IJCRR - Vol 08 Issue 20, October, 2016
CLINICOPATHOLOGICAL AND IMMUNOPHENOTYPIC CHARACTERISTICS OF EWINGS SARCOMA FAMILY OF TUMORS: SPECIAL EMPHASIS ON ROLE OF FRIEND LEUKEMIA INTEGRATION - 1(FLI-1) ANTIBODY AND OCCURRENCE OF TUMOR ON RARE SITES
Author: Alka Mittal, Jayanti Mehta, Kalpana Mangal, Alpana Jain, Neetu Agarwal, Madan Kumar Solanki
Objective: Ewings sarcoma /Primitive neuroectodermal tumor (ES/PNET) is one of the aggressive malignant round cell tumors affecting mainly children and adolescents. This study aimed to emphasize the role of FLI-1 antibody along with CD99 in diagnosing ES/PNET and to keep this tumor in differential diagnosis even at some rare sites.
Material and Method: This descriptive and retrospective study was conducted from January 2014 – May 2016 and included 58 patients of ES/PNET. Histopathological and IHC findings were reviewed and the diagnosis of ES/PNET was confirmed. The tumors were further categorized into classic Ewings, PNET and atypical subtypes. Panel of IHC markers included vimentin, CD99, FLI-1, PanCK, LCA, Desmin, Myogenin, NSE, Synaptophysin, S100 and Chromogranin.
Results: Clinical Findings- We studied 58 patients of ES / PNET of whom 63% (37/58) were males, 37% (21/58) were females. The age ranged between 3- 65 years (mean 20 years).
Histological Findings: 57% of cases (33/58) exhibited classic morphology, 40% (23/58) were classified as PNET. There were only two cases of atypical Ewings sarcoma including large cell and Clear cell variant.
Immunohistochemical Findings: Strong membranous CD99 expression was seen in 100%, FLI -1 nuclear staining in 85% , NSE in 50%, S100 in 30%, Synaptophysin in 10% and EMA in 2% (1/58) cases. All other markers included in the panel were negative.
Tumor on Rare Sites: 10 Cases were of primary extradural spinal PNET, 1 intramedullary spinal, 2 brain parenchyma, 2 nose and nasopharynx , and one rare case of urinary bladder.
Conclusion: Clinical details, histomorphology and IHC includingCD99 and monoclonal FLI-1antibodies may help in supporting diagnosis of EFT if cytogenetic and FISH techniques are not available at any centre.
Keywords: Ewings sarcoma, Primitive neuroectodermal tumor, CD99, FLI-1v
Alka Mittal, Jayanti Mehta, Kalpana Mangal, Alpana Jain, Neetu Agarwal, Madan Kumar Solanki. CLINICOPATHOLOGICAL AND IMMUNOPHENOTYPIC CHARACTERISTICS OF EWINGS SARCOMA FAMILY OF TUMORS: SPECIAL EMPHASIS ON ROLE OF FRIEND LEUKEMIA INTEGRATION - 1(FLI-1) ANTIBODY AND OCCURRENCE OF TUMOR ON RARE SITES International Journal of Current Research and Review. Vol 08 Issue 20, October, 10-16
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