Evaluation and management of adrenal neoplasms: endocrinologist and endocrine surgeon perspectives.

The evaluation and management of adrenal disease is a complex endeavor that relies on an expert knowledge of human physiology and anatomy. Careful and proper patient assessment mandates a balanced approach which marries the disciplines of endocrinology, surgery, and radiology. Any of these three specialties may be on the front line in performing the initial workup when an adrenal neoplasm is discovered. With an ever-increasing volume of cross-sectional imaging, be it CT, MRI, or PET, large numbers of adrenal incidentalomas are being discovered. A close collaboration amongst specialties should strive to streamline the initial evaluation and minimize unnecessary testing and treatment.

Subtotal Parathyroidectomy vs Total Parathyroidectomy with Autotransplantation for Secondary Hyperparathyroidism in Dialysis Patients: Short- and Long-Term Outcomes.

BACKGROUND: Two operations are performed for management of secondary hyperparathyroidism, subtotal parathyroidectomy (SPTX) and total parathyroidectomy with autotransplantation (TPTX-AT). There is no consensus among endocrine surgeons about which operation is the preferred treatment. This study compares the short- and long-term outcomes of SPTX and TPTX-AT for dialysis patients with secondary hyperparathyroidism. STUDY DESIGN: This is a retrospective review of 46 dialysis patients undergoing PTX from 2006 to 2017 at a 719-bed tertiary care hospital. RESULTS: Calcium on postoperative day 1 was 7.7 ± 0.8 mg/dL for SPTX and 7.9 ± 1.3 mg/dL for TPTX-AT (p = 0.49). Parathyroid hormone values on postoperative day 1 were 32.6 ± 26.0 pg/mL for SPTX and 9.5 ± 4.2 pg/mL for TPTX-AT (p ≤ 0.05). Hospital length of stay was 3.7 ± 1.9 days for SPTX and 4.4 ± 3.5 days for TPTX-AT (p = 0.46). The required doses of calcium and calcitriol at discharge did not differ significantly. Reoperation for recurrence or persistence of disease was required in 6 SPTX patients and 2 TPTX-AT patients (p = 0.12). Parathyroid hormone values <15 pg/mL at long-term follow-up occurred in 5.6% of SPTX patients and 26.7% of TPTX-AT patients (p = 0.09). Parathyroid hormone values >200 pg/mL at long-term follow-up occurred in 38.9% of SPTX patients vs 6.7% of the TPTX-AT patients (p ≤ 0.05). Calcium supplementation at more than 6 months was required for 36.8% of SPTX and 71.4% of TPTX-AT patients (p < 0.05). CONCLUSIONS: The long-term control of parathyroid hormone elevation and avoidance of recurrent disease is improved with TPTX-AT, but carries a higher risk of long-term hypocalcemia.

Management of Incidental Adrenal Masses: A White Paper of the ACR Incidental Findings Committee.

The ACR Incidental Findings Committee presents recommendations for managing adrenal masses that are incidentally detected on CT or MRI. These recommendations represent an update to the adrenal component of the JACR 2010 white paper on managing incidental findings in the adrenal glands, kidneys, liver, and pancreas. The Adrenal Subcommittee, constituted by abdominal radiologists and an endocrine surgeon, developed this algorithm. The algorithm draws from published evidence coupled with expert subspecialist opinion and was finalized by a process of iterative consensus. Algorithm branches categorize incidental adrenal masses on the basis of patient characteristics and imaging features. For each specified combination, the algorithm concludes with characterization of benignity or indolence (sufficient to discontinue follow-up) and/or a subsequent management recommendation. The algorithm addresses many, but not all, possible pathologies and clinical scenarios. Our goal is to improve the quality of patient care by providing guidance on how to manage incidentally detected adrenal masses.

The natural history of the benign thyroid nodule: what is the appropriate follow-up strategy?

