Hemangioendotelioma Kapasiforme mediastinal complicado com síndrome de Kasabach-Merrit: relato de caso

• Main Authors: Luanna Ribeiro Campos dos Santos Novais (Author), Alef de Carvalho Vieira (Author), Bruno da Costa Rocha (Author), Erika Patricia Barbosa Correia (Author), Braulio Xavier Pereira Silva-Neto (Author), Breno Soares de Andrade Wanderley (Author), Amanda Gordiano Machado (Author), Mila Simões Alves (Author)
• Format: Book
• Published: Sociedade Brasileira de Pediatria, 2023-03-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 10.25060/residpediatr-2023.v13n1-479; 2236-6814
• Keywords: kasabach-merritt syndrome; cardiac tamponade; hemangioendothelioma; Pediatrics; RJ1-570
• Source: Residência Pediátrica, Vol 13, Iss 1 (2023)

OBJECTIVES: This paper reports the case of a child diagnosed with mediastinal kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt Syndrome at a referral hospital in Salvador, Bahia, Brazil. CASE REPORT: A two-month-old with a history of cyanosis and nasal congestion had an echocardiogram with findings consistent with pericardial effusion. The patient underwent multiple pericardial drainage procedures and was eventually sent to a pediatric referral center in the city of Salvador. Contrast-enhanced computed tomography images showed a mediastinal mass, which was resected. Postoperative pathology revealed the patient had a KHE. He was also suspected with Kasabach-Merritt syndrome due to consumption coagulopathy. Corticoid therapy and chemotherapy with vincristine were performed. The patient progressed well and was discharged and placed on outpatient follow-up, without recurrence of symptoms. COMMENTS: KHE is rare and aggressive, although with a benign histopathology. It can complicate with consumption coagulopathy, characteristically seen in Kasabach-Merritt syndrome.