Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms

• Published in: Clinical imaging 2015-05, Vol.39 (3), p.529-532
• Main Authors: Iacobas, Ionela, Simon, Mitchell L, Amir, Tali, Gribbin, Christopher E, McPartland, Thomas G, Kaufman, Matthew R, Calderwood, Stanley, Nosher, John L
• Format: Article
• Language: English
• Subjects: Age; Antibiotics, Antineoplastic - therapeutic use; Blood platelets; Child, Preschool; De-vascularization; Diagnosis; Disabilities; Hemangioendothelioma - blood supply; Hemangioendothelioma - diagnosis; Hemangioendothelioma - drug therapy; Humans; Imaging; Kaposiform hemangioendothelioma; Kasabach-Merritt Syndrome - blood supply; Kasabach-Merritt Syndrome - diagnosis; Kasabach-Merritt Syndrome - drug therapy; Male; Monitoring; Mortality; Multimodal Imaging; Neovascularization, Pathologic; NMR; Nuclear magnetic resonance; Pain; Prognosis; Quality of life; Radiology; Retroperitoneal; Retroperitoneal Neoplasms - blood supply; Retroperitoneal Neoplasms - diagnosis; Retroperitoneal Neoplasms - drug therapy; Sarcoma, Kaposi - blood supply; Sarcoma, Kaposi - diagnosis; Sarcoma, Kaposi - drug therapy; Sirolimus - therapeutic use; Sirolimus/Rapamycin; Therapy; Tumors; Vascular tumor; Veins & arteries
• ISSN: 0899-7071; 1873-4499
• DOI: 10.1016/j.clinimag.2015.01.003

Abstract Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement.