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Micronodular pneumocyte hyperplasia

T E Muir1, K O Leslie, H Popper

  • 1Department of Pathology, Mayo Clinic, Rochester, Minnesota, USA.

The American Journal of Surgical Pathology
|April 16, 1998
PubMed
Summary
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Micronodular pneumocyte hyperplasia (MNPH) is a rare lung condition often linked to tuberous sclerosis complex (TSC). This study shows MNPH is a distinct hamartoma, not a LAM manifestation, and can occur in men without TSC.

Area of Science:

  • Pulmonary Pathology
  • Oncology
  • Genetics

Background:

  • Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder causing hamartomas.
  • Pulmonary lymphangioleiomyomatosis (LAM) affects 1% of TSC patients (all female).
  • Micronodular pneumocyte hyperplasia (MNPH) is a rare pulmonary manifestation of TSC.

Purpose of the Study:

  • To characterize the clinicopathologic features of MNPH.
  • To determine the relationship between MNPH, TSC, and LAM.
  • To assess the malignant potential of MNPH.

Main Methods:

  • Retrospective analysis of 14 patients with MNPH.
  • Histopathological examination including immunoperoxidase studies.
  • Clinical and follow-up data review.

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Main Results:

  • 14 patients (12 female, 2 male) aged 23-57 years with MNPH.
  • Nine patients had TSC (seven with LAM, two without).
  • Five patients without TSC (three with LAM, two without).
  • MNPH presented as multiple, small nodules of benign type II pneumocytes.
  • Immunoperoxidase studies showed reactivity for cytokeratin, EMA, and surfactant apoprotein B; HMB-45 was negative.
  • Follow-up in 9 patients showed stability or pneumothoraces related to LAM; no deaths attributed to MNPH.

Conclusions:

  • MNPH is a distinct hamartomatous proliferation, separate from LAM.
  • MNPH occurs more frequently in TSC patients but can be seen in non-TSC individuals, including men.
  • MNPH appears to have no malignant potential.