Okumura K, Sasaki Y, Ohyama M, Nishi T
Acta Pathol Jpn. 1986 Feb;36(2):269-77. doi: 10.1111/j.1440-1827.1986.tb01479.x.
This paper reports an autopsy case of a 5-year and 11-month-old girl with generalized subcutaneous lipomatosis, megalencephaly, and macrodactyly. Marked emaciation and poor prognosis were the characteristic findings of this case. We thought that this case was identical with a rare syndrome which was initially reported by BANNAYAN in 1971. Autopsy disclosed diffuse lipomatosis in the thoracic and abdominal cavity, and infiltration of fat tissue to the pancreas. Hyperplasia of the small intestinal mucosa and Peyer's patch, and a pedunculated polyp of the sigmoid colon were the unique findings which could not be seen in the previously reported cases.
本文报道了一例5岁11个月大的女孩尸检病例，该女孩患有全身皮下脂肪瘤病、巨头畸形和巨指（趾）症。明显消瘦和预后不良是该病例的特征性表现。我们认为该病例与1971年BANNAYAN首次报道的一种罕见综合征相同。尸检发现胸腔和腹腔弥漫性脂肪瘤病，脂肪组织浸润至胰腺。小肠黏膜和派尔集合淋巴结增生，以及乙状结肠带蒂息肉是之前报道的病例中未见的独特发现。
