Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature

Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma with characteristic clinical and pathologic features. It typically involves pelvic and abdominal organs of young male patients, and patients usually present at advanced stage with poor prognosis. A few reports are available des...

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Main Authors: Hui Zhu, Emily Marie McMeekin, Charles D. Sturgis
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2015/676894
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author Hui Zhu
Emily Marie McMeekin
Charles D. Sturgis
author_facet Hui Zhu
Emily Marie McMeekin
Charles D. Sturgis
author_sort Hui Zhu
collection DOAJ
description Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma with characteristic clinical and pathologic features. It typically involves pelvic and abdominal organs of young male patients, and patients usually present at advanced stage with poor prognosis. A few reports are available describing the cytopathologic features of DSRCT in serous effusions, with the majority of published cases depicting undifferentiated small blue cells that need to be distinguished from other small blue cell tumors. We report an interesting case of DSRCT involving a pleural effusion with a “floating island” pattern that has been described in hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma. In our case, the epithelioid tumor cells form cohesive aggregates surrounded by a single layer of spindle cells, mimicking the “endothelial wrapping” in other tumors with “floating island” patterns. We demonstrate, by ancillary testing, that these peripheral spindle cells are tapered/flattened DSRCT cells, in contrast to endothelial wrapping cells, as seen in other tumors with this unique cytomorphology. To our knowledge, this is the first case report describing DSRCT showing a “floating island” pattern that needs to be differentiated from metastatic hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma in effusion cytology.
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spelling doaj-art-831087f6bd974e47997af01a98db61652025-02-03T06:11:38ZengWileyCase Reports in Pathology2090-67812090-679X2015-01-01201510.1155/2015/676894676894Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the LiteratureHui Zhu0Emily Marie McMeekin1Charles D. Sturgis2Pathology and Laboratory Medicine Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USAPathology and Laboratory Medicine Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USAPathology and Laboratory Medicine Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USADesmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma with characteristic clinical and pathologic features. It typically involves pelvic and abdominal organs of young male patients, and patients usually present at advanced stage with poor prognosis. A few reports are available describing the cytopathologic features of DSRCT in serous effusions, with the majority of published cases depicting undifferentiated small blue cells that need to be distinguished from other small blue cell tumors. We report an interesting case of DSRCT involving a pleural effusion with a “floating island” pattern that has been described in hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma. In our case, the epithelioid tumor cells form cohesive aggregates surrounded by a single layer of spindle cells, mimicking the “endothelial wrapping” in other tumors with “floating island” patterns. We demonstrate, by ancillary testing, that these peripheral spindle cells are tapered/flattened DSRCT cells, in contrast to endothelial wrapping cells, as seen in other tumors with this unique cytomorphology. To our knowledge, this is the first case report describing DSRCT showing a “floating island” pattern that needs to be differentiated from metastatic hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma in effusion cytology.http://dx.doi.org/10.1155/2015/676894
spellingShingle Hui Zhu
Emily Marie McMeekin
Charles D. Sturgis
Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
Case Reports in Pathology
title Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
title_full Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
title_fullStr Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
title_full_unstemmed Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
title_short Desmoplastic Small Round Cell Tumor, a “Floating Island” Pattern in Pleural Fluid Cytology: A Case Report and Review of the Literature
title_sort desmoplastic small round cell tumor a floating island pattern in pleural fluid cytology a case report and review of the literature
url http://dx.doi.org/10.1155/2015/676894
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