Acta Medica 2013; 2: 59–64 acta medica CASE R EPORT Nasopharyngeal Adenoid Cystic Carcinoma Case Report and Review Melahat DÖNMEZ1*, [MD] Onur ORAL2, [MD] Yıldız ALBAYRAK2, [MD] Pınar NERCIŞ3, [MD] Sema HÜCÜMENOĞLU, [MD] A BST R AC T Adenoid Cystic Carcinoma (ACC) is a rare slow-growing salivary gland neoplasm with malignant behavior and a high recurrence rate. It originates from salivary glands. Nasopharyngeal located ACC (NACC) is a very rare malignancy due to its location with specific biological characteristics. There is no consensus at present regarding clinical characteristics, treatment approaches and prognostic factors. Our aim is to review the literature related to this topic in light of our case about this rare malignancy. A 36-year-old female at 35 weeks pregnancy was admitted to the Gynecology and Obstetrics service for hypertension. The patient had a history of a nasopharyngeal carcinoma for which she underwent radiotherapy 11 years ago. A neurology and eye consultation required for ptosis of her left eye An orbito-cranial MR demonstrated expansive mass lesions that were initially thought to be mucoceles filling the nasal cavity, left maxillary and sphenoid sinuses. A biopsy of the lesions was reported as ‘adenoid cystic carcinoma’. The patient died 6 months later. 1 Department of Pathology, Ministry of Health Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey 2 Department of Pathology, Ministry of Health Ankara Training and Research Hospital, Ankara, Turkey 3 Department of Radiology, Ministry of Health Ankara Training and Research Hospital, Ankara, Turkey * Corresponding Author: Melahat Donmez Ministry of Health Diskapi Yildirim Beyazit Training and Research Hospital, Department of Pathology, Ankara-TURKEY [email protected] Our case is special due to the patient’s arrival with cranial nerve invasion, slow progression, expansive growth pattern, misdiagnosis as a mucocele clinically and radiographically. Because of its high rate of cranial nerve invasion, this neoplasm must be considered in the differential diagnosis. In sinonasal mass lesions with nasal obstructive symptoms, the possibility of malignancy must be eliminated. Despite the fact that papillomas spread expansively, a biopsy must be obtained for prompt diagnosis and treatment. Additionally patients diagnosed with nasopharyngeal carcinoma, even if they are treated and cured, must be called for control in routine intervals because of the malignant behavior and high recurrence rate. Key words: Primary salivary gland type nasopharyngeal carcinoma (SNPC), perineural invasion, distant metastasis, malignancy, salivary gland neoplasm Received 8 October 2013, Accepted 28 October 2013 Published online 31 October 2013 Introduction Adenoid Cystic Carcinoma (ACC) is a rare, slow-growing salivary gland neoplasm with malignant behavior and a high recurrence rate. It originates from secretory glands in the major and minor salivary glands. It is seen in the palate, the buccal mucosa, lips and floor of the mouth in decreasing frequency [1-4] . In nasopharyngeal location, the most common malignant epithelial tumor is ‘nonkeratinized squamous cell carcinoma’. Primary salivary gland type nasopharyngeal carcinoma (SNPC) is highly rare and consists of 0.48% of all nasopharyngeal carcinomas . Therefore, its clinical characteristics, © 2013 Acta Medica. All rights reserved. treatment approaches, and prognostic factors are not completely known  . Nasopharyngeal Adenoid Cystic Carcinoma (NACC) is a primary SNPC and very rare malignancy that has specific biological characteristics. Due to the scarcity of cases, there is no clear consensus in terms of clinical characteristics, treatment approaches and prognostic factors. Our aim is to review the