All the following are features of Von Recklinghausen's Disease except (AIIMS May 2011)
|A||Transmission is autosomal dominant|
|B||Patients develop numerous skin nodules and cafe au lait patches|
|C||Malignant transformation is unheard of|
|D||Treatment is needed only if pain or paresthesia become troublesome or if a tumor become very large|
Malignant transformation is unheard of
a. Von Recklinghausen's disease is usually apparent at birth or may be recognized in adolescence it is characterized by development of one or many neurofibromas producing skin lesions, enlargement of limbs,
b. Secondary skeletal changes by surface erosion or compression, defective focal osteogenesis of a long bone that may result in pseudoarthrosis, osteoporosis, rapidly progressing scoliosis and sometimes neurological deficit. Malignant transformation to a sarcoma is noted.