BACKGROUND: Long-term monitoring of benign thyroid nodules is not addressed in the present American Thyroid Association guidelines. The objective of this study was to determine the appropriate nature and length of follow-up for patients with a benign thyroid nodule. STUDY DESIGN: A retrospective review was performed of all patients referred to single endocrine surgeon for evaluation of thyroid nodules between 2006 and 2012. The review included 263 patients who had benign fine needle aspiration (FNA) cytology and either underwent thyroidectomy or had at least a 1-year follow-up ultrasound. Main outcomes measures were repeat FNA and pathology results. RESULTS: There were 231 women and 32 men. Forty-eight patients underwent immediate thyroidectomy, with pathology showing 2 papillary thyroid cancers (PTC), and 215 patients were followed with annual ultrasounds. During follow-up, 89 (41.3%) nodules underwent repeat FNA after initial biopsy. The repeat FNA cytology showed 91% benign, 7% follicular neoplasm, and 2% PTC. During follow-up, 81 (37.6%) patients underwent thyroidectomy after 3.3±2.8 years. Reasons for surgery included development of symptoms in 58 (71.6%), a non-benign repeat FNA in 8 (9.8%), or patient preference in 15 (18.5%). Surgical pathology identified 70 (86.4%) benign, 7 (8.6%) PTC, 3 (4%) follicular thyroid cancers, and 1 (1.2%) lymphoma. Median time from initial FNA to thyroidectomy in patients who had malignancy was 4.3 years. The maximum initial nodule size and average increase in nodule size did not differ between benign and malignant nodules (p=0.54 and p=0.75, respectively). CONCLUSIONS: Significant numbers of benign thyroid nodules enlarge more than 5 mm over 3 years, triggering repeat FNA or thyroidectomy. Larger diameter nodules and more rapidly growing nodules were not predictive of malignancy. The practice of annually obtaining ultrasound for benign thyroid nodules should be discouraged.

Controversy over radioiodine ablation in thyroid cancer: who benefits?

Recent studies have proposed that overdiagnosis is probably the principal cause of the increased incidence of thyroid cancer. The controversy around radioiodine ablation is complicated by the ever increasing numbers of small, low-risk thyroid cancers being diagnosed. This article examines the history and evolving epidemiology of the disease and treatment.

Radiographic evaluation of nonfunctioning adrenal neoplasms.

Incidental adrenal neoplasms are usually nonfunctioning benign adenomas. Once hormonal production has been assessed, the nonsecreting lesions must be evaluated for the possibility of malignancy. This evaluation relies primarily on the radiographic characteristics. This article focuses on the current state of radiologic technology available to accurately assess nonfunctioning adrenal incidentalomas. As this technology advances, a lesion's malignant potential can more accurately be determined, thereby allowing physicians to make more informed treatment recommendations.

Severe group a streptococcus surgical site infection after thyroid lobectomy.

BACKGROUND: Thyroidectomy is rarely complicated by a surgical site infection (SSI). Despite its low incidence, post-thyroidectomy SSI is especially concerning because of its proximity to vital head and neck structures and the very real potential for airway compromise and death. Severe SSIs frequently are caused by Group A Streptococcus (GAS) because of its potential for developing into necrotizing fascitis. No description of the surgical approach to a necrotizing soft-tissue infection after thyroid resection is available in the current literature. METHODS: Case report and review of the pertinent English-language literature. RESULTS: A 47-year-old male underwent a right thyroid lobectomy and isthmusectomy for a follicular neoplasm. On post-operative day 2, the patient presented to the emergency department with persistent pain, rapid onset of swelling, and airway compromise shown on computed tomography scan. Emergency incision and drainage revealed a severe soft tissue infection. Because of subsequent worsening erythema and soft-tissue swelling, the patient had to be re-explored. The infection, later identified as caused by GAS, might have been transmitted from the patient's daughter. CONCLUSION: To our knowledge, this is the first case reported of exposure to a family member with GAS pharyngitis. Successful treatment requires an appropriately high level of suspicion followed by emergent operative debridement and systemic antibiotics.

Hereditary pheochromocytoma and paraganglioma.

