An autopsy case of systemic hemangiomatosis with honeycomb-like liver and focal splenic sarcomatoid changes

Intern Med. 1998 Oct;37(10):847-52. doi: 10.2169/internalmedicine.37.847.

Abstract

An unusual autopsy case of systemic hemangiomatosis associated with honeycomb-like liver and splenic sarcomatoid changes is presented. The patient suffered from high-output ventricular heart failure secondary to hepatic arteriovenous shunting with hepatic failure, ending fatally due to disseminated intravascular coagulation and pulmonary bleeding. Postmortem examination revealed the characteristic distribution of vasoformative tumors presenting in the hematopoietic system including the liver, bone marrow, and spleen, with the liver appearing characteristically honeycomb-like. While these vasoformative tumors were mostly benign-appearing, partial shifts toward sarcomatoid change were observed in the splenic lesions.

Publication types

  • Case Reports

MeSH terms

  • Angiomatosis / complications
  • Angiomatosis / pathology*
  • Arteriovenous Malformations / pathology
  • Bone Neoplasms / pathology
  • Cell Transformation, Neoplastic / pathology
  • Disseminated Intravascular Coagulation / etiology
  • Dyspnea / etiology
  • Edema / etiology
  • Fatal Outcome
  • Heart Failure / etiology
  • Hemangioma / complications
  • Hemangioma / pathology*
  • Hemangioma, Cavernous / complications
  • Hemangioma, Cavernous / pathology
  • Hemangiosarcoma / pathology
  • Humans
  • Liver / blood supply
  • Liver / injuries
  • Liver Diseases / complications
  • Liver Diseases / pathology*
  • Liver Failure / etiology
  • Liver Neoplasms / complications
  • Liver Neoplasms / pathology*
  • Lumbar Vertebrae / pathology
  • Male
  • Middle Aged
  • Neoplasms, Multiple Primary / complications
  • Neoplasms, Multiple Primary / pathology*
  • Ribs / pathology
  • Spinal Neoplasms / pathology
  • Spleen / injuries
  • Spleen / pathology
  • Splenic Neoplasms / complications
  • Splenic Neoplasms / pathology*
  • Thoracic Vertebrae / pathology