Mucoepidermoid Carcinoma of the Thyroglossal Duct: Two Rare Entities in a Unique Case Report.

BACKGROUND: Mucoepidermoid Carcinoma (MEC) of the thyroid represents less than 0.5% of all thyroid neoplasms. Thyroglossal duct cyst carcinoma is a rare condition with only approximately 300 cases reported. CASE REPORT: A 34-year-old pregnant woman at 37 weeks gestation presented to an endocrinological center for primary autoimmune hypothyroidism. The thyroid ultrasound revealed a pseudonodular pattern. The patient was followed up after two years. She reported a full-term delivery without complications. A new thyroid ultrasound was performed, showing a cystic lesion in the median suprathyroid area, measuring 6 x 9 x 10 mm, not previously reported. After 4 months, the suprathyroid cystic lesion was confirmed by thyroid ultrasound, measuring 6 x 11 x 12 mm. The patient was referred for fine-needle aspiration cytology. Cytological examination showed lymphocytes, red blood cells, and some epithelial aggregates with large cytoplasm and nuclear polymetrism with oxyphilic aspects. The patient underwent the Sistrunk procedure for the suprathyroid lesion. The histological examination revealed lymphocytic thyroiditis in heterotopic thyroid tissue with solid cell nest, epidermoid epithelium, and mucus-secreting cells suggestive of low-grade mucoepidermoid carcinoma. The immunohistochemistry study was positive, exhibiting thyroid transcription factor 1 and cytokeratin-19. No positivity was observed for thyroglobulin, calcitonin, galectin-3, and Hector Battifora mesothelial antigen 1. The recent follow-up examination, 13 months after the surgery, has been found negative for disease recurrence. CONCLUSION: This is the first case of an MEC occurring within a thyroglossal duct. Considering the age of the patient, the histological diagnosis, and the absence of thyroid nodules and metastasis, we decided on the Sistrunk procedure without total thyroidectomy.

Endocrine Toxicities of Antineoplastic Therapy: The Adrenal Topic.

Immune checkpoint inhibitors (ICIs) have improved survival in patients affected by several solid tumours at the cost of new autoimmune adverse events. Endocrine toxicity is frequently reported in patients treated with these agents, mainly as thyroid dysfunction and hypophysitis. Primary adrenal insufficiency is reported in 1-2% of patients receiving a single ICI, but its rate is approximately 5% in patients treated with a combination of two ICIs. The clinical presentation of adrenal insufficiency may be insidious due to symptoms that are not specific. The same symptoms in cancer patients are frequently multifactorial, rendering the early diagnosis of adrenal insufficiency challenging in this group of patients. As adrenal insufficiency can be fatal if not rapidly diagnosed and treated, oncologists should be aware of its clinical presentations to timely involve endocrinologists to offer patients the appropriate management. In parallel, it is essential to educate patients, their caregivers, and relatives, providing them with detailed information about the risk of adrenal insufficiency and how to manage alarming symptoms at their onset. Finally, large collaborative trials are needed to develop appropriate tests to assess better the personal risk of drug-induced adrenal insufficiency and its early diagnosis and treatment, not only in cancer patients.

Cushing's syndrome due to a bronchial ACTH-secreting carcinoid successfully treated with radiofrequency ablation (RFA).

CONTEXT: The ectopic production of ACTH is responsible for approximately 10% of cases of Cushing's syndrome. Whenever possible, once hypercortisolemia is under control with medical therapy, the final treatment consists of surgical excision of the tumor. We report a case of a patient with high surgical risk and poor response to medical therapy in which hypercortisolemia has been successfully treated with radiofrequency ablation of the bronchial carcinoid tumor. CASE PRESENTATION: A 43-year-old woman came to our hospital because of severe and rapidly worsening signs and symptoms of hypercortisolism over the previous 3 months. Hormonal tests suggested the presence of Cushing's syndrome due to ectopic ACTH production. Imaging studies detected an 8-mm pulmonary nodule with fluorine-18-fluorodeoxyglucose uptake localized in the middle right lobe. The patient started therapy with ketoconazole with poor response. Middle right lobectomy was indicated but, due to the patient's very high surgical risk, a thermal ablation with radiofrequency of the bronchial nodule was performed. OUTCOMES AND RESULTS: After the procedure, ACTH and cortisol levels dropped and fluorine-18-fluorodeoxyglucose positron emission tomography showed complete response to treatment. Clinical conditions progressively improved, and 6 weeks later, the patient underwent middle lobectomy without complications. Histology showed a 0.7-cm ACTH-producing typical bronchial carcinoid tumor. CONCLUSIONS: Thermal ablation with radiofrequency allows achieving a rapid control of hypercortisolism with subsequent improvement of symptoms. This procedure should therefore be considered as a viable therapeutic option in those cases of bronchial ACTH-secreting tumors in which the surgical approach is initially contraindicated.

