Low-Grade Mucoepidermoid Carcinoma of the Parotid in a Pediatric Survivor of Hodgkin's Lymphoma

Abstract

Mucoepidermoid carcinoma (MEC) is an uncommon malignancy of the salivary glands in the pediatric population. MEC typically occurs as a secondary malignancy in patients who have undergone radiation therapy for cancer. The occurrence of MEC in patients who have undergone chemotherapy without prior radiotherapy is extremely rare. A 17-year-old female patient with a history of Hodgkin's lymphoma and anemia presented to the clinic with a painless hard lump between her mastoid and mandibular process. The patient's history revealed that at 11 years of age, she was diagnosed with stage IIIB Hodgkin's disease and received the ABVE-PC chemotherapy regimen. This regimen includes doxorubicin (Adriamycin), bleomycin, vincristine, etoposide, prednisone, and cyclophosphamide. The lack of bulk disease and complete response after two cycles of chemotherapy excluded her from receiving involved field radiation therapy (IFRT). Physical examination of the neck revealed a ~1.5 cm firm, non-tender mass near the left parotid. Fine needle aspiration biopsy results revealed spindle-shaped cells and unusual mitoses, and a superficial parotidectomy was recommended. Genetic testing for major oncogenes and tumor suppressor gene mutations was conducted. Results were negative for any significant genetic factors. Alkylating chemotherapy agents and immunosuppression increase the risk of secondary malignancies, although they have not been directly linked to MEC. This case suggests a potential association between chemotherapy treatment and MEC. Furthermore, it raises the question of whether increased vigilance and long-term follow-up are necessary for this vulnerable population of childhood cancer survivors who have not received radiation therapy.

Keywords: hodgkin lymphona; long-term outcome; mucoepidermoid carcinoma (mec); pediatric hematology-oncology; salivary gland cancer; total parotidectomy.

Figure 1. Fine needle aspiration ultrasound image of the mass (indicated by the white arrows)

Needle is apparent in the mass. Tissue was obtained for histological examination.

Figure 2. Gross total parotidectomy with diligence to the facial nerve

Arrows indicate the tumor [6]. Facial nerve was apparent on the tumor, and the real tumor dimensions were 3.3 x 2.9 x 1.3 cm.

Figure 3. Plan for the long-term surveillance of chemotherapy-induced effects

Created in BioRender. Wazir, S. (2025) https://BioRender.com/pcmn9me. Due to the patient's intensive chemotherapy, the patient will remain on triennial pulmonary function tests and biennial echocardiograms for the rest of their life.