Difference between revisions of "Desmoplastic small round cell tumour"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Desmoplastic_small_round_cell_tumour_-_high_mag.jpg
| Width      =
| Caption    = DSRCT. [[H&E stain]].
| Micro      = small (round) cells in nests with an undulating sharp border, road bands of paucicellular fibrous stroma
| Subtypes  =
| LMDDx      = [[Embryonal RMS]], other [[small round cell tumours]]
| Stains    =
| IHC        = AE1/AE3 +ve, desmin +ve, EMA +ve, actin -ve, WT1 (N-terminal) -ve, WT1 (C-terminal) +ve, CD57 +ve.
| EM        =
| Molecular  = t(11;22)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions]] - typically retroperitoneum
| Assdx      =
| Syndromes  =
| Clinicalhx = usu. young adults
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = (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.
'''Desmoplastic small round cell tumour''', abbreviated '''DSRCT''', is a rare malignant soft tissue lesion that tends to afflict young adults.


Revision as of 16:05, 27 September 2013

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 Embryonal RMS, 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:

  • Metastatic germ cell tumour (DDx of location and age).
  • Embryonal RMS.
    • It should be noted that DSRCT, like embryonal RMS, is +ve for desmin!
  • Solid variant of alveolar RMS.
    • Nests in alveolar RMS have round edges.

Images

  • DSRCT - intermed. mag. (WC/Nephron)

  • DSRCT - high mag. (WC/Nephron)

  • DSRCT - very high mag. (WC/Nephron)

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.
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