Intraosseous adenoid cystic carcinoma of maxilla: A rare case report
A 48-year-old female reported to the Department of Oral Medicine and Radiology with swelling over the left side of face since 4 years. There was history of swelling in the same region 5 years ago for which she was operated. The swelling recurred within a year, which gradually increased to attain the present size. Swelling was associated with dull and mild occasional pain on wide opening of the mouth. There was no history of pus/blood discharge, but had caused mild functional difficulty. There was no history of paresthesia, diplopia or change in resonance of the voice. Her past medical and personal histories were non-contributory.\nOn extra oral examination left side revealed gross facial asymmetry, with a diffuse swelling approximately 5 cm × 4 cm extending superoinferiorly from about 1 cm above the ala-tragus line up to the left angle of mouth and mediolaterally from philtrum up to the level of lateral canthus of left eye. Obliteration of left nasolabial fold and deviation of the left ala of the nose was noted. The overlying surface of the swelling was stretched and shiny but was normal in color. On palpation, no raise of local temperature was noted. Swelling was non-tender and firm in consistency with no pulsations evident. Examination of regional lymph nodes showed no evidence of lymphadenopathy [].\nOn intraoral examination an irregular, but well-defined swelling was noted involving the left vestibule and hard palate approximately measuring 7 cm × 6 cm extending anterio posteriorly about 2 cm from the maxillary anterior labial vestibule i.r.t 11.21 until the mesial aspect of 26 and medially crossing the mid palatine raphe on the right side approximately 1 cm away from the marginal gingiva of 13 and extending laterally into the left labial vestibule causing obliteration from 11 to 24. Overlying surface had areas of erythema interspersed with a bluish tint. Swelling was non-tender with firm consistency in the palate and cystic consistency in the left labial vestibule on palpation. Hard tissues in the region of complaint showed spacing in-between 21 and 22 and grade I mobility noted with 22, 23, and 24 [].\nOn correlating the history and clinical findings, a provisional diagnosis of low-grade minor salivary gland malignancy was rendered with mucoepidermoid carcinoma on top of the list followed by the ACC and others. Left maxillary antral malignancies and benign odontogenic tumors namely adenomatoid odontogenic tumor, squamous odontogenic tumor and others were considered in the differential diagnosis list.\nPanoramic image showed an irregular osteolytic lesion in maxillary anterior left region approximately 6 cm × 5 cm extending from mesial aspect of 12 up to the distal aspect of 26 anterio-posteriorly and superiorly had caused erosion and discontinuity of floors of the nasal fossa and maxillary sinus with soft-tissue density seen infiltrating into the respective structures and inferiorly up to interdental alveolus of 22, 23, 24, and 25. The lesion had an irregular moth-eaten internal architecture and had caused diversion of roots of 11, 21 and migration of 22. Intraoral periapical radiographs confirmed radicular findings along with multiplanar root resorptions seen with 21 and 23.\nTo assess the exact boundaries and invasion into the adjacent areas, computed tomography with contrast of mid-face was performed, which revealed a heterogeneous enhancing mass in the left maxilla. The lesion had caused bony destruction and erosions with the epicenter in the maxilla and the soft-tissue density was seen extending into the surrounding structures namely oral cavity, maxillary sinus, nasal cavity, buccal space, and upper lip equidistantly suggestive of intrabony origin [].\nRoutine hematological investigations were normal except erythropenia with microcytic, hypochromic anemia seen on peripheral smear.\nIncisional biopsy was performed from two separate locations namely, palate, and labial vestibule. On histopathological examination, hyperchromatic basaloid tumor cells were arranged in islands with interspersed multiple cystic areas showing eosinophilic material classically resembling cystic “Swiss-cheese pattern” was seen along with a solid pattern. Hence, diagnosis of ACC was rendered [].\nAs a part of metastatic work-up, clinical and imaging investigations were carried out including clinical examination of the oral cavity and neck, chest radiograms, and abdominal ultrasound were carried over, which showed no evidence of secondaries.\nThe patient was referred to higher regional oncology institute where surgery followed by the radiation treatment was planned. However, as the patient was reluctant to undergo surgery, lone radiation therapy with a total dose of 60 Gy was executed in the fractionation of 2 Gy/day for 5 days/week for a period of 6 weeks. As the treatment was incomplete and palliative, the prognosis was considered poor, nevertheless, follow-up of the case for 3 months after treatment showed no evidence of recurrence [].