Hereditary pheochromocytomas (pheo) and paragangliomas (pgl) are caused by identifiable germline mutations. The previously well-known associated syndromes include neurofibromatosis type 1, multiple endocrine neoplasia 2 A and B, and von Hippel-Lindau syndrome. Newly discovered mutations in the succinate dehydrogenase gene complex have been identified as a cause of inherited pgls and pheos. It is now clear that up to 30% of patients presenting with sporadic pheos/pgls harbor a recognizable germline mutation, and therefore directed genetic testing is recommended for many of these patients.

Level VI lymph node dissection does not decrease radioiodine uptake in patients undergoing radioiodine ablation for differentiated thyroid cancer.

BACKGROUND: In patients with well-differentiated thyroid cancer, the incidence of pathologic central compartment lymph node metastases is reported to be approximately 50%. Recently level VI lymph node dissection has been advocated as a means of reducing recurrence rates in these patients, even if there are no clinically apparent nodal metastases. This study investigates whether level VI lymph node dissection decreases the percent radioiodine uptake when patients undergo radioiodine ablation. METHODS: All thyroid cancer patients entered into the endocrine surgery database at a tertiary care center from 2006 to 2010 were reviewed. Those treated with radioactive iodine were analyzed with respect to performance of a central compartment lymph node dissection and the percent uptake of radioiodine ((131)I) on the preablation scan at 72 h. RESULTS: There were 277 patients with well-differentiated thyroid cancer who underwent radioiodine ablation. In all, 75% were female, and the mean age was 47.7 years. A total of 87 patients underwent total thyroidectomy and level VI lymph node dissection (TT + LVIND). The mean number of level VI nodes resected was 6 (1-27), and 60.9% of patients had nodal metastases. Altogether, 190 had a total thyroidectomy (TT) only, and the median number of nodes resected was 0 (0-10). The percent uptake of radioiodine on the preablation scan was 0.93% in patients who had undergone TT + LVIND and 1.2% in those with TT alone (p = 0.17). The median number of radioactive foci noted within the thyroid bed was two in both groups (p = 0.64). The mean preablation thyroglobulin levels, measured after thyroxine withdrawal or thyrogen stimulation, were 4.0 ng/ml in the TT + LVIND group versus 4.7 ng/ml in the TT group (p = 0.07). The average ablative dose of (131)I was 111.8 mCi in the dissection group and 98.5 mCi in the TT-only group. CONCLUSIONS: There is no evidence that uptake of (131)I is reduced by performance of a central neck dissection in patients with well-differentiated thyroid cancer. Preablation thyroglobulin levels were not altered by level VI lymph node dissection.

The phenotype of primary hyperparathyroidism with normal parathyroid hormone levels: how low can parathyroid hormone go?

BACKGROUND: While normocalcemic hyperparathyroidism is well recognized in primary hyperparathyroidism (PHP), less is known about patients with high calcium but normal intact parathyroid hormone (iPTH). We aimed to describe this entity and designated it normohormonal primary hyperparathyroidism (NHPHP). METHODS: From a prospectively maintained database of patients undergoing bilateral parathyroid exploration for PHP, we identified and compared those with preoperative iPTH levels below (NHPHP) and above (typical PHP) normal reference peak (60 pg/mL). RESULTS: NHPHP occurred in 46 of 843 patients (5.5%) undergoing initial parathyroidectomy for PHP. All had hypercalcemia (11.1 mg/dL). Regarding preoperative iPTH, 7 patients (15%) had values <40 pg/mL, 19 (41%) had values <60 pg/mL; and 20 (44%) had intermittent values >60 pg/mL. Unlike patients with elevated iPTH, nearly all NHPHP patients had additional testing delaying the operation. Imaging correctly localized NHPHP parathyroid disease in 80%. At the time of operation, 74% of NHPHP patients had single adenomas. Intraoperatively postmobilization, using the same assay that was used preoperatively, 82% had PTH levels >60 pg/mL (mean, 279 pg/mL). During the follow-up period, iPTH levels remained lower among NHPHP patients (21 pg/mL) compared to 41 pg/mL for patients with preoperative iPTH 60 to 100 pg/mL and 56 pg/mL for patients with preoperative iPTH 100 to 200 pg/mL (P < .0001). CONCLUSION: Lower PTH set points may exist in some patients with otherwise typical PHP features. Although high normal iPTH is inappropriate for hypercalcemia and should suggest PHP, this disorder may occur with iPTH levels as low as 5 pg/mL. Awareness of the unusual phenotype of NHPHP may facilitate earlier diagnosis and surgery.

