Peripheral and central aggressive form of Pindborg tumor of mandible - A rare case report

The calcifying epithelial odontogenic tumor (CEOT), or Pindborg tumor, is a rare and benign odontogenic neoplasm that affects the jaw. Calcifying epithelial odontogenic tumor can be divided into 2 clinico-topographic variants: Intraosseous (central) or Extraosseous (peripheral), with an incidence of 94% and 6%, respectively. The intraosseous tumors have a predilection for the mandible, and most tumors arise in the molar and premolar region of the mandible. When present, extraosseous tumors are often located in the anterior jaw and involve the gingiva.

In this article we present a unique rare variety of Pindborg tumor of mandible with both intraosseous & extraosseous presentation and also locally aggressive behavior.

A 35 year old man presented with Pindborg tumor (CEOT) of mandible with both intraosseous and extraosseous variant in a single lesion, extending from right premolar region to the left molar region.

CEOT is an uncommon neoplasm accounting for less than 1% of all odontogenic tumors. Although CEOT is considered less aggressive clinically than typical infiltrating ameloblastoma, but our case is a rarity which was aggressive and also rapidly progressive in short duration, with intra and extraosseous presentation. Hence it was managed surgically similar to a malignant lesion.

Presentation of two different variants of an odontogenic tumor in a single lesion and aggressive behavior of rather slow growing tumor, mimicking malignant lesion definitely shows possibility of rare variations in different magnitude. Hence proper diagnosis for management with better prognosis of these kinds of tumors is very much essential.