A Case of Diffuse Thyroid Lipomatosis With Amyloid Deposits Presenting With Thyrotoxicosis.

Diffuse thyroid lipomatosis (DTL) is a rare entity of unknown etiology that can be associated with amyloidosis and rarely, thyrotoxicosis. Here, we present a case of DTL with amyloid deposits and concurrent thyrotoxicosis. A 64-year-old South-Asian woman with a several-year history of an enlarging goiter, unintentional weight loss, and work-up 10 months prior suggestive of thyroiditis presented with a viral syndrome in setting of several weeks of progressive fatigue. Her examination was notable for resting sinus tachycardia and massive painless goiter. Initial work-up revealed nephrotic range proteinuria with hypoalbuminemia, which progressed to end-stage-renal disease, elevated inflammatory markers, and elevated free thyroxine (FT4) with a suppressed thyrotropin. Hemodialysis was initiated. Further testing revealed a negative antithyroid antibody panel, an enlarged fatty thyroid per thyroid ultrasound and neck computed tomography, and normal 24-hour uptake on radioactive iodine uptake scan. Both renal and thyroid core biopsies showed amyloid deposits, with the latter confirming benign adipose tissue with entrapped thyroid follicles. Given her rising FT4 levels and persistent tachycardia, methimazole and atenolol were initiated. FT4 levels nearly normalized after uptitration of methimazole and dosing after dialysis. Although the etiopathogenesis and natural history of DTL remain unclear, we discuss the possible mechanisms of thyrotoxicosis in our patient.

Endoscopic Endonasal Transsphenoidal Surgery for Patients with Prolactinomas: Indications and Outcomes.

OBJECTIVE: Clinical paradigms and consensus recommend dopamine agonists (DAs) as the primary treatment for prolactinomas. However, medically treated patients also encounter challenges such as DA resistance, intolerable side effects, and recurrence of hyperprolactinemia after DA withdrawal. Technical advances in transsphenoidal resection, with an endoscopic endonasal approach, have led to improved visualization of tumor, decreased postoperative morbidity, and shortened length of stay. We examined the indications and outcomes in patients with prolactinomas who underwent surgical resection at our center. METHODS: A retrospective analysis was performed of 60 consecutive patients with prolactinomas who underwent endoscopic endonasal transsphenoidal resection between August 2010 and July 2019 and were followed by the same multidisciplinary team. RESULTS: Women comprised 73% of surgical cases, and 60% of the tumors were macroadenomas. The most common primary surgical indication was patient preference (26.6%) followed by DA intolerance (25%) and DA failure (18.3% inadequate shrinkage, 15% persistent hyperprolactinemia, 11.7% both). Gross total resection was noted in 83% and length of stay was 1 day in 92% of patients. Early remission (postoperative day 1 normalization of prolactin off DA therapy) was seen in 71% of all patients, 91% of microadenomas, 56% of macroadenomas, 65% of Knosp grade 0-2 macroadenomas, and 75% of macroadenomas operated on with expectation of a cure. Only 3 patients had recurrence, at 4.3, 3.3, and 1.6 years of follow-up, respectively. CONCLUSIONS: Endoscopic endonasal resection is a viable option for management of patients with prolactinomas in the setting of a high-volume pituitary center, with minimal postoperative complications.

A Remarkable Response of Granulomatous Hypophysitis to Infliximab in a Patient With a Background of Crohn's Disease-A Case Report.

Background: Hypophysitis is primary or idiopathic or secondary to another disease process. The histologic subtypes of hypophysitis are lymphocytic, granulomatous, xanthomatous, xanthogranulomatous, or IgG4-related. Granulomatous hypophysitis is the second most common form and is characterized by multinucleated giant cells with granulomas and histiocytes. It can be idiopathic or secondary to another process such as infection, sarcoidosis, vasculitis, dendritic cell disorders, Crohn's disease (CD) or a reaction to rupture of a Rathke's cyst or pituitary adenoma. We present a case of granulomatous hypophysitis in a patient with CD who had resistance to corticosteroids but a dramatic response to immunosuppressive therapy with anti-tumor necrosis factor (TNF)-α therapy. Case description: A 43-year-old woman with a 9-year history of ileal and colonic CD presented to the Pituitary Center with headaches, visual disturbance, fatigue, nausea, and secondary amenorrhea. She was not on active therapy for her CD at the time of presentation and had no gastrointestinal symptoms. Hormonal evaluation revealed hyperprolactinemia, secondary hypothyroidism and adrenal insufficiency. MRI revealed a 12 × 12 × 19 mm sellar lesion abutting the optic chiasm, reported as a macroadenoma. The patient underwent endoscopic transsphenoidal biopsy of the pituitary mass. Pathology revealed granulomatous hypophysitis. Evaluation for secondary causes of hypophysitis, apart from CD, was negative. Despite a course of high dose prednisone, her symptoms and MRI findings worsened and she developed symptoms consistent with diabetes insipidus. Using a personalized medicine approach, she was started on anti-(TNF)-α therapy with infliximab combined with azathioprine, which are indicated for treatment of CD. Her headaches and polyuria resolved and her menstrual cycles resumed. MRI at 3 months and more than 1.5 years after initiation of anti-TNF-α therapy revealed durable resolution of the pituitary mass. Conclusion: To our knowledge, this is the first report of successful use of anti-TNF-α therapy for a patient with granulomatous hypophysitis, in this case associated with a previous diagnosis of CD. Although glucocorticoids are used frequently as first-line therapy for primary hypophysitis, granulomatous hypophysitis can be corticosteroid resistant and other immunosuppressive approaches may need to be considered within the context of the patient.