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.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Price includes VAT (USA)
Tax calculation will be finalised during checkout.
Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R (1979) Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34:96–101
Belchis D, Cowan M, Mortman K, Rezvani B (2014) Adenocarcinoma arising in an extralobar sequestration: a case report and review of the literature. Lung Cancer 84:92–95
Thway K, Fisher C (2015) PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol 19:359–368
Malinowska I, Kwiatkowski DJ, Weiss S et al (2012) Perivascular epithelioid cell tumors (PEComas) harboring TFE3 gene rearrangements lack the TSC2 alterations characteristic of conventional PEComas: further evidence for a biological distinction. Am J Surg Pathol 36:783–784
Marine LM, Valdes FE, Mertens RM, Bergoeing MR, Kramer A (2011) Endovascular treatment of symptomatic pulmonary sequestration. Ann Vasc Surg 25(5):696.e11–696.e15. doi:10.1016/j.avsg.2010.08.012
Leoncini G, Rossi UG, Ferro C, Chessa L (2011) Endovascular treatment of pulmonary sequestration in adults using Amplatzer® vascular plugs. Interact Cardiovasc Thorac Surg 12(1):98–100. doi:10.1510/icvts.2010.246546 (Epub 2010 Oct 12)
All authors contribute to the diagnosis of the patients and wrote the report. Written consent to publication was obtained.
Conflict of interest
About this article
Cite this article
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). https://doi.org/10.1007/s00408-016-9897-9