Massive Plexiform Neurofibroma of the Neck and Larynx

To report the first case of massive neck plexiform neurofibroma with intralaryngeal (supraglottic) extension in a 5-year-old boy with neurofibromatosis type 1 and to describe its treatment.

Resumed

Report This massive plexiform neurofibroma was surgically removed, relieving its significant respiratory obstructive symptoms without recurrence to date.

Conclusion

Massive neck plexiform neurofibroma with supraglottic part was found in a child with neurofibromatosis type 1; it should be included in differential diagnosis of stridor and neck mass in children. It was diagnosed and removed in early in childhood without recurrence.

plexiform neurofibroma; larynx; neck

Authorship

Mohammad Kamal Mobashir

Department of Otorhinolaryngology-Head and Neck Surgery, Zagazig University, Zagazig, EgyptZagazig UniversityEgyptZagazig, EgyptDepartment of Otorhinolaryngology-Head and Neck Surgery, Zagazig University, Zagazig, Egypt

Abd ElRaof Said Mohamed

Mohammad Waheed El-Anwar

Ahmad Ebrahim El Sayed

Mouhamad A. Fouad

Department of Pathology, Zagazig University, Zagazig, EgyptZagazig UniversityEgyptZagazig, EgyptDepartment of Pathology, Zagazig University, Zagazig, Egypt

Address for correspondence Mohammad El-Anwar, MD, Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, Zagazig University, Zagazig 0020552309843, Egypt (e-mail: mwenteg@yahoo.com).

SCIMAGO INSTITUTIONS RANKINGS

Department of Pathology, Zagazig University, Zagazig, EgyptZagazig UniversityEgyptZagazig, EgyptDepartment of Pathology, Zagazig University, Zagazig, Egypt

Figures

Figures (4)

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Fig. 1
Postoperative images of the patient. (A) Early while tracheostomy in place. (B) After decannulation. (C) Well-healed neck scar at 2 years postoperatively, with no recurrent neck masses and one café au lait patch (blue arrow). (D) Abdomen showing many brown café au lait patches (>6).

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Fig. 2
Endoscopic evaluation of larynx. (A) Preoperative endoscopy shows large supraglottic mass located in the right aryepiglottic fold and covered by apparently normal mucosa, obstructing the view of the vocal cord. The right arytenoid appeared to be pushed anteriorly (yellow arrow). (B and C) Postoperative endoscopy showed no recurrence.

Thumbnail

Fig. 3
Preoperative imaging. (A) Axial MRI shows large diffuse noninfiltrative neck mass. (B) Axial MRI shows the mass encroaching on laryngeal inlet. (C) Axial MRI shows enhancement of the mass. (D) Sagittal MRI shows large mass extending to lower neck. (E) Axial computed tomography (CT) shows large heterogeneous density noninfiltrative neck mass. (F) Axial CT shows encroachment on laryngeal inlet and supraglottic region.

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Fig. 4
Postoperative axial magnetic resonance imaging (MRI) showing (A) normal laryngeal inlet and (B) removal of mass from the neck. Postoperative histopathology of the plexiform neurofibroma showing (C) neurofibromas unencapsulated and showing zonation with a more cellular central region containing residual nerve twigs and more myxoid areas at the periphery (×100). (B) Typically, the Schwann cells predominate and are spindled to ovoid and slender with characteristic wavy nuclei (×200). (F) Neurofibromas are composed of several elements, including Schwann cells, perineurial cells, intraneural fibroblasts, bundles of collagen arrayed in a characteristic “shredded carrot” pattern, and scattered enlarged cells with hyperchromatic nuclei representing degenerative change (×400).

Fig. 1 Postoperative images of the patient. (A) Early while tracheostomy in place. (B) After decannulation. (C) Well-healed neck scar at 2 years postoperatively, with no recurrent neck masses and one café au lait patch (blue arrow). (D) Abdomen showing many brown café au lait patches (>6).

Fig. 2 Endoscopic evaluation of larynx. (A) Preoperative endoscopy shows large supraglottic mass located in the right aryepiglottic fold and covered by apparently normal mucosa, obstructing the view of the vocal cord. The right arytenoid appeared to be pushed anteriorly (yellow arrow). (B and C) Postoperative endoscopy showed no recurrence.

Fig. 3 Preoperative imaging. (A) Axial MRI shows large diffuse noninfiltrative neck mass. (B) Axial MRI shows the mass encroaching on laryngeal inlet. (C) Axial MRI shows enhancement of the mass. (D) Sagittal MRI shows large mass extending to lower neck. (E) Axial computed tomography (CT) shows large heterogeneous density noninfiltrative neck mass. (F) Axial CT shows encroachment on laryngeal inlet and supraglottic region.

Fig. 4 Postoperative axial magnetic resonance imaging (MRI) showing (A) normal laryngeal inlet and (B) removal of mass from the neck. Postoperative histopathology of the plexiform neurofibroma showing (C) neurofibromas unencapsulated and showing zonation with a more cellular central region containing residual nerve twigs and more myxoid areas at the periphery (×100). (B) Typically, the Schwann cells predominate and are spindled to ovoid and slender with characteristic wavy nuclei (×200). (F) Neurofibromas are composed of several elements, including Schwann cells, perineurial cells, intraneural fibroblasts, bundles of collagen arrayed in a characteristic “shredded carrot” pattern, and scattered enlarged cells with hyperchromatic nuclei representing degenerative change (×400).

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Massive Plexiform Neurofibroma of the Neck and Larynx

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[1] Address for correspondence Mohammad El-Anwar, MD, Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, Zagazig University, Zagazig 0020552309843, Egypt (e-mail: mwenteg@yahoo.com).