Computed tomographic diagnosis of pneumatosis intestinalis: clinical measures predictive of the need for surgical intervention.

OBJECTIVE: To determine which clinical, laboratory, and radiographic parameters predict positive operative findings in patients with pneumatosis intestinalis on computed tomography (CT). DESIGN: Retrospective record review. SETTING: Tertiary care hospital and affiliated community hospital. PATIENTS: One hundred fifty consecutive patients diagnosed as having pneumatosis intestinalis on CT. MAIN OUTCOME MEASURES: Presence or absence of abdominal pathological findings at laparotomy and mortality rates. RESULTS: Of the 150 patients studied, 54 (36%) were managed nonoperatively, 72 (48%) were managed operatively, and 24 (16%) were considered unsalvageable and given comfort measures only. Sixty patients (47%) improved with nonoperative management or had negative intraoperative findings. In the nonoperative group, 50 (93%) improved (n = 50) and 3 (5%) crossed over to surgery. One patient (2%) died. In the operative group, 63 patients (87%) had operative findings requiring intervention and 9 (13%) had negative results on exploration. Twenty-one patients (28%) died. Univariate analysis identified numerous predictors of positive intraoperative findings, including history of coronary artery disease, tachycardia, tachypnea, hypotension, peritonitis, abdominal distention, and lactic acidemia. The significant radiographic findings included dilated loops of bowel, portal venous gas, and atherosclerosis on CT. On multivariate analysis, only abdominal distention (odds ratio = 13.19; P = .001), peritonitis (odds ratio = 9.35; P = .007), and lactic acidemia (odds ratio = 2.29; P = .02) were predictive of positive intraoperative findings. CONCLUSIONS: Many patients with pneumatosis intestinalis on CT can be successfully treated nonoperatively. In determining a management strategy, abnormal physical examination findings were more predictive of the need for surgical intervention than laboratory values or radiographic findings.

Laparoscopic adrenalectomy: balancing the operative indications with the technical advances.

Laparoscopic adrenalectomy contributed significantly to reduction of morbidity and improvement of postoperative patient recovery time. The adoption of this technique had substantial impact on the management of adrenal incidentalomas. Although laparoscopic adrenalectomy should be in general avoided for known primary adrenal cancers, it is appropriate for metastasectomy of isolated adrenal metastatic disease.

Normalization of 2-week postoperative parathyroid hormone values in patients with primary hyperparathyroidism: four-gland exploration compared to focused-approach surgery.

BACKGROUND: Elevation of parathyroid hormone (PTH) levels is commonly seen in patients with primary hyperparathyroidism (PHPT) who have undergone parathyroidectomy. This study evaluates differences in 2-week postoperative PTH levels in patients having focused-approach surgery versus four-gland exploration. METHODS: Over 6 years, patients at Rhode Island Hospital (RIH) and the Cleveland Clinic (CCF) who had PHPT and underwent localization studies suggestive of single adenoma were analyzed. At RIH patients underwent focused-approach surgery, and at CCF routine four-gland exploration was performed. Postoperative calcium supplementation was routine at RIH and selective at CCF. RESULTS: There were 308 patients at RIH and 370 at CCF. They were similar in age (59.2 +/- 13.0 years at RIH and 60.4 +/- 12.9 years at CCF), and sex (76.9 and 80.0% female at RIH and CCF, respectively). The mean preoperative serum calcium measured 10.9 +/- 0.7 mg/dl at RIH and 11.1 +/- 0.7 mg/dl at CCF (P < 0.001). Preoperative PTH values were similar, measuring 143.8 +/- 104.8 pg/ml in the focused-approach group (RIH) and 157.6 +/- 150.3 pg/ml in the four-gland exploration group (CCF). Preoperative 25-hydroxyvitamin D (vitamin D-25) levels were 24.1 +/- 12.0 ng/ml at RIH and 27.4 +/- 10.6 ng/ml at CCF; and the prevalence of vitamin D-25 deficiency (level <20 ng/ml) was 43.9% at RIH and 27% at CCF (P = 0.017). The proportion of patients whose intraoperative PTH value dropped by >or=50% prior to completion of surgery was 95.0% at RIH and 95.5% at CCF. The total gland weight resected per patient was 942 mg at RIH versus 1,394 mg at CCF (P = 0.003). The 2-week postoperative serum PTH was >65 pg/ml in 18.8% at RIH and in 38.7% at CCF (P < 0.001). The 2-week postoperative serum calcium values dropped to 9.2 +/- 0.6 mg/dl at RIH and to 9.5 +/- 0.8 mg/dl at CCF (P < 0.001). The incidence of multigland disease was 5.8% at RIH and 21.9% at CCF (P

