Adenoid cystic carcinoma of the parotid gland - A case report and review of literature

Adenoid cystic carcinoma of the parotid gland ISSN: 2394-0026 (P)Case Report ISSN: 2394-0034 (O) Adenoid cystic carcinoma of the parotidgland - A case report and review of literatureNupur Singla1, Gunvanti Rathod2*, Disha Singla3 1P.G. Student, Radiology Department, SBKS MI & RC, Sumandeep Vidyapeeth, Vadodara, India2Assistant Professor, Pathology Department, SBKS MI & RC, Sumandeep Vidyapeeth, Vadodara, India 3P.G. Student, Radiology Department, SBKS MI & RC, Sumandeep Vidyapeeth, Vadodara, India*Corresponding author email: [email protected] to cite this article: Nupur Singla, Gunvanti Rathod, Disha Singla. Adenoid cystic carcinoma ofthe parotid gland - A case report and review of literature. IAIM, 2015; 2(4): 182-186.Available online at www.iaimjournal.comReceived on: 08-03-2015 Accepted on: 23-03-2015AbstractAdenoid cystic carcinoma (ADCC) is a relatively rare malignant salivary gland tumor comprising lessthan 1% of all malignancies of head and neck. It can arise in any salivary gland site, butapproximately 50–60% develops within the minor salivary glands. On fine needle aspiration cytology(FNAC), hyaline globules surrounded by neoplastic cells forming a cell ball are the diagnostic.Adenoid cystic carcinoma, being rare, was not suspected at the first instance. The final diagnosis wasmade from the cytological, radiological, and histopathological reports.Key wordsAdenoid cystic carcinoma, Malignant salivary gland tumor, Hyaline globules.Introduction Solid pattern is associated with increased local recurrence, high metastatic rate and higherAdenoid cystic carcinoma (ADCC) is a relatively mortality [4]. Here, we have reported a case ofrare malignant salivary gland tumor comprising adenoid cystic carcinoma of parotid gland in 42less than 1% of all malignancies of head and years old male patient with multiple lungneck. It is the 5th most common malignancy of metastases.salivary gland origin, representing 5-10% of allsalivary gland neoplasms [1, 2]. It can arise in Case reportany salivary gland site, but approximately 50–60% develops within the minor salivary glands. A 42 years old, systemically healthy male patientIn the parotid gland, the ADCC is relatively rare, presented with a complaint of pain and swellingconstituting only 2–3% of all tumors [3]. on the right side of the face since 5–6 months.Histologically, the tumor consists of three The patient reported a rapid increase in size ofdifferent patterns: cribriform, tubular and solid. the lesion over the past 2 months.International Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 182Copy right © 2015, IAIM, All Rights Reserved.

Adenoid cystic carcinoma of the parotid gland ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)On examination, a well-defined swelling on the [9, 10, 11]. Smears were stained with H&E stainright side of the face in the parotid region, with (Hematoxylin and Eosin) and Papanicolaouan intact surface, measuring approximately stains. The diagnosis was made on smears7×6.5 cm in size with no facial nerve palsy was stained with H&E stain as these showed thenoted. (Photo – 1) The lesion was not fixed to characteristic findings of hyaline globulesthe underlying bone and was entirely within the surrounded by the neoplastic cells forming a cellsoft tissues. Hematological investigations ball. (Photo – 3) These globules stained palerevealed a normal complete blood count, with Papanicolaou stain and were virtuallyerythrocyte sedimentation rate, and blood invisible. Individual cell cytology showed cellschemistry. with small ovocid nuclei, mild to moderate pleomorphism and hyperchromasia andPhoto – 1: Well-defined swelling on the right prominent nucleoli. (Photo – 4) Cells appearedside of the face in the parotid region. in small groups, tight clusters and plugs, while some were seen encircling the hyaline globule. Overall features were that of ADCC. Photo – 2: CECT showing large infiltrative soft tissue mass in the parotid region of the right side of the face.Plain and contrast-enhanced scans with axial Discussionand coronal sections revealed a multifocal, mildperipherally enhancing conglomerated lesion of Adenoid cystic carcinoma (ADCC) is a malignantsize approximately 74×68 mm in axial plane is salivary gland tumor that was first described byseen involving the right parotid gland. (Photo – Billroth in 1859 [12] under the name cylindroma2) Bilateral lung field showed multiple soft tissue attributing to its cribriform appearance formednodular lesion of average size 10x12 mm, by the tumor cells with cylindrical pseudoluminasuggestive of metastasis. The overall features or pseudospaces. The term “adenoid cysticwere suggestive of a mass lesion involving right carcinoma” was introduced by Ewing (Foote andparotid gland along with preauricular soft tissue Frazell) in 1954. Adenoid cystic carcinoma (ACC)nodular lesion. There was presence of multiple is a slow-growing, but aggressive neoplasm withlung metastases. a remarkable capacity for recurrence.The patient was referred to FNAC clinic. FNACwas performed using a 22-guage needleattached to a 10 ml syringe [5, 6, 7, 8]. Bloodmixed particulate material was obtained, airdried and 95% ethanol fixed smears were madeInternational Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 183Copy right © 2015, IAIM, All Rights Reserved.

