title: 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 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.
authors: Drexel University College of Medicine, Pediatric Hematology-Oncology, Saint Peter's University Hospital, 254 Easton Avenue, New Brunswick, NJ 08901. Electronic address: ixiacoba@txch.org.
authors: Ionela
authors: I
authors: Iacobas
authors: UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903-0019. Electronic address: msimon@univrad.com.
authors: Mitchell L
authors: ML
authors: Simon
authors: UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903-0019. Electronic address: amirta@umdnj.edu.
authors: Tali
authors: T
authors: Amir
authors: UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903-0019. Electronic address: cgribbin@univrad.com.
authors: Christopher E
authors: CE
authors: Gribbin
authors: Saint Peter's University Hospital, Pediatric Orthopedic Surgery, 254 Easton Avenue, New Brunswick, NJ 08901. Electronic address: tmcpartland@saintpetersuh.com.
authors: Thomas G
authors: TG
authors: McPartland
authors: Drexel University College of Medicine, Saint Peter's University Hospital, Department of Plastic Surgery, 254 Easton Avenue, New Brunswick, NJ 08901. Electronic address: matthewrkmd@gmail.com.
authors: Matthew R
authors: MR
authors: Kaufman
authors: Drexel University College of Medicine, Pediatric Hematology-Oncology, Saint Peter's University Hospital, 254 Easton Avenue, New Brunswick, NJ 08901. Electronic address: scalderwood@saintpetersuh.com.
authors: Stanley
authors: S
authors: Calderwood
authors: UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903-0019. Electronic address: nosher@umdnj.edu.
authors: John L
authors: JL
authors: Nosher
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D002675:Child, Preschool; D006390:Hemangioendothelioma / Q000098:blood supply* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D059885:Kasabach-Merritt Syndrome / Q000098:blood supply* / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D064847:Multimodal Imaging; D009389:Neovascularization, Pathologic; D012186:Retroperitoneal Neoplasms / Q000098:blood supply* / Q000175:diagnosis / Q000188:drug therapy*; D012514:Sarcoma, Kaposi / Q000098:blood supply* / Q000175:diagnosis / Q000188:drug therapy*; D020123:Sirolimus / Q000627:therapeutic use*
chemical_list: D000903:Antibiotics, Antineoplastic; D020123:Sirolimus
keywords: De-vascularization; Kaposiform hemangioendothelioma; Retroperitoneal; Sirolimus/Rapamycin; Vascular tumor