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sclerosing rhabdomyosarcoma

Monday 27 March 2006

Definition: Sclerosing rhabdomyosarcoma, a particular phenotypic variant of rhabdomyosarcoma initially described in the adult population, has emerged as a potential pitfall in the evaluation of pediatric sarcomas.

Sclerosing rhabdomyosarcoma (RMS) is characterized by a hyalinizing, matrix-rich stroma. It is a newly described variant of rhabdomyosarcoma with a predilection for the head and neck.

Synopsis

- prominent sclerosing hyaline matrix
- pseudovascular and microalveolar architectural foci
- focal positivity for desmin, smooth muscle actin, and myogenin.

Ultrastructure

- irregularly distributed disorganized filaments without clear evidence of Z-bands
- richly collagenized matrix

Differential diagnosis

Because of its densely hyalinized collagenous matrix and its occasional expression of a pseudovascular pattern of growth, sclerosing rhabdomyosarcoma has been at times misdiagnosed as chondrosarcoma, osteosarcoma, or angiosarcoma. (12218574)

Comparative genomic hybridization

- loss of chromosomal region 10q22 (17016719)
- loss of chromosome Y (17016719)
- gain of chromosome 18 (trisomy) (17016719)

Cytogenetics

- mild hyperdiploidy (15630526)
- near-tetraploidy (15630526)

- del(1)(p22) (15660283)
- trisomy 11 (+11) (15660283)
- trisomy 16 (+16) (15660283)
- tumoral trisomy 18 (+18) (15660283)
- tumoral trisomy 21 (+21) (15660283)
- monosomy 22 (-22) (15660283)

CGH

- 10q22 loss (17016719)
- Chr.Y loss (17016719)
- Chr.18 gain (trisomy 18) (17016719)

See also

- rhabdomyosarcomas

References

- Kuhnen C, Herter P, Leuschner I, Mentzel T, Druecke D, Jaworska M, Johnen G. Sclerosing pseudovascular rhabdomyosarcoma-immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma. Virchows Arch. 2006 Nov;449(5):572-8. PMID: 17016719

- Zambrano E, Perez-Atayde AR, Ahrens W, Reyes-Mugica M. Pediatric sclerosing rhabdomyosarcoma. Int J Surg Pathol. 2006 Jul;14(3):193-9. PMID: 16959698

- Croes R, Debiec-Rychter M, Cokelaere K, De Vos R, Hagemeijer A, Sciot R. Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma. Virchows Arch. 2005 Jan;446(1):64-7. PMID: 15660283

- Knipe TA, Chandra RK, Bugg MF. Sclerosing rhabdomyosarcoma: a rare variant with predilection for the head and neck. Laryngoscope. 2005 Jan;115(1):48-50. PMID: 15630365

- Vadgama B, Sebire NJ, Malone M, Ramsay AD. Sclerosing rhabdomyosarcoma in childhood: case report and review of the literature. Pediatr Dev Pathol. 2004 Jul-Aug;7(4):391-6. PMID: 15383934

- Chiles MC, Parham DM, Qualman SJ, Teot LA, Bridge JA, Ullrich F, Barr FG, Meyer WH; Soft Tissue Sarcoma Committee of the Children’s Oncology Group. Sclerosing rhabdomyosarcomas in children and adolescents: a clinicopathologic review of 13 cases from the Intergroup Rhabdomyosarcoma Study Group and Children’s Oncology Group.
Pediatr Dev Pathol. 2004 Nov-Dec;7(6):583-94. PMID: 15630526

- Vadgama B, Sebire NJ, Malone M, Ramsay AD. Sclerosing rhabdomyosarcoma in childhood: case report and review of the literature. Pediatr Dev Pathol. 2004 Jul-Aug;7(4):391-6. PMID: 15383934

- Sclerosing rhabdomyosarcoma in adults: report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma. Folpe AL, McKenney JK, Bridge JA, Weiss SW. Am J Surg Pathol. 2002 Sep;26(9):1175-83. PMID: 12218574

- Mentzel T, Katenkamp D. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases. Virchows Arch. 2000 Apr;436(4):305-11. PMID: 10834531

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