Neurofibroma of sinus maxillae

otolaryngologia polska 68 (2014) 94–98

Available online at www.sciencedirect.com

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

Case report/Kazuistyka

Neurofibroma of sinus maxillae Nerwiakowłókniak zatoki szczękowej Józef Komorski, Łukasz Petz *, Jan Nienartowicz, Łukasz Pałka Klinika Chirurgii Szczękowo-Twarzowej, Akademicki Szpital Kliniczny, Kierownik Kliniki: Dr hab. n.med. Hanna Gerber, Wrocław, Poland

article info

abstract

Article history:

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

Received: 19.05.2012

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

Accepted: 23.07.2012

Surgery Clinic in Wrocław.

Available online: 27.07.2012

© 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Keywords:  Neurofibroma  Sinus maxillae  Schwann cells  Bening tumor Słowa kluczowe:  nerwiakowłókniak  zatoka szczękowa  komórki Schwanna  guz łagodny

Introduction Neurofibroma is a benign tumor originating from peripheral nerves sheath. It may have varied cell structure and most frequently originates from Schwann cells or fibroblasts [1, 2]. Other authors claim that neurobibroma is a combination of Schwann cells, fibroblasts, peripheral nerves cells and intermedial cells [2–4]. WHO classified neurofibromas into: dermal and plexiform. Dermal neurobiromas are located mainly in skin area and are composed of single peripheral nerves and resemble soft

pedanculated masses of skin in a form of bump. Plexiform neurofibromas are composed of many nerve bundles and located mainly in subcutaneous area and discolour the tissue above the tumor. While growing they may expand to a very wide area and become larger. Other histopathological classification of nerofibromas depends on the tumour location (the so called occasional neurofibromas). There are following types of those: 1. Diffuse – This is a type of neurofibroma consists of round or slightly pointed Shwann cells located in collagen fibre layer.

* Corresponding author at: Lek. stom. Łukasz Petz, Akademicki Szpital Kliniczny, Klinika Chirurgii Szczękowo-Twarzowej, ul. Borowska 213, 50-556 Wrocław, Poland. E-mail address: [email protected] (Ł. Petz). 0030-6657/$ – see front matter` 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved. http://dx.doi.org/10.1016/j.otpol.2012.07.001

otolaryngologia polska 68 (2014) 94–98

95

There are often Wagner-Meissner like structures. The tumor usually grows in dermal and subcutaneous layers spreading over taken area and tissue. They are most frequently located in neck and head tissues. 2. Plexiform – This form of neurofibroma is connected with nerves changing them into extensive intircate masses. It is common in patients suffering from von Recklinghausen disease (neurofibromatosis NF1). 3. Epithelioid – Neurofibroma where most Schwann cells are round or polyline shaped. 4. Pacinian – A type of neurofibroma where small round Pacini body-like structures dominate [5]. Localized and single neurofibroma tumors often grow and expand along peripheral nerves as well separated but nonencapsulated tumors. Very rarely in tumor area there is necrosis and cystic degeneration. Sporadically they may be found as a part of NF-1 and most of them become visible at the age of 30 or 40 [6–11].

Case report A female aged 79 case history number 31296/11 was admitted to the Cranio-Surgery Department in July 2011 with symptoms of left sinus tumor. During examination bumpdistention of the left cheek tissue and left suborbital area were diagnosed. The tissue above the lesion was unchanged and of proper temperature. The patient complained about decreasing nose patency for a few years and the presence of white secrete on the back wall of the throat. She also mentioned left suborbital nerve function disorder. Intraorally there was an alveolar bone distention in the maxilla. The tumor side maxilla was toothless. The patient did not report pain while palpation examination. Coexistent diseases: hypertension, pleuritis in the past, family history irrelevant. After performing tumor biopsy histopathological result showed neurofibroma atypicum. After surgery this was confirmed in the whole removed tissue. In CT scan 7213/CT/2011 of 27.09.2011: in CT examination of the craniofacial region with contrast extensive tumor-like structure occupying left sinus maxilla was identified, spreading to nearby bone structures destroying them completely. To the bottom the tumor is located in upper jaw bone also destroying it completely. All walls of maxillary sinus are completely damaged. The tumor penetrates the nasal cavity damaging lower and lateral of the orbital and zygomatic bone, leaving only the frontal processes unaffected. Tumor outline is polycyclic. Defined tumor outline with hypodense area in the centre are visible. The lack of the teeth in the upper jaw excluding two teeth on the right side. There were no changes in the intraorbital structures (Figs. 1–6) description: B. Hendrich MD, PhD. En-block surgical excision was performer including the left side of upper jaw and orbital floor. Afterward the floor of orbital bone was reconstructed using titanium grid. The size of the removed tumor was 5 cm  3 cm, 5 cm  2 cm. After the surgery the patient underwent prosthodontic rehabilitation and is still under outpatient department control.