"Occult" ovarian Leydig cell tumor: when laboratory tells more than imaging.

Hyperandrogenism is a common finding in premenopausal age and is generally caused by polycystic ovarian syndrome or other benign disease. Androgen-secreting tumors represent only 0.2 % of the causes of hyperandrogenism and usually present with severe clinical features, abrupt onset, and very high androgens levels. We describe here three cases of occult ovarian Leydig cell tumors suspected on the basis of severe clinical features of hyperandrogenism rapidly worsening, with elevated serum total testosterone levels, in which bilateral ovariectomy was performed and tumor was confirmed by post-operative histology. In all three cases, imaging was negative for ovarian tumor. Moreover, in one case the confounding concomitant finding of bilateral adrenal masses posed an additional challenge. Our experience highlights that testosterone levels represent the most helpful marker in the diagnosis of androgen-secreting ovarian tumor. In the absence of imaging findings, bilateral ovariectomy should be indicated, if supported by unequivocal clinical and laboratory data.

Differentiated thyroid cancer in two patients with resistance to thyroid hormone.

BACKGROUND: Resistance to thyroid hormone (RTH) is a genetic disease characterized by a reduced responsiveness of the pituitary and peripheral target tissues to TH. We describe two patients with RTH in whom differentiated thyroid cancer was diagnosed. PATIENT FINDINGS: In both patients, RTH was unequivocally diagnosed and both underwent thyroidectomy for multinodular goiter. In Patient 1, histology showed a papillary thyroid carcinoma pT2. Because serum thyrotropin (TSH) levels were elevated even while the patient was taking 150 µg daily of levothyroxine (LT(4)), the patient was treated with (131)I 100 mCi for ablation of the thyroid remnant without discontinuing his LT(4) therapy. We obtained a clinically adequate response by administering LT(4) 175 µg/day (2.18 µg/kg), but the serum TSH was persistently elevated on this dose. The patient was considered "free of disease" after 8 years of follow-up. In Patient 2, histology revealed a papillary microcarcinoma (0.6 cm). Diagnostic whole-body scan was performed while the patient was taking 100 µg/day LT(4), a time that his serum TSH was 38 µU/mL. Only a small remnant was revealed, so (131)I remnant ablation was not performed. While taking LT(4) at a dose of 175 µg/day (3 µg/kg), the serum TSH was persistently high, serum TH levels were in the normal-high range, and he appeared to be clinically euthyroid. There has been no evidence of persistent or recurrent thyroid carcinoma in ultrasonography and thyroglobulin measurements that have been performed on a yearly basis for 3 years. CONCLUSION: Patients with thyroid carcinoma and RTH are a unique model of thyroid cancer in which follow-up likely occurs in the setting of constantly elevated serum TSH concentrations. The concern in these patients is that their persistent elevation of serum TSH may have an adverse effect on their thyroid cancer, and management choices in terms of the dose of LT(4) that provides the optimum lowering of serum TSH without toxicity are difficult, particularly in the situation wherein, as was the case with one of our patients, there is cardiac disease.

Post-surgery severe hypocalcemia in primary hyperparathyroidism preoperatively treated with zoledronic acid.

Zoledronic acid is a newly FDA-approved bisphosphonate for the treatment of hypercalcemia of malignancy. Although the safety and efficacy of this drug in treating hypercalcemia associated with hyperparathyroidism have not yet been established in clinically controlled trials, its off-label use is not uncommon. We describe a patient with primary hyperparathyroidism treated with zoledronic acid who developed severe postoperative hypocalcemia. A 64-yr-old woman was admitted with severe hypercalcemia. She was treated with rehydration, calcitonin, methylprednisolone, furosemide as well as 4 mg/day of zoledronic acid for two consecutive days. Primary hyperparathyroidism caused by a right inferior parathyroid lesion was diagnosed. While awaiting surgery, she continued furosemide, methylprednisolone and hydration: after one week, serum calcium had fallen to such a low level that a short-term calcium carbonate supplementation was required. Three weeks after admission, the patient underwent selective right inferior parathyroidectomy, followed by reduction of PTH. During the postoperative period the patient presented severe hypocalcemia resistant to the usual treatment. Serum calcium levels returned to normal three months after surgery. The severity of hypocalcemia and the resistance to conventional treatments suggest that the effect of hungry bone syndrome could be worse in patients treated with bisphosphonates in the preoperative phase.