Case report/Kazuistyka

Neurofibroma of sinus maxillae

Nerwiakowłókniak zatoki szczękowej

Józef Komorski, Łukasz Petz *, Jan Nienartowicz, Łukasz PałkaKlinika Chirurgii Szczękowo-Twarzowej, Akademicki Szpital Kliniczny,Kierownik Kliniki: Dr hab. n.med. Hanna Gerber, Wrocław, Poland

o t o l a r y n g o l o g i a p o l s k a 6 8 ( 2 0 1 4 ) 9 4 – 9 8

a r t i c l e i n f o

Article history:

Received: 19.05.2012

Accepted: 23.07.2012

Available online: 27.07.2012

Keywords:� Neurofibroma� Sinus maxillae� Schwann cells� Bening tumor

Słowa kluczowe:� nerwiakowłókniak� zatoka szczękowa� komórki Schwanna� guz łagodny

a b s t r a c t

Neurofibroma is a benign tumour composed from Schwann cells. Localization in sinus

maxillea is very rare. Authors presenting case which was treatment in Maxillo-Facial

Surgery Clinic in Wrocław.

© 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by

Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Available online at www.sciencedirect.com

journal homepage: www.elsevier.com/locate/otpol

Introduction

Neurofibroma is a benign tumor originating from peripheralnerves sheath. It may have varied cell structure and mostfrequently originates from Schwann cells or fibroblasts [1, 2].Other authors claim that neurobibroma is a combination ofSchwann cells, fibroblasts, peripheral nerves cells and inter-medial cells [2–4].

WHO classified neurofibromas into: dermal and plexiform.Dermal neurobiromas are located mainly in skin area and arecomposed of single peripheral nerves and resemble soft

* Corresponding author at: Lek. stom. Łukasz Petz, Akademicki Szpita213, 50-556 Wrocław, Poland.

E-mail address: ania_lukasz@poczta.onet.pl (Ł. Petz).0030-6657/$ – see front matter̀ 2012 Polish Otorhinolaryngology - Head and Neck Sur

http://dx.doi.org/10.1016/j.otpol.2012.07.001

pedanculated masses of skin in a form of bump. Plexiformneurofibromas are composed of many nerve bundles andlocated mainly in subcutaneous area and discolour the tissueabove the tumor. While growing they may expand to a verywide area and become larger.

Other histopathological classification of nerofibromasdepends on the tumour location (the so called occasionalneurofibromas). There are following types of those:

1. Diffuse – This is a type of neurofibroma consists of round orslightly pointed Shwann cells located in collagen fibre layer.

l Kliniczny, Klinika Chirurgii Szczękowo-Twarzowej, ul. Borowska

gery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Fig. 1 – CT scan of the tumor in frontal plane of the maxillarysinusRyc. 1 – Skan TK guza w płaszczyźnie czołowym region zatokiszczękowej

Fig. 2 – CT scan of the tumor in frontal plane of the cheek regRyc. 2 – Skan TK guza w płaszczyźnie czołowym regionpoliczka

o t o l a r y n g o l o g i a p o l s k a 6 8 ( 2 0 1 4 ) 9 4 – 9 8 95

There are often Wagner-Meissner like structures. Thetumor usually grows in dermal and subcutaneous layersspreading over taken area and tissue. They are mostfrequently located in neck and head tissues.

2. Plexiform – This form of neurofibroma is connected withnerves changing them into extensive intircate masses. It iscommon in patients suffering from von Recklinghausendisease (neurofibromatosis NF1).

3. Epithelioid – Neurofibroma where most Schwann cells areround or polyline shaped.

4. Pacinian – A type of neurofibroma where small round Pacinibody-like structures dominate [5].

Localized and single neurofibroma tumors often grow andexpand along peripheral nerves as well separated but non-encapsulated tumors. Very rarely in tumor area there isnecrosis and cystic degeneration. Sporadically they may befound as a part of NF-1 and most of them become visible at theage of 30 or 40 [6–11].

Case report

A female aged 79 case history number 31296/11 was admittedto the Cranio-Surgery Department in July 2011 with symp-toms of left sinus tumor. During examination bump-distention of the left cheek tissue and left suborbital areawere diagnosed. The tissue above the lesion was unchangedand of proper temperature. The patient complained aboutdecreasing nose patency for a few years and the presenceof white secrete on the back wall of the throat. She alsomentioned left suborbital nerve function disorder. Intrao-rally there was an alveolar bone distention in the maxilla.The tumor side maxilla was toothless. The patient did notreport pain while palpation examination. Coexistent disea-ses: hypertension, pleuritis in the past, family historyirrelevant.

