Localized pleuropulmonary crystal-storing histiocytosis: 5 cases of a rare histiocytic disorder with variable clinicoradiologic features

Am J Surg Pathol. 2013 Jun;37(6):906-12. doi: 10.1097/PAS.0b013e31827b1618.

Abstract

Crystal-storing histiocytosis (CSH) localized to the thoracic region is a rare occurrence, often secondary to lymphoproliferative or plasma cell diseases. About 10 case reports have been previously published, and 3 of these have no relationship with clonal hematologic disorders. We collected here the first series of 5 consecutive cases of CSH involving lungs (4 cases) and pleura (1 case). There were 3 women and 2 men with a mean age at diagnosis of 65 years. All cases had an underlying hematologic disorder (2 B-cell marginal-zone lymphomas, 2 monoclonal gammopathy of undetermined significance and 1 pulmonary plasmacytoma). Despite a common morphology characterized by a dense and irregular growth of large eosinophilic histiocytes with intracytoplasmic refractile crystals, 2 cases presented with cystic changes at gross and imaging examinations, calcified amyloid was found in 2 cases, and 1 case showed an interstitial lung disease with nonspecific interstitial pneumonia pattern. Histiocytes were immunoreactive for CD68 (clones PGM-1 and KP-1) but were not for CD1a and S100; the associated lymphoplasmacellular disorder had a clonal profile on molecular analysis with κ light-chain restriction. Two cases were originally misdiagnosed as cystic fibrohistiocytic tumor and carcinoid tumor, thus confirming that CSH localized to this site may result in a diagnostic challenge with a broad spectrum of differential diagnoses. The presence of intracytoplasmic crystals and a plasma cell infiltrate around a histiocytic proliferation should alert the pathologist to consider CSH and to carefully investigate the presence of clonal hematologic disease.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Female
  • Histiocytosis / complications
  • Histiocytosis / diagnostic imaging
  • Histiocytosis / pathology*
  • Humans
  • Lung Diseases / complications
  • Lung Diseases / diagnostic imaging
  • Lung Diseases / pathology*
  • Lymphoma, B-Cell, Marginal Zone / complications
  • Male
  • Middle Aged
  • Paraproteinemias / complications
  • Plasmacytoma / complications
  • Pleural Diseases / complications
  • Pleural Diseases / diagnostic imaging
  • Pleural Diseases / pathology*
  • Radiography