D'Costa Grace Francis, Taksande Rahul V, Pandya Bhanumati Sumant, Najmi Shaheen, Dua Seema, Patil Yoganand Vishwasrao
Department of Pathology, Grant Medical College, Byculla, Mumbai.
Indian J Pathol Microbiol. 2007 Jul;50(3):572-4.
Macrodystrophia lipomatosa is a rare congenital form of localized gigantism, characterized by an increase in all mesenchymal elements, particularly fibroadipose tissue. The areas of predilection are segments supplied by the median or plantar nerves. We report such a rare case in a thirty four year old male patient, who presented with a swelling of the right upper limb and a marked increase in the size of the right thumb, index finger and radial half of the right hand, present since birth, with progressive increase to the present size. Amputation of the right thumb and debulking of the palm was done. The specimen measured 26 x 18 x 12 cms. and the thumb alone measured 13 x 7 x 7 cm. histology revealed hypertrophy of adipose tissue in the subcutaneous compartment and infiltration into the nerve sheaths and muscles.
巨脂性营养障碍是一种罕见的先天性局限性巨人症，其特征是所有间充质成分增加，尤其是纤维脂肪组织。好发部位是由正中神经或足底神经支配的节段。我们报告了一例罕见病例，患者为34岁男性，自出生以来右上肢肿胀，右手拇指、示指及桡侧明显增粗，且逐渐增大至目前大小。对其进行了右手拇指截肢及手掌减容手术。标本大小为26×18×12厘米，仅拇指就有13×7×7厘米。组织学检查显示皮下脂肪组织肥大，并浸润至神经鞘和肌肉。
