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Neurofibroma.

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Diagnosis, Differential↗

Pacinian neurofibroma.

A 40-year-old woman developed a tender, greyish-white nodule of the index finger. Histologic examination disclosed mature and immature pacinian corpuscles.

Adult↗

A dumbbell (hourglass) neurofibroma of the spine in a patient with von Recklinghausen's disease. A case report with twelve-year follow-up.

A five-and-one-half-year-old boy, referred for orthopaedic evaluation of spinal curvature, was diagnosed as having neurofibromatosis (von Recklinghausen's disease) with a 44-degree right thoracolumbar curve from T-10 to L-3. A soft-tissue mass was identified in the area of T-7 to T-12 and, when neurological signs developed, the mass was removed by extraperitoneal laparothoracotomy. Tumor root was noted to extend into the spinal canal, and subsequent surgery was undertaken to remove this tissue. There was no postoperative increase in the neurologic deficit, although the scoliosis progressed to the point where spinal fusion was required. This was followed by brace treatment to maintain correction during the growth years. Long-term follow-up (12 years) revealed that the patient's spine was balanced, the fusion mass appeared to be solid radiographically, and the neurological examination was normal.

Adolescent↗

Some electron microscopic and autoradiographic results concerning cutaneous neurofibromas in von Recklinghausen's disease.

The cutaneous tumors of 5 patients with clinical symptoms of Recklinghausens neurofibromatosis were investigated by light and electron microscopy. Special reference was given to the study of initial changes of the disease and the structure of the nerve fascicles which end in the tumor. In 2 patients the proliferating cells were determined by using an autoradiographic technique. It was discovered that all cellular elements of a peripheral nerve are involved by the tumor formation. The results were discussed and compared with the alterations during nerve regeneration after traumatic injury.

Cell Division↗

The vascular features of plexiform neurofibroma with some observations on the importance of pre-operative angiography and the value of pre-operative intra-arterial embolisation.

The presence of abnormal feeding vessels in plexiform neurofibromata of the head and neck has been demonstrated by angiography in three patients. Attention is drawn to the possibility of dangerous bleeding in the treatment of these lesions. Two other patients presented with a similar lesion over the trunk: in one patient there was excessive bleeding during surgical excision but not in the other, despite their very similar clinical appearances. It is suggested that two varieties of plexiform neurofibromata exist which are distinguishable only by angiography: those with a normal vasculature and those with a plethora of abnormal vessels forming an integral part of the lesion along with the neural and fibrous elements (haemangioneurofibroma). A method of treatment is proposed based on pre-operative embolisation followed by complete surgical excision. The futility of pre-operative vessel ligation is confirmed.

Adult↗

Non-von Recklinghausen's neurofibromatosis presenting as hemifacial neurofibromas and contralateral café au lait spots.

The term neurofibromatosis designates a spectrum of disorders, the most common of which is von Recklinghausen's disease. We describe two patients with forms of neurofibromatosis other than von Recklinghausen's neurofibromatosis, characterized by involvement of half of the face and the contralateral lower extremity. Neither patient had a family history of neurofibromatosis, and a thorough evaluation of both patients failed to reveal evidence of von Recklinghausen's disease. The classification of neurofibromatosis is briefly summarized, with special emphasis placed on the need for a specific diagnosis regarding the type of neurofibromatosis for purposes of estimating prognosis and providing genetic counseling. The relationship between pregnancy and neurofibromatosis is also discussed.

Adult↗