Fig. 1 – CT scan of the tumor in frontal plane of the maxillary sinus Ryc. 1 – Skan TK guza w płaszczyźnie czołowym region zatoki szczękowej

Fig. 2 – CT scan of the tumor in frontal plane of the cheek reg Ryc. 2 – Skan TK guza w płaszczyźnie czołowym region policzka

96

otolaryngologia polska 68 (2014) 94–98

Fig. 3 – CT scan of the tumor in axial plane of the maxillary sinus reg Ryc. 3 – Skan TK guza w płaszczyźnie osiowej reg zatoki szczękowej

Fig. 5 – CT scan of the tumor in axial plane of the maxillary sinus reg Ryc. 5 – Skan TK guza w płaszczyźnie osiowej reg zatoki szczękowej

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

Fig. 6 – 3D reconstraction Ryc. 6 – Rekonstrukcja 3D

otolaryngologia polska 68 (2014) 94–98

Discussion The neurofibroma of the jaw bone is very rare [12]. According to Das Gupta et al. [11] only 44,8% out of 303 cases of benign peripheral nerves tumors of the sheath were located in the neck and head region out of which only 9% in oral cavity area where the tongue is usually affected. In the article written by Robitaille et al. [13] in 1975 only 25% of all Schwannom's tumors were located in the neck and head. Only 4 tumors out of 15 located in nasal sinuses were neurofibromas. Also Rakshit et al. [14] examining 266 cases of peripheral nerves tumors fund only one case of neurofibroma in nasal cavity [14–16]. This location of tumor is very rare but some authors suggest its increasing presence in head and neck [17–21]. Only 10% of neurofibromas are single cases, remaining 90% are the symptoms of type 1 neurofibromatosis [9, 22–31]. The treatment of single lesions is usually surgical trying to preserve the nerve where the tumor originates [8, 15, 25]. The excission should be radical though, since recurrences are possible. Neurofibromas have a tendency to expand drastically damaging nearby structures and become malignant [15, 24]. In comparison to NF-1 neurofibromas, where there are 5–16% cases of malignant tumor, single one becoming malignant is rare, although more frequent then in case of Shwanomm tumor [15, 26, 27]. This case proves how aggressive the tumor of this type may be, showing few clinical signs. At the beginning symptoms are uncharacteristic, such as sporadic neuralgia or periodical parestheses of the tissues connected to the particular nerve, which are usually diminished by both the doctor and the patient. Symptoms become stronger with the tumor's growth and damage done to nearby structures and depend on the tumor location [6, 15, 32]. It is also important to pay attention to the single tumor as it may be the beginning of type 1 neurofibromatosis. Modern visualization methods should help diagnosing the tumor in its initial stages.

Authors' contributions/Wkład autorów J. Komorski – study design, Ł. Petz – data collection, statistical analysis, data interpretation, J. Nienartowicz – statistical analysis, data interpretation, acceptance of final manuscript version, 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 in accordance with The Code of Ethics of the World Medical

97

Association (Declaration of Helsinki) for experiments involving humans; EU Directive 2010/63/EU for animal experiments; Uniform Requirements for manuscripts submitted to Biomedical journals. The own research were conducted according to the Good Clinical Practice guidelines and accepted by local Bioethics Committee, all patients agreed in writing to participation and these researches.

