A rare case of perivascular epithelioid cell tumour metastases to the brain

Abstract

Perivascular epithelioid cell tumour is a rare mesenchymal tumour with distinct immunohistochemical profile. While it is known to occur in various anatomical sites, the central nervous system had always been a protected site for primary or secondary perivascular epithelioid cell tumours. We describe a 61-year-old lady who presented with symptoms of raised intracranial pressure, 3 months after the resection of duodenal and thoracic tumours which were histologically consistent with perivascular epithelioid cell tumour. She was investigated and then subsequently subjected to resection of two metastatic intracranial lesions. The radiological, intraoperative as well as histopathological findings of the metastatic lesions are discussed. Metastatic perivascular epithelioid cell tumour of the brain is extremely rare. However, patients who are stratified as high risk for recurrence or metastases should undergo an early magnetic resonance imaging/computed tomography of the brain in addition to a whole-body positron emission tomography scan, to allow for early detection and management of these tumours.

Keywords: Perivascular epithelioid cell tumour; brain; metastasis.

Figure 1.

Contrast-enhanced CT of the brain: right cerebellar mass (left) and left posterior temporal mass (right) showing a good contrast uptake.

Figure 2.

Post-gadolinium contrasted MRI images showing a distinct right cerebellar lesion with central necrosis (black arrow). The left temporal lesion is entirely solid, and the vein of Labbe is seen traversing along the tumour (white arrow).

Figure 3.

(a) Tumour cells clustering around a blood vessel: haematoxylin and eosin (original magnification: 10× objective), (b) spindle and epithelioid tumour cells with increased mitotic activity (red arrow): haematoxylin and eosin (original magnification: 20× objective).

Figure 4.

(a) Tumour cells showing positivity for HMB45 (original magnification: 10× objective), (b) smooth muscle actin; SMA (original magnification: 20× objective) and (c) high Ki67 proliferative index (original magnification: 10× objective).