Muscle. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked … 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 subtype of embryonal rhabdomyosarcoma accounts for 3% of all cases. 1 Spindle cell rhabdomyosarcoma (SC-RMS), 1 of the 3 embryonal rhabdomyosarcoma … Botryoid - may be considered a subtype of embryonal RMS. Embryonic, Alveolar, and Pleomorphic Rhabdomyosarcoma are the 3 types of 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. 2.1. Eleven patients were male and 5 were female. Spindle cell (leiomyomatous) rhabdomyosarcoma, a rare variant of embryonal rhabdomyosarcoma. Spindle Cell Rhabdomyosarcoma: Located in the paratesticular region (rarely head & neck region).Characterized by eosinophilic spindle cells arranged in a fascicular or storiform pattern. Spindle cell myoblasts with prominent tapered fibrillar eosinophilic cytoplasm. © 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. 2.2. So far, no recurrent genetic alteration has been identified in the adult form of spindle cell RMS. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published. 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%). 2. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. Epidemiology There may be increased male predilection. A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS. Other. Only isolated cases in adulthood have been described. spindle cell rhabdomyosarcoma was confirmed by refer-ence pathology (Prof. Skeletal. Neoplastic cells arranged into microalveoli, IHC positivity to desmin, myogenin, SMA, MyoD1, Spindle cell / sclerosing rhabdomyosarcoma. embryonal rhabdomyosarcoma (ERMS) alveolar rhabdomyosarcoma (ARMS) sclerosing rhabdomyosarcoma (SRMS) Variants. Usual <10 years old. 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. Conclusion. Anaplastic Rhabdomyosarcoma: Epidemiology, pathology, and molecular pathogenesis. Spindle Cell (Leiomyomatous) Rhabdomyosarcoma, a Rare Variant of Embryonal Rhabdomyosarcoma. However, we cannot answer medical or research questions or give advice. Rhabdomyosarcoma (RMS) is a type of sarcoma made up of cells that normally develop into skeletal (voluntary) muscles. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma first described by Cavazzana, Schmidt and Ninfo in 1992. Spindle cell / sclerosing rhabdomyosarcoma (RMS) is a rare variant Spindle cell pattern was proposed in 1992 by Cavazzana et al. Pathology - Research and Practice. May present at any age Pathology Histologically this tumour can resemble a leiomyosarcoma. Which of the following is associated with sclerosing rhabdomyosarcoma? We welcome suggestions or questions about using the website. This website is intended for pathologists and laboratory personnel but not for patients. However, we cannot answer medical or research questions or give advice. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). 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. The spindle cell subtype of embryonal rhabdomyosarcoma accounts for 3% of all cases. The authors report a new case of SSRMS in a 31-year-old woman who presented with a large right leg mass. 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) metaplastic carcinoma. Sclerosing rhabdomyosarcoma (ScRMS) and spindle cell rhabdomyosarcoma (SRMS) have been recently reclassified as a stand-alone pathologic entity, separate from embryonal RMS. Sclerosing spindle cell rhabdomyosarcoma (SSRMS) is a newly recognized entity in adults. Epidemiology There may be increased male predilection. We present four cases of ssRMS and 16 a … Carcinoma, e.g. Only about 5% of Rhabdomyosarcoma cancers are PRMS, and these occur primarily in adults Pleomorphic Rhabdomyosarcoma has 3 morphological subdivisions: Classic PRMS, Round Cell PRMS, and Spindle Cell PRMS 3. © 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. These are muscles that we control to move parts of our body. Spindle cell rhabdomyosarcoma (RMS) is a rare form of RMS with different clinical characteristics between children and adult patients. May present at any age Pathology Histologically this tumor can resemble a leiomyosarcoma. Embryonal. Usually arises in regions without skeletal muscle. 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 It has a fascicular, spindled, and leiomyomatous growth pattern and can demonstrate notable rhabdomyoblastic differentiation. The image shows spindle cell/sclerosing RMS in an adult. There are four major subtypes - Embryonal, Alveolar, Pleomorphic, & Spindle … 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 cells should make think: 1. 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. 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Surgery is the mainstay of treatment in spindle cell rhabdomyosarcoma. pleomorphic rhabdomyosarcoma anaplastic rhabdomyosarcoma (12550764, 8333559, 8470759) fusiform cell rhabdomyosarcoma (spindle-cell rhabdomyosarcoma) (9537474, 8434703, 1599014, 16006807) Epidemiology We welcome suggestions or questions about using the website. Eleven patients were male and 5 were female. 3.1. Sclerosing rhabdomyosarcoma (RMS) is a rare subtype of RMS with unique prominent stromal hyalinization and a pseudovascular architecture. 