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CLC number: R445; R734.2

On-line Access: 2013-06-03

Received: 2012-12-13

Revision Accepted: 2013-04-01

Crosschecked: 2013-08-13

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Journal of Zhejiang University SCIENCE B 2013 Vol.14 No.9 P.859-865


Primary pulmonary rhabdomyosarcoma in an adult: a case report and review of the literature

Author(s):  Gui-yi Ji, Hui Mao

Affiliation(s):  Department of Respiratory Medicine, West China Hospital of Sichuan University, Chengdu 610041, China

Corresponding email(s):   merrymh@126.com

Key Words:  Adult, Pulmonary, Rhabdomyosarcoma

Gui-yi Ji, Hui Mao. Primary pulmonary rhabdomyosarcoma in an adult: a case report and review of the literature[J]. Journal of Zhejiang University Science B, 2013, 14(9): 859-865.

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Primary pulmonary rhabdomyosarcoma (RMS) is one of the most common soft-tissue malignancies in children, but is rare in persons aged >45 years (Triche, 1997; Ferrari et al., 2003). RMS is considered to result from the malignant transformation of primitive mesenchymal cells. The head, neck, and limbs are the principal locations of RMS. Primary pulmonary RMS is extremely rare and is frequently misdiagnosed as small cell lung cancer.

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[1]Barr, F.G., 2001. Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma. Oncogene, 20(40):5736-5746.

[2]Besnard-Guerin, C., Newsham, I., Winquist, R., Cavenee, W.K., 1996. A common region of loss of heterozygosity in Wilms’ tumor and embryonal rhabdomyosarcoma distal to the D11S988 locus on chromosome 11p15.5. Hum. Genet., 97(2):163-170.

[3]Burke, M., Anderson, J.R., Kao, S.C., Rodeberg, D., Qualman, S.J., Wolden, S.L., Meyer, W.H., Breitfeld, P.P., 2007. Assessment of response to induction therapy and its influence on 5-year failure-free survival in group III rhabdomyosarcoma: the intergroup rhabdomyosarcoma study-IV experience—a report from the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. J. Clin. Oncol., 25(31):4909-4913.

[4]Cessna, M.H., Zhou, H., Perkins, S.L., Tripp, S.R., Layfield, L., Daines, C., Coffin, C.M., 2001. Are myogenin and MyoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics. Am. J. Surg. Pathol., 25(9):1150-1157.

[5]Chang, W.Y., Yuan, L., Feng, L., Hensle, T., Tycko, B., 1996. Chromosome 11p15.5 regional imprinting: comparative analysis of KIP2 and H19 in human tissues and Wilms’ tumors. Hum. Mol. Genet., 5(8):1101-1108.

[6]Crist, W.M., Garnsey, L., Beltangady, M.S., Gehan, E., Ruymann, F., Webber, B., Hays, D.M., Wharam, M., Maurer, H.M., 1990. Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II—Intergroup Rhabdomyosarcoma Committee. J. Clin. Oncol., 8(3):443-452.

[7]Crist, W.M., Anderson, J.R., Meza, J.L., Fryer, C., Raney, R.B., Ruymann, F.B., Breneman, J., Qualman, S.J., Wiener, E., Wharam, M., et al., 2001. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J. Clin. Oncol., 19(12):3091-3102.

[8]Davis, R.J., D′Cruz, C.M., Lovell, M.A., Biegel, J.A., Barr, F.G., 1994. Fusion of PAX7 to FKHR by the variant t(1;13)(p36;q14) translocation in alveolar rhabdomyosarcoma. Cancer Res., 54(11):2869-2872.

[9]Dias, P., Chen, B., Dilday, B., Palmer, H., Hosoi, H., Singh, S., Wu, C., Li, X., Thompson, J., Parham, D., et al., 2000. Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. Am. J. Pathol., 156(2):399-408.

[10]Ferrari, A., Dileo, P., Casanova, M., Bertuli, R., Gandola, L., Navarria, P., Collini, P., Cronchi, A., Olmi, P., Fossati-Bellani, F., et al., 2003. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. J. Cancer, 98(3):571-580.

[11]Folpe, A.L., McKenney, J.K., Bridge, J.A., Weiss, S.W., 2002. 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. Am. J. Surg. Pathol., 26(9):1175-1183.

[12]Fredericks, W.J., Galili, N., Mukhopadhyay, S., Rovera, G., Bennicelli, J., Barr, F.G., Rauscher, F.J., 1995. The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3. Mol. Cell Biol., 15(3):1522-1535.

