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Malignant perivascular epitheloid cell tumour (PEComa) of the lung - a rare entity.

ABSTRACT: Malignant Perivascular Epitheloid Cell Tumour (PEComa) of the lung is very rare, with only six cases reported in literature. This case presented with a large mass originating from right upper lobe of the lung with dilemma in its histopathological diagnosis and management. Postoperative histopathology after a right upper and middle lobectomy describes a tumour with an alveolar/nested pattern of growth and epitheloid morphology with expression of TFE-3 and diagnosed as PEComa. After 6 months the patient had a local recurrence inside the thorax & chest wall.This case qualifies it as a rare type of malignant PEComa with younger age of presentation, aggressive clinical behaviour & malignant histological features along with TFE3 positivity on immunohistochemistry. This case is probably the first of its kind with the largest reported size involving two lobes of the lung.

SUBMITTER: Chakrabarti A

PROVIDER: S-EPMC6754007 | biostudies-other | 2017 Mar

REPOSITORIES: biostudies-other

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Malignant perivascular epitheloid cell tumour (PEComa) of the lung - a rare entity.

Chakrabarti Amitabha A Bandyopadhyay Manujesh M Purkayastha Biswarup B

Innovative surgical sciences 20170223 1

Malignant Perivascular Epitheloid Cell Tumour (PEComa) of the lung is very rare, with only six cases reported in literature. This case presented with a large mass originating from right upper lobe of the lung with dilemma in its histopathological diagnosis and management. Postoperative histopathology after a right upper and middle lobectomy describes a tumour with an alveolar/nested pattern of growth and epitheloid morphology with expression of TFE-3 and diagnosed as PEComa. After 6 months the p ...[more]

PMID: 31579733

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