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August 1956

Neurofibromatosis: A Case Report of Elephantiasis Neuromatosa of the Right Lower Extremity, with Invasion of the Popliteal Artery

Author Affiliations

Brookline, Mass.

AMA Arch Surg. 1956;73(2):279-284. doi:10.1001/archsurg.1956.01280020093018

The problem of neurofibromatosis is essentially the problem of its pathogenesis and clinical pathological correlation. This is further complicated by the multiplicity of terms used to describe the various clinical and pathological manifestations, and the erroneous concepts of its behavior, particularly in relation to malignancy. It is not the purpose of this communication to be concerned specifically with an attempt at a precise clinical pathological evaluation. This phase of neurofibromatosis and its relationship to malignancy will be the subject of a subsequent paper.

Among the terms used to describe neurofibromatosis are the following: forme fruste, molluscum fibrosum, fibroma molluscum, plexiform neuroma, multiple neuroma, neurinomatosis, neurofibromatosis, multiple neurofibromatosis, neuroblastomatosis, neuromatosis, hourglass tumors of the spine, dumb-bell tumor, elephantiasis neuroma, elephantiasis neuromatosa, pachydermatocele, neurofibroma, and von Recklinghausen's disease.

Neurofibromatosis is frequently referred to as von Recklinghausen's disease,* since he first reported it in 1882.1 Classically the disease is characterized by multiple