IP Journal of Otorhinolaryngology and Allied Science

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Singh, Poonia, Kumari, Bansal, and Aman: Adenoid cystic carcinoma of nasal septum: A review of literature

Case Report

A 43-year-old male patient came to ENT OPD with complaints of left nasal obstruction and bleeding from nose since 6 months. Nasal obstruction was insidious in onset and gradually progressive and commencing to complete left nasal obstruction since 2 months. Bleeding from nose was scanty in amount with 1-2 episodes per day. There were no complaints of recurrent cold, excessive sneezing, headache, facial fullness. Patient was a chronic smoker with 20 pack years, non alcoholic and vegetarian. On anterior rhinoscopy, a mass was seen in left nasal cavity which was sensitive to touch. Probe couldnot be passed on medial side. On nasal endoscopy, mass of size 3*2 cm was seen arising from nasal septum from mid of cartilaginous septum to mid of bony septum (Figure 1). On noncontrast computed tomography of paranasal sinuses soft tissue attenuation lesion in noted obstructing left nasal cavity and lesion in contact with partially visualised left inferior turbinate. Bony left inferior turbinate is not visualised, lesion is causing remodelling of medial wall of left maxillary sinus (Figure 2). On contrast enhanced MRI of nose and paranasal sinuses, there was well defined soft tissue lesion showing moderate heterogenous enhancement in left nasal cavity involving nasal septum, causing obstruction of left nasal cavity, abutting left inferior turbinate with obstruction of left maxillary sinus causing sinusitis, suggestive of neoplastic lesion (Figure 3).

Endoscopic nasal biopsy was taken under local anaesthesia which on histopathological examination showed luminal and adluminal proliferation of cells with scant cytoplasm forming pseudoglandular spaces with basement membrane material and mucin suggestive of adenoid cystic carcinoma. On immunohistochemistry, tumour cells were positive for CK-7, S-100 and CD-117 and negative for CK-20 and CEA.

Surgical excision was planned. By lateral rhinotomy incision, nasal septum was removed after taking 1 cm margins. Post operative radiotherapy was given to patient in 33 cycles over 6.5 weeks with 2Gy per cycle. After 2 years of follow up, there was no recurrence.

Figure 1
Figure 2
Figure 3


Nasal septal malignancies constitutes 2.4%-8.7% of all sinonasal malignancies. Adenoid cystic carcinoma is a common sinonasal malignancy but is very rare in nasal septum.6 It presents with non specific symptoms such as nasal obstruction and epistaxis. It is a slow growing tumour with invasion of perineurallymphatics. It is known to produce blood borne metastasis most commonly to lung. Metastasis to regional lymphatics is very rare.2 43 cases of septal tumours were described by young, out of which only 1 was ACC.7 Another study by Beatte et. Al. stated only one case of setal ACC out of total 85 cases.8

It is histologically of 3 types cribriform, tubular and solid. Cribriform is the most common type with best prognosis while solid is least common with worst prognosis. There is high incidence of recurrence and distant metastasis with solid type.9 Solid type ACC is further subdivided into 3 types by szantoet. al as grade 1 with 0 % solid component, garde 2 with less than 30% solid component and garde 3 with solid component more than 30%. 5 year survival rate was 95%, 65% & 14% respectively and 10 year survival rate as 76%,26% an d 5% respectively.10 On immunohistochemistry these stains positive for smooth muscle actin, S100,vimentin, smooth muscle myosin heavy chain, CD117 and myeloblastosis oncogene.11

Its treatment is generally combined surgery and radiotherapy.12 Surgical approach depend on the tumour size and location. Small and localised tumours can be excised via endoscopic approach while larger tumours requires lateral rhinotomy approach. Mid facial degloving and lateral rhinotomy with sublabialincisons may be required for lower septal and posterior septal tumours.13 Post op radiotherapy is given with a total dose of 66-70 Gy in 2Gy per fraction with five fractions per week. In a study by Wiseman et. al , it was seen that local recurrence rate was lower in group of surgery with post op radiotherapy than surgery alone.12 In a study by Horiuchi et. al, he stated that minimum 50 Gy radiation must be given post operatively to effectively reduce local recurrence.14 Chemotherapy is ineffective in its treatment. Radiotherapy doesn’t have any effect on cure but it reduces its recurrence by treating residual microscopic disease.15 Long term follow up is required for its slow growth and high local recurrence and distant metastais.

