In this case report, we describe a solitary lingual ganglioneuroma that presented as a painful submucosal mass in a 61-year-old male patient with a recent history of Hodgkin lymphoma treated with chemo-radiotherapy. There are multiple possible causes of submucosal masses of the tongue, and a differential diagnosis is necessary. Final diagnosis was made by biopsy and histopathological examination. Ganglioneuromas are benign tumors that rarely occur in the tongue, with only two such cases previously described in the English literature.
Ganglioneuroma presenting as a painful submucosal mass of the tongue.
Solitary ganglioneuroma not associated with MEN type 2b.
Patient with history of Hodgkin lymphoma treated with chemo-radiotherapy.
Ganglioneuromas are benign tumors comprising mature ganglion and Schwann cells [ ]. Neuroblastomas, ganglioneuroblastomas, and ganglioneuromas constitute the spectrum of tumors deriving from the primordial neural crest cells that establish the sympathetic nervous system. In 1999, one study reported that ganglioneuroblastomas and ganglioneuromas only constitute 3% of neuroblastic tumors [ ]. In general, ganglioneuromas are more common among women than men, and most often occur in the pediatric population [ ]. Ganglioneuromas are most frequently located in the posterior mediastinum (41.5% of cases), retroperitoneum (37.5%), adrenal gland (21%), and neck (8%) [ ]. Unusual sites include the heart, bone, spermatic cord, intestine, and tongue. Intraoral presentation is rare, and necessitates further investigation to exclude the possibility of multiple endocrine neoplasia type 2b (MEN2b), neurofibromatosis type 1, or Cowden’s disease [ ]. The English literature includes only two reported cases of a solitary ganglioneuroma of the tongue [ , ]. In the present report, we describe the rare case of a lingual ganglioneuroma in a patient who, five years earlier, had undergone treatment for Hodgkin lymphoma of the tongue.
Presentation of case
A 61-year-old man presented with oral pain, bilaterally located on the right lateroposterior part of the tongue, and was referred to the Maxillofacial Surgery Department. The patient’s pain had been continuing for several weeks, and was most pronounced while speaking.
His oncological history included stage IIa Epstein-Barr virus-associated Hodgkin lymphoma at the base of the tongue and the cervical lymph nodes, on the right side. Complete remission had been obtained after four cycles of chemotherapy using Adriamycin-Bleomycin-Vinblastine-Dacarbazine (ABVD). Additional radiotherapy had been administered, with a total dose of 30 Gy, in 15 fractions of 2 Gy. The patient’s previous diagnosis and treatment had occurred 5 years prior to the current presentation with oral pain.
The patient’s medical history also revealed benign prostatic hyperplasia, hypercholesterolemia, and hypertonia. The patient consumed two alcohol units a day, and was previously a heavy smoker (25 pack-years) but had reduced his smoking habit to a maximum of 5 cigarettes/day since the diagnosis of Hodgkin lymphoma.
Extraoral physical examination revealed no palpable cervical lymphadenopathy, or any other abnormalities. Intraoral examination revealed a submucosal mass of approximately 1 cm at the lateroposterior part of the tongue, on the right side (see Fig. 1 ). The lesion appeared to be erythematous. No other intraoral mucosal lesions or abnormalities were observed.
Considering the patient’s oncological history, an excision biopsy was performed. Histopathological examination revealed mature ganglion cells and numerous Schwann cells, positive for S-100 staining. These characteristics were consistent with a diagnosis of ganglioneuroma [ ]. Fig. 2 presents the histopathological images.