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Advanced Surgical Techniques and Procedures (ASTP) © All rights are reserved by Salina Husain, et al.

Case Report Open Access

An Cystic Carcinoma of Origin Loo Lit Yee1, Salina Husain1, Farah Dayana Zahedi1, Revadi Govindaraju2, Shahrul Hitam2 1Department of Otorhinolaryngology Head and Neck Surgery, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur. 2Department of Otorhinolaryngology, Hospital Ampang, Ampang, Selangor.

Abstract Background: Adenoid cystic carcinoma (ACC) of the nasal septum is a rare disease of the head and neck. This elusive is despite its locoregional control improvement over the years may cause distant metastasis years after definitive treatment. Reliable prognostic factors were identified over the years and their implications are immense especially with regards counseling and therapeutic planning. Case Report: We present a case of nasal septum ACC with perineural invasion. A 54 years old male presented with recurrent mild epistaxis for two months duration with associated right nasal obstruction. Endoscopic excision of the tumor was performed. The patient received adjuvant radiotherapy due to involved resection margins and perineural invasion. Conclusion: Nasal septal ACC is a rare entity. Adjuvant radiotherapy is advocated as a part of treatment due to its characteristic perineural spread. Lifelong follow up is necessary due to its high recurrence rate. Keywords: Adenoid Cystic Carcinoma; Septum; Epistaxis

Introduction Magnetic resonance imaging (MRI) and computed tomography (CT) of the (PNS) showed an enhancing soft tissue Adenoid cystic carcinoma (ACC) was first described as cylindro- mass confined in the right causing septal deviation to the ma by Bilroth in 1856 [1]. ACC is a rare form of malignancy left [Figure 1 & 2]. The mass measured 33 x 17 x 28mm. It extended representing about 5% of all head and neck carcinomas [2]. Sino nasal superiorly to the right anterior ethmoid air cells with no intracranial ACC accounts for 10-20% of all head and neck ACC [3]. The most extension. He was staged T3N0M0. common site of the disease is the followed by the nasal cavity, nasopharynx and [4]. It has a tendency to A right endoscopic sinus surgery with wide local excision of the progress slowly with a characteristic perineural invasion. We present nasal septal mass was performed. The histopathological examination a rare case of nasal septum ACC. of the mass confirmed the diagnosis of adenoid cystic carcinoma, Case Report predominantly of cribriform type with perineural infiltration [Figure 3] and positive margin. He received radiotherapy with a total dose of A 54 years old male presented with recurrent mild epistaxis for 66 Gy in 33 fractions. The patient was asymptomatic until at 15 two months duration with associated right nasal obstruction. He is a months follow up when a CT Thorax revealed lung metastasis. He smoker with a background history of hypertension, benign prostatic was counseled for further treatment but he refused. hyperplasia and prolapsed intervertebral disc at the level between the fourth and fifth lumbar vertebrae. The patient had no family history Discussion of malignancy. Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm Naso-endoscopic examination revealed a friable mass originating representing about 5% of all head and neck carcinomas [2]. Sinonasal from the posterior part of the right nasal septum extending to the ACC can affect any sites within the nasal cavity. The commonest site floor of the nose. The ostiomeatal complex, spheno-ethmoidal recess of sinonasal ACC was the ethmoid sinus [4]. However, other studies and nasopharynx were normal. There were no palpable cervical found that the most common sinonasal ACC was in the maxillary nodes. Per oral and otoscopic examinations were unremarkable. sinus [3]. In this case, the presence of ACC arising from the septum made it more interesting. Six reports of ACC of the nasal septum had been available in the literature rendering the rarity of the disease [5]. *Address for Correspondence: Dr. Salina Husain, Department of Otorhi- nolaryngology Head and Neck Surgery, National University of Malaysia, Histologically, ACC is classified into the commonest cribriform Malaysia Medical Center, JalanYaacob Latiff, Bandar TunRazak, 56000, type, tubular type and the least common solid type. The classification Cheras, Kuala Lumpur, Fax :+60391456675; Tel: +6039146599; E-mail: correlates with the disease prognosis. The cribriform type reported to [email protected] have the best outcome and the solid type had the worst prognosis [6, Received: February 15, 2018; Accepted: April 27, 2018; Published: April 30, 7]. In addition, the staging of the tumour, involvement of perineural 2018 invasion and margin of resection also play an important role in the

