Embryonal and Alveolar Rhabdomyosarcoma of Parameningeal Sites in Adults: A Report of 13 Cases

Abstract

This study reports 13 adult parameningeal rhabdomyosarcomas. Ages ranged from 18 to 86 years. Nine lesions were alveolar, 3 were embryonal, and 1 could not be further classified. All cases were positive for desmin and/or myogenin and 1 tumor was strongly positive for cytokeratin. PAX3-FKHR or PAX7-FKHR fusion transcripts or FKHR breaks were identified in 5 cases confirming a diagnosis of alveolar rhabdomyosarcoma. Three cases were negative supporting a diagnosis of embryonal rhabdomyosarcoma. A total of 10 patients were treated with chemotherapy and radiation. Three patients are alive with no disease, 3 are alive with disease, 3 died of disease, and 4 patients are lost to follow-up. Adult sinonasal rhabdomyosarcoma is uncommon and should be considered in the differential of sinonasal neoplasms. Disease can occur in the elderly. Desmin and myogenin can aid in the diagnosis but cytokeratin reactivity can be seen and care must be taken not to diagnose carcinoma.

Keywords

rhabdomyosarcoma sinonasal tumors alveolar myogenin

Get full access to this article

View all access options for this article.

References

Barr FG , Chatten J. , D'Cruz CM , et al. Molecular assays for chromosomal translocations in the diagnosis of pediatric soft tissue sarcomas. JAMA. 1995 ;273:553-557.

Barr FG , Xiong QB , Kelly K. A consensus polymerase chain reaction-oligonucleotide hybridization approach for the detection of chromosomal translocations in pediatric bone and soft tissue sarcomas. Am J Clin Pathol. 1995;104:627-633.

Xia SJ , Pressey JG , Barr FG Molecular pathogenesis of rhabdomyosarcoma. Cancer Biol Ther . 2002;1:97-104.

Biegel JA , Nycum LM , Valentine V. , Barr FG , Shapiro DN Detection of the t(2;13)(q35;q14) and PAX3-FKHR fusion in alveolar rhabdomyosarcoma by fluorescence in situ hybridization. Genes Chromosomes Cancer . 1995;12:186-192.

Callender TA , Weber RS , Janjan N. , et al. Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children. Otolaryngol Head Neck Surg . 1995;112:252-257.

Chan JK , Ng HK , Wan KY , Tsao SY , Leung TW , Tse KC Clear cell rhabdomyosarcoma of the nasal cavity and paranasal sinuses . Histopathology. 1989;14:391-399.

Durham L. , Mackenzie IJ , Foy P. Embryonal rhabdomyosarcoma of the sphenoid sinus. J Larnygol Otol. 1988;102:836-837.

Grieman RB , Katsikeris NF , Symington JM Rhabdomyosarcoma of the maxillary sinus: review of the literature and report of a case. J Oral Maxillofac Surg. 1988;46:1090-1096.

Koenigsberg RA , Noah R. , Turtz A. , et al: Rhabdomyosarcoma of the paranasal sinuses in and adult. Clin Imaging. 1995;19:234-236.

10.

Lee JH , Lee MS , Lee BH , et al. Rhabdomyosarcoma of the head and neck in adults: MR and CT findings . AJNR Am J Neuroradiol. 1996;17:1923-1928.

11.

Manucha V. , Castellani R. , Sun CC Alveolar rhabdomyosarcoma of the paranasal sinuses in a 57 year-old woman with 1:16 translocation. Int J Surg Pathol. 2006;14:238-242.

12.

Moon, HS , Kwon SW , Lee JH A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult. Korean J Ophthalmol. 2006;20:70-75.

13.

Muto E. , Shioyama Y. , Nakamura K. , et al. Adult rhabdomyosarcoma in the nasal and paranasal sinuses showing complete local response to a combination of chemotherapy and radiotherapy using 3D-CRT and IMRT. Fukuoka Igaku Zasshi. 2005;96:363-369.

