Metastasizing epithelioid hemangioendothelioma of the nose in childhood.
Epithelioid hemangioendothelioma (EHE) arising in the skin is extremely rare, and the majority of documented cases have developed in soft tissues or parenchymatous organs. We report on a 9-year-old boy who presented with a painful erythematous plaque on the nose. Histopathological findings showed a dermal tumor composed of epithelioid cells with moderate cellular atypia and characteristic intracytoplasmic vacuoles. Immunohistochemical stainings confirmed the vascular nature of the tumor. On the basis of these findings, the diagnosis of an EHE was made. Then, two cervical lymph nodes were excised and adjuvant therapy with interferon alpha 2b was initiated. Eighteen months after diagnosis, there is no evidence of local tumor recurrence or metastases. To our knowledge, this is the first report of a metastasizing cutaneous EHE on the nose in childhood. As our case shows, this rare tumor entity has to be considered as a differential diagnosis in neoplasms of the skin, even in childhood.
Department of Dermatology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany. fegberts@dermatology.uni-kiel.de
Friederike
F
Egberts
Thomas
T
Mentzel
Iuo
I
Leuschner
Gisela
G
Metzler
Regina
R
Fölster-Holst
D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D003560:Cysts / Q000473:pathology; D003937:Diagnosis, Differential; D018323:Hemangioendothelioma, Epithelioid / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D008207:Lymphatic Metastasis / Q000473:pathology*; D008297:Male; D018613:Microscopy, Confocal; D009669:Nose Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D018296:Pilomatrixoma / Q000473:pathology; D011994:Recombinant Proteins; D012878:Skin Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*
D000970:Antineoplastic Agents; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins