Desmoplastic Small Round Cell Tumor



Desmoplastic Small Round Cell Tumor


Saul Suster, MD

Elizabeth A. Montgomery, MD

Cyril Fisher, MD, DSc, FRCPath









In desmoplastic small round cell tumor, islands of neoplastic cells proliferate in dense desmoplastic stroma. The tumor cells are markedly hyperchromatic in contrast to the stromal myofibroblasts image.






The neoplastic cells in desmoplastic round cell tumor are quite uniform, a feature of sarcomas with balanced reciprocal translocations. Nucleoli are inconspicuous, and cytoplasm is scanty.


TERMINOLOGY


Abbreviations



  • Desmoplastic small round cell tumor (DSRCT)


Synonyms



  • Desmoplastic small round cell tumor with divergent differentiation


  • Polyphenotypic small round cell tumor


  • Desmoplastic primitive neuroectodermal tumor


  • Intraabdominal desmoplastic small round cell tumor


Definitions



  • Primitive malignant neoplasm arising in serosal surfaces with distinctive histology composed of primitive small round blue cells embedded in abundant desmoplastic stroma


CLINICAL ISSUES


Presentation



  • Male predilection


  • Pain and weight loss


  • Most common in children and young adults (2nd or 3rd decade; median age: 20 years)


  • Most commonly present in peritoneal cavity



    • Also reported in paratesticular region, ovary, thoracic cavity, lung, central nervous system, and head and neck


Treatment



  • Surgical excision


  • Chemotherapy


Prognosis



  • Poor prognosis



    • Nearly uniformly fatal


  • Frequent local recurrence; rarely metastasizes


  • Median survival is 24 months


MACROSCOPIC FEATURES


General Features



  • Large, bulky tumors (> 10 cm in greatest dimension)


  • May also grow in multinodular fashion


  • Firm homogeneous cut surface


  • Hemorrhage and necrosis


MICROSCOPIC PATHOLOGY


Histologic Features



  • Nests, trabeculae, or sheets of small round blue cells embedded in abundant desmoplastic fibrous stroma


  • Nests may display peripheral palisading of tumor cells


  • Rosette-like structures


Cytologic Features



  • Small to intermediate-sized round to oval cells with scant cytoplasm


  • Round to oval, hyperchromatic nuclei


  • Small nucleoli


  • High mitotic rate, with typical mitoses (translocation sarcoma)

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Desmoplastic Small Round Cell Tumor
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