This subtype has a more favorable clinical course and prognosis than usual embryonal rhabdomyosarcoma.Spindle cell rhabdomyosarcoma typically occurs in young males and most commonly occurs in paratesticular soft tissue, followed by the head and neck. This website is intended for pathologists and laboratory personnel but not for patients. UpToDate. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. Spindle cell rhabdomyosarcoma (RMS) is a rare form of RMS with different clinical characteristics between children and adult patients. Spindle cell myoblasts with prominent tapered fibrillar eosinophilic cytoplasm. Smooth. The spindle cell subtype of embryonal rhabdomyosarcoma accounts for 3% of all cases. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Spindle cell / sclerosing rhabdomyosarcoma (RMS) is a rare variant Spindle cell pattern was proposed in 1992 by Cavazzana et al. © Copyright PathologyOutlines.com, Inc. Click, Contributed by Dr. Saroona Haroon, Case #457, IARC: WHO Classification of Tumours of Soft Tissue and Bone, 4th edition, 2013, Indian J Otolaryngol Head Neck Surg 2016;68:384, CAP: Protocol for the Examination of Specimens From Patients With Rhabdomyosarcoma [Accessed 25 April 2018], Spindle cell / sclerosing rhabdomyosarcoma (RMS) is a rare variant, Spindle cell pattern was proposed in 1992 by Cavazzana et al. Spindle cell and sclerosing rhabdomyosarcoma (ssRMS) is a rare variant of rhabdomyosarcoma, which includes three distinct subtypes. Eleven patients were male and 5 were female. 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. Genetically, a subset of the congenital cases display NCOA2 gene rearrangements, whereas tumors occurring in older children or … Typically locally invasive. Eleven patients were male and 5 were female. 1 Spindle cell rhabdomyosarcoma (SC-RMS), 1 of the 3 embryonal rhabdomyosarcoma … May present at any age Pathology Histologically this tumor can resemble a leiomyosarcoma. Rhabdomyosarcoma (RMS) is a type of sarcoma made up of cells that normally develop into skeletal (voluntary) muscles. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked … Conclusion. Its genetic hallmark remains unknown and it remains debatable if there is pathogenetic relationship between the spindle cell and the so‐called sclerosing RMS. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma. The authors report a new case of SSRMS in a 31-year-old woman who presented with a large right leg mass. Author links open overlay panel G. Edel 1 a P. Wuisman 2 R. Erlemann 3. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma. Nerve. Refers in patient 1. Spindle cell variant of RMS in adults, however, shows predilection for head and neck region and extremities, has greater cytologic atypia, and is more aggressive than pediatric lesions. Rhabdomyosarcoma (RMS) is the most common childhood and adolescent sarcoma showing features of skeletal muscle differentiation. Sclerosing rhabdomyosarcoma (ScRMS) and spindle cell rhabdomyosarcoma (SRMS) have been recently reclassified as a stand-alone pathologic entity, separate from embryonal RMS. Spindle cell rhabdomyosarcoma. There are four major subtypes - Embryonal, Alveolar, Pleomorphic, & Spindle … 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. Only isolated cases in adulthood have been described. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Neoplastic cells arranged into microalveoli, IHC positivity to desmin, myogenin, SMA, MyoD1, Spindle cell / sclerosing rhabdomyosarcoma. Skeletal. It is classified into 3 histologic subtypes (embryonal, alveolar, and pleomorphic) by their unique characteristics, with the embryonal type comprising most of these cases. 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. May present at any age Pathology Histologically this tumour can resemble a leiomyosarcoma. Spindle cell - may … Other. 3.1. The degree of cellularity and the lack of collagenous stroma creates an appearance resembling fibrosarcoma. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. 3. 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. Histologic types show markedly different clincal features (select type for criteria) and the sclerosing pattern was described in 2000 by Mentzel and Katenkamp (Am J Surg Pathol 1992;16:229, Virchows Arch 2000;436:305) Predilection for the head and neck / extremities Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published. The spindle cell subtype of embryonal rhabdomyosarcoma accounts for 3% of all cases. Diagnosis usually occurs with advanced disease frequently already with metastatic spread. Epidemiology There may be increased male predilection. Anaplastic Rhabdomyosarcoma: 1 If tissue is limited, the pathologist can keep the frozen tissue aliquot used for frozen section (usually done to determine sample Usually arises in regions without skeletal muscle. Less common types: Undifferentiated rhabdomyosarcoma. However, we cannot answer medical or research questions or give advice. Epidemiology There may be increased male predilection. Epidemiology, pathology, and molecular pathogenesis. embryonal rhabdomyosarcoma (ERMS) alveolar rhabdomyosarcoma (ARMS) sclerosing rhabdomyosarcoma (SRMS) Variants. and the sclerosing pattern was described in 2000 by Mentzel and Katenkamp (, Predilection for the head and neck / extremities, Variant of rhabdomyosarcoma with dense hyalinizing collagenous matrix with rounded or spindle shaped tumor cells arranged in small nests present in at least 80% of the tumor (, Spindle cell / sclerosing are considered the same diagnostic variant of rhabdomyosarcoma, Previously categorized as a part of embryonal rhabdomyosarcoma, Sclerosing: more common in adults in the extremities and head and neck region (, Spindle cell: most common in the paratestis, followed by head and neck, More common in the pediatric population in the extremities (, In adults, recurrence and metastasis rate of 40 - 50% (, In pediatric population, similar behavior to embryonal variant except parameningeal spindle cell is more aggressive (, Prognosis of tumors in the head and neck is worse than those located in the extremities, 26 year old woman with a large chest wall tumor (, 32 year old man with swelling of right masseter (, 35 year old man presenting with knee tumor (, 36 year old Latino man with parotid gland tumor (, 50 year old man with oral rhabdomyosarcoma (, 55 year old woman with a right thigh mass (, 67 year old Japanese woman with tumor in the masticator space (, Mainstay therapeutic method should be similar to most soft tissue tumors including surgery and adjuvant chemotherapy or radiotherapy can be added (, Poorly circumscribed, fleshy, expanding masses, Neoplastic cells are arranged in lobules, small nests, microalveoli and single file arrays in an abundant hyalinized, eosinophilic to basophilic matrix that closely resembles primitive osteoid or chondroid material, Mitotic rate is very high (> 20 mitotic figures / 20 high power fields), Spindle cells or small primitive-like cells embedded in abundant in collagen stroma, Scattered rhabdomyoblasts with eccentric nuclei and significant ample eosinophilic cytoplasm, In some cases tumor cells focally display a cribriform pattern closely mimicking adenoid cystic carcinoma, Some cases can also show positive expression of, Irregularly distributed disorganized filament in richly collagenized matrix, Loss of 10q22, loss of chromosome Y and gain of 18 (, Pattern of numerical changes similar to embryonal rhabdomyosarcoma in one case (, In an adult case: gain of chromosome 11 and loss of chromosome 22 (, Cytogenetic findings in 2 pediatric cases suggest a link with embryonal rhabdomyosarcoma (. 3 year old boy with paratesticular rhabdomyosarcoma presenting as thickening of tunica vaginalis (Pediatr Radiol 2009;39:1010) 10 year old boy with pediatric paratesticular rhabdomyosarcoma with epididymitis (World J Mens Health 2012;30:146) 13 and 15 year old boys with spindle cell variant (Acta Cytol 2005;49:331, Case #145) Cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and clinical! 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