Dados do Trabalho

Título

METASTATIC PSEUDOMYOGENIC HEMANGIOENDOTHELIOMA: A CASE REPORT

Apresentação do caso

A 26-years-old female patient, with a medical history of acute lymphocytic leukemia
treated in early childhood, was diagnosed with a metastatic pseudomyogenic
hemangioendothelioma. The immunohistochemistry corroborated the diagnosis, with
positivity for: ERG, INI 1, CD31, AE1/AE3. At the time of diagnosis, the patient already
presented metastatic bone lesions in the hip and thoracic spine. She was treated in another
service with Denosumab for 5 months, and showed symptoms of clinical worsening.
Progressive disease was confirmed by findings of diffuse uptake in the axial skeleton by
18F-FDG PET. She underwent first-line systemic treatment with gemcitabine and docetaxel
day 1 and 8 every-3-weeks for 6 cycles. The patient tolerated the treatment well and
currently has stable disease on new imaging tests.

Discussão

Pseudomyogenic hemangioendothelioma is a very rare entity, with few case reports in the
literature. This disease rarely metastasizes, presenting itself as a low-grade vascular tumor
that can histologically mimic epithelioid sarcoma and often presents as multiple
discontinuous nodules in the extremities. The immunohistochemical characteristics
consistently show immunoreactivity for cytokeratins and endothelial markers.

Comentários finais

This case shows an exceedingly rare disease with an aggressively atypical presentation and
exceptionally good response to chemotherapy with gemcitabine and docetaxel.

Palavras-chave

PSEUDOMYOGENIC; HEMANGIOENDOTHELIOMA; METASTATIC