Atypical case of primary intraosseous adenoid cystic carcinoma of mandible

Abstract

The Primary central salivary gland neoplasms of the mandible are infrequent. Their clinical and radiographic features may be similar to odontogenic tumors, which are otherwise common. Their accurate diagnosis becomes troublesome. Hence, diagnosis should depend on stringent diagnostic criteria. Adenoid cystic carcinoma is well known for its prolonged clinical course and its tendency for delayed onset of distant metastases. The long-term survival of these patients is therefore poor. Treatment modalities include surgery, radiotherapy and chemotherapy. The purpose of this paper is to report a case of primary central adenoid cystic carcinoma of mandible with an atypical presentation.

Primary Intraosseous Adenoid Cystic Carcinoma with Widespread Skeletal Metastases Showing Features of High-Grade Transformation

“…PIACC generally occurs in 4th to 6th decade without gender predilection [8]. The most common site involved is the posterior body and angle of mandible [2]. Pain and swelling are the most common symptoms reported followed by paraesthesia, tooth mobility and trismus [8].…”

“…Mucoepidermoid carcinoma and adenoid cystic carcinoma (ACC) are the commonly seen salivary gland malignancies. Salivary gland carcinomas located centrally within the mandible are rare, comprising less than 0.4% of all salivary gland carcinomas [2]. Primary intraosseous adenoid cystic carcinoma (PIACC) of the jaws is extremely uncommon.…”

Rehabilitation of an Advanced Case of Adenoid Cystic Carcinoma

“…Of these, only 10% are diagnosed as ACC [ 3 , 4 ]. This neoplasm presents numerous clinical and microscopic characteristics similar to other tumors; thus differential diagnosis should always be carried out by histological and immunohistochemical exams [ 7 , 8 ]. The differences between the ACC and other glandular tumors, such as adenocarcinoma, are only revealed by specific markers of collagen IV, laminins and integrins.…”

“…The differences between the ACC and other glandular tumors, such as adenocarcinoma, are only revealed by specific markers of collagen IV, laminins and integrins. These characteristics demonstrate that the diagnosis must be made quickly in order to establish the most appropriate treatment [ 7 , 10 ].…”

“…Treatment for ACC consists of four distinct modalities: surgical therapy, radiotherapy, chemotherapy, and combined therapies (surgery and radiotherapy; radio and chemotherapy). The latter has been the modality of choice in most cases, because only surgical removal or radiation therapy does not discard the possibility of recurrence in the margins nor metastases to occur mainly in the cervical lymph nodes, lungs, bones, and brain [ 5 – 7 ].…”