Primary cutaneous Ewing’s sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings Authors
First Online: 12 February 2010 Received: 22 August 2009 Revised: 27 December 2009 Accepted: 28 December 2009 DOI:
Cite this article as: Bahk, W., Chang, E., Bae, J.M. et al. Skeletal Radiol (2010) 39: 595. doi:10.1007/s00256-009-0872-x Abstract
Extraskeletal Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them.
Keywords Primary cutaneous Ewing’s sarcoma/primitive neuroectodermal tumor MRI findings Multiple huge masses Clinical outcome References
Delattre O, Zucman J, Melot T, Garau XS, Zucker JM, et al. The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331:294–9.
Ehrig T, Billings SD, Fanburg-Smith JC. Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity? Ann Diagn Pathol. 2007;11:153–9.
Ezinger FM, Weiss SW. Primitive neuroectodermal tumors and related lesions. In Soft tissue tumor. 3rd ed. St Louis: Mosby; 1995. p. 929–64.
Chow E, Pappo A, Jenkin JJ, Shah AB, Kun LE. Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys. 2000;46:433–8.
Hasegawa SI, Davison J, Rutten A, Fletcher JA, Fletcher CDM. Parimary cutaneous Ewing’s sarcoma. Immunophenotypic and molecular cytogenic evaluation of five cases. Am J Surg Pathol. 1998;22:310–8.
Sanguenza OP, Sangeuza P, Valda LR, Meshul CK, Requena L. Multiple primitive neuroectodermal tumors. J Am Acad Dermatol. 1994;31:356–61.
Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer. 1975;36:240–51.
Jaffe R, Santamaria M, Yunis EJ, Tannery NH, Agostini RM Jr, Medina RM, et al. The neuroectodermal tumor of bone. Am J Surg Pathol. 1984;8:885–98.
Aurias A, Rimbaut C, Buffe D, Dubousset J, Mazabraud A. Chromosomal translocation in Ewing’s sarcoma. N Engl J Med. 1983;309:496–8.
Dehner LP. Primitive neuroectodermal tumor and Ewing’s sarcoma. Am J Surg Pathol. 1993;17:1–13.
Moll R, Lee I, Gould VE, Berndt R, Roessner A, Franke WW. Immunocytochemical analysis of Ewing’s tumors. Patterns of expression of intermediate filaments and desmosomal proteins indicate cell type heterogeneity and pluripotential differentiation. Am J Pathol. 1987;127:288–304.
Hess E, Cohen C, DeRose PB, Yost B, Costa MJ. Nonspecificity of P30/32M/C2 immunolocalization with the O13 monoclonal antibody in the diagnosis of Ewing’s sarcoma: application of an algorithmic immunohistochemical analysis. Appl Immunohistochem. 1997;5:94–103.
Stevenson AJ, Chatten J, Bertoni F, Mietinnen M. CD99 (p30/32M/C2) neuroectodermal/Ewing’s sarcoma antigen as an immunohistochemical marker: review of more than 600 tumors and the literature experience. Appl Immunohistochem. 1994;2:231–40.
Weidner N, Tjoe T. Immunohistochemical profile of monoclonal antibody O13: antibody that recognizes glycoprotein P30/32MIC2 and is useful in diagnosing Ewing’s sarcoma and peripheral neuroepithelioma. Am J Surg Pathol. 1994;18:486–94.
Shingde MV, Buckland M, Busam KJ, McCarthy AW, Wilmott J, Thompson JF et al. Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumor: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis. J Clin Pathol. 2009;62:915–9.
Kim JH, Kim JY, Chun KA, Jee WH, Sung MS. MR imaging manifestations of skin tumors. Eur Radiol. 2008;18:2652–61.