Indian Journal of Pathology and Microbiology (Jan 2018)

Sporadic lymphangioleiomyomatosis with multiple atypical features: A case report and literature review

  • Xiaokang Wang,
  • Fei Su,
  • Fenfang Zhou,
  • Maohui Feng

DOI
https://doi.org/10.4103/ijpm.ijpm_843_16
Journal volume & issue
Vol. 61, no. 1
pp. 94 – 97

Abstract

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Lymphangioleiomyomatosis (LAM) is a rare, genetically determined, progressive interstitial lung disease, which almost exclusively affects women, especially at the childbearing age. The initial symptoms and radiographic changes in a patient with LAM are always associated with the respiratory system. Here, we present a case of mediastinal and abdominal LAM of a 22-year-old male, where LAM cells are negative for human melanoma black-45 ( HMB-45). The report of this uncharacterized LAM case will make a significant contribution to the realization of LAM associated clinical features, diagnostic approaches, and its afterward treatments.

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