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

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.

Single-center Experience in the Diagnosis and Treatment of Hepatic Perivascular Epithelioid Cell Neoplasm

“…Furthermore, postoperative complications or recurrence have rarely been reported. 21 In our study, all patients underwent surgical resection, including laparotomy and laparoscopic partial hepatectomy. Two patients had received TACE before the surgery.…”

Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review

“…Review of an article by Chen et al, 3 and a PubMed search of articles published up to July 2020, using the following search terms: ("perivascular epithelioid cell tumor" or "PEComa") and ("gastrointestinal tract" or "GI" or "oral " or "mouth" or "esophagus" or "gullet" or "gastric" or "stomach" or "duodenum" or "jejunum" or "ileum" or "cecum" or "colon" or "colorectal" or "sigmoid" or "rectum" or "anus" or "mesentery"), revealed 62 cases of gastrointestinal PEComa-NOS in the literature. 3,12,[15][16][17][18][19][20][21][22][23] the colon was the most commonly affected part of the abdominal cavity, followed by the mesentery, rectum, stomach, duodenum, ileum, cecum, and other locations. 3 The GI tract is the second most frequent location of PEComa, preceded only by the gynaecological tract.…”