Two Cases of Ectopic Adrenocorticotropic Hormone Syndrome with Olfactory Neuroblastoma and Literature Review

Abstract

are equally contributed to this journal.Abstract. Olfactory neuroblastomas are rare, slow-growing malignant tumors, usually diagnosed at advanced stages. Ectopic adrenocorticotropic hormone (ACTH) syndrome caused by an olfactory neuroblastoma is extremely rare. We reported two Korean women who suffered from ectopic ACTH syndrome (EAS) caused by olfactory neuroblastomas. The first patient was a 66-year-old woman who had been diagnosed as olfactory neuroblastoma and refused the management two years before and the second patient was a 37-year-old woman on chemotherapy for olfactory neuroblastoma. In the first case, she presented the Cushingoid appearance with systemic edema and her tumor was removed surgically. ACTH secretion by the tissue was confirmed by immunohistochemistry. By contrast, the second patient presented as severe pneumonia caused by cytomegalovirus and was treated with anti-viral agent followed by chemotherapy and radiotherapy, and her residual mass remained. However, after treatment, both patients' plasma ACTH and cortisol levels returned to normal without any adrenolytic therapy. Considering the causative tumors of EAS can be rarely cured and EAS increases the susceptibility to infections, it is prudent to suppress any hypercortisolemia initially, apart from treating the causal malignancy.

An unusual manifestation of olfactory neuroblastoma

“…It is important to note that the ACTH-producing ON may pose a diagnostic challenge due to the potential false-positive inferior petrosal sinus result. This may occur more commonly with ON due to the adjacent location of the tumour to the ethmoid sinus causing mixing of effluent from ethmoid sinus to the upstream pituitary venous drainage 7. False-positive results have also been re ported for high-dose dexamethasone suppression and CRH stimulation tests.…”

Intraoperative Hypertensive Crisis Due to a Catecholamine-Secreting Esthesioneuroblastoma

“…Other studies have reported secretion of adrenocorticotropic hormone (ACTH) from esthesioneuroblastomas (17)(18)(19)(20)(21)(22)(23)(24) . Syndrome of inappropriate antidiuretic hormone (SIADH) has also been reported in patients with esthesioneuroblastoma (9,(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36) .…”

Congenital Immature Teratoma Mimicking Cushing’s Disease

“…Amongst the intracranial causes, EAS due to esthesioneuroblastoma has been reported [11]. EAS due to sacrococcygeal teratomas has been found [12].…”