Case of recurrence olfactory neuroblastoma presented with ACTH dependent Cushing syndrome

Faroug Ahmed; Robert Murray

doi:10.1530/endoabs.100.OC6

A-49-year-old female with previous history of Olfactory neuroblastoma treated surgically in 2003 and no adjuvant treatment was given. She has a past medical history of depression, bilateral sensory neural hearing loss which is thought to be genetic. In Feb 2023 presented with uncontrol hypertension associated with hypokalaemia. Clinical examination showed BMI of 48.4, uncontrol high blood pressure despite using multiple antihypertensive medication. The initial assessment for the in Feb 2023 showed:After the biochemical confirmation of ACTH dependent Cushing, she underwent Pituitary MRI which revealed extensive midline skull base enhancing tumour with suprasellar extension, more on the left with elevation of the left side of the chiasm.There is encasement of the carotid arteries bilaterally. She was started on metyrapone and was booked for the Transpheniodal surgery for debulking. She underwent the surgery in May 2023. The Histopathology confirmed the diagnosis of Recurrent olfactory Neuroblastoma. Immunostaining was positive for Synaptophysin, Ki67 very high (>30%), P53 20%, and it was negative to AE1/3, ACTH, EMA, GH, LH, MNF, Prolactin, S100. She had hydrocortisone post op and metyrapone was continued. She then received radical radiotherapy which was completed on 06.11.2023. Three months after the surgery, BMI was 41, her blood pressure well controlled. ACTH was 59 ng/l (<47) and the mean cortisol in Cortisol Day Curve was 143 nmol/l. MRI pituitary after the surgery showed a good anterior tumour debulk. With prominent posterior tumoral remnant within the suprasellar cistern with persistent although slightly reduced, chiasmatic tenting and ongoing envelopment of the left internal carotid artery within the cavernous sinus She remains on the hydrocortisone and the metyrapone.

Table 1.
Test	Value	Normal range
Aldosterone	<55 pmol/l	100-850
Renin	<0.02 nmol/lH	0.5-3.5
TSH	0.10 miu/l	2.0-4.0
T4	15.9 pmol/l	10-20
T3	1.1 nmol/l	0.9-2.5
Prolactin	249 mu/l	<600
IGF-1	4.5 nmol/l	6.9-28.0
GH	<0.1 mg/l
DHA sulphate	14.6 umol/l	1.3-8.5
LH	<0.3 iu/l
FSH	<0.3 iu/l
Androstenedione	31.7 nmol/l	1.1-5.7
Overnight dexamethasone suppression test	2728 nmol/l	<50
Morning Cortisol	1984 nmol/l
ACTH	572 ng/l	<47
Potassium	2.8 mmol/l	3.5-5.3
HBa1c	38 mmol/mol	20-41

Conclusion: This case highlights the rare association between olfactory neuroblastoma and ACTH dependent Cushing syndrome. The significant improvement after the transsphenoidal surgery make the focus of this lady’s Cushing’s does seem to be the olfactory neuroblastoma despite not having ACTH staining.

Endocrine Abstracts

OC6