Clinico-pathologic profile and outcomes of pediatric endocrine patients managed by endocrine surgeons: Experience over three decades in a tertiary center in India.

BACKGROUND: Pediatric endocrine disorders requiring surgical intervention are rare and so are experienced surgeons dealing with these. The aim of the current study was to investigate disease profile and perioperative outcome of pediatric patients with surgical endocrine disorders in an endocrine surgery unit. METHODS: This retrospective study (Sep 1989-Aug 2019) consisted of pediatric endocrine surgery patients (<18 years) who were managed by a team of pediatric endocrinologists and endocrine surgeons at our center. Patients were divided into three cohorts consisting of a decade each. Clinico-pathologic variables, perioperative events operative and follow-up details were recorded. RESULTS: A total of 332 children were included and their mean age was 14.6 ± 3.9 years (M:F = 1:1.6). Thyroid disorders were most prevalent (59.8%), followed by adrenal (28.2%), parathyroid (10.4%), and pancreas (1.5%). Incidence of benign, malignant, and congenital/developmental disorders were 65.4, 28.1 and 8.3, respectively. Familial association was observed in 8.9% children, which is highest among pheochromocytoma patients. Overall, 201 thyroidectomies + associated procedures, 35 parathyroidectomies, 96 adrenal and paraganglioma resections, and 5 pancreatic procedures were performed. Median hospital stay was 5.6 ± 4.1 days. The number of cases increased significantly over 3 decades. Clinical profile and outcome did not vary except for significant decrease in incidence of malignant pathology (p = 0.04) and increase in VHL cases (p = 0.04) in the last decade though overall increase in familial cases was nonsignificant (p = 0.11). No perioperative mortality was observed except for 3% after adrenalectomy. CONCLUSION: A team of dedicated endocrine surgeons and pediatric endocrinologists is effective in management of pediatric endocrine surgery.

Pediatric Primary Hyperparathyroidism: Experience in a Tertiary Care Referral Center in a Developing Country Over Three Decades.

BACKGROUND: There is limited experience in managing pediatric primary hyperparathyroidism (PHPT). The aim of this study was to analyze the clinical presentation and outcome of surgery in children with PHPT managed at a tertiary referral center. METHODS: This retrospective study (September 1989-August 2019) consisted of 35 pediatric PHPT patients (< 18 years) who underwent parathyroidectomy. Clinico-pathologic profile and outcome were noted. RESULTS: The mean age of cohort was 15.2±2.9 years and girls outnumbered boys (M:F = 1:1.9). Familial and symptomatic disease was noted in 8.5 and 94.3% cases, respectively. Skeletal manifestations (83%) were the commonest followed by renal (29%). Fifty-four percent children had skeletal fractures, and 23% were bed-ridden. Among rare manifestations, hypercalcemic crisis, recurrent pancreatitis and stigmata of rickets were observed in 2.8, 11.4 and 14.2% children, respectively. Mean calcium concentration was 12.1 ± 2.0 mg/dl and PTH 91.8 ± 66.5 pmol/L. The sensitivity of preoperative imaging in parathyroid localization was 91.4%. Minimally invasive parathyroidectomy (MIP) was performed in 40% cases. Parathyroid adenoma was observed in 91.4% patients, whereas remaining had hyperplasia. Thirty-four percent suffered from Hungry bone syndrome in postoperative period. The cure rate following primary surgery was 97%. One child with persistent PHPT had successful re-operation. Median follow-up was 5 (1-17) years, and no recurrence or familial disease was revealed during this period. CONCLUSION: Majority of pediatric patients present with symptomatic PHPT. Despite relatively high incidence of familial disease select pediatric patients can undergo successful MIP.

Changing Profile of Primary Hyperparathyroidism Over Two and Half Decades: A Study in Tertiary Referral Center of North India.

