Medullary Thyroid Carcinoma: Ectopic ACTH Production by Liver Metastases (Report of Two Cases)

1993 ◽  
pp. 156-161
Author(s):  
D. Bergant ◽  
M. Auersperg ◽  
M. Us-Krašovec ◽  
G. Petrič ◽  
M. Hočevar
Keyword(s):  
Thyroid Carcinoma ◽  
Liver Metastases ◽  
Medullary Thyroid Carcinoma ◽  
Ectopic Acth ◽  
Medullary Thyroid ◽  
Ectopic Acth Production

MEN 2A with double mutation in RET gene: Ectopic ACTH production in medullary thyroid carcinoma

2016 ◽  
Vol 146 (9) ◽  
pp. 419-420
Author(s):  
Jessica Ares ◽  
Lucía Díaz-Naya ◽  
Alicia Martín-Nieto ◽  
Joaquín Pertierra
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Ectopic Acth ◽  
Double Mutation ◽  
Medullary Thyroid ◽  
Ret Gene ◽  
Ectopic Acth Production ◽  
Men 2A

Presentation of a Medullary Endocrine Neoplasia 2A Kindred with Cushing's Syndrome

2008 ◽  
Vol 74 (7) ◽  
pp. 659-661
Author(s):  
Victor Zaydfudim ◽  
Daniel G. Stover ◽  
Susan W. Caro ◽  
John E. Phay
Keyword(s):  
Genetic Testing ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Ectopic Acth ◽  
Medullary Thyroid ◽  
Endocrine Neoplasia ◽  
Ectopic Acth Production ◽  
Men 2A

Although medullary thyroid cancer (MTC) can produce adrenocorticotropic hormone (ACTH) in up to 40 per cent of cases as determined by immunohistochemistry, clinical hypercortisolism is rarely seen. We report a medullary endocrine neoplasia 2A (MEN 2A) kindred whose proband case presented with Cushing's syndrome (CS). This 51-year-old woman presented with debilitating weakness, exertional dyspnea, 50 pound weight gain, moon facies, worsening hypertension, striae, and hirsutism. A comprehensive evaluation diagnosed ectopic ACTH production from unresectable metastatic MTC to the liver. Genetic testing revealed a germline RET proto-oncogene mutation at codon 609. Further genetic testing identified six family members with the same mutation. The patient underwent palliative bilateral laparoscopic adrenalectomies with significant improvement in major comorbidities. Overall CS resulting from ectopic ACTH overproduction by MTC is rare, occurring in 0.6 per cent of all patients with medullary thyroid carcinoma. About 50 cases have been previously reported in the literature, but only three in families with MEN 2A. We describe the first case of a MEN 2A kindred presenting with CS from ectopic ACTH production by metastatic medullary thyroid carcinoma. We advocate consideration of early bilateral laparoscopic adrenalectomies in patients with symptomatic hypercortisolism from unresectable metastatic medullary thyroid carcinoma.

scholarly journals Synchronous Lung and Liver Metastases from Medullary Thyroid Carcinoma

2003 ◽  
Vol 10 (1) ◽  
pp. 39-41 ◽  
Author(s):  
H Yanardag ◽  
C Tetikkurt ◽  
S Tetikkurt
Keyword(s):  
Thyroid Carcinoma ◽  
Liver Metastases ◽  
Medullary Thyroid Carcinoma ◽  
Metastatic Disease ◽  
Serum Markers ◽  
Initial Presentation ◽  
Medullary Thyroid ◽  
Common Causes ◽  
High Concentrations ◽  
First Time

Metastatic disease is one of the most common causes of calcified nodules in the lung or liver. The incidence of calcified metastasis mainly to the lung and liver is high at the initial presentation in patients with medullary thyriod carcinoma. Synchronous calcified metastasis in the lung and liver is reported for the first time. The diagnosis of medullary thyroid carcinoma may be evident from the synchronous presence of miliary calcified nodules in two different sites if they are associated with high concentrations of serum markers.

