Malignant peripheral nerve sheath tumour (MPNST) is a very rare entity in head and neck with high rate of recurrences and local invasiveness. This tumour is usually found in lower extremities and only 10-12% occur in head and neck region. The diagnosis is considered as the most elusive and difficult among soft tissue sarcomas because of its non specific presentation, both clinically as well as pathologically. This difficulty has now been overcome by immunohistochemistry. We report here a case of MPNST in a 50 years old male with a localized right maxillary growth

Aftab, Kanwal

Ali, Naeem Sultan

Junaid, Montasir

eCommons@AKU

eCommons@AKUSection of Otolaryngology, Head & Neck Surgery Department of SurgeryJuly 2011Malignant peripheral nerve sheath tumour ofmaxillaNaeem Sultan AliAga Khan UniversityMontasir JunaidAga Khan UniversityKanwal AftabAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neckPart of the Otolaryngology CommonsRecommended CitationAli, N., Junaid, M., Aftab, K. (2011). Malignant peripheral nerve sheath tumour of maxilla. Journal of the College of Physicians andSurgeons Pakistan, 21(7), 420-2.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neck/1420 Journal of the College of Physicians and Surgeons Pakistan  2011, Vol. 21 (7): 420-422INTRODUCTIONMalignant peripheral nerve sheath tumours (MPNST)are rare soft tissue sarcomas of ectomesenchymalorigin. They are considered highly malignant sarcomaswhich are locally invasive, associated with high rates ofrecurrences and metastatic spread.1 This tumour isusually seen in lower extremities and retroperitonealregion and is rare in the head and neck region. TheWorld Health Organization coined the term MPNST tosubstitute the previously heterogeneous and oftenconfusing terms, such as malignant schwannoma,malignant neurilemmoma, neurogenic sarcoma, andneurofibrosarcoma. Cases of MPNSTs have beenreported mostly in the soft tissues, less common in thebones and very rare in the maxilla.2,3 We report a caseof MPNST in a 50 years old male with a right maxillarymass.CASE REPORTA 50-year-old male patient complained of swelling onthe right side of face since 4 months. He gave a historyof swelling, which was initially small, roughly marblesized and progressively increased in size over the pastfour months. It was not associated with any dental pain.There was no fluctuation in the size of the swelling.Patient also did not have any history of epistaxis,speech, chewing or breathing difficulty, numbness overthe face or blurring of vision. Past medical, dental andsurgical histories were insignificant. His personal historyrevealed that he was a chronic tobacco chewer andsmoker.Extra oral examination showed an ovoid swellingmeasuring 6x5 cm on the right middle third of face justbelow the eye. The skin over the swelling was stretchedand surrounding tissue appeared normal. This swellingwas firm, non-compressible, nonfluctuant and non-reducible. Upon intra oral examination no visible ulcer orswelling or loose teeth identified. Mouth opening wasadequate. Nasal endoscopy showed a soft tissue bulgeon its lateral wall.Based on history and clinical examination, a provisionaldiagnosis of antral malignancy was given and differentialdiagnosis included squamous cell carcinoma, muco-epideroid carcinoma, adenoid cystic carcinoma, chondro-sarcoma and osteosarcoma.CT scan showed destruction of maxilla on right side,which was associated with soft tissue replacement(Figure 1). The lesion was extending into orbit, ethomoidsinus and pterygopalatine fossa. Incisional biopsy wastaken via sublabial approach. Histopathological findingswere suggestive of a spindle cell malignancy. Thepatient then underwent hemimaxillectomy. The tumourwas visualized bulging out from the antrum extendingposteriorly up to pterygoid muscles, superiorly orbitalfat, laterally buccal fat and cheek muscles (not involvingthem) and medially eroding the medial wall of maxillarysinus. Floor of the sinus was found spared.Histopathologic studies revealed a circumscribed cellularneoplastic lesion composed of spindle cells arranged ina storiform pattern. Lesion consisting of fusiform cellsand epithelial cells with marked pleomorphism andmultinucleated giant cells. The diagnosis was sarcomaof a high grade malignancy. Immunohistochemicalstudies showed tumour cells negative for cytokeratines,actin, desmin, myoglobin and factor VIII, however, it waspositive to Protein S-100 (Figure 2). This led to the finaldiagnosis of malignant peripheral nerve sheath tumourof the maxilla.The patient was then referred to radiation oncologyservice for adjuvant radiation. He is on regular follow-upfor past 8 months and has presented no evidence oflocal recurrence. ABSTRACTMalignant peripheral nerve sheath tumour (MPNST) is a very rare entity in head and neck with high rate of recurrencesand local invasiveness. This tumour is usually found in lower extremities and only 10-12% occur in head and neck region.The diagnosis is considered as the most elusive and difficult among soft tissue sarcomas because of its non specificpresentation, both clinically as well as pathologically. This difficulty has now been overcome by immunohistochemistry. Wereport here a case of MPNST in a 50 years old male with a localized right maxillary growth.Key words: Malignant peripheral nerve sheath tumour (MPNST).    Neurofibromatosis.   Soft tissue sarcoma.   