Rev Bras Otorrinolaringol

2007;73(2):284. CASE REPORT

Neck Neurofibromatosis:
clinical and surgical
features
Clarissa L. Buono Lehoczki 1, Ronny Tah Yen Ng 2,
Reinaldo Jordão Gusmão 3 Keywords: neck, neurofibromatosis, treatment.

INTRODUCTION DISCUSSION CONCLUSIONS

Type I Neurofibromatosis (NF1) is a Patients with NF1 have increased Neurofibromatosis is an entity to
dominant autosomal disease. Its prognosis chance of developing benign and malig- be considered by otolaryngologists when
is related to the development of tumors that nant tumors, which may in some cases performing the differential diagnosis of
may evolve to malignancy. It is characteri- infiltrate and lead to compression of vital cervical tumors.
zed by multiple café-au-lait spots, skeletal structures and evident deformities.2 It usually manifests itself in the form
defects, optical gliomas, Lisch nodules and Images are key for diagnostic pur- of deeply located benign tumors that may
neurofibromas.1 poses, and tissue biopsy is used to confirm compress vital structures. In such cases,
Plexiform neurofibromas (PN) are the diagnosis. surgical management is required.6
one of the many possible findings on pa- Although most tumors are benign, There is consensus that surgery
tients affected by the disease. must be postponed until compressive
This paper aims at reporting on a symptoms are present or signs of malig-
case of a pediatric patient with cervical nancy have been identified.
plexiform neurofibromas and the difficul-
REFERENCES
ties inherent to the treatment.
1. White AK. Head and Neck manifesta-
CASE STUDY tions of neurofibromatosis. Laryngoscope
A four months old male patient, 1996;96:732-37.
with high respiratory discomfort, was 2. Rapado F. Neurofibromatosis Type 1 of the
diagnosed with a mass in the rhinopha- head and neck: dilemmas in management.
J Laryngol Otol 2001;115:151-4.
rynx that extended all the way to the
3. Bruchhage KL, Bockmuh U, Dahm MC.
oropharynx, later described as a plexiform Zervikale manifestation eines malig-
neurofibroma in the pathologist’s report. nen Schwannom. Laryngorhinootologie
The CT scan of the area revealed 1998;77(4):235-7.
Cervical Neurofibromatosis - see arrow for tumor in neck CT scan
a mass located in the left parotid region. axial view. 4. Wise JB, Patel SB. Management issues in
The tumor was diverting the internal massive pediatric facia plexiform neurofi-
carotid artery from its original position the risk of malignancy ranges between 4 broma with neurofibroma type 1. Head &
and compressing the internal jugular vein. and 12% as described in the literature.3 Neck 2002;24(2):207-11.
(Picture 1) Surgery is the only effective option 5.Chung CJ, Armfield KB, Mukherj SK, For-
At 16 months of age the patient to manage plexiform neurofibromas. Suc- dham LA, Krause WL. Cervical neurofibro-
underwent a tracheotomy to address the cess is however limited, as this is a highly mas in children with NF-1. Pediatr Radiol
respiratory difficulties. At 18 months he infiltrating tumor with elevated relapse 1999;29(5):353-6.
rates. Besides, the difficulty in identifying 6.Sobol SE, Tewefik TL, Qteuberg J. Otolarin-
was submitted to a partial excision of the
gologic manifestations on neurofibromato-
tumor as it was infiltrating in the cervical the main nervous plexus during surgery
sis in children. J Otolaryngol 1997;26(1):13-
plexus, brachial plexus, and facial nerve. increases postoperative morbidity and inci- 9.
Biopsy findings also pointed to plexiform dence of neurological complications. 4

neurofibroma. It seems prudent to postpone sur-

The child is being monitored for gery in symptom-free patients with head
worsening of compressive symptoms and and neck PN until clearly obstructive
signs of malignancy. symptoms are present.5

1
SBORL Specialist 2004, otolaryngologist, MD at the Pediatric Otolaryngology Department at Unicamp.
2
Resident of Otolaryngology at Unicamp.
3
PhD in Otoralyngology at FCM UNICAMP, Head of the Pediatric Otolaryngology Department at UNICAMP.
Universidade Estadual de Campinas.
Mailing address: Clarissa Buono Lehoczki - Al. Arapanés 113 Moema São Paulo SP 04524-000.
Paper submitted to the ABORL-CCF SGP (Management Publications System) on May 21th, 2005 and accepted for publication on June 20th, 2006.cod. 351.

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