scholarly journals The role of hepatic trans-arterial chemoembolization in metastatic medullary thyroid carcinoma: a specialist center experience and review of the literature

2017 ◽  
Vol 176 (4) ◽  
pp. 463-470 ◽  
Author(s):  
S Grozinsky-Glasberg ◽  
A I Bloom ◽  
N Lev-Cohain ◽  
A Klimov ◽  
H Besiso ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Liver Metastases ◽  
Medullary Thyroid Carcinoma ◽  
Clinical Improvement ◽  
Tumor Response ◽  
Liver Volume ◽  
Hepatic Metastases ◽  
Case Series ◽  
Medullary Thyroid ◽  
Arterial Chemoembolization

BackgroundLiver metastases are relatively common in patients with metastatic medullary thyroid carcinoma (MTC), carrying a negative impact on disease prognosis. The options for selective therapy of liver metastases in MTC patients are limited to catheter-guided procedures such as trans-arterial chemoembolization (TACE). Data regarding the effectiveness and safety of this procedure in MTC are limited.AimTo explore the clinical outcome, survival and safety profile of TACE for liver metastases in a group of MTC patients.MethodsRetrospective case series of patients treated at a single tertiary University Medical Center from 2005 to 2015.ResultsSeven consecutive patients (mean age 64.5 ± 10.9 years, 5 females) with histologically confirmed MTC with liver metastases were included. Metastatic involvement of the liver was less than 50% of the liver volume in all patients. The median size of the largest liver lesion was 40 ± 6.9 mm. The patients underwent in total 20 sessions of TACE. Clinical improvement as well as tumor response (PR) were observed in all patients. The median time to tumor progression was 38 months (range 8–126). Three patients were still alive at the end of the follow-up period (a median overall survival rate of 57 ± 44 months).ConclusionTACE in MTC patients with hepatic metastases is usually well tolerated and induces both clinical improvement and tumor response for prolonged periods of time in the majority of patients. This therapeutic option should always be considered, irrespective of the presence of extrahepatic metastasis.

scholarly journals A Case of Medullary Thyroid Carcinoma with Ectopic ACTH Syndrome

1977 ◽  
Vol 53 (11) ◽  
pp. 1279-1291 ◽  
Author(s):  
Shin-ichiro TAKAI ◽  
Toshio OGIHARA ◽  
Akira MIYAUCHI ◽  
Hiromu HIGASHI ◽  
Tsuyoshi NISHIMURA ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Medullary Thyroid

scholarly journals Metastatic medullary thyroid carcinoma presenting as ectopic Cushing’s syndrome

2021 ◽  
Vol 2021 ◽  
Author(s):  
Hannah E Forde ◽  
Niamh Mehigan-Farrelly ◽  
Katie Ryan ◽  
Tom Moran ◽  
Megan Greally ◽  
...  
Keyword(s):  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Recurrent Disease ◽  
Neuroendocrine Tumour ◽  
List Type ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Serum Calcitonin ◽  
Ectopic Acth Secretion ◽  
Medullary Thyroid

