title: Retiform hemangioendothelioma developed on the site of an earlier cystic lymphangioma in a six-year-old girl.
abstract: Retiform hemangioendothelioma (RH) is a rare low-grade malignancy angiosarcoma, with a high rate of local recurrence and a low metastatic risk. A 6 year-old girl with a large cervical cystic lymphangioma diagnosed by ultrasound and Doppler ultrasound, which showed a large multiloculated anechoic cyst with no flow. The lymphangioma was treated with injections of Picibanil (OK-432). The tumor regressed, but after a year, she developed a poorly limited infiltrated plaque spreading out regularly over her chest, back, and shoulder. The biopsy showed a poorly limited dermal and subcutaneous vascular proliferation composed of elongated arborising vessels lined with ovoid endothelial cells in a hobnail pattern. In addition, the deep part of the lesion showed typical features of a papillary intralymphatic angioendothelioma pattern (PILA) or Dabska tumor. The endothelial cells strongly expressed podoplanin (D2-40). A diagnosis of RH with focal areas of PILA was reached. The girl died 8 months after surgery of hypovolemic shock in a context of diffuse lymphangiomatosis with pulmonary localization. To our knowledge, RH has hardly ever been described in children. This entity exhibits a continuum with the PILA, sharing not only morphological and immunohistochemical similarities but also its ability to develop in a context of a vascular anomaly, particularly a lymphangioma. The role of Picibanil in the development of this tumor can be discussed.
authors.affiliation: Department of Pathology, Necker-Enfants Malades Hospital, APHP, Université Paris Descartes, Paris, France.
authors.forename: Anne-Fore
authors.initials: AF
authors.lastname: Albertini
authors.forename: Nicole
authors.initials: N
authors.lastname: Brousse
authors.forename: Christine
authors.initials: C
authors.lastname: Bodemer
authors.forename: Eduardo
authors.initials: E
authors.lastname: Calonje
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage; D002648:Child; D005260:Female; D006390:Hemangioendothelioma / Q000473:pathology*; D006801:Humans; D018191:Lymphangioma, Cystic / Q000188:drug therapy / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000473:pathology*; D010844:Picibanil / Q000008:administration & dosage; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*
chemical_list: D000970:Antineoplastic Agents; D010844:Picibanil