Unusual Presentation of a Thyroid Malignancy: A Case Report

Medullary thyroid carcinoma is a rare malignancy. We report a case of Medullary thyroid carcinoma in a 56-year-old male patient. Patient presented with lower limb paresis and severe hypokalemia. Cushing’s syndrome is a rare complication of Medullary thyroid carcinoma [MTC] and is due to ectopic Adrenocorticotropic [ACTH] secretion by tumor cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Entire clinical history, laboratory investigations, microscopic pictures are discussed in detail.

A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years. Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome

Medullary thyroid carcinoma (MTC) may rarely present with paraneoplastic syndromes. Among the most frequent ones are the appearance of diarrhea and ectopic Cushing syndrome (ECS). The ECS in the context of MTC is usually present in patients with distant metastatic disease. The use of drugs such as ketoconazole, metyrapone, somatostatin analogs and etomidate have been ineffective alternatives to control hypercortisolism in these patients. Bilateral adrenalectomy is often required to manage this situation. Recently, the use of tyrosine kinase inhibitors has been shown to be a useful tool to achieve eucortisolism in patients with metastatic MTC and ECS. We present a patient with sporadic advanced persistent and progressive MTC with lymph node and liver metastases, which after 16 years of follow-up developed an ECS. After one month of 300 mg/day vandetanib treatment, a biochemical and clinical response of the ECS was achieved but it did not result in significant reduction of tumor burden. However the patient reached criteria for stable disease according to response evaluation criteria in solid tumors (RECIST 1.1) after 8 months of follow-up.

Utilidad del PET/CT con análogos de somatostatina marcados con Ga68 en el diagnóstico de síndrome de Cushing secundario a tumor neuroendocrino pancreático / Pancreatic neuroendocrine tumor diagnosed with positron emission tomography/ct scan (PET/CT): report of one case

We report a 28 years old woman consulting for weight gain, progressive muscle weakness and appearance of facial plethora. Laboratory showed high cortisol and ACTH levels. Magnetic resonance of sella turcica was normal and an abdominal magnetic resonance showed a pancreatic mass, suggestive of a neuroendocrine tumor. A PET/CT with somatostatin analogues demonstrated an intense over-expression of somatostatin receptors in the tumor. The patient was subjected to a pancreatectomy and the day after surgery, ACTH levels decreased to less than 5 pg/ml. The patological study of the surgical piece showed a neuroendocrine carcinoma. The patient had a good postoperative evolution.

A Case of Ectopic ACTH Syndrome Associated with Bronchial Carcinoid / 대한내분비학회지

Fifteen percent of patients with Cushing's syndrome could be accompanied with nonpituitary tumors that secrets ACTH-the ectopic ACTH syndrome. Among them, bronchial carcinoid is one of the rare causes of the ectopic ACTH syndrome. We experienced a 60-year-old woman who showed conditions of weight gain, generalized edema, hypokalemia, diabetes mellitus, and right middle lung mass. Histologically, the tumor was diagnosed with bronchial carcinoid and was confirmed as an ectopic ACTH syndrome through immunohistochemical staining of ACTH. After resection of the lung mass, blood sugar, electrolytes, generalized weakness, and blood pressure improved.