After performing tumor biopsy histopathological resultshowed neurofibroma atypicum. After surgery this wasconfirmed in the whole removed tissue.

In CT scan 7213/CT/2011 of 27.09.2011: in CT examination ofthe craniofacial region with contrast extensive tumor-likestructure occupying left sinus maxilla was identified, sprea-ding to nearby bone structures destroying them completely. Tothe bottom the tumor is located in upper jaw bone alsodestroying it completely. All walls of maxillary sinus arecompletely damaged. The tumor penetrates the nasal cavitydamaging lower and lateral of the orbital and zygomatic bone,leaving only the frontal processes unaffected. Tumor outlineis polycyclic. Defined tumor outline with hypodense area inthe centre are visible. The lack of the teeth in the upper jawexcluding two teeth on the right side. There were no changesin the intraorbital structures (Figs. 1–6) description: B.Hendrich MD, PhD.

En-block surgical excision was performer including the leftside of upper jaw and orbital floor. Afterward the floor oforbital bone was reconstructed using titanium grid. The size ofthe removed tumor was 5 cm � 3 cm, 5 cm � 2 cm. After thesurgery the patient underwent prosthodontic rehabilitationand is still under outpatient department control.

Fig. 6 – 3D reconstractionRyc. 6 – Rekonstrukcja 3D

Fig. 5 – CT scan of the tumor in axial plane of the maxillarysinus regRyc. 5 – Skan TK guza w płaszczyźnie osiowej reg zatokiszczękowej

Fig. 4 – CT scan of the tumor in axial plane of the palate regRyc. 4 – Skan TK guza w płaszczyźnie osiowej reg podniebienia

Fig. 3 – CT scan of the tumor in axial plane of the maxillarysinus regRyc. 3 – Skan TK guza w płaszczyźnie osiowej reg zatokiszczękowej

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Discussion

The neurofibroma of the jaw bone is very rare [12]. According toDas Gupta et al. [11] only 44,8% out of 303 cases of benignperipheral nerves tumors of the sheath were located in theneck and head region out of which only 9% in oral cavity areawhere the tongue is usually affected. In the article written byRobitaille et al. [13] in 1975 only 25% of all Schwannom'stumors were located in the neck and head. Only 4 tumors outof 15 located in nasal sinuses were neurofibromas. AlsoRakshit et al. [14] examining 266 cases of peripheral nervestumors fund only one case of neurofibroma in nasal cavity[14–16]. This location of tumor is very rare but some authorssuggest its increasing presence in head and neck [17–21]. Only10% of neurofibromas are single cases, remaining 90% are thesymptoms of type 1 neurofibromatosis [9, 22–31]. Thetreatment of single lesions is usually surgical trying topreserve the nerve where the tumor originates [8, 15, 25].The excission should be radical though, since recurrences arepossible. Neurofibromas have a tendency to expand dras-tically damaging nearby structures and become malignant[15, 24]. In comparison to NF-1 neurofibromas, where there are5–16% cases of malignant tumor, single one becomingmalignant is rare, although more frequent then in case ofShwanomm tumor [15, 26, 27]. This case proves how aggressivethe tumor of this type may be, showing few clinical signs. Atthe beginning symptoms are uncharacteristic, such assporadic neuralgia or periodical parestheses of the tissuesconnected to the particular nerve, which are usually diminishedby both the doctor and the patient. Symptoms become strongerwith the tumor's growth and damage done to nearby structuresand depend on the tumor location [6, 15, 32]. It is also importantto pay attention to the single tumor as it may be the beginningof type 1 neurofibromatosis. Modern visualization methodsshould help diagnosing the tumor in its initial stages.

Authors' contributions/Wkład autorów

J. Komorski – study design, Ł. Petz – data collection, statisticalanalysis, data interpretation, J. Nienartowicz – statisticalanalysis, data interpretation, acceptance of final manuscriptversion, Ł. Pałka – literature search.

Conflict of interest/Konflikt interesu

None declared.

Financial support/Finansowanie

None declared.

Ethics/Etyka

The work described in this article have been carried out inaccordance with The Code of Ethics of the World Medical

Association (Declaration of Helsinki) for experiments invol-ving humans; EU Directive 2010/63/EU for animal experiments;Uniform Requirements for manuscripts submitted to Biome-dical journals.

The own research were conducted according to the GoodClinical Practice guidelines and accepted by local BioethicsCommittee, all patients agreed in writing to participation andthese researches.

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