literature related to this topic by presenting a case with NACC. Different from the nasopharyngeal squamous cell carcinomas, NACC shows perineural invasion that is a specific biological behavior. The tumor progresses through the cranial nerve; it reaches the orbital and cranial cavity, 59 Nasopharyngeal Adenoid Cystic Carcinoma Case Report and Review Picture 1. T2-weighted axial cross-section. Mass observed here holds left compartment of Sfenid Sinus And left posterior ethmoid sinus cells, spreading the posterior orbita, middle cranial fossa, along the sphenoid wing, multi compartmental, expansile, hyperintence. Optic foramen and the superior orbital fissure is seen that the mass was obliterated. which worsen the patients’ prognosis. Nasal cavity, skull floor, pterygopalatine fossa, and zygomatic fossa are common locations that can render surgical resection more difficult [6-9] . Case Presentation A 36 year-old female patient at 35 weeks pregnancy presented with hypertension to the Gynecology and Obstetrics Department. The patient reported smoking 10 cigarettes a day. She reported left eyelid droop Picture 2. T1-weighted fat-suppressed axial cross-sectional. Because of the pregnancy opaque material could not be granted. Isointense structure is observed. two months ago. She also reported that she was diagnosed as nasopharyngeal cancer eleven years ago and she underwent radiotherapy (RT). Additionally, neurology and opthalmology were consulted for ptosis of the left eyelid. On examination, ptosis of her left eye lid, limitation in eye movement and 2nd, 3rd and 6th cranial nerve paralysis were noted. On orbito-cranial MR there were expansile mass lesions filling the nasal cavity, left maxillary and sphenoid sinus; eroding the concha and hard palate; obliterating the optic canal, superior-inferior orbital fissure, entering mid-left cranial fossa and orbits at the orbital apex; surrounding the left cavernous sinus, that were at first thought to be in accordance with the diagnosis of mucocele (Picture 1 and 2). Nasopharyngeal incisional biopsy was then taken. Figure 1 (A) and (B). Microscopic appearance of the ACC showing neoplastic proliferating cells were of uniform basaloid character, and formed cribriform structures in wide spaces. (Hematoxylin-Eosin) (A) 60 (B) © 2013 Acta Medica. All rights reserved. Acta Medica 2013; 2: 59–64 On macroscopic examination of the biopsy material, two pieces of grey-white irregular tissue sized 1.5x0.5x0.5 cm and 0.8x0.3x0.3 cm were observed. On serial sections in the microscopic examination, it was found that the entire specimen contained neoplastic proliferation. Proliferating cells were of uniform basaloid character, and formed cribriform structures in wide spaces (Figure 1 (A) and (B), Figures 2 and 3). Hyaline or mucoid material was present in the lumens. In some focal areas, mucoid stroma was observed. On histochemical studies with PAS and mucin, the pseudocystic areas were positive, and negative on Pas/Diastase staining (Figures 4 and 5). Thus the image was diagnosed as ‘adenoid cystic carcinoma’. The patient delivered a baby girl of 2400 grams via caesarean section. There were no early complications. The patient was referred to obstetric/gynecology, otorhinolaryngology and internal medicine. However, the patient died 6 months later. Figure 2. Imunhistochemical PanCK staining is positive Donmez et al. Discussion ACC is a tumor with different morphologic configurations of basal epithelial and myoepithelial cells. Despite a grossly solid appearance with an infiltrative pattern, it can sometimes have a well-demarcated, Figure 3. Immunhistochemical S100 staining is positive Figure 4. Immunohistochemical GFAP staining is positive Figure 5 (A) and (B). Histochemical