[Show abstract][Hide abstract] ABSTRACT: Thymic neuroendocrine (NE) tumors are a rare manifestation of multiple endocrine neoplasia syndrome type 1 (MEN-1). They are malignant and aggressive tumors and form a major cause of mortality in MEN-1. Transcervical thymectomy (TCT) at the time of parathyroid surgery for primary hyperparathyroidism (PHPT) in MEN-1 usually prevents thymic NE tumors. We report a 56-year-old nonsmoker male with sporadic MEN-1 who presented with thymic NE carcinoma developing rapidly within a span of 8 months after subtotal parathyroidectomy and TCT for PHPT. We present a brief review of literature on this rare NE malignancy, focusing on its occurrence despite TCT. This case highlights the fact that thymic NE carcinoma may develop even after TCT in MEN-1. Regular surveillance for these aggressive thymic NE tumors is mandatory even after TCT in MEN-1 setting.
Indian journal of endocrinology and metabolism. 07/2013; 17(4):743-6.
[Show abstract][Hide abstract] ABSTRACT: PURPOSE: The intraoperative parathyroid hormone (IOPTH) monitoring is a useful adjunct for predicting whether a cure has been obtained during parathyroidectomy. We studied the influence of vitamin D status and parathyroid tumor weight on the IOPTH dynamics for predicting a cure in patients with symptomatic primary hyperparathyroidism. METHODS: Fifty-nine primary hyperparathyroidism patients with a single adenoma underwent curative surgery. Patients were grouped according to their serum 25-hydroxy vitamin D levels (deficient, insufficient and sufficient) and tumor weights (small, large and giant). The IOPTH results in patient groups were compared, and the percentage of the IOPTH decrease was examined for a correlation with the serum 25-hydroxy vitamin D level and tumor weight. RESULTS: The sensitivity, specificity and overall accuracy of IOPTH in predicting a cure of hyperparathyroidism were 94.8, 100 and 93.2%, respectively. The percentage decrease in the IOTPH was significantly higher in the vitamin D deficient, compared to the vitamin D sufficient patients (p = 0.012); and in the patients with larger tumors, compared to those with smaller parathyroid tumors (p = 0.02). A statistically significant correlation was found between the percentage decrease in the IOPTH at 10 min post-tumor excision and the serum 25-hydroxy vitamin D level (p = 0.037), but not with the tumor weight (p = 0.208). CONCLUSIONS: The IOPTH can accurately predict a cure in patients with severe primary hyperparathyroidism. The percentage of decrease in the IOPTH is steeper in patients with lower serum 25-hydroxy vitamin D levels and larger parathyroid tumors.
[Show abstract][Hide abstract] ABSTRACT: Cardiovascular (CV) dysfunction and cardiomyopathy can cause perioperative morbidity in pheochromocytoma patients, but have not been studied systematically. This prospective, case-control study evaluated nature and extent of CV dysfunctions and their reversal following curative pheochromocytoma surgery.
Thirty-five pheochromocytoma patients, 9 normotensive nonpheochromocytoma adrenal tumors and 10 essential hypertensives were evaluated with 2-dimensional echocardiography, tissue Doppler, and serum N-terminal pro-brain natriuretic peptide (s-NTpro-BNP, a sensitive myocardial damage biomarker) serially before and after treatment.
Pheochromocytoma patients had systolic and diastolic dysfunction, reduced left ventricular (LV) ejection fraction (EF), increased LV end-diastolic and systolic dimensions and volumes, myocardial performance index, and decreased transmitral early/late velocity ratio, which were worse compared with controls. All indices improved significantly with α-blockade and after pheochromocytoma resection, and normalized over 3-6 months. Tissue Doppler early velocity was lower (P = .04) and s-NT-proBNP higher (P = .0001) in pheochromocytoma patients compared with controls. Seven pheochromocytoma patients (20%) had significant LV dysfunction (LVEF <45%; s-NTpro-BNP levels >500 pg/mL) and had more marked postoperative improvement.
Global LV diastolic and systolic dysfunctions specific to pheochromocytoma are common and improve early postoperatively, with sustained improvement upon follow-up. Detailed cardiac evaluation with echocardiography, tissue Doppler, and s-NTpro-BNP may help to reduce perioperative morbidity and monitor recovery in pheochromocytoma patients.
Surgery 12/2011; 150(6):1202-11. · 3.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: PURPOSE: Laparoscopic excision is preferred for small non-invasive pheochromocytoma over open approach. Applicability of laparoscopic procedures for large organ-contained pheochromocytoma is unclear. A database of 137 pheochromocytoma patients managed during 1990-2010 was reviewed to compare outcomes of open and laparoscopic procedures for 101 unilateral organ-contained pheochromocytoma patients in this retrospective non-randomized study. PATIENTS AND METHODS: Forty-nine patients underwent open procedures, and 52 underwent laparoscopic procedures. Laparoscopic procedure was converted to open in 19 due to bleeding (n = 12), concern for malignancy (n = 5), hypertensive crisis (n = 1), and equipment failure (n = 1). Outcome measures were compared between open, laparoscopic, and conversion patient groups. RESULTS: Patient groups were well matched for age, gender, BMI, and clinical and pathological characteristics. Mean tumor size was insignificantly larger in the open (7.6 ± 2.7 cm) than the laparoscopic group (6.6 ± 2 cm, p = 0.06). There were no significant differences in periop hemodynamic events. Mean blood loss, blood transfusion and analgesic requirements, and postop ICU and hospital stay were significantly lesser in laparoscopic than open and conversion groups (p < 0.05). There was no periop mortality. Morbidity occurred more frequently in the open (n = 12) than in the laparoscopic group (n = 3). At follow-up (mean, 44 ± 33.7; range, 6-160 months), no patient had recurrent pheochromocytoma. Outcomes in terms of cure of pheochromocytoma and hypertension were not different between the three groups. CONCLUSIONS: Laparoscopic procedures are feasible and as safe and effective as open procedures for patients with organ-contained pheochromocytoma. In a patient cohort where majority of the patients had large (>6 cm) pheochromocytoma, laparoscopic procedures resulted in lesser morbidity and shorter convalescence and provided equal chance for cure of pheochromocytoma and hypertension as conventional open surgical procedures.
Langenbeck s Archives of Surgery 11/2011; · 1.89 Impact Factor