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Background:Thyroid nodules are exceedingly common, leading to costly interventions for many lesions that ultimately prove benign. Therefore, a reliable, noninvasive method to identify which nodules warrant fine needle aspiration and/or follow-up on the basis of a reasonable likelihood of malignancy is highly desirable. American College of Radiology (ACR) created a standard terminology (lexicon) to describe all thyroid nodules on sonography and standardized TI-RADS risk-stratification system to identify nodules that warrant biopsy and/or follow-up. Many healthcare institutions including UPMC adapted the TI-RADS scoring system in order to identify most clinically significant malignancies while reducing the number of biopsies and follow-up ultrasounds performed on benign nodules. According to ACR, TI-RADS category 3 nodules <1.5 cm and TI-RADS category 4 nodules <1 cm do not warrant follow-up imaging. There are no validation studies on TI-RADS follow-up recommendations. Methods:We completed a retrospective chart review from UPMC endocrine surgery thyroid nodule database from 2002 to 2012. We identified 57 nodules that showed a change in size during follow-up and had surgical data. Patient demographics, nodule baseline TI-RADS category, size, follow-up volume change and histopathological data were recorded. We reviewed ultrasound images and calculated TI-RADS category at baseline and during follow-up. Results:TI-RADS category 1-2 (TR1 and TR2) nodules(n=4) did not show any change in size over an average of 6.5 years confirming the recommendations that TR1 and TR2 nodules do not need follow-up.TI-RADS category (TR3) nodules (n=22) showed an average 225% change in volume over 4 years of follow-up. TR3 nodules <1.5 cm showed 397% volume change; 3 out of 15 (20%) nodules that showed a change in size proved to have thyroid cancer >1cm. TI-RADS category (TR4) nodules(n=31) showed a 786% volume change over 2.6 years of follow-up. TR4 nodules <1 cm, 5/14(35%) proved to have thyroid cancer >1 cm in follow up. Conclusions:TR1 and TR2 nodules did not show thyroid cancer during follow-up validating ACR recommendations not to follow these nodules. 3/15(26.5%) TR3 nodules <1.5 cm that showed a change in volume proved to have thyroid cancer. 5/14(35%) TR4 nodules <1cm that changed in volume were found to have thyroid cancer. Further studies are needed to identify nodules that require follow-up in order to decrease the misdiagnosis of thyroid cancer.

Introduction: Beckwith-Wiedemann Syndrome (BWS) is an autosomal dominant disorder of chromosome 11p15 that results in increased IGF-2 and CDK1NC. This leads to excessive cell proliferation and tumor formation. The following highlights a case of metastatic pheochromocytoma in a patient with BWS. Clinical Case: A 30-year-old male presented with sudden onset blurry vision without any associated complaints. His past medical history was significant for BWS. His family history was negative for uncontrolled hypertension, sudden death, thyroid cancer or hyperparathyroidism. Physical examination was notable for an elevated systolic blood pressure of 200/160 mm of hg and fundoscopy revealed features of hypertensive emergency. Laboratory investigations revealed an elevated plasma normetanephrine [10445 pg/ml (normal: <148)], metanephrine [93 pg/ml (normal: <57)], total metanephrine [10538 pg/ml (normal: <205)], epinephrine [134 pg/ml (normal:<50)], norepinephrine [23526 pg/ml (normal: 112-658), total catecholamine level [23660 (normal: 123-671pg/ml)] and dopamine [403 pg/ml (normal<30)] levels. His PTH, corrected serum calcium, gastrin, insulin, carcinoembryonic antigen, calcitonin levels and basal metabolic panel were all normal. MRI of the abdomen demonstrated bilateral adrenal nodules with a large mass encasing the celiac axis along with evidence of hepatic lesions. I-123 MIBG scan showed mild radioactive tracer uptake in the adrenal nodules and mass near the celiac axis but not in the hepatic lesions. PET scan confirmed MRI