Çocukluk Çağının Ender Bir Neoplastik Hastalığı: Multifokal Epiteloid Hemanjioendotelyoma Vakası

Abstract

On yedi yaşında kız hasta, karaciğerde dört farklı segmentte insidental olarak saptanmış epiteloid hemanjioendotelyoma tanısıyla tarafımıza yönlendirildi. Karaciğer tru-cut biyopsi materyalinin yeniden incelenmesi sonucunda epiteloid hemanjioendotelyoma ile uyumlu olduğu görüldü. Yapılan tetkiklerinde akciğer ve beyinde multipl metastatik odaklar saptanan hasta asemptomatik olduğundan tedavisiz izlemine karar verildi. Bir yıllık izlem süresi boyunca herhangi bir semptomu olmayan hastamızın bir yıl sonundaki kontrol görüntülemelerinde kitlelerin boyutlarının artmadığı ve yeni bir metastaz olmadığı görüldü. Multipl metastazı olan epiteloid hemanjioendotelyoma vakaları için bevacizumab terapisi veya cerrahi tedavi yöntemleri uygulanabilmektedir. Hastamızın seyri, kitle boyutlarına ve metastaz yaygınlığına bakılmaksızın, asemptomatik epiteloid hemanjioendotelyoma hastalarının tedavisiz izlemi için cesaret verici olmuştur.

Keywords

• Metastaz, multifokal epiteloid hemanjioendotelyoma, vasküler tümör

A Rare Neoplastic Disease of Childhood: Multifocal Epithelioid Hemangioendothelioma

Abstract

A 17-year-old girl in whom the diagnosis of epithelioid hemangioendothelioma was established in four different segments of the liver incidentally was referred to us. Further examination of tru-cut biopsy material showed multiple metastatic lesions in the brain and both lungs. Since the patient was asymptomatic, she was followed up without treatment for a year. At the first-year control visit, the radiologic examination indicated a stable epithelioid hemangioendothelioma without any new metastatic lesion, and increase in the dimensions of the mass lesion. The management of epithelioid hemangioendothelioma consists of surgical treatment, drug administration such as bevacizumab or follow-up without treatment. The course of the disease in our patient encouraged follow-up of asymptomatic patients with epithelioid hemangioendothelioma without treatment, regardless of the extent of metastatic lesions,and dimensions of the mass lesion

Keywords

• Metastasis, multifocal epithelioid hemangioendothelioma, vascular tumor

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