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Desmoplastic small round cell tumor.

Nausheen Yaqoob·Sheema H Hasan
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Abstract

Desmoplastic small round cell tumour (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects adolescent male and young adults and most frequently presents as a large abdominal mass with widespread peritoneal involvement at the time of diagnosis. Histologically, it is composed of nests of small, undifferentiated round or oval hyperchromatic cells embedded in abundant desmoplastic stroma. Co-expression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence of origin from a primitive pluripotent stem cell with multiphenotypic differentiation. A multidisciplinary treatment including high-dose chemotherapy, aggressive debulking surgery, radiation and myeloablative chemotherapy with stem cell rescue might be the proper approach to treat this rare malignancy and may improve progression-free survival.

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Abdominal NeoplasmsCarcinoma, Small CellCombined Modality TherapyDiagnosis, DifferentialHumansPrognosisJournal ArticleReview

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Nausheen Yaqoob, Sheema H Hasan. 2006. Desmoplastic small round cell tumor.. https://doi.org/9.2006/jcpsp.614616

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Sister Mary Joseph's nodule as presenting sign of a desmoplastic small round cell tumor.

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PubMed2007

Cytology of desmoplastic small round-cell tumor: comparison of pre- and post-chemotherapy fine-needle aspiration biopsies.

BACKGROUND: Desmoplastic small round-cell tumor (DSRCT) is an aggressive malignancy of young adults, which is amenable to fine-needle aspiration biopsy (FNAB). As this entity is increasingly recognized and biopsied, cytopathologists are compelled to become familiar with the range of cytologic features of DSRCT. In addition, postchemotherapy tumors may be sampled to confirm disease recurrence before planning additional therapy. This study was designed to compare prechemotherapy and postchemotherapy cytomorphology of DSRCT and to evaluate for distinct chemotherapy-induced changes. METHODS: The authors searched their respective institutional databases for all DSRCT cases with an associated FNAB. FNAB slides, immunocytochemistry, and cytogenetic results were reviewed. RESULTS: Six aspirates from 5 patients were identified, 3 of which were postchemotherapy. The postchemotherapy cases demonstrated cytologic findings not typically described in DSRCTs, including prominent and conspicuous nucleoli, discohesive single-cell architecture, and slightly larger cell size. CONCLUSIONS: Cytomorphologic variability was prominent in prechemotherapy cases, and no case could be classified as DSRCT on cytology alone; immunohistochemistry was necessary for definitive diagnosis. Chemotherapy increased the spectrum of cytologic features. The most notable difference between the 2 groups was a predominantly discohesive single-cell pattern with conspicuous nucleoli in the postchemotherapy group, instead of the clustering pattern of medium-sized cells with inconspicuous nucleoli typically attributed to de novo cases reported in the literature.

Abdominal Neoplasms↗