Diagnosis
Laryngeal neurofibromatosis
Discussion
Neurogenic tumors including neurofibromas of the larynx are uncommon.
Some of these have been associated with VRD but the majority of reports
do not mention associated features of von Recklinghausen's disease (VRD).
The cases of laryngeal plexiform neurofibromas are less common than solitary
neurofibromas and some reports do not identify whether or not the neurofibromas
are of the plexiform variety. Plexiform neurofibroma exists with and without
associated VRD. Extensive involvement of many structures with these tumors
is the usual situation. Nearly all of the solitary tumors are supraglottic
with a predilection for the aryepiglottic fold or false vocal cord. Based
on this anatomic association, Nanson has speculated that they most commonly
arise from the superior laryngeal nerve and its terminal ramifications.
Schwannomas and neurofibromas have been reported to arise throughout the
larynx, involving the false vocal cord, epiglottis, and subglottis. Management
of these tumors may be as simple as endoscopic removal or may involve
extensive radical surgery to restore function. Plexiform tumors infiltrate
widely and complete removal is usually not a realistic goal.
The management of these patients is highly individualized. The variable
but always progressive nature of this disease necessitates a cautious,
conservative approach whenever possible.
Dr.
Bhujang Pai, Hinduja Hospital, Mumbai
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