S-100 protein.Department of ENT1 / Pathology2, The Aga Khan UniversityHospital, Karachi.Correspondence: Dr. Naeem Sultan Ali, DD-115, KarimabadColony, Block No. 3, F.B. Area, Karachi-75950.E-mail: naeem.sultanali@gmail.comReceived  April 20, 2010;  accepted  May 10, 2011.Malignant Peripheral Nerve Sheath Tumour of Maxilla Naeem Sultan Ali1, Montasir Junaid1 and Kanwal Aftab2CASE REPORTDISCUSSIONMPNST is an aggressive sarcoma of neural originshowing close association with peripheral nervesor neurofibroma or may show features of neuraldifferentiation. Most of these nerve sheath tumours innose and paranasal sinuses arise from ophthalmic andmaxillary branches of trigeminal nerve and its branchesalthough it is difficult to identify exactly which nerve isinvolved.4 The most common sites involved aremaxillary and ethmoid sinuses.5Malignant peripheral nerve sheath tumours can arise denovo or as a malignant transformation of neuro-fibromatosis-1 (NF-1) gene. Approximately 50% ofMPNST are associated with a family history of vonRecklinghausen's disease. Since this patient denied ofany such family history it was possibly a de novo case.The risk of developing MPNST in patients with NF-1 isapproximately 2-4%. It is also seen to arise in patientspreviously treated with radiotherapy.6These cases have been reported in all age groups, butpeek incidence is seen commonly in second and fourthdecade of life with an equal male and femalepredilection.7 MPNSTs are commonly seen in lowerextremities and retroperitonium followed by trunk, upperextremities and head and neck regions. In head andneck, frequent sites include nasopharynx, paranasalsinus, nasal cavity, orbit, oral cavity, parapharyngealregion, neck, thyroid gland and larynx.8,9The clinical presentations differ as per site involved. Butin case of maxilla, they may present with facial and/ororbital swelling, paraesthesia of cheek, epiphora, nasaldischarge or epistaxis (if involving nasal cavity). Sincethese tumours are aggressive they have tendency toerode into adjacent bones and tissue. Two third of theselesions are greater than 5 cm at the time of presentation.This tumour can spread through hematogenous routeand by perineural extension. Lymph node metastasis israre. Roof of orbit and floor of maxilla was not erodedin this case. However, the anterior wall was deficientcompletely. No nodal metastasis was found in thispatient.Histologically, these tumours have no defined, classicappearance. Commonly described findings are thepresence of spindle cells with high mitotic rate andindistinct cytoplasmic borders arranged in bundles orfascicles. Immunohistochemistry plays an important partin the diagnosis and in excluding fibrosarcoma, synovialsarcoma and fibrous histiocytoma. MPNST specificallydemonstrates S-100 positivity.The recommended treatment of MPNST is the surgicalextirpation including wide margins. In this patienthemimaxillectomy via Weber-Ferguson incision wasopted. Elective neck dissection is not recommendedunless there is positive radiological and/or clinicalevidence of nodal metastasis. The role of radiotherapyand chemotherapy is still controversial. This tumour hasbeen reported radioresistant, but some authors reporteduse of complementary radiotherapy.10 However, theprognosis is poor and even worse in patient with NF-1disease.REFERENCES1. Patil K, Mahima VG, Ambika L. Malignant peripheral nervesheath tumour: an elusive diagnosis. Indian J Dent Res 2007; 18:19-22.2. Kar M, Deo SV, Shukla NK, Malik A, DattaGupta S, Mohanti BK,et al. Malignant peripheral nerve sheath tumours (MPNST) -clinicopathological study and treatment outcome of twenty-fourcases. World J Surg Oncol 2006; 4:55.3. Lesic A, Bumbasirevic M, Atkinson HD, Maksimovic R, Sopta J,Atanackovic M. Malignant intraosseous peripheral nerve sheathtumour of the proximal femur: a case report. J Orthop Surg 2006;14:84-9.4. Beegun I, Bottrill ID, Hollowood K. Malignant peripheral nervesheath tumours of the infraorbital nerve: case report andJournal of the College of Physicians and Surgeons Pakistan  2011, Vol. 21 (7): 420-422 421Malignant peripheral nerve sheath tumour of maxillaFigure 1:  Pre-operative axial CT shows right maxillary mass.Figure 2: Patchy immunoreactivity for S-100 protein in the tumour cells(immunohistochemical stain, 20x).literature review. J Laryngol Otol 2009; 123:466-70. Epub 2008May 20.5. Kim HJ, Kim CH, Kim H, Yoon JH. Malignant peripheral nervesheath tumour of the paranasal sinus. J Otolaryngol 2007; 36:E9-11.6. Makimoto Y, Yamamoto S, Takano H, Motoori K, Ueda T,Kazama T, et al. Imaging findings of radiation-induced sarcomaof the head and neck. Br J Radiol 2007; 80:790-7. Epub2007 Oct 1.7. Martinez Devesa P, Mitchell TE, Scott I, Moffat DA. Malignantperipheral nerve sheath tumours of the head and neck: twocases and a review of the literature. Ear Nose Throat J 2006;85:392-6.8. Cheng SF, Chen YI, Chang CY, Peng Y, Liao SL. Malignantperipheral nerve sheath tumour of the orbit: malignanttransformation from neurofibroma without neurofibromatosis.Ophthal Plast Reconstr Surg 2008; 24:413-5.9. Sabesan T, Hussein K, Ilankovan V. Malignant peripheral nervesheath tumour of the parapharyngeal space in a patient withneurofibromatosis type 1. Br J Oral Maxillofac Surg 2008; 46:585-7.Epub 2008 Mar 18.10. Minovi A, Basten O, Hunter B, Draf W, Bockmühl U. Malignantperipheral nerve sheath tumours of the head and neck:management of 10 cases and literature review. Head Neck 2007;29:439-45.422 Journal of the College of Physicians and Surgeons Pakistan  2011, Vol. 21 (7): 420-422Naeem Sultan Ali, Montasir Junaid and Kanwal Aftabl l l l lOl l l l l

Malignant peripheral nerve sheath tumour of maxilla

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Malignant peripheral nerve sheath tumour of maxilla

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