BACKGROUND: Most of the cases of PHPT in developing countries present in symptomatic stage, some even in very advanced stage but in recent years the trend seems to be changing. This has been corroborated from few recently published literature from developing countries. The scope of this study is to further carry out an in-depth analysis of various clinical and biochemical parameters of PHPT patients at a tertiary care center of northern India. METHODS: In this retrospective analysis, a total of 333 patients with PHPT from the year 1990 to 2016 were studied. The study population was divided into three subgroups based on the time span; 1990-1999 (n = 34), 2000-2009 (n = 112), 2010-2016 (n = 187), and clinical and biochemical parameters were compared. RESULTS: The clinical presentation has evolved progressively with increase in older age group (35 vs 39 vs 43.85, p < 0.001), less patients with musculoskeletal symptoms (85.3 vs 76.8 vs 61%, p = 0.002) and less patients with severe bone disease (29.4 vs 10.7 vs 10.7%, p = 0.088). Biochemical parameters also showed a changing trend with significant decrease in mean S. Alkaline phosphatase (1393 vs 965 vs 414.8 IU/L, p < 0.001) and S. iPTH (837.52 vs 812.89 vs 635.74 pg/mL, p = 0.02). Vitamin D nutrition status is still suboptimal but shows improvement, and more patients are insufficient as compared to previous deficient state (mean S. Vitamin D-10.31 vs 16.16 vs 25.30 ng/mL, p < 0.001). CONCLUSIONS: Our study reveals a change in trend in PHPT which is similar to evolution of this disease in western population and positively corroborated with observations from China, Hong Kong and Turkey.

Correlation of digital mammography and digital breast tomosynthesis features of self-detected breast cancers with human epidermal growth factor receptor type 2/neu status.

CONTEXT: Breast cancer is a heterogeneous disease with several histological subtypes. Its prognosis and management are influenced by human epidermal growth factor receptor type 2 (HER2/neu) expression. Varying grades of HER2/neu overexpression are likely to have different morphological features. Digital breast tomosynthesis (DBT) enhances lesion visibility and hence that it may reveal features closer to histomorphological findings. AIMS: The aim of this study is to correlate digital mammography (DM) and DBT findings of self-detected tumors with HER2/neu status, to determine whether differences in imaging features can help predict the degrees of HER2/neu overexpression. SETTINGS AND DESIGN: Prospective study conducted in a tertiary care hospital. METHODS: For 100 consecutive patients with self-detected lumps, DM and DBT data were reviewed by two radiologists who were blinded to histopathology. Of these, 63 patients with histologically proven breast cancer were recruited and their DM and DBT findings compared and correlated with HER2neu status (scores 0-3+). STATISTICAL ANALYSIS: Pearson's Chi-squared test and Fisher's exact test were used (SPSS version 22.0, IBM). RESULTS: Morphology of lesions at both DM and DBT varied with HER2/neu status (P = 0.04 and 0.015, respectively). HER2-0 tumors mostly presented as masses without microcalcifications (88.8%), while most of HER2-3+ tumors as masses or asymmetries with microcalcifications (61.9%). The presence or absence of calcifications varied significantly with HER2/neu status. Breast imaging-reporting and data system (BI-RADS) scoring varied significantly (P < 0.001) with higher HER2 signal, more frequently associated with BI-RADS 5 score. CONCLUSION: DM and DBT features vary with the intensity of HER2 immunostaining. Higher BI-RADS scores, microcalcifications, and spiculated margins are frequently associated with HER2/neu 3+ lesions.

Surgical Management of Primary Hyperparathyroidism in the Era of Focused Parathyroidectomy: A Study in Tertiary Referral Centre of North India.

BACKGROUND: Despite the benefits of focused parathyroidectomy (FPTx), few studies have questioned its durability with lower long-term cure rates than bilateral or conventional parathyroidectomy (CPTx). The objective of this study is to bring out the information on the type of surgical management versus cure rate, recurrence, and role of intra-operative parathyroid hormone (IOPTH) level monitoring of PHPT patients. MATERIAL AND METHODS: This was a retrospective study of all PHPT patients treated at our center based on operative approach (CPTx vs FPTx) or use of IOPTH. Treatment failure was divided into persistent or recurrent disease, based on documentation of hypercalcemia in combination with an inappropriate PTH within 6 months or more of surgery, respectively. RESULTS: Overall, 50.78% patients underwent CPTx and 49.32% FPTx. 29 FPTx were converted to CPTx. Intention to treat analysis between CPTx and FPTx showed that the persistence rate was not statistically different at 2.54% and 4%, respectively (P = 0.98). Furthermore, when the persistence rate was scrutinized by a treatment received (TR) instead of ITT analysis, the persistence rate was higher for the patients who underwent TR-CPTX than for the patients subjected to TR-FPTX (3.22% vs 1.08%) but not significant statistically. We further analyzed the outcome of FPTx with IOPTH (n = 213) and FPTx without IOPTH (n = 28). The outcome did not differ between two groups statistically. CONCLUSION: FPTx yields a similar success rate as compared to CPTx even in symptomatic PHPT patients and can be performed safely even without intra-opeartive adjunct IOPTH in selected patients.