studies with PAS (A) and Mucin (B), the pseudocystic areas were positive. (A) © 2013 Acta Medica. All rights reserved. (B) 61 Nasopharyngeal Adenoid Cystic Carcinoma Case Report and Review expansive pattern. In most cases, it does not have a capsule, and consists of pseudoglandular, “cribriform” structures with small glandular lumens . Some cases with ACC present with a newly identified, rare, sclerosant’ pattern [7,8,9]. A combination of these two patterns can be seen in the original or recurrent tumors. Additionally, this neoplasm can also present in a hybrid pattern in combination with other tumors . With regards to the cell type, a combination of intercalated ductal epithelial cells, myoepithelial cells, secretory cells and pluripotential reserve cells exists. Thus, the combination of cells in a tumor is no different from that in a benign mixed tumor and has a similar histogenesis. On incisional biopsies, if the periphery of the lesion is satisfactorily sampled, when perineural invasion is not observed, the diagnosis should be questioned  . NACC’s are tumors with a long, slow progressing history that are generally diagnosed late. The time between the appearance of the lesion and the first symptom is 2-5 years. Sometimes, computerized tomography can be helpful to diagnose bone erosion on the skull floor. Sometimes, with MR, involvement of the infra-temporal fossa and cavernous sinus, or perineural and perivascular infiltration can also be noted and suggest a separate diagnosis . However, in our case, this was not considered a different diagnosis radiologically. In our case, a biopsy was obtained due to an initial diagnosis of a mucocele, clinically and radiologically. NACC occur more often in females, approximately 49 years of age. The most common symptoms in decreasing frequency include tinnitus, nasal obstruction, epistaxis, headache, facial numbness, eye weakness, diplopia, vision loss and vertigo [12). Studies of Liu et al and Cao et al [12,13] about nasopharyngeal ACC and clinical features are shown in detail table 1. Studies show that cranial nerve Table 1. Clinical features and treatment NUMBER OF PATIENTS FACTOR Liu et al Cao et al GENDER Males Females 11 15 18 18 AGE Older Younger 17* 9 18** 18 SKULL BASE INVASION No Yes 15 11 8 28 CRANIAL NERVE INVASION No Yes 19 7 15 21 STAGE Early Advanced 14 12 8 28 *Older: ≥40 years, ** Older: ≥45 years invasion occurs, which differs from other nasopharyngeal neoplasms. The tumor progresses through the cranial nerve, reaches the orbital cavity and cranial cavity and this worsens the patients’ prognosis. [12,14] . NACC rarely spreads to lymph nodes. At the same time, the tumor has the tendency for hematogenous spread and can demonstrate distant metastasis without cervical node metastasis (Table 2) [12,13] . Cao et al defined clinical features of all nasopharyngeal SNPC subtypes with ACC. They are shown in detail in Table 3. Among cases of SNPC, the most common subtype is NACC, and the subtype with the highest metastasis is ACC . Among all the nasopharyngeal SNPC cases, cervical lymph node metastasis of ACC is observed in less than 20% of cases  . Table 2. Published Clinical Studies of Nasopharyngeal Adenoid Cystic Carcinoma CASES LYMPH NODE METASTASES (%) DISTANT METASTASES (%) TREATMENT S SURVIVAL Lee at al.15 11 N 60.0 R, S+R N Wang et al.14 5 15.0 35.0 R,S 78.0%, 5-yr OS Schramm and Immola16 11 N N S+R 100.0%, 3-yr OS Wen et al.17 21 14.3 30.0 S+R, R 42.9%, DFS Liu et al.12 26 7.7 26.9 S+R,R 54.8%, 5-yr OS Cao et al.13 36 13.9 30.6 S+R, R 61.3%, 5-yr OS AUTHOR R = radiotherapy; S = surgery; N = no result; OS = overall survival; DFS = disease free survival 62 © 2013 Acta Medica. All rights reserved. Acta Medica 2013; 2: 59–64 Donmez et al. Table 