Pheochromocytoma: a changing perspective and current concepts.

This article aims to review current concepts in diagnosing and managing pheochromocytoma and paraganglioma (PPGL). Personalized genetic testing is vital, as 40-60% of tumors are linked to a known mutation. Tumor DNA should be sampled first. Next-generation sequencing is the best and most cost-effective choice and also helps with the expansion of current knowledge. Recent advancements have also led to the increased incorporation of regulatory RNA, metabolome markers, and the NETest in PPGL workup. PPGL presentation is highly volatile and nonspecific due to its multifactorial etiology. Symptoms mainly derive from catecholamine (CMN) excess or mass effect, primarily affecting the cardiovascular system. However, paroxysmal nature, hypertension, and the classic triad are no longer perceived as telltale signs. Identifying high-risk subjects and diagnosing patients at the correct time by using appropriate personalized methods are essential. Free plasma/urine catecholamine metabolites must be first-line examinations using liquid chromatography with tandem mass spectrometry as the gold standard analytical method. Reference intervals should be personalized according to demographics and comorbidity. The same applies to result interpretation. Threefold increase from the upper limit is highly suggestive of PPGL. Computed tomography (CT) is preferred for pheochromocytoma due to better cost-effectiveness and spatial resolution. Unenhanced attenuation of >10HU in non-contrast CT is indicative. The choice of extra-adrenal tumor imaging is based on location. Functional imaging with positron emission tomography/computed tomography and radionuclide administration improves diagnostic accuracy, especially in extra-adrenal/malignant or familial cases. Surgery is the mainstay treatment when feasible. Preoperative α-adrenergic blockade reduces surgical morbidity. Aggressive metastatic PPGL benefits from systemic chemotherapy, while milder cases can be managed with radionuclides. Short-term postoperative follow-up evaluates the adequacy of resection. Long-term follow-up assesses the risk of recurrence or metastasis. Asymptomatic carriers and their families can benefit from surveillance, with intervals depending on the specific gene mutation. Trials primarily focusing on targeted therapy and radionuclides are currently active. A multidisciplinary approach, correct timing, and personalization are key for successful PPGL management.

Calcitonin: current concepts and differential diagnosis.

Calcitonin (CT) is most effectively produced by the parafollicular cells of the thyroid gland. It acts through the calcitonin receptor (CTR), a seven-transmembrane class II G-protein-coupled receptor linked to multiple signal transduction pathways with its main secretagogues being calcium and gastrin. It is clinically used mostly in the diagnosis and follow-up of medullary thyroid carcinoma (MTC). Hypercalcitoninemia can be attributed to primary (e.g. CT-secreting tumor) or secondary (e.g. due to hypercalcemia) overproduction, underexcretion (e.g. renal insufficiency), drug reaction (e.g. ß-blockers), or false-positive results. In clinical practice, elevated basal calcitonin (bCT) is indicative, but not pathognomonic, of MTC. Current literature leans toward an age as well as gender-specific cutoff approach. bCT >100 pg/ml has up to 100% positive prognostic value (PPV) for MTC, whereas bCT between 8 and 100 pg/ml for adult males and 6 and 80 pg/ml for adult females should be possibly further investigated with stimulation calcitonin (sCT) tests. Calcium is showing similar efficacy with pentagastrin (Pg) sCT; however, the real value of these provocative tests has been disputed given the availability of new, highly sensitive CT immunoassays. Anyhow, evidence concludes that sCT <2 times bCT may not be suggestive of MTC, in which case, thyroid in addition to whole body workup based on clinical evaluation is further warranted. Moreover, measurement of basal and stimulated procalcitonin has been proposed as an emerging concept in this clinical scenario. Measuring bCT levels in patients with thyroid nodules as a screening tool for MTC remains another controversial topic. It has been well established, though, that bCT levels raise the sensitivity of FNAB (Fine Needle Aspiration Biopsy) and correlate with disease progression both pre- and postoperatively in this situation. There have been numerous reports about extrathyroidal neoplasms that express CT. Pancreatic, laryngeal, and lung neuroendocrine neoplasms (NENs) are most frequently associated with hypercalcitoninemia, but CT production has also been described in various other neoplasms such as duodenal, esophageal, cutaneous, and paranasal NENs as well as prostate, colon, breast, and lung non-NENs. This review outlines the current biosynthetic and physiology concepts about CT and presents up-to-date information regarding the differential diagnosis of its elevation in various clinical situations.

