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Rapidly enlarging malignant abdominal PEComa with hepatic metastasis: a promising initial response to sirolimus following surgical excision of primary tumor.

Authors
  • Uhlenhopp, Dustin J1
  • West, Jacob1
  • Heckart, Jonathan1
  • Campbell, Ruth1
  • Elhaddad, Abdelaziz2
  • 1 Department of Internal Medicine, MercyOne Des Moines Medical Center, Des Moines, IA 50314, USA.
  • 2 Department of Hematology/Oncology, MercyOne Cancer Center, Des Moines, IA 50314, USA.
Type
Published Article
Journal
Oxford medical case reports
Publication Date
Mar 01, 2020
Volume
2020
Issue
3
Identifiers
DOI: 10.1093/omcr/omaa013
PMID: 32257249
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Intra-abdominal perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Although no effective therapies have been agreed upon, mTOR inhibitors are currently being investigated as a potential therapy for this extremely rare tumor. We present a case of a 64-year-old male found to have a large intra-abdominal PEComa with multiple metastatic lesions in the liver. Patient underwent surgical resection of the primary lesion in the abdomen and sigmoid colon followed by adjuvant therapy with the mTOR inhibitor, sirolimus. Initial response was noted with a decrease in size and number of lesions found in the patient's liver. After 8 months of therapy, restaging imaging showed disease progression in the liver lesions. Patient subsequently failed treatments with pazopanib, investigational therapy TAK-228 (Sapanisertib) and nivolumab and ipilimumab. Overall the patient died after 22 months of disease. PEComas generally follow a benign course. This case is a much rarer entity given the malignant features/outcome. © The Author(s) 2020. Published by Oxford University Press.

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