Neurofibromatosis type 1 (NF 1) is an inherited autosomal dominant disorder with an estimated incidence of 1 in 2,500 to 3,000 live births [1]. Neurofibromas are benign peripheral nerve sheath tumors developed from the proliferation of fibroblasts and Schwann cells. Wallac [2] categorized neurofibromas into four types: cutaneous neurofibromas of the epidermis or dermis, subcutaneous neurofibrom...

Macrodystrophia lipomatosa is a rare congenital nonhereditary developmental anomaly. It is characterized by hamartomatous proliferation of the soft tissue leading to disproportionate enlargement of the limbs and digits. Since it leads to diagnostic dilemma, it has to be differentiated from various other conditions as they differ in course, prognosis, complications, and treatment. Herein, we pre...

http://www.sajr.org.za doi:10.4102/sajr.v19i2.886 Fibrolipomatous hamartoma is a rare benign tumour most commonly affecting the median nerve and is characterised by fibro-fatty nerve infiltration. It results in fusiform nerve enlargement with a pathognomonic ‘spaghetti-like’ imaging appearance. Patients present with numbness and paraesthesia and later with motor deficits in the affected nerve d...

Macrodystrophia lipomatosa is a rare nonhereditary congenital form of localised gigantism usually involving the 2nd or 3rd digit of the hand or foot. Pathologically an increase in adipose tissue involving subcutaneous tissue periosteum and bone marrow is present. Typical clinical andradiological appearances are described in this case report. Case Report A 36-year-old female presented with enlar...