Non-stenting treatment versus endoscopic stent placement in staple line leaks after laparoscopic sleeve gastrectomy.

PURPOSE: Presentation of results of non-stenting treatment versus endoscopic stenting placement in gastric staple leaks after laparoscopic sleeve gastrectomy (LSG). METHODS: Between January 2007 and August 2020, 1371 eligible patients underwent LSG. After gastric leak detection, patients were classified into treatment groups A (endoscopic stent placement) and B (non-stenting management). Overall hospital stay, the time to complete gastric leak resolution and the incidence of further operative management constituted the main outcome measures. Statistical analysis included descriptive statistics and linear regression tests as needed. RESULTS: A total of 27 patients (19 F/8 M, median age: 44.8 years (range: 36-58) with median preoperative BMI: 43.5 kg/m2 (range: 37.0-48.7)) presented with gastric staple line leak (1.9%) - mean detection day 5.8 postop (range: 1-12). Eight patients enrolled in group A and 19 patients in group B. The mean hospital stay for group A was 41.2 days (range: 24-60) versus 15 days (range: 12-18) for group B (p < 0.001). Complete leakage resolution was observed at mean 42.4 days (range 25-60) for group A and 34.5 days (range: 28-40) for group B patients, (p = 0.025). Only 2 group A patients accomplished complete leak resolution without additional intervention. Five group A patients (62.5%) versus 4 group B patients (21.1%) needed operative intervention during the treatment course (p = 0.037). CONCLUSIONS: Conservative, non-stenting treatment of staple line leaks after LSG is feasible and is associated with superior results in terms of hospital say and leak resolution in comparison to endoscopic stenting.

Medullary thyroid carcinoma in children: current state of the art and future perspectives.

Medullary thyroid carcinoma (MTC) is a distinct type of malignant thyroid tumor in cell origin, biological behavior, and natural history. It accounts for 1.6% of all thyroid cancers and presents either sporadically or as a hereditary disease, the latter occurring as a part of multiple endocrine neoplasia (MEN) 2A and MEN2B syndromes or as a familial MTC disease with no other manifestations. The gene responsible for the hereditary form is the rearranged during transfection (RET) gene, a proto-oncogene located to human chromosome 10. Most pediatric MTC cases have been discovered after genetic testing investigations, leading to the concept of prophylactic surgery in presymptomatic patients. Therefore, the genetic status of the child, along with serum calcitonin levels and ultrasonographic findings, determine the appropriate age for prophylactic surgical intervention. Nevertheless, a diagnosis at an early stage of MTC warrants total thyroidectomy and central lymph node dissection with the addition of lateral/contralateral lymph node dissection depending on the tumor size, ultrasonographic evidence of neck disease, or calcitonin levels. Conversely, locally advanced/unresectable or metastatic MTC is primarily treated with multikinase inhibitors, while more specific RET inhibitors are being tested in clinical trials with promising results.

Long-standing hypercalcemia in a 78-years old woman.

A 78 years-old woman was found with worsening hypercalcemia, osteopenia and memory loss during the past 2 years. Multiple, repeated imaging studies failed to reveal the etiology of the primary hyperparathyroidism. Bilateral neck exploration revealed a 4.5 × 2.3 cm right superior parathyroid adenoma in an ectopic position.

Large adrenal tumor in a 76-year-old man.

Among various adrenal tumors, metastatic ones are the most common. PET/CT scanning facilitates early detection. Occurrence of isolated and synchronous metastasis is very rare and poses serious diagnostic and therapeutic challenges.

ENSAT registry-based randomized clinical trials for adrenocortical carcinoma.

