Desmoplastic small round cell tumour

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Desmoplastic small round cell tumour
Diagnosis in short

DSRCT. H&E stain.

LM small (round) cells in nests with an undulating sharp border, road bands of paucicellular fibrous stroma
LM DDx solid variant of alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, other small round cell tumours
IHC AE1/AE3 +ve, desmin +ve, EMA +ve, actin -ve, WT1 (N-terminal) -ve, WT1 (C-terminal) +ve, CD57 +ve.
Molecular t(11;22)
Site soft tissue lesions - typically retroperitoneum

Clinical history usu. young adults
Prevalence very rare
Prognosis poor
Clin. DDx (metastatic) germ cell tumour, lymphoma

Desmoplastic small round cell tumour, abbreviated DSRCT, is a rare malignant soft tissue lesion that tends to afflict young adults.

General

  • Males > females.
  • Usu. affects young adults.
  • Typically retroperitoneal.
  • Poor prognosis.

Microscopic

Features:[1]

  1. Broad bands of paucicellular fibrous stroma with:
  2. Small round cells in nests with an undulating sharp border.

Notes:

  • Usu. abundant mitoses.
  • +/-Necrosis.

DDx:

Images

IHC

Features:

  • AE1/AE3 +ve.
  • Desmin +ve.
  • EMA +ve.
  • Actin -ve.
  • WT1 (N-terminal) -ve.
  • WT1 (C-terminal) +ve.
  • CD57 +ve.

Molecular

See also

References

  1. Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC (March 1999). "Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation". Radiology 210 (3): 633–8. PMID 10207460. http://radiology.rsna.org/content/210/3/633.full.
  2. Lee YS, Hsiao CH (October 2007). "Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients". J. Formos. Med. Assoc. 106 (10): 854–60. doi:10.1016/S0929-6646(08)60051-0. PMID 17964965.
  3. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP (January 2005). "Results of multimodal treatment for desmoplastic small round cell tumors". J. Pediatr. Surg. 40 (1): 251–5. doi:10.1016/j.jpedsurg.2004.09.046. PMID 15868593. http://www.dsrct.com/JPS%20Article.pdf.