Difference between revisions of "Desmoplastic small round cell tumour"

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#redirect [[Soft_tissue_lesions#Desmoplastic_small_round_cell_tumour]]
{{ 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      = solid variant of [[alveolar rhabdomyosarcoma]], [[embryonal rhabdomyosarcoma]], 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.
 
==General==
*Males > females.
*Usu. affects young adults.
*Typically retroperitoneal.
*Poor prognosis.
 
==Clinics==
*Rare cases in the CNS reported.
 
==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.
*Other [[small round cell tumours]].
 
===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.
*CAM5.2 usu -ve.
*Desmin +ve (strongly).
*[[EMA]] +ve.
*Actin -ve.
*[[WT-1]] (N-terminal) -ve.
*[[WT-1]] (C-terminal) +ve (nuclear staining).
*CD57 +ve.
*[[Synaptophysin]] focal, patchy +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]].
*[[Small round cell tumours]].
*[[Stromal desmoplasia]].
 
==References==
{{Reflist|2}}
 
[[Category:Soft tissue lesions]]
[[Category:Neuropathology]]
[[Category:Diagnosis]]

Latest revision as of 11:10, 21 January 2020

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.

Clinics

  • Rare cases in the CNS reported.

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

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

  • DSRCT - high mag. (WC/Nephron)

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

IHC

Features:

  • AE1/AE3 +ve.
  • CAM5.2 usu -ve.
  • Desmin +ve (strongly).
  • EMA +ve.
  • Actin -ve.
  • WT-1 (N-terminal) -ve.
  • WT-1 (C-terminal) +ve (nuclear staining).
  • CD57 +ve.
  • Synaptophysin focal, patchy +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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