[13]Galili, N., Davis, R.J., Fredericks, W.J., Mukhopadhyay, S., Rauscher, F.J., Emanuel, B.S., Rovera, G., Barr, F.G., 1993. Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma. Nat. Genet., 5(3):230-235.

[14]Golub, T.R., Slonim, D.K., Tamayo, P., 1999. Molecular classification of cancer: class discovery and class prediction by gene expression monitoring. Science, 286(5439):531-537.

[15]Gurney, J.G., Young, J.L., Roffers, S.D., Smith, M.A., Bunin, G.R., 2005. Soft Tissue Sarcomas. National Cancer Institute, Bethesda, MD, USA.

[16]Houghton, P., Horton, J., Houghton, J., 1991. Drug Sensitivity and Resistance in the Xenograft Model. In: Maurer, H., Ruymann, R., Pochedly, C. (Eds.), Rhabdomyosarcoma and Related Tumors in Children. Boca Raton, FL, CRC, p.187-204.

[17]Koumarianou, A., Karageorgopoulou, S., Machairas, A., Liakakos, T., Chranioti, S., Dimitriadis, G., Misiakos, E.P., 2012. A pleomorphic rhabdomyosarcoma mimicking an inguinal hernia: a case report and review of the literature. Oncology, 5(1):74-79.

[18]Ladanyi, M., 1995. The emerging molecular genetics of sarcoma translocations. Diagn. Mol. Pathol., 4(3):162-173.

[19]Minn, A.Y., Lyden, E.R., Anderson, J.R., Million, L., Arndt, C.A., Brown, K., Hawkins, D.S., Donaldson, S.S., 2010. Early treatment failure in intermediate-risk rhabdomyosarcoma: results from IRS-IV and D9803—a report from the Children’s Oncology Group. J. Clin. Oncol., 28(27):4228-4232.

[20]Newton, W.A.Jr., Gehan, E.A., Webber, B.L., Marsden, H.B., van Unnik, A.J.M., Hamoudi, A.B., Tsokos, M.G., Shimada, H., Harms, D., Schmidt, D., et al., 1995. Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification— an intergroup rhabdomyosarcoma study. Cancer, 76(6):1073-1085.

[21]Ordonez, N.G., 1998. Desmoplastic small round cell tumor: II: an ultrastructural and immunohistochemical study with emphasis on new immunohistochemical markers. Am. J. Surg. Pathol., 22(11):1314-1327.

[22]Pappo, A.S., Shapiro, D.N., Crist, W.M., Maurer, H.M., 1995. Biology and therapy of pediatric rhabdomyosarcoma. J. Clin. Oncol., 13(8):2123-2139.

[23]Pappo, A.S., Lyden, E., Breneman, J., Wiener, E., Teot, L., Meza, J., Crist, W., Vietti, T., 2001. Up-front window trial of topotecan in previously untreated children and adolescents with metastatic rhabdomyosarcoma: an intergroup rhabdomyosarcoma study. J. Clin. Oncol., 19(1):213-219.

[24]Parham, D.M., Ellison, D.A., 2006. Rhabdomyosarcoma in adults and children: an update. Arch. Pathol. Lab. Med., 130(10):1454-1465.

[25]Sorensen, P.H., Triche, T.J., 1996. Gene fusions encoding chimaeric transcription factors in solid tumours. Semin. Cancer Biol., 7(1):3-14.

[26]Sorensen, P.H., Lynch, J.C., Qualman, S.J., Tirabosco, R., Lim, J.F., Maurer, H.M., Bridge, J.A., Crist, W.M., Triche, T.J., Barr, F.G., 2002. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children’s Oncology Group. J. Clin. Oncol., 20(11):2672-2679.

[27]Triche, T.J., 1997. Pathology and Molecular Diagnosis of Pediatric Malignancies. In: Pizzo, P.A., Poplack, D.G. (Eds.), Principles and Practice of Pediatric Oncology, 3rd Ed. Lippincott-Raven Publishers, Philadelphia, p.54-156.

[28]Wexler, L.H., Helman, L.J., 1997. Rhabdomyosarcoma and the Undifferentiated Sarcomas. In: Pizzo, P.A., Poplack, D.G. (Eds.), Principles and Practise of Pediatric Oncology, 3rd Ed. Lippincott-Raven Publishers, Philadelphia, p.238-329.

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