Malignant PEComa: a case report with emphasis on clinical and morphological criteria

Abstract

BackgroundMalignant perivascular epitheliod cell tumor (PEComa) is a very rare entity composed of distinctive perivascular epitheliod cells with variable immunoreactivity for melanocytic and muscle markers. At present this neoplasm does not have a known normal cellular counterpart and the natural history is often unpredictable. Up to now, few cases of PEComa have been described and treatment modalities are still controversial, particularly in advanced conditions.Case presentationWe handled the case of a 42-year-old man with unresectable PEComa of the abdomen. A 7 cm hepatic hypodense lesion between segment V and VIII of the liver and diffuse intraperitoneal nodules of 0,3-3,5 cm along the right subcapsular hepatic region, were documented by a CT scan. Radiological images showed abnormal lymph nodes of the right internal mammary chain and anterior mediastinum. The patient underwent an explorative laparotomy for uncontrolled intrabdominal hemorrhage without a well-defined preoperative tumor diagnosis. At surgery, multiple lobulated nodules containing hemorrhagic fluid on the liver surface, peritoneum and omentum were confirmed. The procedure had a palliative intent and consisted of hemostasis, hematomas evacuation and omentectomy. The diagnosis of PEComa was made after surgery on the basis of morphological and immunohystochemical criteria. Radiological and intra operative findings suggest that the mass has an hepatic origin with diffuse involvement of hepatic capsule and suspensory ligaments. The patient received medical support care with blood and plasma transfusions. In our experience, PEComa was clinically malignant, leading to a fatal outcome 25 days after hospital admission of patient.ConclusionsHere we report and discuss the peculiar clinical, radiological and morphological presentation of unresectable PEComa. Although in the majority of the reported series, PEComas show a more better prognosis, our case presents with a particular aggressive biological behaviour. The importance of a correct preoperative diagnosis, the need for more effective targeted therapies based on tumor molecular knowledge and evidence-based clinical studies are emphasized together with a revision of the concerning scientific literature.

Primary malignant gastric PEComa – Diagnostic and technical dilemmas

“…A recent case by Slevaggi et al reported 25 day mortality in a 42 year old male with a malignant PEComa which was hepatic in origin. 7 This has reflected our own experience in the case outlined here with our patient not surviving greater than 3 months since diagnosis.…”

Hepatic PEComa: a potential pitfall in the evaluation of hepatic neoplasms

“…They are primarily benign tumours, but they may rarely behave malignantly 12. Clinical manifestations of PEComa in general, including hepatic PEComa, are uncommon.…”

Angiomyolipoma of the kidney: Clinicopathological and immunohistochemical study

“…A second hypothesis is that PEC has a myoblastic, smooth muscle origin with a molecular alteration that leads to the expression of melanogenesis and melanocytic markers; a third hypothesis is that PEC has a pericytic origin [7,30].…”