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

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 the...

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Bibliographic Details
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
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Description
Summary: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.
ISSN:0899-7071
1873-4499
DOI:10.1016/j.clinimag.2015.01.003