Adrenocortical carcinoma (ACC) is an orphan disease lacking effective systemic treatment options. The low incidence of the disease and high cost of clinical trials are major obstacles in the search for improved treatment strategies. As a novel approach, registry-based clinical trials have been introduced in clinical research, so allowing for significant cost reduction, but without compromising scientific benefit. Herein, we describe how the European Network for the Study of Adrenal Tumours (ENSAT) could transform its current registry into one fit for a clinical trial infrastructure. The rationale to perform randomized registry-based trials in ACC is outlined including an analysis of relevant limitations and challenges. We summarize a survey on this concept among ENSAT members who expressed a strong interest in the concept and rated its scientific potential as high. Legal aspects, including ethical approval of registry-based randomization were identified as potential obstacles. Finally, we describe three potential randomized registry-based clinical trials in an adjuvant setting and for advanced disease with a high potential to be executed within the framework of an advanced ENSAT registry. Thus we, therefore, provide the basis for future registry-based trials for ACC patients. This could ultimately provide proof-of-principle of how to perform more effective randomized trials for an orphan disease.

Intravascular B-large cell lymphoma: an unexpected diagnosis of an incidental adrenal mass.

Adrenal incidentalomas originally defined as tumors discovered serendipitously in the course of diagnostic evaluation or follow-up of unrelated disorders, may occasionally pose serious diagnostic challenges. Intravascular large B-cell lymphoma (IVLBCL) may be a rare example of such a case. We present an IVLBCL confined to the adrenal gland in a 52-year-old man focusing on its diagnostic and therapeutic aspects. On endocrine work up, the tumor was hormonally inactive and exhibited inconclusive imaging characteristics without signs of locoregional spread. After a left laparoscopic adrenalectomy, histologic sections revealed the presence of tumor cells inside dilated, thin-walled vascular spaces. Immunohistochemical stains confirmed the diagnosis of IVLBCL. The patient was then referred to a Hematology Unit for further staging and treatment and received six cycles of R-CHOP. Despite the fact that IVLBCL carries a dismal prognosis our patient remains alive and in complete remission 6 years after the initial diagnosis.

Carney Syndrome Presented as a Pathological Spine Fracture in a 35-Year-Old Male.

BACKGROUND Carney complex (CNC) is a genetic disorder that presents as an adrenocorticotropic hormone (ACTH)-independent variant of endogenous Cushing syndrome. It was first reported in 1985 and was described as a form of multiple endocrine hyperplasia associated with mutations of the c-AMP-dependent protein kinase (PRKAR1A) gene that causes bilateral adrenal hyperplasia. We report a case of an incidentally found CNC in a 35-year-old male, and this case report focuses on the diagnostic scheme as well as the surgical treatment of this rare challenging condition. CASE REPORT A-35-year-old male presented with pathological thoracic spine fracture. The patient exhibited obesity, facial flushing, red-purplish streaks on the abdominal wall, multiple pigmented nevi of the trunk, and hypertension. Family history was positive for cardiac myxoma. Laboratory investigation showed ACTH-independent Cushing syndrome. Abdominal magnetic resonance imaging and computed tomography scan showed bilateral adrenal hyperplasia. The ensuing Liddle test revealed the characteristic paradox increase of 24-hours urine cortisol for CNC. After a bilateral retroperitoneoscopic adrenalectomy, histologic examination confirmed the presence of bilateral primary pigmented nodular adrenocortical disease (PPNAD). Genetic testing revealed a unique mutation of the responsible PRKAR1A gene. CONCLUSIONS CNC presence was suspected due to the family history. Its characteristic pathologic manifestation called PPNAD, clinically presents as an ACTH-independent Cushing syndrome with paradoxical positive response of urinary glucocorticosteroid excretion after dexamethasone administration (Liddle's test). Bilateral retroperitoneoscopic adrenalectomy constitutes an acceptable surgical option for PPNAD.

Thyroid paraganglioma: a case series of a rare head and neck tumor.