Adenoid cystic carcinoma of the parotid gland ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)Photo – 3: Hyaline globules surrounded by grows slowly but infiltrates widely. The mode ofneoplastic cells forming a cell ball. (H & E Stain, spread of this tumor is characteristic. It has a20X) tendency for perineural spread accounting for pain in about 50% of cases. The propensity of this tumor to invade bone and spread along the base of the skull results in extensive intracranial invasion and involvement of the cranial nerves. Lymphatic spread being very rare, lymphadenopathy is seldom encountered. Lymph nodes, however, in very extensive cases, may be involved by direct extension [14]. In long standing cases distant metastasis occurs via the blood stream to the lungs and bones.Photo – 4: Tumor cells having uniform and Diagnosis of ADCC on FNAC can be made byhyperchromatic nuclei, fine to coarse chromatin presence of biphasic cells population: epithelialpattern with prominent nucleoli. (Pap Stain, (usually basaloid) and myoepithelial cells. There40X) is also presence of basement membrane material and hyaline globules which are surrounded by tumor cells. The tumor cells show usually small, uniform and hyper chromatic nuclei, fine to coarse chromatin pattern with prominent nucleoli [15].Clinically, it appears as a slow-growing mass The differential diagnosis of ADCC includeswith early local pain, facial nerve paralysis in the polymorphous low grade adenocarcinomacase of parotid tumors, fixity to deeper (PLGA), basal cell adenoma (BCA) and mixedstructures and local invasion. tumor (pleomorphic adenoma). PLGA shows uniform cell population with cytologically bland,This is an interesting tumor with two distinct round or oval vesicular nuclei and paleclinical entities [13]. One group, as in the eosinophilic cytoplasm where as cells in ADCCpresent case, has a relentless fulminating course have clear cytoplasm, angular, hyper chromaticwith early metastasis and fatal outcome within a nuclei and may show mitotic activity. The Ki-67short period of 2-3 years. The second group has index is reported to be 10 times higher in ADCCan insidious natural history and long survival compared to PLGA. Basophilic pools ofperiod despite local recurrences. Most of the glycosaminoglycans are seen in ADCC but not incases fall in the second category, “the patient PLGA. Smooth muscle markers of myoepithelialand the tumor existing in symbiosis”. The tumor differentiation are positive in ADCC but negative in PLGA [16]. Occasional foci in pleomorphic adenoma (PA) can resemble ADCC but the presence of typical myxochondroid matrix and plasmacytoid or spindle shaped cells helps to avoid confusionInternational Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 184Copy right © 2015, IAIM, All Rights Reserved.

