Difference between revisions of "Desmoplastic small round cell tumour"
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| Micro = small (round) cells in nests with an undulating sharp border, road bands of paucicellular fibrous stroma | | Micro = small (round) cells in nests with an undulating sharp border, road bands of paucicellular fibrous stroma | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[ | | LMDDx = solid variant of [[alveolar rhabdomyosarcoma]], [[embryonal rhabdomyosarcoma]], other [[small round cell tumours]] | ||
| Stains = | | Stains = | ||
| IHC = AE1/AE3 +ve, desmin +ve, EMA +ve, actin -ve, WT1 (N-terminal) -ve, WT1 (C-terminal) +ve, CD57 +ve. | | IHC = AE1/AE3 +ve, desmin +ve, EMA +ve, actin -ve, WT1 (N-terminal) -ve, WT1 (C-terminal) +ve, CD57 +ve. | ||
| Line 35: | Line 35: | ||
*Typically retroperitoneal. | *Typically retroperitoneal. | ||
*Poor prognosis. | *Poor prognosis. | ||
==Clinics== | |||
*Rare cases in the CNS reported. | |||
==Microscopic== | ==Microscopic== | ||
| Line 52: | Line 55: | ||
*Solid variant of [[alveolar RMS]]. | *Solid variant of [[alveolar RMS]]. | ||
**Nests in alveolar RMS have round edges. | **Nests in alveolar RMS have round edges. | ||
*Other [[small round cell tumours]]. | |||
===Images=== | ===Images=== | ||
| Line 62: | Line 66: | ||
==IHC== | ==IHC== | ||
Features: | Features: | ||
*AE1/AE3 +ve. | *[[AE1/AE3]] +ve. | ||
*Desmin +ve. | *CAM5.2 usu -ve. | ||
*EMA +ve. | *Desmin +ve (strongly). | ||
*[[EMA]] +ve. | |||
*Actin -ve. | *Actin -ve. | ||
* | *[[WT-1]] (N-terminal) -ve. | ||
* | *[[WT-1]] (C-terminal) +ve (nuclear staining). | ||
*CD57 +ve. | *CD57 +ve. | ||
*[[Synaptophysin]] focal, patchy +ve. | |||
==Molecular== | ==Molecular== | ||
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==See also== | ==See also== | ||
*[[Soft tissue lesions]]. | *[[Soft tissue lesions]]. | ||
*[[Small round cell tumours]]. | |||
*[[Stromal desmoplasia]]. | |||
==References== | ==References== | ||
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[[Category:Soft tissue lesions]] | [[Category:Soft tissue lesions]] | ||
[[Category:Neuropathology]] | |||
[[Category:Diagnosis]] | [[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]
- 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
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
- ↑ 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.
- ↑ 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.
- ↑ 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.