Work

Pulmonary Lymphangioleiomyomatosis: A Case Report and Literature Review.

Year: 2020

Type: article

Abstract: Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease characterized by diffuse cystic changes caused by a destructive proliferation of smooth muscle-like cells or LAM cells. It is a part of the perivascular epithelioid cell family of tumors. LAM may be associated with the genetic disorder tuberous sclerosis complex or may occur sporadically. Individuals affected by LAM are typically females of child-bearing age who present with recurrent spontaneous pneumothorax. The microscopic findings can be subtle and careful examination is needed to identify the neoplastic cells of LAM. Immunohistochemical markers in cases of LAM demonstrate a characteristic co-expression of myogenic and melanocytic markers. We report a case of a 41-year-old woman who presented with multiple episodes of spontaneous pneumothorax and microscopic findings characteristic of LAM.

Source: PubMed

Authors: Sakda Sathirareuangchai, David Shimizu, Koah Robin Vierkoetter

Institutions: University of Hawaiʻi at Mānoa, University of Hawaii System, Queen's Medical Center

Language: English

Topic: Tuberous Sclerosis Complex Research

Subfield: Genetics (clinical)

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: green

OpenAlex ID: W3042373415

FWCI: 1.608

Cites: 20

Cited by: 15

Related to: 10