Adenoid cystic carcinoma of the parotid gland ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)[17]. The three recognized histological variants survival. Am J Clin Pathol., 1979; 138:of ADCC are cribriform, tubular and solid 579–83.although, cribriform is the most commonest and 3. Neville, Damm, Allen, Bouquot. Oral andsolid is the least common. Tumor nuclei are Maxillofacial Pathology. 2nd edition, p.typically small, angulated and hyper chromatic 426–8.with scanty cytoplasm. Mitotic figures are 4. Huang MX, Ma DQ, Sun KH, Yu GY, Guogenerally scarce in cribriform and tubular areas; CB, Gao F. Factors influencing survivalhowever, they are easily visualized in solid rate in adenoid cystic carcinoma of thestandards that have been associated with the salivary glands. Int J Oral Maxillofacworst prognosis [18]. Surg., 1997; 26: 435–9. 5. Rathod GB, Ghadiya V, Shinde P, TandanRadiological investigations, especially CT scans RK. Pleomorphic sarcoma in 60 years oldare important to delineate the tumor, to plan male – A case report. Internationalextent of surgery and to look out for recurrences Journal of Current Microbiology andas a follow up postoperatively. Pulmonary and Applied Sciences, 2014; 3(8): 510-517.skeletal surveys are important to rule out distant 6. Gunvanti Rathod, Pragnesh Parmar,metastasis. Treatment of these tumors includes Sangita Rathod, Ashish Parikh.surgical excision and postoperative radiation. Suprascapular malignant fibrousThe role of chemotherapy for metastatic histiocytoma – A case report. Discovery,Adenoid cystic carcinoma is still controversial. 2014, 12(31): 50-53. 7. Rathod GB, Goyal R, Bhimani RK,Conclusion Goswami SS. Metaplastic carcinoma ofADCC is a rare malignant tumor of the parotid breast in 65 years old female - A casegland. The primary treatment objective in ADCC report. Medical Science, 2014; 10(39):patients is local control, normal functionality 77-81.and distant metastasis prevention. On FNAC 8. Disha Singla, Gunvanti Rathod.characteristic hyaline globules surrounded by Cytodiagnosis of renal cell carcinoma – Aneoplastic cells forming a cell ball are the case report. IAIM, 2015; 2(2): 133-137.diagnostic. Adenoid cystic carcinoma, being rare, 9. Gunvanti Rathod, Pragnesh Parmar. Finewas not suspected at the first instance. The final needle aspiration cytology of swellingsdiagnosis was made from the cytological, of head and neck region. Indian Journalradiological, and histopathological reports. of Medical Sciences, 2012; 66: 49-54. 10. Gunvanti Rathod, Sangita Rathod,References Pragnesh Parmar, Ashish Parikh. 1. Matsuba HM, Spector GJ, Thawley SE, Diagnostic efficacy of fine needle Simpson JR, Mauney M, Pikul FJ. aspiration cytology in cervical Adenoid cystic salivary gland carcinoma: lymphadenopathy – A one year study. A histomorphologic review of treatment International Journal of Medical and failure patterns. Cancer, 1986; 67: 519– Pharmaceutical Sciences, 2014; 4(5): 1- 24. 8. 2. Spiro RH, Huvos AG, Strong EW. Adenoid 11. Mobeen Alwani, Gunvanti B. Rathod. cystic carcinoma: Factors influencing Diagnosis of anaplastic thyroid carcinoma on fine needle aspirationInternational Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 185Copy right © 2015, IAIM, All Rights Reserved.

Adenoid cystic carcinoma of the parotid gland ISSN: 2394-0026 (P) ISSN: 2394-0034 (O) cytology - A rare case report. IAIM, benign and malignant salivary gland 2015; 2(3): 183-187. tumors other than pleomorphic12. Lucas RB. Pathology of tumors of the adenoma. Arch Pathol Lab Med., 1999; oral tissues. 4th edition, London: 123: 801–6. Churchill Livingstone; 1998, p. 330–5. 17. Ogawa I, Miyauchi M, Matsuura H.13. Eby L. S., Johnson D. S., Baker H. W. Pleomorphic adenoma with extensive Adenoid cystic carcinoma of the head adenoid cystic carcinoma – like and neck. Cancer, 1972; 29: 1160-1168. cribriform areas of parotid gland. Pathol14. Allen M. S., Marsh W. L. Lymph node Int., 2003; 53: 30–4. involvement by direct extension in 18. Eveson JW, Cawson RA. Tumours of the advanced Adenoid cystic carcinoma. minor (oropharyngeal) salivary glands: A Cancer, 1976; 38: 2017-2021. demographic study of 336 cases. J Oral15. Orell S. R., et al. Manual and Atlas of fine Pathol., 1985; 14: 500–9. Needle Aspiration Cytology. Churchill Livingstone.16. Prasad AR, Savera AT, Gown AM. The myoepithelial immunophenotype in 135Source of support: Nil Conflict of interest: None declared.International Archives of Integrated Medicine, Vol. 2, Issue 4, April, 2015. Page 186Copy right © 2015, IAIM, All Rights Reserved.