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Ectopic ACTH Production Leading to Diagnosis of Underlying Medullary Thyroid Carcinoma.

Matheny LN, Wilson JR, Baum HB - J Investig Med High Impact Case Rep (2016)

Bottom Line: Among these, it is even more unusual for an initial diagnosis of hypercortisolism to lead to the discovery of underlying MTC.Bilateral adrenalectomy was ultimately required.Medullary thyroid carcinoma should be considered in patients presenting with Cushing's syndrome due to ectopic ACTH secretion, and a multimodality treatment approach is often required.

View Article: PubMed Central - PubMed

Affiliation: Vanderbilt University Medical Center, Nashville, TN, USA.

ABSTRACT
Medullary thyroid carcinoma (MTC) has been described as a source of ectopic ACTH secretion in patients with Cushing's syndrome. This is an infrequent association, occurring in less than 1% of MTC cases. Among these, it is even more unusual for an initial diagnosis of hypercortisolism to lead to the discovery of underlying MTC. Here we present a case of a patient with weakness, diarrhea, and hypokalemia who was found first to have Cushing's syndrome and later diagnosed with metastatic MTC. The patient was treated initially with oral agents to control his hypercortisolism, then with an etomidate infusion after experiencing intestinal perforation. He also received vandetanib therapy targeting his underlying malignancy, as this has been shown to reverse clinical signs of Cushing's syndrome in patients with MTC and subsequent ectopic ACTH secretion. Bilateral adrenalectomy was ultimately required. Medullary thyroid carcinoma should be considered in patients presenting with Cushing's syndrome due to ectopic ACTH secretion, and a multimodality treatment approach is often required.

No MeSH data available.


Related in: MedlinePlus

CT abdomen/pelvis with contrast obtained for evaluation of hypercortisolism. Note the presence of new low-density liver lesions not seen 3 months prior, consistent with metastases. Also, new bilateral adrenal hyperplasia.
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fig2-2324709616643989: CT abdomen/pelvis with contrast obtained for evaluation of hypercortisolism. Note the presence of new low-density liver lesions not seen 3 months prior, consistent with metastases. Also, new bilateral adrenal hyperplasia.

Mentions: Due to his degree of cortisol excess and failure to suppress after high-dose dexamethasone, further evaluation for ectopic ACTH secretion was performed. This included normal levels of vasoactive intestinal polypeptide and 5-hydroxyindoleacetic acid, but calcitonin returned elevated at >5840 pmol/L (0-3.36), as did chromogranin A at 1089 µg/L (<96). Inferior petrosal sinus sampling was considered clinically unnecessary. Computed tomography (CT) scan of the chest and abdomen with contrast showed new adrenal hypertrophy and low-density liver lesions not seen 3 months prior (Figures 1 and 2). Given the elevated calcitonin level, thyroid imaging was pursued and revealed an irregular 2 cm left thyroid mass and lymphadenopathy. Left neck lymph node and liver biopsies confirmed the diagnosis of metastatic MTC at both sites (Figures 3 and 4).


Ectopic ACTH Production Leading to Diagnosis of Underlying Medullary Thyroid Carcinoma.

Matheny LN, Wilson JR, Baum HB - J Investig Med High Impact Case Rep (2016)

CT abdomen/pelvis with contrast obtained for evaluation of hypercortisolism. Note the presence of new low-density liver lesions not seen 3 months prior, consistent with metastases. Also, new bilateral adrenal hyperplasia.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License 1 - License 2 - License 3
Show All Figures
getmorefigures.php?uid=PMC4837669&req=5

fig2-2324709616643989: CT abdomen/pelvis with contrast obtained for evaluation of hypercortisolism. Note the presence of new low-density liver lesions not seen 3 months prior, consistent with metastases. Also, new bilateral adrenal hyperplasia.
Mentions: Due to his degree of cortisol excess and failure to suppress after high-dose dexamethasone, further evaluation for ectopic ACTH secretion was performed. This included normal levels of vasoactive intestinal polypeptide and 5-hydroxyindoleacetic acid, but calcitonin returned elevated at >5840 pmol/L (0-3.36), as did chromogranin A at 1089 µg/L (<96). Inferior petrosal sinus sampling was considered clinically unnecessary. Computed tomography (CT) scan of the chest and abdomen with contrast showed new adrenal hypertrophy and low-density liver lesions not seen 3 months prior (Figures 1 and 2). Given the elevated calcitonin level, thyroid imaging was pursued and revealed an irregular 2 cm left thyroid mass and lymphadenopathy. Left neck lymph node and liver biopsies confirmed the diagnosis of metastatic MTC at both sites (Figures 3 and 4).

Bottom Line: Among these, it is even more unusual for an initial diagnosis of hypercortisolism to lead to the discovery of underlying MTC.Bilateral adrenalectomy was ultimately required.Medullary thyroid carcinoma should be considered in patients presenting with Cushing's syndrome due to ectopic ACTH secretion, and a multimodality treatment approach is often required.

View Article: PubMed Central - PubMed

Affiliation: Vanderbilt University Medical Center, Nashville, TN, USA.

ABSTRACT
Medullary thyroid carcinoma (MTC) has been described as a source of ectopic ACTH secretion in patients with Cushing's syndrome. This is an infrequent association, occurring in less than 1% of MTC cases. Among these, it is even more unusual for an initial diagnosis of hypercortisolism to lead to the discovery of underlying MTC. Here we present a case of a patient with weakness, diarrhea, and hypokalemia who was found first to have Cushing's syndrome and later diagnosed with metastatic MTC. The patient was treated initially with oral agents to control his hypercortisolism, then with an etomidate infusion after experiencing intestinal perforation. He also received vandetanib therapy targeting his underlying malignancy, as this has been shown to reverse clinical signs of Cushing's syndrome in patients with MTC and subsequent ectopic ACTH secretion. Bilateral adrenalectomy was ultimately required. Medullary thyroid carcinoma should be considered in patients presenting with Cushing's syndrome due to ectopic ACTH secretion, and a multimodality treatment approach is often required.

No MeSH data available.


Related in: MedlinePlus