Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2014 May 21:2014:bcr2014204232.
doi: 10.1136/bcr-2014-204232.

Suspected hydatid cyst of liver harbouring an aggressive desmoplastic small round cell tumour

Feroz Alam  1 , Kiran Alam  1 , Shagufta Qadri  1 , Wasif Mohd Ali  2
Affiliations
Case Reports

Suspected hydatid cyst of liver harbouring an aggressive desmoplastic small round cell tumour

Feroz Alam et al. BMJ Case Rep. .

Abstract

Among the group of small round cell tumours, there is a distinct and rare tumour known as desmoplastic small round cell tumour (DSRCT). DSRCT presents as multiple, widespread masses in the abdomen and pelvis and may be accompanied by extensive tumour implants throughout the peritoneum as the tumour is known to spread diffusely along serosal surfaces. We discuss a case of DSRCT in a 16-year-old boy who presented with abdominal pain since 2 years, a non-tender mass was palpable on the right upper quadrant of the abdomen, ultrasonographic and CT findings suggested hydatid cyst of liver. Laparotomy revealed multiple small peritoneal deposits along with a single mass in the liver. On histopathology, the lesion was found to be neoplastic and composed of predominantly clusters of small round blue cells, in a desmoplastic stroma; tumour cells were diffusely positive for cytokeratin, vimentin and neuron-specific enolase, thus confirming the diagnosis of DSRCT.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Contrast-enhanced CT scan showing involvement of the segment 2 of the left lobe of the liver.
Figure 2
Figure 2
(A) Small round tumour cells in a desmoplastic stroma, few cords of hepatocytes are also seen (H&E, ×100). (B) Clusters of small round blue tumour cell with scant cytoplasm surrounded by desmoplastic stroma (H&E, ×400).
Figure 3
Figure 3
Pan-cytokeratin positivity by tumor cells (pan-cytokeratin, ×100).
Figure 4
Figure 4
Vimentin positivity by tumour cells (vimentin, ×100).
Figure 5
Figure 5
Neuron-specific enolase (NSE) positivity by tumour cells (NSE, ×100).
See this image and copyright information in PMC

References

    1. Sesterhenn I, Davis CJ, Mostofi FK. Undifferentiated malignant epithelial tumors involving serosal surfaces of escrotum and abdomen in young males. J Urol 1987;137:214A
    1. Wong HH, Hatcher HM, Benson C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res 2013;3:14. - PMC - PubMed
    1. La Quaglia MP, Brennan MF. The clinical approach to desmoplastic small round cell tumor. Surg Oncol 2000;9:77–81 - PubMed
    1. Schmidt D, Koster E, Harms D. Intraabdominal desmoplastic small-cell tumor with divergent differentiation: clinicopathological findings and DNA ploidy. Med Pediatr Oncol 1994;22:97–102 - PubMed
    1. Gerald WL, Rosai J, Ladanyi M. Characterization of genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor. Proc Natl Acad Sci USA 1995:92:1028–32 - PMC - PubMed

Publication types