Pituitary adenomas in the setting of multiple endocrine neoplasia type 1: a single-institution experience.

OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed. METHODS: A retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes. RESULTS: A review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pituitary, pancreatic, adrenal, and, finally, rare carcinoid tumors. CONCLUSIONS: Asymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have significant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study.

Cranial Tumor Surgical Outcomes at a High-Volume Academic Referral Center.

OBJECTIVE: To determine adverse event rates for adult cranial neuro-oncologic surgeries performed at a high-volume quaternary academic center and assess the impact of resident participation on perioperative complication rates. PATIENTS AND METHODS: All adult patients undergoing neurosurgical intervention for an intracranial neoplastic lesion between January 1, 2009, and December 31, 2013, were included. Cases were categorized as biopsy, extra-axial/skull base, intra-axial, or transsphenoidal. Complications were categorized as neurologic, medical, wound, mortality, or none and compared for patients managed by a chief resident vs a consultant neurosurgeon. RESULTS: A total of 6277 neurosurgical procedures for intracranial neoplasms were performed. After excluding radiosurgical procedures and pediatric patients, 4151 adult patients who underwent 4423 procedures were available for analysis. Complications were infrequent, with overall rates of 9.8% (435 of 4423 procedures), 1.7% (73 of 4423), and 1.4% (63 of 4423) for neurologic, medical, and wound complications, respectively. The rate of perioperative mortality was 0.3% (14 of 4423 procedures). Case performance and management by a chief resident did not negatively impact outcome. CONCLUSION: In our large-volume brain tumor practice, rates of complications were low, and management of cases by chief residents in a semiautonomous manner did not negatively impact surgical outcomes.

Biologic Agents Are Associated with Excessive Weight Gain in Children with Inflammatory Bowel Disease.

BACKGROUND: Children with active inflammatory bowel disease (IBD) are frequently underweight. Anti-tumor necrosis factor (anti-TNF) agents may induce remission and restore growth. However, its use in other autoimmune diseases has been associated with excess weight gain. Our aim was to examine whether children with IBD could experience excess weight gain. METHODS: A centralized diagnostic index identified pediatric IBD patients evaluated at our institution who received anti-TNF therapy for at least 1 year between August 1998 and December 2013. Anthropometric data were collected at time of anti-TNF initiation and annually. Excess weight gain was defined as ΔBMI SDS (standard deviation score) where patients were (1) reclassified from "normal" to "overweight/obese," (2) "overweight" to "obese," or (2) a final BMI SDS >0 and ΔSDS >0.5. RESULTS: During the study period, 268 children received anti-TNF therapy. Of these, 69 had sufficient follow-up for a median of 29.3 months. Median age at first anti-TNF dose was 12.8 years. At baseline, mean weight SDS was -0.7 (SD 1.4), while mean BMI SDS was -0.6 (1.3). Using baseline BMI SDS, 11.6% were overweight/obese. At last follow-up (LFU), however, the mean ΔBMI SDS was 0.50 (p < 0.0001). However, 10 (17%) patients had excess weight gain at LFU; 3 patients were reclassified from "normal" to "obese," and 7 had a final BMI SDS >0 and ΔSDS >0.5. CONCLUSIONS: Pediatric patients with IBD may experience excess weight gain when treated with anti-TNF agents. Monitoring for this side effect is warranted.

Polymyalgia Rheumatica and its Association with Cancer.

OBJECTIVE: Polymyalgia rheumatica (PMR) is a common rheumatologic disease in the elderly population. Studies on the relationship between PMR and cancer have yielded mixed results and have been limited by multiple factors. This study examined the association between PMR and development of cancer in a community cohort. METHODS: A population-based cohort of 359 patients with PMR diagnosed between 1/1/1970 and 12/31/1999 and followed to 12/31/2013 was assembled along with a comparison cohort of 357 subjects. Records of the PMR and comparator subjects were reviewed for details concerning diagnosis of cancer. The cumulative incidence of malignancy in patients with and without PMR, adjusted for the competing risk of death, was estimated and compared using methods of Gray. Cox proportional hazards models were used to assess the trends in malignancy over time. RESULTS: There was no significant difference in the prevalence of malignancy prior to PMR incidence date/index date between the two groups with prior malignancies in 41 (11%) of patients with PMR, and 50 (14%) of non-PMR subjects (p-value=0.31). As well, there was no difference in the cumulative incidence of malignancy at 10 years following PMR incidence between patients with PMR and non-PMR subjects (cumulative incidence at 10 years ± SE: PMR 13.8 ± 2.0, control 13.1 ± 2.0; p-value=0.89). CONCLUSION: There is no increased risk of malignancy in patients who are diagnosed with PMR when compared to subjects without PMR in this population-based cohort.