Primary salivary gland-type carcinoma of the nasopharynx is a rare malignancy with aggressive clinical behavior and poor prognosis. Few cases have been reported in the literature. We report the case of a salivary gland-type carcinoma of the nasopharynx diagnosed in a 70-year-old woman managed in department of radiotherapy at Hassan II University Hospital in Morocco. The patient received a total of 70 Gy of radiations, which were divided into 2 Gy per session during 7 weeks. Follow-up was performed regularly, including nasal endoscopic examination and nasopharyngeal CT. Evolution was marked by disease control after 12 months of follow-up. The aim of this work is to show the rarity of this entity, describe clinical, radiological and histological features and finally discuss the treatment management.
Adenoid cystic carcinoma (ACC) is the most common histological subtype of salivary gland malignancies, but it is very rare in the nasopharynx, with relatively few cases reported in the literature. Some of these cases are poorly documented and not well characterized given to the rarity of cases. The ACC is characterized by a slow evolution but locally aggressive and high risk of recurrence. The optimal treatment remains debated, even though recent evidence provides support for a multimodality approach [
We report a case of a 70-year-old woman, diagnosed and treated in radiotherapy department at Hassan II University Hospital in Morocco. The patient had in her medical history a cardiac failure, but no prior smoking or radiation. She presented 6 months before the diagnosis, unilateral hearing loss with nasal obstruction. Clinical examination found a 3-centimeter lymph node of the IVth left cervical area. Nasopharyngeal endoscopy had showed a tumor process occupying totally the nasopharynx. Chest x-ray and blood tests were normal. A contrast computed tomography (CT) of the facial cranium and neck had showed an extensive lesion occupying all the nasopharynx with absence of bony erosion or involvement of adjacent structures. It revealed also two cervical ganglions with central necrosis in the jugulo-carotid area. The patient underwent a biopsy of the nasopharyngeal mass, under general anaesthesia. Histology revealed a cribriform adenocarcinoma salivary gland-type (
Regarding treatment strategy, Patient received radiotherapy alone without chemotherapy given to the geriatric assessment and co-morbidities. She received a total of 70 Gy of the radiations, divided into 2 Gy per session over a period of 7 weeks. Follow-up with nasal endoscopic examination and nasopharyngeal CT was performed and showed no recurrence 12 months after the end of treatment.
Primary salivary gland type carcinoma is one of the most common malignancies of salivary gland origin, with widely varied histological subtypes, diverse clinical behaviours, and different prognoses [
ized material also surrounds these cribriform islands, or small strands of tumor are found embedded within this hyalinized stroma. The tumor cells are small and cuboidal exhibiting deeply basophilic nuclei and little cytoplasm. These cells are fairly uniform in appearance, and mitotic activity is rarely seen [
Adenoid cystic carcinoma of the nasopharynx has tendency to invade the nerves and to propagate perineurally (80% of the specimens found invaded). Even if some studies showed that there is not strong correlation between survival and perineural invasion alone, the majority believes that the invasion of a main nerve trunk or at least a large nerve by the tumor means a poor prognosis [
Multiple treatment modalities exist for the treatment of salivary gland tumors of the nasopharynx [
Given the proven radiosensitivity of the ACC, this tumour is, in fact, regarded as a radiosensitive, even if not radiocurable, neoplasm, thus exclusive radiotherapy can determine a reduction in tumour volume with a meaningful improvement in the clinical symptoms [
In our patient, given to the impossibility of total resection, because of the locoregional extension, furthermore its geriatric assessment and co-morbidities, an exclusive radiotherapy was indicated. The evolution was marked by disease control at the imaging and endoscopy.
Nasopharyngeal cribriform adenocarcinoma salivary gland-type is an uncommon tumor with only very few cases reported in the literature. The treatment management must be discussed in a multidisciplinary approach for each case. Radiotherapy alone seems to be a good option in case of localized disease especially when chemotherapy is not applicable. But data are still insufficient for an appropriate treatment protocol or an accurate prognosis prediction for the disease and we still need larger clinical series and longer follow-up periods to establish the best treatment strategy for these patients.
The authors declare any conflict of interests.