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Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product.
Mod Pathol. 2003 Mar; 16(3):229-35.MP

Abstract

Desmoplastic small round cell tumor is a rare tumor typically involving peritoneum. Although the histogenesis of desmoplastic small round cell tumor has yet to be elucidated, immunophenotypical and morphological analysis shows a characteristic divergent phenotype overlapping with other round cell tumors such as Ewing's sarcoma/primitive neuroectodermal tumor, rhabdomyosarcoma, small cell mesothelioma, and carcinoma. Detection of the EWS-WT1 gene fusion is characteristic of desmoplastic small round cell tumor and has been used reliably in tumor diagnosis. In this study, we evaluated the immunophenotype of 23 desmoplastic small round cell tumor cases with the EWS-WT1 gene fusion product identified by reverse transcription-polymerase chain reaction. Paraffin sections were stained with antibodies against calretinin, WT1 (C19), desmin, myoglobin, MyoD, Myf5, myogenin, placental alkaline phosphatase, cytokeratins, MIC2, HER2/neu and c-kit using standard immunohistochemical methods. Immunoreactivity was evaluated semiquantitively by light microscopy. Desmoplastic small round cell tumors showed reactivity with calretinin in 4/21, desmin in 21/23, myoglobin in 5/17, placental alkaline phosphatase in 17/21, HER2/neu in 7/18 (3+ in 1 and 1+ in 6), c-kit in 2/14, MIC2 in 13/23, WT1 in 16/23, CAM5.2 in 21/23, and AE1/3 in 16/23 cases. The most sensitive myogenic and epithelial markers are desmin and CAM 5.2. Although nuclear reactivity of the early myogenic regulatory factors (MyoD, myogenin, Myf5) was not detected, myoglobin immunoreactivity was present in 29% of desmoplastic small round cell tumors. HER2/neu overexpression (3+) and c-kit expression are uncommon in desmoplastic small round cell tumors. A panel of myogenic and epithelial markers should be used to detect the divergent phenotype in desmoplastic small round cell tumors, a key feature in the differential diagnosis. Detection of EWS-WT1 fusion becomes critical for the diagnosis when the characteristic divergent phenotype cannot be detected immunohistochemically.

Authors+Show Affiliations

Anatomic Pathology, 6 Founders Pavilion, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19147, USA. pjz@mail.med.upenn.edu.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

12640103

Citation

Zhang, Paul J., et al. "Immunophenotype of Desmoplastic Small Round Cell Tumors as Detected in Cases With EWS-WT1 Gene Fusion Product." Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, vol. 16, no. 3, 2003, pp. 229-35.
Zhang PJ, Goldblum JR, Pawel BR, et al. Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product. Mod Pathol. 2003;16(3):229-35.
Zhang, P. J., Goldblum, J. R., Pawel, B. R., Fisher, C., Pasha, T. L., & Barr, F. G. (2003). Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product. Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, 16(3), 229-35.
Zhang PJ, et al. Immunophenotype of Desmoplastic Small Round Cell Tumors as Detected in Cases With EWS-WT1 Gene Fusion Product. Mod Pathol. 2003;16(3):229-35. PubMed PMID: 12640103.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Immunophenotype of desmoplastic small round cell tumors as detected in cases with EWS-WT1 gene fusion product. AU - Zhang,Paul J, AU - Goldblum,John R, AU - Pawel,Bruce R, AU - Fisher,Cyril, AU - Pasha,Teresa L, AU - Barr,Frederic G, PY - 2003/3/18/pubmed PY - 2003/10/15/medline PY - 2003/3/18/entrez SP - 229 EP - 35 JF - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc JO - Mod Pathol VL - 16 IS - 3 N2 - Desmoplastic small round cell tumor is a rare tumor typically involving peritoneum. Although the histogenesis of desmoplastic small round cell tumor has yet to be elucidated, immunophenotypical and morphological analysis shows a characteristic divergent phenotype overlapping with other round cell tumors such as Ewing's sarcoma/primitive neuroectodermal tumor, rhabdomyosarcoma, small cell mesothelioma, and carcinoma. Detection of the EWS-WT1 gene fusion is characteristic of desmoplastic small round cell tumor and has been used reliably in tumor diagnosis. In this study, we evaluated the immunophenotype of 23 desmoplastic small round cell tumor cases with the EWS-WT1 gene fusion product identified by reverse transcription-polymerase chain reaction. Paraffin sections were stained with antibodies against calretinin, WT1 (C19), desmin, myoglobin, MyoD, Myf5, myogenin, placental alkaline phosphatase, cytokeratins, MIC2, HER2/neu and c-kit using standard immunohistochemical methods. Immunoreactivity was evaluated semiquantitively by light microscopy. Desmoplastic small round cell tumors showed reactivity with calretinin in 4/21, desmin in 21/23, myoglobin in 5/17, placental alkaline phosphatase in 17/21, HER2/neu in 7/18 (3+ in 1 and 1+ in 6), c-kit in 2/14, MIC2 in 13/23, WT1 in 16/23, CAM5.2 in 21/23, and AE1/3 in 16/23 cases. The most sensitive myogenic and epithelial markers are desmin and CAM 5.2. Although nuclear reactivity of the early myogenic regulatory factors (MyoD, myogenin, Myf5) was not detected, myoglobin immunoreactivity was present in 29% of desmoplastic small round cell tumors. HER2/neu overexpression (3+) and c-kit expression are uncommon in desmoplastic small round cell tumors. A panel of myogenic and epithelial markers should be used to detect the divergent phenotype in desmoplastic small round cell tumors, a key feature in the differential diagnosis. Detection of EWS-WT1 fusion becomes critical for the diagnosis when the characteristic divergent phenotype cannot be detected immunohistochemically. SN - 0893-3952 UR - https://www.unboundmedicine.com/medline/citation/12640103/Immunophenotype_of_desmoplastic_small_round_cell_tumors_as_detected_in_cases_with_EWS_WT1_gene_fusion_product_ L2 - https://doi.org/10.1097/01.MP.0000056630.76035.F3 DB - PRIME DP - Unbound Medicine ER -