references/pi smiennictwo

[1] Rosenbaum T, Rosenbaum C, Winner U, Meller HW, Lenard HG, Hanemann CO. Long-term culture and characterization of human neurofibroma-derived Schwann cells. J Neurosci Res 2000;61:524–526. [2] Apostolidis C, Anterriotis D, Rapidis AD, Angelopoulos AP. Solitary intraosseous neurofibroma of the inferior alveolar nerve: report of a case. J Oral Maxillofac Surg 2001;59:232–235. [3] Ide F, Shimoyama T, Horie N, Kusama K. Comparative ultrastructural and immunohistochemical study of perineurioma and neurofibroma of the oral mucosa. Oral Oncol 2004;40:948–953. [4] Gomez-Oliveira G, Fernandez-Alba Luengo J, Martin-Sastre R, Patino-Seijas B, Lopez-Cedrun-Cembranos JL. Plexiform neurofibroma of the cheek mucosa. A case report. Med Oral 2004;9:263–267. [5] Weiss SW, Sobin LH. Histologica typing of soft tissue tumours. WHO; 1969. [6] Sankowska M, Sąsiadek M, Sosnowska-Pacuszko D. Contemporary diagnostic imaging of spinal canal tumors. Adv Clin Exp Med 2006;15(4):711–722. [7] Handschel J, Herbst H, Brand B, Meyer U, Piffko J. Intraoral sebaceous carcinoma. Br J Oral Maxillofac Surg 2003;41: 84–87. [8] Alatli C, Oner B, Unur M, Erseven G. Solitary plexiform neurofibroma of the oral cavity. A case report. Int J Oral Maxillofac Surg 1996;25:379–380. [9] Badger GR. Solitary neurofibromatosis in the maxilla: report of oral findings. J Am Dent Assoc 1980;100:213–214. [10] Che Z, Nam W, Park WS, Kim HJ, Cha IH, Kim HS, et al. Intraosseous nerve sheath tumors in the jaws. Yonsei Med J 2006;47:264–270. [11] Das Gupta TK, Brasfield RD, Strong EW, Hajdu SI. Bening solitary Schwannomas. Cancer 1969;24(2):355–366. [12] Larsson A, Praetorius F, Hjörting-Hansen E. Intraosseous neurofibroma of the jaws. Int J Oral Surg 1978;7: 494–499. [13] Robitaille Y, Seemayer TA, El Deiry A. Peripheral nerve tumours involving paranasal sinuses: case raport and review of literature. Cancer 1975;35:1254–1258. [14] Rakshit BK, Sundareshwar B, Reddy CR. Nerve sheath tumours. Int Surg 1973;58(8):557–560. [15] Tandon DA, Deka RC. Neurofibromas of the Maxillary Sinus. Indian J Otolaryngol 1988;40(1):28–29. [16] Neville B, Damm D, Allen C, Bouquot J. Oral and maxillofacial pathology. Philadelphia: W.B. Saunders; 1995. [17] Wise JB, Patel SG, Shah JP. Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type 1. Head Neck 2002;24: 207–211. [18] Takahama Jr A, Leon JE, de Almeida OP, Kowalski LP. Nonlymphoid mesenchymal tumors of the parotid gland. Oral Oncol 2008;44:970–974. [19] Becelli R, Renzi G, Cerulli G, Saltarel A, Perugini M. Von recklinghausen neurofibromatosis with palatal localization.

98

[20]

[21]

[22]

[23]

[24]

[25]

otolaryngologia polska 68 (2014) 94–98

Diagnostic and surgical problems in two clinical cases. Minerva Stomatol 2002;51:391–397. Meyer U, Wiesmann HP, Berr K, Kubler NR, Handschel J. Cell-based bone reconstruction therapies-principles of clinical approaches. Int J Oral Maxillofac Implants 2006;21:899–906. Kim YD, Bai CH, Suh JS, Song KW. Transnasal endoscopic excision of an isolated neurofibroma of nasal septum. Rhinology 1997;35:89–91. Mori H, Kakuta S, Yamaguchi A, Nagumo M. Solitary intraosseous neurofibroma of the maxilla: report of a case. J Oral Maxillofac Surg 1993;51:688–690. Sharma P, Narwal A, Rana AS, Kumar S. Intraosseous neurofibroma of maxilla in a child. J Indian Soc Pedod Prev Dent 2009;27:62–64. Skouteris CA, Sotereanos GC. Solitary neurofibroma of the maxilla: report of a case. J Oral Maxillofac Surg 1988;46: 701–705. Huang GS, Lee CH, Lee HS, Chang WC, Juan CJ, Chen CY. Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol 2005;34:303–306.

[26] Vivek N, Manikandhan R, James PC, Rajeev R. Solitary intraosseous neurofibroma of mandible. Indian J Dent Res 2006;17:135. [27] Poupard RJ, Mintz S. Solitary intrabony neurofibroma of the maxilla. J Oral Maxillofac Surg 1997;55:768–772. [28] Sisson GA, Goldstein JC. Otolaryngology, vol. 3. Philadelphia: W.B. Saunders Company; 1973. p. 123. [29] Depprich R, Singh DD, Reinecke P, Kübler NR, Handschel J. Solitary submucous neurofibroma of the mandible: review of the literature and report of a rare case. Head Face Med 2009;5:24. http://dx.doi.org/10.1186/1746160X-5-24. [30] Gooder P, Farrington T. Extracranial neurilemmomata of the head and neck. J Larygol Otol 1980;94:243–249. [31] Polak M, Polak G, Brocheriou C, Vigneul J. Solitary neurofibroma of the mandible: case report and review of the literature. J Oral Maxillofac Surg 1989;47:65–68. [32] Deichler J, Martínez R, Niklander S, Seguel H, Marshall M, Esguep A. Solitary intraosseous neurofibroma of the mandible. Apropos of a case. Med Oral Patol Oral Cir Bucal 2011;16(6):704–707.