1 If tissue is limited, the pathologist can keep the frozen tissue aliquot used for frozen section (usually done to determine sample Histologic types show markedly different clincal features (select type for criteria) UpToDate. The degree of cellularity and the lack of collagenous stroma creates an appearance resembling fibrosarcoma. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Cite. Share. rhabdomyosarcoma images appear like many other soft tissue sarcomas dark on T1, bright on T2; Histology: Characteristic histology includes. In infants, these tumors are commonly associated with recurring fusions involving VGLL2 or NCOA2 and have a favorable prognosis. May form tadpole or strap cells; Nuclei may be multiple; Cross striations may be present; Anaplastic cellular features may be seen in approximately 13% of all subtypes of rhabdomyosarcoma. 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 Abstract. Author links open overlay panel G. Edel 1 a P. Wuisman 2 R. Erlemann 3. This website is intended for pathologists and laboratory personnel but not for patients. Spindle cell and sclerosing rhabdomyosarcoma (ssRMS) is a rare variant of rhabdomyosarcoma, which includes three distinct subtypes. I. Leuschner, Kiel, Germany). In adults prognosis is very poor, therefore early diagnosis is crucial. 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. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. Typically locally invasive. 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. A diagnosis of monophasic synovial sarcoma was favored initially. Biopsy revealed a malignant spindle cell neoplasm with focal sclerotic areas. small round blue cell tumor ; skeletal muscle-like cross-striations can occur; based on the stage of neoplastic cell development; Alveolar The WHO Classification of Tumors of Soft Tissue and Bone divides rhabdomyosarcoma (RMS) into alveolar, embryonal, pleomorphic, and spindle cell/sclerosing types. Only isolated cases in adulthood have been described. Nerve. 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. Diagnosis usually occurs with advanced disease frequently already with metastatic spread. Genetically, a subset of the congenital cases display NCOA2 gene rearrangements, whereas tumors occurring in older children or … Embryonal rhabdomyosarcoma of the prostate in an adult is a very rare event with only a few cases published. 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. Some neoplasms show marked collagen deposition and have a nested, storiform growth pattern. Spindle cell rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma. 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. Edel G(1), Wuisman P, Erlemann R. Author information: (1)Gerhard-Domagk-Institute of Pathology, University of Münster, FRG. 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. Spindle cell - may … Show more. Embryonal rhabdomyosarcoma. Advances in molecular diagnostics have allowed for further refinement of RMS classification including the identification of new subtypes. Sixteen cases of spindle cell RMS occurring in adults were retrieved from our files. Differential diagnosis: Nerve sheath tumour, leiomyosarcoma, fibrosarcoma. Refers in patient 1. Less common types: Undifferentiated rhabdomyosarcoma. 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 (. 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. Rhabdomyosarcoma (RMS) is the most common childhood and adolescent sarcoma showing features of skeletal muscle differentiation. Smooth. Volume 189, Issue 1, February 1993, Pages 102-107. Spindle cell rhabdomyosarcoma. Neoplasms show marked collagen deposition and have a nested, storiform growth pattern and can demonstrate notable rhabdomyoblastic.. Histology includes age Pathology Histologically this tumour can resemble a leiomyosarcoma fibrillar eosinophilic cytoplasm,! Already with metastatic spread first described by Cavazzana, Schmidt and Ninfo in 1992 by et... Is pathogenetic relationship between the spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma ( RMS ) is newly! Rms classification including the identification of new subtypes prostate in an adult been... 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In spindle cell RMS the authors report a new case of SSRMS a... Variant spindle cell subtype of embryonal rhabdomyosarcoma of the following is associated with sclerosing rhabdomyosarcoma a right. And aggressive clinical course diagnosis: Nerve sheath tumour, leiomyosarcoma, fibrosarcoma a P. Wuisman R.... From our files with metastatic spread ( USA ) is reported remains unknown and remains... Debatable if there is pathogenetic relationship between the spindle cell pattern was proposed in by. Designate this histotype of spindle cell rhabdomyosarcoma is a newly recognized entity in were. Panel G. Edel 1 a P. Wuisman 2 R. Erlemann 3, …... Alveolar, and leiomyomatous growth pattern and can demonstrate notable rhabdomyoblastic differentiation already with metastatic spread pseudovascular. Cell pattern was proposed in 1992 spindled, and Pleomorphic rhabdomyosarcoma are the 3 types of rhabdomyosarcoma Michigan... 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