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INTRODUCTION

Adenoid cystic carcinoma (ACC) accounts for approximately 10% of
salivary gland tumours. It is the most common malignant tumour of the
submandibular gland and minor salivary glands however, rare in nasal cavity and
paranasal sinuses accounting for less than <5% cases.1,2 The age of the patients range from 20 to 84 years, with a median age of 52 years.1 It is common in females compared to males with a ratio of 2:1.3 The most common site affected is the maxillary sinus followed by nasal cavity.4Tumours of the sinonasal tract commonly present with symptoms that are identical to those caused by inflammatory sinus disease, such as nasal obstruction, nasal discharge, epistaxis, headache, facial pain and cheek swelling.3Histologically,growth patterns are characterized as cribriform, tubular and solid. As the cribriform pattern of the tumour forms cylindrical accumulations of basal lumina, glycosaminoglycans and stroma, the term cylindroma had been applied in the past.5 Although slow growing, these tumours have a propensity for frequent local recurrence and early perineural and haematogenous spread to lungs.6 Tumours showing perineural invasion, cervical lymph node metastasis, solid histological features and distant metastasis are associated with increased treatment failures and recurrences.3 Until today, most sources agree that aggressive treatment of these tumours is necessary. The mainstay of treatment of ACC is surgery; adjuvant radiation therapy is reserved in case of positive margins or advanced stage.3,4 CASE REPORT Here we present a case of 13year old Indian boy with mass in the nasal cavity and epistaxis. The naso-endoscope examinations revealed a mass in the posterior right nasal cavity which was   friable and bleeding easily. Clinically, possibility of juvenile nasopharyngeal angiofibroma was given. Computed tomography (CT) scan revealed heterogeneously enhancing lobulated mass lesion in the nasopharynx/ posterior nasal cavity (Fig. 1). Thin bony septae are seen within it. Anteriorly, the mass is extending into bilateral posterior part of nasal cavity mainly into the right side, through posterior choanae and causing lateral bowing of medial wall of the right maxillary sinus and left deviation of nasal septum. Destruction of floor of sphenoid sinus was noted superiorly. The mass measured 5x4x3.5cm. There were no neck nodes or distant metastases. Neoplastic lesion in nasopharyngeal region was suggested. The patient then underwent endoscopic removal of mass and sent for histopathological examination. On gross examination, multiple grey brown to grey tan soft to form tissue pieces were noted altogether measuring 5x4x2cm with two turbinates measuring 3x0.6cm each.  Microscopy revealed presence of a malignant tumour predominantly arranged in cribriform pattern comprising of islands of small uniform basaloid tumour cells with presence of microcystic spaces filled with basophilic mucoid material (Fig. 2,3).Periodic acid Schiff (PAS) stain was positive in mucoid luminal material. Focally, tubular pattern was also seen showing smaller duct like arrays of basaloid epithelial cells surrounding a central lumen like space. Surrounding stroma was hyalinised and showed mild chronic inflammatory cell infiltrate. Perineural invasion noted (Fig. 4). Rare mitosis was seen.  Bony spicules and turbinates were free of tumour. The final diagnosis of Adenoid cystic carcinoma was made. The patient was referred to higher centre for postoperative radiationtherapy. DISCUSSION ACC was first described by Billroth in 1856.5ACC is a rare tumour of epithelial cell origin comprising 3 to 5% of all head and neck malignancies.7They usually arise in the major and minor salivary glands, but can occur in all sites comprising secretory glands (breast, cervix, colon, prostate).2 The peak incidence is from the fourth decade to the sixth decade, although cases have been reported in between ages of 11 to 90 years.3,6 The maxillary sinus is the most common site of these tumours followed by the nasal cavity, as reported previously.8 Common symptoms of sinonasal ACC include nasal obstruction, facial pain, epistaxis, nasal discharge, headache and loss of smell, the same symptoms observed in patients with sinusitis and inflammatory nasal conditions. This can cause a delay in diagnosis and treatment.3,8 ACC can be composed of ³1 histopathologic subtype(s), including tubular, cribriform, and solid. Some studies have emphasized that histologic pattern correlates with prognosis (tubular = best, cribriform = intermediate and solid = worst).1Cribriform is known as the most common type,8 which also was demonstrated in the current study. ACC of the head and neck, and specifically of the nasal cavity and paranasal sinuses, poses numerous treatment challenges for many reasons: it has a high propensity for local invasion to adjacent structures, making resection more difficult; it is commonly diagnosed late due to its insidious growth; and in 50% of cases it has already exhibited perineural spread at the time of diagnosis.7as noted in our case also. There are studies which indicate that surgery followed by radiotherapy is the most common treatment for patients with sinonasal ACC,5 which was the treatment given in our case. Overall 5-year survival rates for patients with sinonasal ACC from 50% to 86% have been reported.9,10 ACC of the nasal cavity is a rare clinical entity posing diagnostic and therapeutic challenges. Therefore, any patient with suspicious malignancy needs to be investigated thoroughly by histological diagnosis. It exhibits extensive local tissue infiltration and perineural spread, which results in a high rate of recurrence despite aggressive surgical resection. High index of suspicion is required for early diagnosis and optimal management.   CONFLICT OF INTEREST None REFERENCES 1.           Mills SE. Salivary Glands. Ch 20. In: Mills SE, Carter D, Greenson JK, Reuter VE, Stoler MH (eds).Sternberg's Diagnostic Surgical Pathology. 5thed, Vol 1. Philadelphia: Lippincott Williams & Wilkins ; 2010:836 2.           Michel G, Joubert M, Delemazure AS, Espitalier F, Durand N, Malard O. Adenoid cystic carcinoma of the paranasal sinuses: Retrospective series and review of the literature. European Annals of Otorhinolaryngology, Head and Neck diseases 2013;130: 257—262 3.           Ghani NMSNA, Shukri NM, Mohamad A. 2 Case reports of sinonasal adenoid cystic carcinoma: Review of the literature on surgical approaches. Egyptian Journal of Ear, Nose, Throat and Allied Sciences 2016; 17:173–176 4.           Amit M, Binenbaum Y, Sharma K, Naomi R, Ilana R, Abib A et al. Adenoid cystic carcinoma of the nasal cavity and paranasal sinuses: a meta- analysis.J Neurol Surg  2013;74:118–125 5.           Purushothaman PK, Ramakrishnan R, Sindhu P. Adenoid Cystic Carcinoma of Nasal Cavity: A Rare Presentation Case Report. International Journal of Scientific and Research Publications 2015;5(2): 1–3 6.           Kumar VP, Rao PN, Kumar GA. Adenoid cystic carcinoma of nasal cavity- a case report. Indian J Otolaryngol Head Neck Surg 2003 Mar;55(1):43-46 7.         Gill KS, Fratalli MA. An unusual presentation of Adenoid Cystic Carcinoma. Case Reports in Otolaryngology 2015:1-4 8.         Lupinetti AD, Roberts DB, Williams MD, Kupferman ME, Rosenthal DI, Demonte F,et al. Sinonasal adenoid cystic carcinoma: The M. D. Anderson Cancer Center experience. Cancer 2007;110(12):2726–2731 9.           Rhee CS, Won TB, Lee CH, Min YG, Sung MW, Kim KH, Shim WS, Kim YM, Kim JW. Adenoid cystic carcinoma of the sinonasal tract: treatment results. Laryngoscope. 2006; 116(6):982–986 10.   Myers LL, Nussenbaum B, Bradford CR, Teknos TN, Esclamado RM, Wolf GT. Paranasal sinus malignancies: an 18-year single institution experience. Laryngoscope. 2002; 112: 1964–1969

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