presence of distant metastases at diagnosis, Number of metastatic sites or tissues involved, Presence of regional lymph node involvement (N1), Histopathologic subtype: pleomorphic worse than alveolar, worse than embryonal (, Intergroup Rhabdomyosarcoma Study group's International Classification of Rhabdomyosarcomas (, 3 year old boy with paratesticular rhabdomyosarcoma presenting as thickening of tunica vaginalis (, 10 year old boy with pediatric paratesticular rhabdomyosarcoma with epididymitis (, 13 and 15 year old boys with spindle cell variant (, 16 year old boy with mixed type paratesticular rhabdomyosarcoma (, 18 year old man with paratesticular embryonal rhabdomyosarcoma (, 19 year old man with adult paratesticular rhabdomyosarcoma (, Multimodal approach of surgical excision, VAC based chemotherapy is standard of care, While most authors support use of chemotherapy, there is significant toxicity, Ferrari et al suggested that low risk cases receive low dose anthracycline free regimens without any loss of benefit, Their study of 216 cases of pediatric paratesticular rhabdomyosarcoma had overall 5 year survival of 85.5%, 95% for localized disease, 2% for metastatic disease (, Radiotherapy for local disease control may be given, Retroperitoneal lymph node dissection is not advocated unless there is evidence of lymph node enlargement / involvement by imaging, Detailed approaches and stratification for treatment are available (, Encapsulated, lobulated, smooth, gray white glistening mass that displaces testicular parenchyma but typically does not invade testicular tissue, 1 to 20 cm, with foci of hemorrhage and cystic degeneration, Embryonal RMS: fleshy grayish white to pinkish tan mass, 4 - 6 cm and may be mucoid, Spindled RMS: mean 5.8 cm, median: 4.6 cm, similar to classical embryonal RMS (mean: 6.4 cm, median: 6 cm) as reported in the IRS I & II studies (, Mixture of haphazardly arranged rhabdomyoblasts and undifferentiated primitive cells, Primitive cells are small and round with minimal cytoplasm, dark nuclei, Variable numbers of strap cells, with or without cross striations and bizarre "tadpole" cells, Embryonal RMS: small cells with hyperchromatic nuclei, minimal cytoplasm as well as cells with rims of eosinophilic cytoplasm and spindle cells with cytoplasmic tails and variable cross striations; myxoid or collagenous stroma, Predominant cell type is elongated, spindle cell arranged in fascicles or whorls; herringbone growth pattern may be seen, Cells have eosinophilic fibrillar cytoplasm, centrally located nuclei with blunted or fusiform ends, small to inconspicuous or prominent nucleoli, Mitotic figures are easily appreciated, including atypical forms, A smaller proportion of admixed immature rhabdomyoblasts are usually seen, with bright cytoplasmic eosinophilia, eccentric nuclei and occasionally cytoplasmic cross striations (useful for diagnosis and to differentiate from leiomyosarcoma), Variable collagen fibers intermingled between the spindle cells, Some authors, including the IRS I & II studies, identified collagen rich and collagen poor spindle cells, Collagen poor: cells are arranged in bundles or fascicles with abundant cellularity and little or no stroma, resembling leiomyosarcoma, Collagen rich: lower cellularity due to abundant fine collagen fibers with a "storiform" pattern giving it a more sclerotic appearance, This subclassification does not appear to affect clinical outcome, Spindle cell variant: mumerous spindle cells and large fragments of cytoplasmic processes with cross striations (, Thin actin and thicker myosin filaments are seen, Spindled variant is more differentiated with higher proportion of cases expressing markers of mature muscles (myoglobin, troponin T and muscle specific actin) compared to nonspindle cell variants (, Partial monosomy of chromosome 11; loss of heterozygosity (LOH) at 11p characterizes embryonal RMS, LOH by loss of maternal copy and duplication of paternal copy of 11p results in activation of IGF2 (IGF2 is known to show genomic imprinting, with silencing of the maternal allele), Alveolar RMS: characterized by t(2;13)(q35;q14) or t(1;13)(p36;q14), resulting in PAX3-FKHR or the PAX7-FKHR fusion proteins, detectable in 75 - 80% of alveolar RMS but absent in other subtypes; some cases have loss of imprinting of the IGF2 gene with re-expression of the normally silent maternal allele, Spindle cell RMS: PAX3-FKHR / PAX7-FKHR fusion (RMS1 / RMS2) associated with alveolar RMS are classically absent; cytogenetically, spindle cell RMS is close to embryonal RMS, based on presence of sporadic small gains of chromosome 2, 8, 11, 13 & 20. Embryonal. Embryonic, Alveolar, and Pleomorphic Rhabdomyosarcoma are the 3 types of Rhabdomyosarcoma. Rhabdomyosarcoma (RMS) is a type of sarcoma made up of cells that normally develop into skeletal (voluntary) muscles. Background Documentation Pediatric • Rhabdomyosarcoma 4.0.0.0 Resection 5 Explanatory Notes A. Submission of Tissue A minimum of 100 mg of viable tumor should be snap-frozen for potential molecular studies. Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma. Only isolated cases in adulthood have been described. I. Leuschner, Kiel, Germany). Diagnosis usually occurs with advanced disease frequently already with metastatic spread. Smooth. Sclerosing rhabdomyosarcoma (ScRMS) and spindle cell rhabdomyosarcoma (SRMS) have been recently reclassified as a stand-alone pathologic entity, separate from embryonal RMS. 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. Therapy and outcome After initial staging the patient was treated according to the therapy guidelines of the CWS Cooperative Soft Tissue Sar-coma (“Weichteilsarkom”)Group(formoredetailssee May present at any age Pathology Histologically this tumor can resemble a leiomyosarcoma. 2. Spindle Cell Rhabdomyosarcoma: Located in the paratesticular region (rarely head & neck region).Characterized by eosinophilic spindle cells arranged in a fascicular or storiform pattern. A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. rhabdomyosarcoma images appear like many other soft tissue sarcomas dark on T1, bright on T2; Histology: Characteristic histology includes. Rhabdomyosarcoma (RMS) is the most important malignant soft tissue tumor of childhood (Enzinger and Weiss 1988).Following the first detailed description of an RMS of the skeletal muscles by Stout (1946), this entity was subdivided by histologic criteria into embryonal (Stobbe and Dargeon 1950), alveolar (Riopelle and Theriault 1956), botryoid (Horn et al. Author links open overlay panel G. Edel 1 a P. Wuisman 2 R. Erlemann 3. Edel G(1), Wuisman P, Erlemann R. Author information: (1)Gerhard-Domagk-Institute of Pathology, University of Münster, FRG. The spindle cell variant of rhabdomyosarcoma (RMS) is uncommon and is most often encountered in the paratesticular region of children in whom it has a good prognosis. The spindle cell subtype of embryonal rhabdomyosarcoma accounts for 3% of all cases. May present at any age Pathology Histologically this tumour can resemble a leiomyosarcoma. 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