Surgeon-performed ultrasound in patients referred for thyroid disease improves patient care by minimizing performance of unnecessary procedures and optimizing surgical treatment.

BACKGROUND: Ultrasonography has become an indispensable tool in the evaluation of thyroid nodular disease, and most patients will have had a thyroid ultrasound prior to initial surgical evaluation. This study examines the added benefit of office-based, surgeon-performed ultrasonography in patients referred for thyroid disease. METHODS: All patients referred to a single endocrine surgeon for evaluation of thyroid disease over a 2-year period were reviewed. Outside ultrasonographic findings were compared to the surgeon-performed ultrasound that was used to formulate treatment decisions. RESULTS: Of 286 consecutive patients referred for surgical evaluation of thyroid disease, 261 had an outside ultrasound available for comparison. There were 239 women and 47 men. Mean age was 54.7 +/- 16.6. In 46 patients (17.6%), differences between the two ultrasounds were significant enough to alter treatment plans. For 18 patients no distinct nodule was identified and biopsy was avoided. Nine of these patients had ultrasound characteristics of Hashimoto's disease. In five patients the nodule was significantly smaller than reported and biopsy was not warranted. Twelve patients had nonpalpable, enlarged lymph nodes not previously identified; these were biopsied. Three were positive for metastatic thyroid cancer, which prompted the addition of neck dissection to the operative procedure. In 8 of 132 patients undergoing thyroidectomy, the surgical procedure was significantly altered by the ultrasound findings. CONCLUSIONS: This study demonstrates a clear advantage for patients who undergo a surgeon-performed ultrasound. For many, unnecessary procedures were prevented. For others, substantial modifications to the extent of surgery were made when new ultrasonographic findings were identified during the preoperative investigation.

Does failure to perform prophylactic level VI node dissection leave persistent disease detectable by ultrasonography in patients with low-risk papillary carcinoma of the thyroid?

BACKGROUND: There is controversy regarding the need for prophylactic level VI central node dissection in patients with low-risk papillary thyroid carcinoma (PTC). This study focuses on the incidence of persistent level VI nodal disease in low-risk PTC without prophylactic central node dissection. METHODS: PTC was known at the time of thyroidectomy in 304 of the 761 patients who had initial thyroid surgery from 2001 to 2007. Therapeutic level VI node dissection was performed for suspicious or positive nodes. A prophylactic central node dissection was not done if suspicious nodes were not identified. All patients had a high-resolution ultrasonography, and almost all patients had a suppressed serum thyroglobulin level 4-6 months after thyroidectomy. RESULTS: A total of 112 of 304 patients (37%) had a therapeutic level VI node dissection. A prophylactic central node dissection was not performed in the remaining 192 patients. One hundred and sixty-one of the 192 patients (84%) were low risk. Biopsy-proven persistent disease was identified at the 4-6-month postoperative ultrasonography in only 3 of the 161 low-risk patients (1.8%). The suppressed serum thyroglobulin level was increased in these 3 patients and 2 additional patients. CONCLUSION: Failure to perform a prophylactic central node dissection in low-risk PTC resulted in both a very low incidence of persistent level VI nodal disease and elevated suppressed thyroglobulin 4-6 months after thyroidectomy.