Difference between revisions of "Desmoplastic small round cell tumour"

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#redirect [[Soft_tissue_lesions#Desmoplastic_small_round_cell_tumour]]
'''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:<ref name=pmid10207460>{{cite journal |author=Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC |title=Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation |journal=Radiology |volume=210 |issue=3 |pages=633–8 |year=1999 |month=March |pmid=10207460 |doi= |url=http://radiology.rsna.org/content/210/3/633.full}}</ref>
#Broad bands of paucicellular fibrous stroma with:
#Small round cells in nests with an undulating sharp border.
#*The small round cells lack distinct nucleoli and have scant cytoplasm; they are ''[[small round cell tumour]]'' cells.
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===
<gallery>
Image:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg | DSRCT - intermed. mag. (WC/Nephron)
Image:Desmoplastic_small_round_cell_tumour_-_high_mag.jpg | DSRCT - high mag. (WC/Nephron)
Image:Desmoplastic_small_round_cell_tumour_-_very_high_mag.jpg | DSRCT - very high mag. (WC/Nephron)
</gallery>
==IHC==
Features:
*AE1/AE3 +ve.
*Desmin +ve.
*EMA +ve.
*Actin -ve.
*WT1 (N-terminal) -ve.
*WT1 (C-terminal) +ve.
*CD57 +ve.
==Molecular==
*t(11;22)(p13;q12).<ref name=pmid17964965>{{cite journal |author=Lee YS, Hsiao CH |title=Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients |journal=J. Formos. Med. Assoc. |volume=106 |issue=10 |pages=854–60 |year=2007 |month=October |pmid=17964965 |doi=10.1016/S0929-6646(08)60051-0 |url=}}</ref><ref>{{cite journal |author=Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP |title=Results of multimodal treatment for desmoplastic small round cell tumors |journal=J. Pediatr. Surg. |volume=40 |issue=1 |pages=251–5 |year=2005 |month=January |pmid=15868593 |doi=10.1016/j.jpedsurg.2004.09.046 |url=http://www.dsrct.com/JPS%20Article.pdf}}</ref>
==See also==
*[[Soft tissue lesions]].
==References==
{{Reflist|2}}
[[Category:Soft tissue lesions]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Revision as of 15:58, 27 September 2013

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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