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

Abstract

Adenoid cystic carcinoma (ACC) is a rare malignant tumor that mostly occurs in minor glands, especially in the palate. Intraosseous adenoid cystic carcinoma (IACC) is rarer. There is no clear conclusion on the clinical, radiologic and pathological characteristics of IACC because of few reported IACC cases, leading to insufficient understanding of IACC. We reviewed 52 previous reports of primary IACC (PIACC) and analyzed the clinical features of those patients involved, attempting to provide a better understanding of PIACC. Moreover, we present a case of primary PIACC and a case of recurrent IACC (RIACC). The two patients showed similarities in clinical and pathological results, along with slight differences in radiological and immunohistochemical results. The patient of case 1 seemed to display a worse prognosis, which can only be proved after long term follow-up.

Keywords: clinical; immunohistochemical; intraosseous adenoid cystic carcinoma; pathological; prognosis; radiologic.

Figure 1

Extraoral examination showed swelling on the buccal side of the right mandibular second molar while the overlying mucous was normal.

Figure 2

Axial CT of soft tissue (A) and bone windows (B) demonstrated a soft tissue mass on the body, angle and ramus of right mandibular involving the mandibular canal with extension to adjacent muscles. Additional axial soft tissue (C) and axial bone window (D) images demonstrated expansion of the mass to the body of the left mandibular. Wormlike bone resorption of buccal and lingual bone cortex of the right mandible can be seen.

Figure 3

Three-dimensional reconstruction image showed honeycomb-like bone destruction of the buccal bone cortex.

Figure 4

Axial DWI (A), T1-weighted (B) and T2-weighted (C) images demonstrated a 6.8 × 2.6 cm lesion on the right side from the body of mandibular to the condylar neck involving the mandibular canal, the right internal pterygoid and masseter muscles. The mass extended slightly to the left mandibular anterior tooth area, with an ill-defined margin. This lesion is hypointense on T1 and hyperintense on T2 and DWI.

Figure 5

Sagittal (A) and coronal (B) MRI images demonstrated an enlarged lymph node in the right submandibular area, with a diameter of 0.8 cm.

Figure 6

Sagittal (A) and axial (B) views of the mandibular section specimen. The mandibular section specimen included the entire body, the angle, the condyle and the coronoid process of the right mandible and partial body of the left mandible. (C) A design of the anterolateral thigh flap with a size of 13 × 6 cm.

Figure 7

Pathological findings. (a). Tumor cells arranged mainly in solid nests, surrounded by the bone (black asterisk) (×4, H&E. Bar: 125 μm). (b). Low-power view of invasion and encirclement of vascular (×4, H&E. Bar: 125 μm). (c). High-power view of necrosis (red asterisk), hyperchromasia and cellular atypia (×40, H&E. Bar: 10 μm). (d). Invasion of tumor cells inside vascular (arrows) (×20, H&E. Bar: 20 μm). H&E: Hematoxylin and eosin.

Figure 8

High-power view of (a). invasion and encirclement of nerve (green asterisk) and (b). mitosis of tumor cells (box) (×40, H&E. Bar: 10 μm). H&E: Hematoxylin and eosin.

Figure 9

The solid nest indicated by the yellow oval was the largest, with a MinAmax of 0.225 mm (×4, H&E. Bar: 80 μm). H&E: Hematoxylin and eosin.

Figure 10

Extraoral examination showed no abnormal signs. The gingival mucosa was clear and normal with no red or swelling.

Figure 11

Panoramic radiograph imaging revealed a cystic lesion on the left mandible ramus with ill-defined margin infiltrating to the mandibular canal.

Figure 12

Coronal CT soft tissue (A), sagittal soft windows (B) and axial soft windows (C) demonstrated a soft tissue mass on the ramus of the left mandible involving the left mandibular canal. Coronal CT bone tissue (D), sagittal bone windows (E) and axial bone windows (F) demonstrated defection of lingual bone cortex and perforation of buccal bone cortex.

Figure 13

Sagittal image (A) and coronal image (B) of three-dimension reconstruction showed bone destruction of the lingual and buccal bone cortex on the left ramus of the left mandible.

Figure 14

Axial DWI (A), T1-weighted (B) and T2-weighted (C) images demonstrated a 2.4 × 2.9 cm lesion on the left mandibular ramus involving the mandibular canal. The mass invaded the left pterygopalatine fossa and parapharyngeal space inward and extended outward to the left masseter muscle space. Patchy signal in the parapharyngeal space and the masticatory muscle space can be seen. This lesion is hypointense on T1 and hyperintense on T2 and DWI. Axial T2-weighted (D) image demonstrated loss of left submandibular gland.

Figure 15

PET–CT examination showed increased SUV in (A) submental lymph node, (B) lymph nodes in deep surface of left sternocleidomastoid muscle and (C) left cervical parasheath lymph nodes.

Figure 16

(A) Mandibular resection specimen included the partial body, the angle, the condyle and the coronoid process of the left mandible. (B) Muscle groups and the tumor tissue (white circle) attached to the inner surface of the left mandible. (C) A design of the free fibula flap.

Figure 17

Pathological findings. (a). Tumor cells arranged in tubular and cribriform pattern (×4, H&E. Bar: 125 μm). (b). Tumor cells arranged in solid nests (×4, H&E. Bar: 125 μm). (c). Invasion of tumor cells in vascular wall(arrows) (×20, H&E. Bar: 20 μm). (d). High-power view of necrosis (red asterisk) and nuclear atypia (×40, H&E. Bar: 10 μm). H&E: Hematoxylin and eosin.

Figure 18

Low-power view of (a). invasion and encirclement of nerve (green asterisk) (×10, H&E. Bar: 40 μm) and high-power view of (b). hyperchromasia and cellular atypia of tumor cells around bone (black asterisk) (×40, H&E. Bar: 10 μm). H&E: Hematoxylin and eosin.

Figure 19

The solid nest indicated by the yellow oval was the largest, with a MinAmax of 0.404 mm (×4, H&E. Bar: 80 μm). H&E: Hematoxylin and eosin.