Loredana Sana1, Pietro Betalli2, Michela Bravi1, Paola Stroppa1, Maurizio Cheli3, Aurelio Sonzogni4, Lisa Licini4, Roberto Agazzi5, Michele Colledan6, Filippo Parolini7, Daniele Alberti7, Lorenzo D'Antiga1. 1. Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII di Bergamo, Piazza OMS1, 24127, Bergamo, Italy. 2. Paediatric Surgery, Hospital Papa Giovanni XXIII, Bergamo, Italy. pbetalli@asst-pg23.it. 3. Paediatric Surgery, Hospital Papa Giovanni XXIII, Bergamo, Italy. 4. Pathology Department, Hospital Papa Giovanni XXIII, Bergamo, Italy. 5. Interventional Radiology, Hospital Papa Giovanni XXIII, Bergamo, Italy. 6. General Surgery and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy. 7. Paediatric Surgery, University of Brescia, Brescia, Italy.
Abstract
PURPOSE: 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. METHODS: We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. RESULTS: 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 < 0.01). Histology showed type 1 lesion in 3/8, type 2 in 3/8, and type 3 in 2/8 with foci of angiosarcoma. CONCLUSION: Most patients with symptomatic HHE can be managed successfully with a combination of medical, radiological and surgical treatments. Patients with diffuse lesions, late presentation, cardiac failure and type II histology have a poor outcome. LEVEL OF EVIDENCE: Diagnostic level IV. Therapeutic level IV.
PURPOSE: 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. METHODS: We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. RESULTS: 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 < 0.01). Histology showed type 1 lesion in 3/8, type 2 in 3/8, and type 3 in 2/8 with foci of angiosarcoma. CONCLUSION: Most patients with symptomatic HHE can be managed successfully with a combination of medical, radiological and surgical treatments. Patients with diffuse lesions, late presentation, cardiac failure and type II histology have a poor outcome. LEVEL OF EVIDENCE: Diagnostic level IV. Therapeutic level IV.
Authors: Ionela Iacobas; Thuy L Phung; Denise M Adams; Cameron C Trenor; Francine Blei; Douglas S Fishman; Adrienne Hammill; Prakash M Masand; Steven J Fishman Journal: J Pediatr Date: 2018-09-21 Impact factor: 4.406