ECEESPE2025 ePoster Presentations Endocrine Related Cancer (100 abstracts)
Unexpected evolution of an ACTH secreting pitnet
Samir Mkaouer 1 , Elyes Kamoun 1 , Meriem Yazidi 1 , Awatef Mekaouer 1 , Nadia Khessairi 1 , Ibtissem Oueslati 1 , Fatma Chaker 1 , Sonia Nagi 2 & melika Chihaoui 1
1CHU La Rabta, Endocrinology Department, Tunis, Tunisia; 2Neurology Institute of Tunisia, Radiology Department, Tunis, Tunisia
JOINT3291
Introduction: Pituitary carcinoma (PC) is a rare tumor and represents only 0,1 - 0,2% of all pituitary tumors. Early diagnosis of PC is often challenging and requires the presence of metastases.
Observation: A 59-Year-old diabetic patient was referred to our clinic. A Cushing syndrom was suspected due to hypercatabolic signs and was confirmed by elevated urinary free cortisol (UFC)= 800µg/24h [22-110]. Due to elevated ACTH levels (95pg/ml [10-50]), a pituitary MRI was performed and showed a 7mm pituitary adenoma. The patient underwent a complete transsphenoidal resection. Pathology examination showed a Corticotrop benign pitnet with a Ki67= 1%. After surgery, diabetes and hypertension have improved, and the patient had a corticotropin deficiency. Three months after surgery, due to worsening of blood pressure and diabetes, dexamethasone suppression test was performed with cortisol levels at 1,9 µg/dL, suggesting the relapse of the Cushing disease with a normal pituitary MRI. The patient went through Radiotherapy (54 Gy) and was put on Ketoconazole 400mg per day for two years with normal UFC levels. After withdrowal of ketoconazole and increase of UFC levels, the patient underwent bilateral adrenalectomy and was under hydrocortisone and fludrocortisone replacement. After six months, she developped hyperpigmentation with ACTH levels of 1890pg/ml suggesting Nelson’s syndrom, later associated with ptosis and mydriasis. A pituitrary MRI showed a 25*21*13mm macroadenoma with cavernous and optic chiasma extension. She went a second Transsphenoidal Surgery followed by gamma night radiotherapy. Pathological examination showed an atypic pitnet with Ki67=10%>3%, complicated by dural metastases after two years. She was treated by temozolomide for 15 months.
Conclusion: Although ACTH pitnet is a disease with frequent relapse, the Transformation of a benign pitnet to a PC is rare. A review showed that the mean time interval from initial diagnosis to diagnosis of PC was 10.7 years. This suggests to be careful and repeat MRIs if there are signs of aggressivity.
Volume 110
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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