Gnathic variant of primary adenoid cystic carcinoma

Abstract

Adenoid Cystic Carcinoma (AdCC) is an uncommon malignant epithelial salivary gland neoplasm, which is indolent yet persistent. Intraosseous /central variant of AdCC of the jaw is poorly understood owing to its innocuous clinical presentation thus posing a diagnostic challenge. AdCC is characterized by perineural invasion, late distant metastasis and a high recurrence rate, thus having a major effect on the mortality and morbidity rate. It is often diagnosed at an advanced stage which is chiefly achieved through histological examination and complete clinic-radiological work up. A clinical case of an intraosseous variant of AdCC, involving the mandible of a 49 year old male patient is discussed herewith, encompassing the entire spectrum of clinicopathological, radiological and treatment modalities rendered.

Primary and Recurrent Intraosseous Adenoid Cystic Carcinoma—Analysis of Two Cases and Literature Review

“…Since 1955, there have been only 52 publicly reported cases of PIACC, with case 1 being the 53rd case (Table 1). Among all selected articles, Hu et al [14] summarized a total of 47 PIACC cases before 2017, while Indu [15], Reddy [16], Savithri [17], Shome [18] and Sasaki [19] reported the 48th, 49th, 50th, 51st and 52nd case, respectively. The patient did not receive any adjuvant therapy after surgery and a 14-month follow-up showed no evidence of recurrence or metastasis.…”

A Case of Primary Intraosseous Adenoid Cystic Carcinoma of the Mandible

“…In addition, primary intraosseous ACC (PIACC) of the jaw is rare. To our knowledge, only 51 cases have been previously reported from 1955 to 2022, with 47 cases from the review of Hu et al (2017) and four recent cases reported in the English literature [ 2 – 6 ]. PIACC of the jaw has rarely been reported and is poorly understood.…”