Thoracic Epithelioid Hemangioendothelioma: clinical demonstration and therapeutic procedures

Port J Card Thorac Vasc Surg. 2022 Jul 3;29(2):39-44. doi: 10.48729/pjctvs.276.


Epithelioid hemangioendotheliomais a low to intermediate grade malignant vascular tumors that can involve any organ. About 60-80%of patients are women, patient ages range 7 to 81 years, with a median age of 38 years. Four cases of thoracic epithelioid hemangioendotheliomas with different clinical presentation and disease progression are reported. Cases 1 and 2 are pulmonary epithelioid hemangioendotheliomas diagnosed at different advanced stages and patients died after 6 and 2,5 months of medical treatment, respectively. Case 3 corresponds to pleural epithelioid hemangioendothelioma, submit- ted to left lung decortication and pleuro-pericardial window; patient is free either from symptoms and radiographic manifestations for 10 months of follow-up. Case 4, of mediastinal epithelioid hemangioendothelioma, represented by a mass in the upper left mediastinum adherent to the aortic arch; patient underwent block excision of the mass followed by chemotherapy; subsequent recurrence 41 months later and the patient died 8 months after. The reported 4 cases reveal the heterogeneous clinical presentation of epithelioid hemangioendotheliomas with behavior in between benign and high-grade tumors, raising difficulty in either differentiating from other vascular tumors and previewing clinical outcome.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Bone Neoplasms* / pathology
  • Child
  • Female
  • Hemangioendothelioma, Epithelioid* / diagnosis
  • Humans
  • Male
  • Mediastinal Neoplasms* / diagnosis
  • Middle Aged
  • Sarcoma*
  • Vascular Neoplasms*
  • Young Adult