Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis

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Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis
Diagnosis in short

Micronodule of pneumocyte hyperplasia in multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis. H&E stain.
LM DDx atypical adenomatous hyperplasia of the lung
Site lung
Associated Dx lymphangioleiomyomatosis - also assoc. with tuberous sclerosis
Syndromes tuberous sclerosis
Prevalence rare
Prognosis benign

Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis, also multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis, is the presence of a rare relatively distinctive hamartomatous lesion of the lung in multiple foci in a person with tuberous sclerosis.[1]

General

  • Rare.

Clinical:

Gross

Features:[3]

  • Multiple small lung nodules.
    • Random distribution.
    • Up to 5 mm in size.

Radiology:

  • May have an emphysema-like picture due to the obstruction of lymphatics and alveolar ducts from mass effect.[2]
  • Nodules have ground-glass appearance on CT.[3]

Microscopic

Features:

  • Macrophages within the air spaces.
  • Enlarged alveolar lining cells with:
    • Hobnail morphology - free (luminal) surface area > attached/basal surface area.
    • Round or oval nuclei.

DDx:

Images

  • MMPH - intermed. mag.

  • MMPH - high mag.

IHC

Features:[3]

  • Cytokeratin +ve.
  • Surfactant apoprotein A +ve.
  • Surfactant apoprotein B +ve.

Others:[3]

  • HMB-45 -ve.
  • SMA (alpha) -ve.
  • p53 -ve.

See also

References

  1. ↑ Nagar, AM.; Teh, HS.; Khoo, RN.; Morani, AC.; Vrishni, K.; Raghuram, J. (Feb 2008). "Multifocal pneumocyte hyperplasia in tuberous sclerosis.". Thorax 63 (2): 186. doi:10.1136/thx.2006.076604. PMID 18234663.
  2. ↑ 2.0 2.1 Popper, HH.; Juettner-Smolle, FM.; Pongratz, MG. (Apr 1991). "Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis.". Histopathology 18 (4): 347-54. PMID 2071093.
  3. ↑ 3.0 3.1 3.2 3.3 Kobashi, Y.; Sugiu, T.; Mouri, K.; Irei, T.; Nakata, M.; Oka, M. (Jun 2008). "Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis: differentiation from multiple atypical adenomatous hyperplasia.". Jpn J Clin Oncol 38 (6): 451-4. doi:10.1093/jjco/hyn042. PMID 18535095.
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