Cuching's Syndrome By Ectopics Corticotropic Hormone Secretion By Metastatic Medullary Thyroid Carciinoma
Presentation Number: MON 407
Date of Presentation: April 3rd, 2017
Faiza Abdulaziz Qari*
King Abdulaziz University, Jeddah, Saudi Arabia
Background :- Cuching's syndrome by ectopics corticotropic hormone can mimics pituitary dependent Cushing disease . It can be a life threatening hypercortisolemia . The Curative resection of tumour is possible only in a small proportion of patients (12%). The Surgical treatment is bilateral adrenalectomy , which has better advantage over medical treatment ketoconazole, metyrapone in controlling hypercortisolemia
- A 45 year old Saudi male known case of sporadic medullary thyroid carcinoma with metastasis to lymph nodes and bone. He underwent total thyroidectomy and modified neck dissection and external radiation 2010 . He had diabetes type 2 for 8 years on oral hypoglycaemic agents, with poor blood sugar control as his HbA1c 13.1%.
- He was a admitted with MTC which was progressive with rising calcitonin from 9000 to 40000 mcg/L as well he had upper airway obstruction and type 2 respiratory failure with pulmonary oedema.
- He underwent urgent tracheostomy.He was treated with diuretics, oral antibiotics, bronchodilators and assessed by ENT and found to have bilateral vocal cord palsy.
- Patient showed some improvements , however on further assessment he was found to have severe proximal muscle weakness and he was unable to stand from the sitting position.
On Examination: He had severe proximal muscle weakness of arms and thighs, however there were no tenderness, wasting, or fasciculation . His power was 3/5 with intact sensations intact and normal reflexes .
Hormonal assay revealed the following .
TSH was < 0.06 iu/L, FT4 18 pmol/L, ACTH =61pg/mL, Serum am cortisol AM was 1089 nmol/,Serum pm cortisol was 1004 nmol/L،24 hr U for free cortisol was 562 nmol/L (N5-55) , No suppression with Dexamethazone suppression test,
Radiological investigations :-
- Pituitary MRI was normal .
- MRI of thoracic Spine showed multiple bony metastatic, with no evidence of cord compression or syringomyelia.
The diagnosis was most properly Cuching's syndrome resulted from ectopics corticotropic hormone secretion by metastatic Medullary Thyroid Carcinoma .
In order to confirm the diagnosis by bilateral inferior petrosal sinus sampling (IPSS) for ACTH levels was done by CRH + vasopressin stimulation
The results showed
- ACTH 117 -----max response 127.
- Cortisol 1003 ----- max response 104
- The patient underwent Surgical Resection of
- Tumor secreting ACTH
- Bilateral Adrenalectomy
This is the rare case demonstrating Cushing Syndrome Due to Ectopic ACTH Production by metastatic Medullary Thyroid Carcinoma
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