title: Response to Paclitaxel in an Adult Patient with Advanced Kaposiform Hemangioendothelioma.
abstract: BACKGROUND
  Kaposiform hemangioendothelioma (KHE) is a rare neoplasm of vascular origin that typically arises from the skin or soft tissues as a solitary tumor. The optimal therapy for this disease is still unknown. We report the case of an adult patient presenting with metastatic KHE of the spleen, who had a partial response after treatment with paclitaxel.
  
  
  METHODS
  A 36-year-old man presented in November 2012 with a nontraumatic rupture of the spleen. A splenectomy was performed, and the pathology was consistent with a nonspecific vascular proliferation. Follow-up scans revealed lytic bone lesions and liver metastasis. A biopsy of the liver was performed and confirmed KHE. The decision was made to proceed with treatment with gemcitabine and docetaxel, which was discontinued due to myelotoxicity. The patient was then transferred to our institution, and a pathology review supported the diagnosis of metastatic KHE. His disease remained stable until February 2014, when he developed progression in the liver. Chemotherapy was restarted with paclitaxel, and a partial response was documented after 3 cycles. Unfortunately, disease progression occurred after 24 weeks, and subsequent treatments included prednisone, doxorubicin, interferon-α, gemcitabine, and ifosfamide, without any response. The patient developed Kasabach-Merritt phenomenon and passed away 1 week later due to a major gastrointestinal bleeding.
  
  
  CONCLUSIONS
  This case report suggests that paclitaxel could be considered as a treatment option for advanced KHE, a rare condition for which no standard treatment exists.
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: José Maurício
authors.initials: JM
authors.lastname: Mota
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Mariana
authors.initials: M
authors.lastname: Scaranti
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Leonardo G
authors.initials: LG
authors.lastname: Fonseca
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Diego Araújo
authors.initials: DA
authors.lastname: Tolói
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Veridiana Pires
authors.initials: VP
authors.lastname: de Camargo
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Rodrigo Ramella
authors.initials: RR
authors.lastname: Munhoz
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Olavo
authors.initials: O
authors.lastname: Feher
authors.affiliation: Medical Oncology Division, Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Paulo M
authors.initials: PM
authors.lastname: Hoff
keywords: Kaposiform hemangioendothelioma; Paclitaxel; Sarcoma