title: Malignant Pigmented Mass "Sequestrated" in the Lung: A Unique Case Report.
abstract: 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.
authors.affiliation: Pathologic Anatomy, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
authors.forename: Maria Cecilia
authors.initials: MC
authors.lastname: Mengoli
authors.affiliation: Unit of Thoracic Surgery, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy. filippo_lococo@yahoo.it.
authors.forename: Filippo
authors.initials: F
authors.lastname: Lococo
authors.affiliation: Oncology Unit, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Depenni
authors.affiliation: Thoracic Surgery, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
authors.forename: Uliano
authors.initials: U
authors.lastname: Morandi
authors.affiliation: Pathologic Anatomy, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
authors.forename: Giulio
authors.initials: G
authors.lastname: Rossi
mesh_terms: D000328:Adult; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000378:metabolism; D001998:Bronchopulmonary Sequestration / Q000150:complications; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008940:Mitotic Index; D054973:Perivascular Epithelioid Cell Neoplasms / Q000150:complications / Q000378:metabolism / Q000473:pathology*
chemical_list: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; C069203:TFE3 protein, human
keywords: Immunohistochemistry; Lung; PEComa; Sequestration; TFE3