The neuroendocrine characteristics of Esthesioneuroblastoma: a case report

Esthesioneuroblastoma (ENB) is a rare, malign tumor, often without manifestation of symptom, which emerges from undifferentiated neuroectodermal tissue of the olfactory epithelium. It is most commonly localised within the nasal cavity, the rima olfactoria, the lamina cribrosa and the concha nasalis superior.

Syndrome of inadequate antidiuretic hormone (ADH) secretion (SIADH) is triggered by a dysregulation of the pituitary gland, but can also be induced by tumorous paraneoplastic activity. It leads to an ADH increase and consecutive elevated renal fluid retention, resulting in hyponatraemia which can cause hypertension, craving for salty food or muscular spasm.

We were involved in the case of a 60-year-old female patient, who was treated with Tolvaptan due to a SIADH of unknown origin. She suffered from hypertensive crises, spasms and cravings for salty food. The patient was regularly examined by means of positron emission tomography-computed tomography (PET-CT) to rule out a paraneoplastic process. In the last PET-CT a light mucosal swelling was observed in the right nasal cavity affecting the right concha nasalis superior.

Within the scope of a clinical examination we noticed a blueish mucosal swelling above the right Concha nasalis medialis. Epistaxis and nasal congestion were denied. We performed a right-sided endonasal ethmoidectomy to extirpate the tumour. Histopathologically, the localisation, histomorphology and immunohistological staining profile suggest the presence of an ENB.

Immediately after the surgical tumor excision the patient was able to stop taking Tolvaptan without recurrence of symptom. This instance shows that in case of SIADH of unknown origin an ENB should always be taken into consideration.

Publication History

Publication Date:
23 April 2019 (online)