3. Clinical features of primary salivary gland-type carcinomas of the nasopharynx  . NUMBER OF PATIENTS (%) FACTOR ACC MEC AC GENDER Males Females 18 (50.0%) 4 (36.4%) 18 (50.0%) 7 (63.6%) 4 (57.1%) 3 (42.9%) AGE ≥45 y Younger 18 (50.0%) 5 (45.5%) 18 (50.0%) 6 (54.5%) 4 (57.1%) 3 (42.9%) SKULL BASE INVASION No Yes 8 (22.2%) 9 (81.8%) 28 (77.8%) 2 (18.2%) 3 (42.9%) 4 (57.1%) CRANIAL NERVE INVASION No Yes 15 (55.6%) 11 (100.0%) 4 (57.1%) 21 (44.4%) 0 (0.0%) 3 (42.9%) STAGE Early Advanced 8 (22.2%) 8 (62,7%) 28 (77.8%) 3 (27.3%) HISTOLOGIC GRADE Low High - 5 (45.5%) 6 (54.5%) 3 (42.9%) 4 (57.1%) 5 (71,4%) 2 (28.6%) LYMPH NODE METASTASES No Yes 31 (86.1%) 10 (90.9%) 3 (42.9%) 5 (13.9%) 1 (9.1%) 4 (57.1%) DISTANT METASTASES No Yes 25 (69.4%) 11 (100.0%) 5 (71,4%) 11 (30.6%) 0 (0.0%) 2 (28.6%) MEC = MUCOEPIDERMOİD CARCINOMA; AC = ADENOCARCINOMA In contrast to non-keratinized and keratinized carcinoma, the sensitivity to RT is low, and therefore, surgery is accepted as the main treatment in stages 1, 2, 3 NACCs . The optimal treatment is radical surgery followed by RT. However, the anatomy of the nasopharynx brings additional risks and technical problems related to involvement of critical neural and vascular structures . Due to a low incidence of SNPC, not many studies on chemotherapy have been performed previously  . In studies comparing nasopharyngeal keratinized and non-keratinized carcinomas, survival rates of SNPC malignancies involving the nasopharynx have a better prognosis. However, cranial nerve involvement and the presence of an advanced stage can affect survival dramatically. Because the incidence of NACC is very low, and statistical studies on the number of cases are very few, multi-centric studies should be conducted in order to evaluate future treatment strategies and prognostic factors  . In reports by Lin Yu-Chin et al , cases of salivary and nonsalivary ACC were investigated clinicopathologically. When major salivary gland, minor salivary gland and nonsalivary ACC were compared, sinonasal and tracheo-bronchial ACC have an advanced course with a more positive margin in cases of sinonasal and lacrimal ACC (Table 4). There was no difference between groups in terms of perineural invasion. Sinonasal, lacrimal and trachebronchial ACC have a worse prognosis than ACCs originating from the major salivary gland. Thus they concluded that a bad prognosis of sinonasal, lacrimal Table 4. Pathological features of salivary and nonsalivary ACC  Site No. of patients Advanced stage* High grade** Positive margin Perineural invasion Major salivary Parotid Submandibular Sublingual 32 (total) 21 8 3 34.4% (11/32) 15.6% (5/32) 53.1% (17/32) 65.6% (21/32) Minor salivary Intraoral Tongue Buccal Mauth floor Palate Sinonasal 51 (total) 26 5 5 2 14 25 57.2% (15/26) 76% (19/25) 11.5% (3/26) 12.0% (3/25) 53.8% (14/26) 84% (19/25) 61.5% (16/26) 76% (21/25) 50% (1/2) 44.4% (4/9) 71.4% (5/7) 100% (2/2) 0% (0/9) 14.3% (1/7) 50% (1/2) 77.8% (7/9) 42.9% (3/7) 100% (2/2) 66.7% (6/9) 100% (7/7) Nonsalivary Ear Lacrimal Tracheeobronchial 18 2 9 7 *Stage 3,4; **Histological grade 3 © 2013 Acta Medica. All rights reserved. 63 Nasopharyngeal Adenoid Cystic Carcinoma Case Report and Review and tracheobronchial ACC can result from late di- a slow-growing tumor that spreads in an expansive agnosis, difficult resection and tumor biology in dif- way, rather than in an invasive pattern, causing a diferent locationsThe origin of ACC plays role in de- agnosis of mucocele to be skipped clinically and raterming the prognosis. It is reported that sinonasal diologically. This led to a delay in diagnosis, leading ACC has a worse prognosis than ACC of the major to her death. salivary gland . 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