Adv Surg Tech Proc, 2018 Volume 1(1): 1 - 3 Husain S, Yee LL, Zahedi1 FD, Govindaraju R,Hitam S, Zahedi FD. An Adenoid Cystic Carcinoma of Nasal Septum Origin. Adv Surg Tech Proc. 2018; 1(1): 1-3. prognosis of the disease. T3 and T4 tumours are associated with much higher Perineural invasion maybe demonstrated on MRI scan as fat incidence of perineural invasion compare to T1 and T2 tumours (60% vs. planes obliteration, enlargement or destruction of foramina, enla- 23.5%) [8]. rgement or enhancement of , neuropathic atrophy and convexity of the lateral sinus wall and replacement of the trigeminal subarachnoid cistern with soft tissue [9]. However, the imaging studies in our patient revealed the mass confined to the nasal cavity without perineural invasion. Endoscopic sinus surgery and wide local excision of the mass on the posterior part of the septum was performed. The nasal septum in our case was not involved. Should the nasal septum cartilage be involved, there are several approaches to gain margin clearance depending on the localization of the lesion on the septum. If the anterior part of the septum is involved, lateral rhinotomy would be the option of choice. Mid-facial degloving approach can be utilized for lower nasal septum involvement with good cosmesis. If the posterior nasal septal area is involved, adequate exposure is allowed by using lateral rhinotomy with sublabial incision. To resect tumor of the nasal septum and floor of the nose, lateral rhinotomy with -splitting incision is the choice of approach [5]. Figure 1: MRI coronal view showing a mass arising from the nasal septum with deviation to the left. Due to the neurotropism nature of the ACC, current treatment recommendation is complete surgical resection with post-operative radiotherapy (RT) [10]. This was supported by the findings by a study by Garden et al demonstrating local control rates of 95%, 86% and 79% at 5, 10, and 15 year in 198 patients with ACC of the head and neck treated with surgery and then followed by RT [11]. Total removal of the tumour with negative tissue margin is vital to a successful combined therapy. Adjuvant RT was indicated in our patient in view of positive resection margin and the presence of perineural invasion on histopathological examination. Recurrence rate of ACC proved to be a notorious. It typically recurs locally. Recurrence rate is reported to be as high as 65% in sinonasal ACC despite surgical excision followed by RT [12]. Combined treatment for ACC of head and neck origin achieved 85% freedom from relapse and disease-specific survival at 5, 10, and 15 years was 89%, 67.4% and 39.6% respectively. Distant failure occurs predominantly within 5 years but it was registered even after 15 years [8]. Figure 2: MRI axial view showing an enhanced mass arising from the nasal septum. Our patient whom unfortunately had T3 tumour, perineural invasion and a positive margin may have an unfavorable prognosis. He predictably developed distant failure 15 months after the initial treatment. Conclusion Nasal septal ACC is a rare entity. Adjuvant radiotherapy is advocated as a part of treatment due to its characteristic perineural spread. Lifelong follow up is necessary due to its high recurrence rate.

References

1. Bertel Grahne, Christian Laurén and Lars R Holsti. Clinical and Histological Malignancy of Adenoid Cystic Carcinoma. J Laryngol Otol. 1977; 91:743-749. [Crossref] 2. PavelDulguerov, Michael S Jacobsen, Abdelkarim S Allal, Willy Lehmann and Thomas Calcaterra. Nasal and Paranasal Sinus Carcinoma: Are We Making Progress?Cancer. 2001; 92:3012-3029. [Crossref] 3. Chae‐Seo Rhee, Tae‐Bin Won, ChulHee Lee, Yang‐Gi Min, Myung‐Whun Sung, Figure 3: Histopathological slide showing adenoid cystic carcinoma with Kwang‐Hyun Kim, et al. 'Adenoid Cystic Carcinoma of the Sinonasal Tract: perineural infiltration. Treatment Results'. Laryngoscope. 2006; 11. [Crossref]

Adv Surg Tech Proc, 2018 Volume 1(1): 2 - 3 Husain S, Yee LL, Zahedi1 FD, Govindaraju R,Hitam S, Zahedi FD. An Adenoid Cystic Carcinoma of Nasal Septum Origin. Adv Surg Tech Proc. 2018; 1(1): 1-3.

4. BS Gendeh, FD Zahedi, H Ahmad and TY Kew. Adenoid Cystic Carcinoma of the 9. Karen S Caldemeyer, Vincent P Mathews, Paul D Righi and Richard R Smith. Imaging Sinonasal Tract: Outcome of Endonasal Endoscopic Surgery at Five-Year Follow up. J Features and Clinical Significance of Perineural Spread or Extension of Head and Laryngol Otol. 2013; 127:511-515. [Crossref] Neck Tumors. Radiographics. 1998; 18:97-110. [Crossref] 5. Shu-Yu Tai, Chen-Yu Chien, Chih-Feng Tai, Wen-ReiKuo, Wan-Ting Huang, Ling- 10. Chunying Shen, TingtingXu, Caiping Huang, Chaosu Hu and Shaoqin He. Treatment Feng Wang, et al. Nasal Septum Adenoid Cystic Carcinoma: A Case Report. Outcomes and Prognostic Features in Adenoid Cystic Carcinoma Originated from the Kaohsiung J med sci. 2007; 23:426-430. [Crossref] Head and Neck. Oral oncol. 2012; 48: 445-449. [Crossref] 6. Jim Fordice, Corey Kershaw, Adel El-Naggar and HelmuthGoepfert. Adenoid Cystic 11. Adam S Garden, Randal S Weber, William H Morrison, K KianAng and Lester J Carcinoma of the Head and Neck: Predictors of Morbidity and Mortality. Arch Peters. The Influence of Positive Margins and Invasion in Adenoid Cystic Otolaryngol Head Neck Surg. 1999; 125:149-152. [Crossref] Carcinoma of the Head and Neck Treated with Surgery and Radiation. Int J Radiation Oncol Biol Physics. 1995; 32:619-626. [Crossref] 7. Sam M Wiseman, Saurin R Popat, Nestor R Rigual, Wesley L Hicks, Orner JB, Wein RO, et al. Adenoid Cystic Carcinoma of the Paranasal Sinuses or Nasal Cavity: A 40- 12. Allison D Lupinetti, Dianna B Roberts, Michelle D Williams, Michael E Kupferman, Year Review of 35 Cases. Ear Nose J. 2002; 81:510-514. [Crossref] David I Rosenthal, Franco Demonte, et al. Sinonasal Adenoid Cystic Carcinoma. Cancer. 2007; 110:2726-2731. [Crossref] 8. H Kokemueller, AEckardt, P Brachvogel and J-E Hausamen. Adenoid Cystic Carcinoma of the Head and Neck-a 20 Years’ Experience'. Int J Oral Maxillofac Surg. 2004; 33:25-31. [Crossref]

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