Metachromic leukodystroph and liver tumor association in two siblings
Osman Faruk Şenyüz 1, Egemen Eroğlu 2, Cengiz Yalçınkaya 3, Mehmet Eliçevik 1, Sait Albayram 4, Sergülen Dervişoğlu 5
1İ. Ü. Cerrahpaşa Tıp Fakültesi, Çocuk Cerrahisi Anabilim Dalı, İstanbul
2Amerikan Hastanesi, Çocuk Cerrahisi Bölümü, İstanbul
3İ. Ü. Cerrahpaşa Tıp Fakültesi, Nöroloji Anabilim Dalı, İstanbul
4İ. Ü. Cerrahpaşa Tıp Fakültesi, Radyolojii Anabilim Dalı, İstanbul
5İ. Ü. Cerrahpaşa Tıp Fakültesi,Patolojik Anatomi Anabilim Dalı, İstanbul
Keywords: Hepatobiliary mucinous cystadenoma, metachromatic leukodystrophy, siblings, hepatic tumors
Abstract
The authors present two siblings with rare hepatic tumors in association with metachromatic leukodystrophy. The presence of metachromatic bodies in the microscopic examination of the tumour extirpated during hepatectomy dictates the close follow-up of neurologic status of these patients regarding the signs and symptoms of metachromatic leukodystrophy.