14.

Nakhleh RE , Swanson PE , Dehner LP Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults: a clinical, pathologic, and immunohistochemical study of 12 cases . Cancer. 1991;67:1019-1024.

15.

Ogawa I. , Takata T. , Nikai H. , Suei Y. , Ishikawa T. Alveolar rhabdomyosarcoma (solid variant) of the maxillary sinus in an adult . Int J Oral Maxillofac Surg. 1996 ;25:122-123.

16.

Suzuki M. , Kobayashi Y. , Harada Y. , et al. Rhabdomyosarcoma of the maxillary sinus. A case report. J Laryngol Otol. 1984; 98:405-415.

17.

Wharam MD Jr. Rhabdomyosarcoma of parameningeal sites. Semin Radiat Oncol. 1997;7:212-216.

18.

Wurm J. , Constantinidis J. , Grabenbauer GG , Iro H. Rhabdomyosarcoma of the nose and paranasal sinuses: treatment results in 15 cases. Otolaryngol Head Neck Surg. 2005 ;133:42-50.

19.

Yamaguchi S. , Nagasawa H. , Suzuki T. , et al. Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years. Clin Oral Investig . 2004;8:52-55.

20.

Barr FG , Nauta LE , Hollows JC Structural analysis of PAX3 genomic rearrangements in alveolar rhabdomyosarcoma . Cancer Genet Cytogenet. 1998;102:32-39.

21.

Das K. , Mirani N. , Hameed M. , Pliner L. , Aisner SC Fine-needle aspiration cytology of alveolar rhabdomyosarcoma utilizing ThinPrep liquid-based sample and cytopspin preparations: a case confirmed by FKHR break apart rearrangement by FISH probe. Diagn Cytopathol . 2006;34:704-706.

22.

Ca?lar K. , Varan A. , Akyüz C. , et al. Second neoplasms in pediatric patients treated for cancer: a center's 30-year experience. J Pediatr Hematol Oncol. 2006;28:374-378.

23.

Esnaola NF , Rubin BP , Baldini EH , et al. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg. 2001;234:215-223.

24.

Sorensen PH , Lynch JC , Qualman SJ , et al. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group. J Clin Oncol . 2002 ;20:2672-2679.

25.

Davicioni E. , Finckenstein FG , Shahbazian V. , Buckley JD , Triche TJ , Anderson MJ Identification of a PAX-FKHR gene expression signature that defines molecular classes and determines the prognosis of alveolar rhabdomyosarcomas. Cancer Res. 2006;66:6936-6946.

26.

Wachtel M. , Dettling M. , Koscielniak E. , et al.Gene expression signatures identify rhabdomyosarcoma subtypes and detect a novel t(2;2)(q35;p23) translocation fusing PAX3 to NCOA1. Cancer Res. 2004;64: 5539-5545.

27.

Berkes CA , Tapscott SJ MyoD and the transcriptional control of myogenesis. Semin Cell Dev Biol. 2005;16: 585-595.

28.

Dias P. , Chen B. , Dilday B. , et al. Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. Am J Pathol . 2000;156:399-408.

29.

Hostein I. , Andraud-Fregeville M. , Guillou L. , et al. Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype: an analysis of 109 paraffin-embedded specimens. Cancer. 2004;101:2817-2824.

30.

Miettinen M. , Rapola J. Immunohistochemical spectrum of rhabdomyosarcoma and rhabdomyosarcoma-like tumors. Expression of cytokeratin and the 68-kD neuro-filament protein. Am J Surg Pathol. 1989;13:120-132.

31.

Coindre JM , de Mascarel A. , Trojani M. , de Mascarel I. , Pages A. Immunohistochemical study of rhabdomyosarcoma. Unexpected staining with S100 protein and cytokeratin. J Pathol. 1988;155:127-132.

32.

Ferrari A. , Dileo P. , Casanova M. , et al. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer. 2003;98:571-580.