Gargantuan carcinoma ex-pleomorphic adenoma of the submandibular gland: Case report and review of literature

Introduction: Pleomorphic adenoma is the most common neoplasm of the major salivary glands, and can undergo malignant transformation to carcinoma ex-pleomorphic adenoma (CXPA) in untreated patients.

Case presentation: Our case describes a 56-year-old male with a 15-year history of slowly growing expansive right-sided neck. He underwent excision of the neck mass, neck dissection, and pectoralis flap reconstruction. Final pathology revealed a 22 cm CXPA with myoepithelial and salivary duct carcinoma components. The patient was recommended radiation therapy to receive 66 Gy in 33 fractions.

Discussion: CXPA is rare, typically aggressive, and carries a poor prognosis. This case is unique given the location of the tumor arising from the submandibular gland and delayed presentation leading to its massive size. Surgical planning for a lesion of this nature is complex due to structures in the vicinity and additional needs for reconstruction. Another important consideration highlighted by this case includes the need to assess patient reliability and provide resources to reduce the odds of being lost to follow up and enhance patient care.