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Malignant Pigmented Mass “Sequestrated” in the Lung: A Unique Case Report


Primary lung tumors arising in pulmonary sequestration is an exceptional event, usually consisting of common histologic types. On the other hand, malignant perivascular epithelioid cell (PEComatous) tumors with deposition of melanin pigment have never been reported in the lung so far. In this study, we report a challenging case of a 34-year-old man presented with recurrent hemoptysis and CT scan detection of a pulmonary mass at the left lower lobe, vascularized by aberrant communication with the left diaphragmatic artery. After surgical resection, we documented a malignant PEComatous tumor (characterized by TFE3 expression and high mitotic rate) that had arisen in the context of an extralobar sequestration.

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Author Contribution

All authors contribute to the diagnosis of the patients and wrote the report. Written consent to publication was obtained.

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Correspondence to Filippo Lococo.

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Mengoli, M.C., Lococo, F., Depenni, R. et al. Malignant Pigmented Mass “Sequestrated” in the Lung: A Unique Case Report. Lung 194, 699–701 (2016).

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  • PEComa
  • Sequestration
  • Lung
  • Immunohistochemistry
  • TFE3