Table 1










Tai et. Al6 ,2007





Surgery by lateral rhinotomy Radiotherapy

1.5 yrs follow up, no recurrence

Akiyama et. Al15 ,2013






Anterior canialfossa surgery *ensoscopic nasal surgery*transpalatal approach radiotherapy

9 months follow up, no reccurence

Lit Yee et. Al16, 2018





Endoscopic excision with radiotherpay

15 months follow up , lung metastasis were found

Handa et. Al17, 1992





Nasal pain

Lateral rhinotomy No radiotherapy

2 yrs follow up, no recurrence

Schneiderman et. Al18 ,2002





Intranasal resection No radiotherapy

9 months

Sivaji et. Al19 ,2003





Lateral rhinotomy with radiotherapy

Presented in very few days of follow up after treatment

Konadir A &Hassoumi K20, 2018





Endoscopic resection with post op radiotherapy

1yr and 9 months, no recurrence

Beladavar B, Batra R5 ,2018

60 yr




No treatment explained

Priya SR, Chaukar D, D’Cruz A,2016





Excision by lateral rhinotomy

15 months, no reccurence

Priya SR, Chaukar D, D’Cruz A,2016





Excision was done at some other center 3 years ago ,technique not explained

3 yrs of follow up, no recurrence

Review of Literature

On reviewing literature on ACC of nasal septum, we could find only 10 cases out of which 4 were females and 6 were males. Average age of presentation was 53.7 years which is 56.5 years in males and 49.5 years in females. Thus, female have an early age of presentation of this tumour. In reported cases from india, out of four, three are males showing male predominance with average age of presentation 56.75 years and of males 53.3 years and only female was of 64 years of age. Treatment was given in 9 patients. Maximum duration of follow up was of 3 years by Priya SR and minimum duration of follow up was just after the treatment with an average of 16.33 months. Recurrence was seen in only one report after a follow up of 15 months to lung.


ACC of nasal septum is a very rare malignancy. ACC of nasal septum has early presentation because of early nasal obstruction and early nasal bleeding, in comparison to ACC of maxillary sinus which appears usually in t3/t4 stage after bony erosions and rhinological, opthalmic and palatal symptoms.

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J Hicham M Rachid Giant Adenoid cystic carcinoma of the nasal cavityJ OtolRhinolLaryngol2005864143


M K Bhayani M Yener A E Naggar Prognosis and risk factors for early-stage adenoid cystic carcinoma of the major salivary glandsCancer20121181128728010.1002/cncr.26549


Z Gil D L Carlson A Gupta Patterns and incidence of neural invasion in patients with cancers of the paranasal sinusesArch Otolaryngol Head Neck Surg200913521738210.1001/archoto.2008.525


B S Gendeh H Zahedi T Y Ahmad Adenoid Cystic Carcinoma of theSinonasal Tract: Outcome of Endonasal Endoscopic Surgery at Five-Year Follow upJ Laryngol Otol20131275511610.1017/S0022215113000480


B P Belaldavar R Batra Adenoid cysticcarcinoma of the nasal septum: A rare case reportJ Sci Soc2013401394010.4103/0974-5009.109699


S Y Tai C Y Chien C F Tai Nasal septum adenoid cystic carcinoma: A case reportKaohsiung J Med Sci20072384265610.1016/S0257-5655(07)70008-1


J R Young Malignant tumors of the nasal septumJ Laryngol Otol19799381749


C W Beatty B W Pearson E B Kern Carcinoma of the nasal septum, experiencewith 85 casesOtolaryngol Head Neck Surg198290190410.1177/019459988209000116


K H Perzin P Gullane A C Clairmont Adenoid cystic carcinomas arising insalivary glands: a correlation of histologic features and clinical courseCancer19784212658210.1002/1097-0142(197807)42:1<265::aid-cncr2820420141>3.0.co;2-z


P A Szanto M A Luna M E Tortoledo R A White Histologic grading of adenoidcystic carcinoma of the salivary glandsCancer198454610627110.1002/1097-0142(19840915)54:6<1062::aid-cncr2820540622>3.0.co;2-e


P M Dillon S Chakraborty C A Moskaluk Adenoid cystic carcinoma: A review of recent advances, molecular targets, and clinical trialsHead Neck2016384620710.1002/hed.23925


S M Wiseman S R Popat N R Rigual Adenoid cysticcarcinoma of the paranasal sinuses or nasal cavity: A 40-yearreview of 35 casesEar Nose Throat J20028185107


P Wardas M Tymowskim P Seweryna Endoscopic approach to the resection of adenoid cystic carcinoma of paranasal sinuses and nasal cavity: case report and own experienceEur J Med Res2015209710.1186/s40001-015-0189-2


J Horiuchi H Shibuya S Suzuki M Takeda M Takagi The role of radiotherapy inthe management of adenoid cystic carcinoma of the head and neckInt J RadiatOncolBiol Phys198713811357610.1016/0360-3016(87)90185-4


K Akiyama M Karaki H Hosikawa A massive adenoid cystic carcinoma of nasal septum progressed into the skull baseAuris Nasus Larynx20134022398110.1016/j.anl.2012.02.006


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Review Article

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Authors Details

Jagat Singh, Usha Poonia, Manisha Kumari, Sukriti Bansal, Aman

Article History

Received : 19-08-2021

Accepted : 08-09-2021

Available online : 01-11-2021

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