Summary A 41-year-old male presented to the Emergency Department with a 6-month history of back and hip pain. Skeletal survey revealed bilateral pubic rami fractures and MRI of the spine demonstrated multiple thoracic and lumbar fractures. Secondary work up for osteoporosis was undertaken. There was no evidence of hyperparathyroidism and the patient was vitamin D replete. Testosterone (T) was low at 1.7 nmol/L (8.6–29.0) and gonadotrophins were undetectable. The patient failed a 1 mg dexamethasone suppression test (DST) with a morning cortisol of 570 nmol/L (<50) and subsequently a low dose DST with a cortisol post 48 h of dexamethasone of 773 nmol/L (<50) and an elevated ACTH 98 ng/L. A corticotropin-releasing factor (CRF) test suggested ectopic ACTH secretion. The patient was commenced on teriparatide for osteoporosis and metyrapone to control the hypercortisolaemia. A positron emission tomography (PET) scan to look for the source of ACTH secretion demonstrated right neck adenopathy. Biopsy and subsequent lymph node dissection were performed and histology revealed a metastatic neuroendocrine tumour. Immunostaining was positive for calcitonin and thyroid transcription factor 1 (TTF1). Serum calcitonin was also significantly elevated at 45 264 ng/L (<10). The patient proceeded to a total thyroidectomy and left neck dissection. Histology confirmed a 7 mm medullary thyroid carcinoma (MTC). Post-operatively, the patient commenced vandetanib therapy and achieved a clinical and biochemical response. After approximately 18 months of vandetanib therapy, the patient developed recurrent disease in his neck. He is currently on LOXO-292 and is doing well 36 months post-diagnosis. Learning points Unexplained osteoporosis requires thorough investigation and the workup for secondary causes is not complete without excluding glucocorticoid excess. MTC should be considered when searching for sources of ectopic ACTH secretion. Resistance to tyrosine kinase inhibitors is well described with MTC and clinicians should have a low threshold for screening for recurrent disease.

scholarly journals Cushing’s Syndrome Due to Medullary Thyroid Carcinoma: Diagnosis by Proopiomelanocortin Messenger Ribonucleic Acid in Situ Hybridization

2003 ◽  
Vol 88 (10) ◽  
pp. 4565-4568 ◽  
Author(s):  
R. C. Smallridge ◽  
K. Bourne ◽  
B. W. Pearson ◽  
J. A. van Heerden ◽  
P. C. Carpenter ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
Thyroid Carcinoma ◽  
Neck Dissection ◽  
Medullary Thyroid Carcinoma ◽  
Cushing's Syndrome ◽  
Plasma Acth ◽  
Mri Scan ◽  
Ectopic Acth ◽  
Medullary Thyroid

Abstract Medullary thyroid carcinoma (MTC) rarely causes ectopic ACTH syndrome. We describe a 38-yr-old man with renal stones who had a 5-cm MTC removed in 1992. He was RET-protooncogene positive (codon 618). Serum calcitonin was 1597 pg/ml postoperatively. In 1996 he had rib fractures, bruising, weakness, and three to four stools per day. Laboratory studies revealed an elevated 24-h urine-free cortisol (780 μg/d), epinephrine (66 μg/d), and calcium (558 mg/d). Baseline serum cortisol was 23.9 μg/dl and decreased to 12.9 and 4.5 μg/dl after 2 mg and 8 mg dexamethasone suppression, respectively. Plasma ACTH was 170 pg/ml and decreased to 75 and 24 pg/ml after dexamethasone. Bone density t-score was −4.3 (trochanter). Computed tomography scans showed multiple cervical nodes and 2-cm right adrenal nodule. Magnetic resonance imaging (MRI) scan showed a prominent, homogeneous pituitary; the adrenal MRI scan was not typical for a pheochromocytoma. Serum CRH was less than 6.6 pg/ml. Bilateral adrenalectomy revealed two adjacent right adrenal pheochromocytomas and corrected the elevated urine cortisol (30 μg/d), epinephrine (0 μg/d), and calcium (281 mg/d) but not plasma ACTH (125 pg/ml). Neck dissection reduced calcitonin by 96% (5300 to 120 pg/ml) and ACTH by 91% (125 to 11 pg/ml). Carcinoembryonic antigen was reduced from 32.0 to 2.3 ng/ml. Immunohistochemical stain was negative for ACTH in the MTC-positive lymph nodes and the pheochromocytoma. Proopiomelanocortin mRNA by in situ hybridization was positive in the MTC but not in the pheochromocytoma. A repeat pituitary MRI scan was normal. The differential diagnosis of ACTH-dependent Cushing’s syndrome in this case included pituitary disease or ectopic ACTH, either from medullary thyroid carcinoma or pheochromocytoma. ACTH stains were unrevealing, but proopiomelanocortin mRNA in situ hybridization in MTC tissue and plasma ACTH response to neck dissection confirmed MTC as the source of ectopic ACTH.

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