1. Hepatic hemangioendothelioma of infancy: clinical features of a large cohort of patients and proposed management
- Author
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Roberto Agazzi, Daniele Alberti, Paola Stroppa, Filippo Parolini, Aurelio Sonzogni, Maurizio Cheli, M. Bravi, Loredana Sana, Lorenzo D'Antiga, Lisa Licini, Pietro Betalli, Michele Colledan, Sana, L, Betalli, P, Bravi, M, Stroppa, P, Cheli, M, Sonzogni, A, Licini, L, Agazzi, R, Colledan, M, Parolini, F, Alberti, D, and D'Antiga, L
- Subjects
Male ,medicine.medical_specialty ,Skin Neoplasms ,Time Factors ,Anemia ,medicine.medical_treatment ,Hemangioendothelioma ,Hemangioma ,Lesion ,Angiosarcoma ,medicine ,Humans ,Embolization ,Children ,Tumor ,business.industry ,Infant ,General Medicine ,medicine.disease ,Embolization, Therapeutic ,Transplantation ,Liver ,Pediatrics, Perinatology and Child Health ,Vomiting ,Female ,Surgery ,Radiology ,medicine.symptom ,business ,Follow-Up Studies - Abstract
The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years. We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. Twenty seven patients (male/female 5/22), median age 13 days (1–1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months’ follow-up (30 days–11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p
- Published
- 2021
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