findings and was negative for any evidence of malignancy in the chest, pelvis and skeleton. MRI of the brain was negative for metastasis as well as pituitary abnormalities. Ultrasound-guided liver biopsy was positive for malignant cells that stained positive for chromogranin and synaptophysin confirming the diagnosis of metastatic pheochromocytoma. He was treated with phenoxybenzamine, diltiazem and lisinopril. He underwent cycles of cyclophosphamide, vincristine and dacarbazine. Genetic testing revealed a variant in SDHD gene which was of uncertain significance. Repeat biochemical testing on follow up after a year and a half showed a decreased plasma normetanephrine [487pg/ml] and metanephrine levels [110 pg/ml] in comparison to his levels on presentation. Repeat imaging revealed a decrease in tumor burden including bilateral adrenal nodules, celiac axis mass and hepatic metastases. Conclusion: This is an unusual case of malignant pheochromocytoma in the absence of SDHB mutation in a patient with BWS. Genetic causes in these patients are yet to be determined. However, genes H19 and KCNQ1OT1 have been implicated in addition to IGF-2 and CDK1NC

Introduction: Hypophysitis is an acute or chronic inflammation of the pituitary gland and is an important diagnostic consideration in a patient with a sellar lesion. The annual incidence of hypophysitis is estimated to be 1 in 7–9 million and it accounts for approximately 0.4% of pituitary surgery cases. The following highlights a rare case of isolated IgG4-related hypophysitis Clinical Case: A 63-year-old Caucasian female presented with sudden onset of diplopia and decreased visual acuity. This was associated with a 3-month history of headaches and 5-lbs weight loss. Past medical history was significant for hypertension and a 1.5cm sellar/suprasellar mass incidentally discovered during the work-up for persistent headaches 1-month prior. Initial anterior pituitary hormone evaluation was normal and the patient was scheduled for endoscopic endonasal resection of a presumed non-functioning pituitary adenoma. Family history was negative for pituitary tumors or hyperparathyroidism. Physical examination was notable for medial deviation of her left eye but neurologic examination was otherwise normal. Laboratory studies were notable for a normal TSH [1.769 uIu/ml (normal: 0.3–5.0)] and low free T4 [0.44 ng/ml (normal: 0.89–1.78)] consistent with central hypothyroidism; an inappropriately normal FSH for a postmenopausal woman [5.6 mIu/ml (normal: 0.3–10.5), and a normal prolactin level [16 ng/ml (0.6–20)]. An 8am cortisol was low at 2mcg/dL (5–21) with an ACTH level of 10 pg/mL (9–46). IGF-1 was normal at 89 ng/mL (41–279). Repeat pituitary MRI imaging demonstrated a homogenously enhancing sellar/suprasellar mass measuring 3.8 cm with displacement of the optic chiasm. Serum IgG4 levels were normal. The patient was started on 50mg IV hydrocortisone every 8 hours for central adrenal insufficiency and levothyroxine 88 mcg daily for central hypothyroidism and underwent an endoscopic endonasal biopsy of the lesion. Surgical pathology was notable for plasma cell-rich lymphohistiocytic hypophysitis and IgG4 plasma cells constituted >40% of the total plasma cell population. The patient subsequently received 1g of rituximab and repeat imaging one week later showed marked improvement in the size and extent of the lesion. The patient was discharged on prednisone and levothyroxine and received a second dose of rituximab at follow-up. The patient reports a decrease in the frequency of her headaches but continues to endorse diplopia. Conclusion: IgG4-related hypophysitis typically presents as part of a multifocal systemic process. This case highlights a rare entity of IgG4-related hypophysitis without other features of systemic disease and with normal serum levels of IgG4. Although glucocorticoids are universally regarded as the first line of therapy, an immunosuppressive agent or B-cell depletion therapy such as Rituximab may improve remission and decrease the risk of relapse.