Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 28, 2020; 26(36): 5520-5526
Published online Sep 28, 2020. doi: 10.3748/wjg.v26.i36.5520
SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma mimicking solid pseudopapillary neoplasm: A case report and review of the literature
Yinan Hua, Piyush Soni, Douglas Larsen, Riyam Zreik, Bing Leng, Debby Rampisela
Yinan Hua, Piyush Soni, Douglas Larsen, Riyam Zreik, Bing Leng, Debby Rampisela, Department of Pathology, Baylor Scott & White Health, Texas A&M University College of Medicine, Temple, TX 76508, United States
Author contributions: All authors contributed to the writing and revising of the manuscript.
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Conflict-of-interest statement: No conflict of interest.
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Corresponding author: Debby Rampisela, MD, Attending Doctor, Department of Pathology, Baylor Scott & White Health, Texas A&M University College of Medicine, 2401 S. 31st Street, MS-01-266, Temple, TX 76508, United States. debby.rampisela@bswhealth.org
Received: June 10, 2020
Peer-review started: June 11, 2020
First decision: June 18, 2020
Revised: June 29, 2020
Accepted: September 5, 2020
Article in press: September 5, 2020
Published online: September 28, 2020
Processing time: 105 Days and 16.5 Hours
Abstract
BACKGROUND

SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a very aggressive tumor that is rarely reported in the literature. The tumor has a predominant rhabdoid cell component and different patterns of growth have been reported.

CASE SUMMARY

A 59-year-old woman presented with diffuse abdominal pain, increasing in severity and accompanied by weight loss, nausea, and vomiting. Imaging showed a pancreatic head mass. Fine needle aspiration demonstrated atypical epithelioid cells with a pseudopapillary growth pattern suggestive of solid pseudopapillary neoplasm. The excised neoplasm showed monotonous epithelioid and focally spindle cells with pseudopapillary structures, rhabdoid features, and loss of SMARCB1 protein expression with wild-type KRAS, consistent with a SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma. The patient’s condition deteriorated rapidly following surgery and she expired 3 mo post operation.

CONCLUSION

In this article, we report the first case of SMARCB1/INI1-deficient undifferentiated pancreatic rhabdoid carcinoma mimicking solid pseudopapillary neoplasm.

Keywords: Pancreatic undifferentiated rhabdoid carcinoma; Solid pseudopapillary neoplasm; SMARCB1/INI1; KRAS; Case report

Core Tip: The presence of a pseudopapillary architectural pattern in pancreatic tumors sampled via fine needle aspiration commonly leads to solid pseudopapillary neoplasm as the primary diagnostic consideration, particularly in middle-aged women; however, an inconclusive immunohistochemistry profile may suggest an alternative diagnosis. Our case highlights that while SMARCB1/INI1-deficient pancreatic undifferentiated carcinomas may mimic solid pseudopapillary neoplasm, recognition of this aggressive malignancy is important as these rare tumors impart a dismal prognosis.