Pulmonary lymphangioleiomyomatosis associated with aggressive renal angiomyolipoma

Allison Cooper, Laura Baugh, Shannon Kelley, Howard Huang, Joseph Guileyardo

Research output: Contribution to journalArticlepeer-review

Abstract

Lymphangioleiomyomatosis (LAM) is a rare cystic pulmonary disease that may occur in association with mutations in the tuberous sclerosis genes or arise sporadically. The histologic hallmark of the disease is the “LAM” cell, a spindled to epithelioid smooth muscle-like cell that bears morphologic and immunohistochemical resemblance to the perivascular epithelioid cell tumors (PEComas). The origin of the “LAM” cell is unknown; emerging theories suggest that a member of the PEComa family, the renal angiomyolipoma, may be the primary source and that both LAM and angiomyolipomas are associated with the genetic syndrome tuberous sclerosis. We present a young woman with LAM with an aggressive renal angiomyolipoma confirmed at autopsy.

Original languageEnglish (US)
Pages (from-to)81-83
Number of pages3
JournalBaylor University Medical Center Proceedings
Volume31
Issue number1
DOIs
StatePublished - 2018
Externally publishedYes

Keywords

  • Angiomyolipoma
  • Lymphangioleiomyomatosis
  • Perivascular epithelioid cell tumor
  • Tuberous sclerosis

ASJC Scopus subject areas

  • Medicine(all)

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