Thyroid paragangliomas are rare neuroendocrine tumors. We present two cases analyzing their clinical presentation and pathology findings. A 44-year-old woman presented with a 33 mm left thyroid lobe mass. A 27-year-old male presented with a 27-mm right thyroid lobe mass and a FNA biopsy suggesting a follicular thyroid tumor. Both patients underwent total thyroidectomy. Vigorous bleeding was noted on the first case. Histologic sections revealed encapsulated tumors, whereas immunochemical stains were positive for chromogranin A, synaptophysin and NSE and negative for thyroglobulin, calcitonin, CEA and S-100. After an 18- and 12-month follow-up, respectively, both patients have no signs of local recurrence or distant metastasis. Preoperative diagnosis of thyroid paragangliomas was never attained in this series. Immunohistochemistry is mandatory for proper differential diagnosis. For the surgeon, the operation is technically demanding mainly due to the increased vascularity and friability of the tumor.

Classic Primary Hyperparathyroidism Versus Normocalcemic and Normohormonal Variants: Do They Really Differ?

BACKGROUND: Normocalcemic (NCpHPT) and normohormonal (NHpHPT) variants have been recognized primary hyperparathyroidism entities that pose serious challenges. We sought to define the differences among them in a series of surgically treated patients. PATIENTS AND METHODS: Between 2011 and 2015, 149 patients were enrolled into three groups: CpHPT (Ca > 10.2 mg/dL, PTH > 65 pg/mL), NCpHPT (normal Ca, PTH > 65 pg/mL) and NHpHPT (Ca > 10.2 mg/dL, normal PTH). Descriptive statistics and inter-group differences were computed, whereas multiple logistic/linear regression tests were used for further analysis. RESULTS: Of these patients 125 were female and 24 male, mean age 56.3 years (range 8-83). A total of 115 (77.2%) patients presented with CpHPT, 23 (15.4%) with NCpHPT and 11 (7.4%) with NHpHPT. MGD was found in 25 (16.8%) patients and SGD in 124 (83.2%); multivariate analysis failed to reveal statistically significant association of MGD with any pHPT variant (CpHPT 16.5% vs NCpHPT 21.7% vs NHpHPT 9.1%, p = 0.726). Conversely, NCpHPT patients exhibited statistically significant smaller adenoma weight (p = 0.023). Moreover, U/S in these patients had smaller positive predictive value (p = 0.278), whereas concordance between U/S and MIBI was also lower (p = 0.669). The utility of MIBI and U/S differed significantly (p < 0.001); more frequent use of U/S was observed for all groups. However, their predictive values did not differ significantly (p = 0.832). CONCLUSIONS: NCpHPT is more similar than different to CpHPT. NCpHPT constitutes the most challenging entity: it is associated with smaller adenoma weight, whereas U/S exhibited lower positive predictive value and lower concordance rate with MIBI. A trend for higher MGD presence in this group of patients was observed, though without statistical significance.

Multifocal Versus Solitary Papillary Thyroid Carcinoma.

BACKGROUND: Papillary Thyroid Carcinoma (PTC) which accounts for >85 % of all thyroid cancers in iodine-rich areas, appears either as a single tumor or as two or more, neoplastic foci within the thyroid gland (Multifocal PTC). We present the comparative results between solitary and MFC PTC. MATERIALS AND METHODS: Demographics, tumor characteristics (size, laterality, foci number, histologic subtype) and TNM staging were compared between solitary and MFPTC patients. The presence of lymphocytic or Hashimoto's thyroditis was also recorded. RESULTS: From January 2008 to December 2012, among 647 PTC patients, 241(37.2 %) had MFPTC 177 females (73.4 %) and 64 males (26.6 %), mean age 48.5 years (range 12-87). Mean number of tumor foci was 3.3 (range 2-26). MFPTC patients presented with more advanced T stage (28.2 vs. 18.7 %, p = 0.01) and more LN metastases (28.6 vs. 15.5 %, p < 0.001). Foci number correlates with male gender and LN metastases (p = 0.014 and p = 0.019, respectively). Central (N1a) or lateral (N1b) LN involvement correlates strongly with male gender (p  = 0.024) and younger age (p < 0.001). The follicular variant was the next most frequent histologic subtype associated with extremely rare LN metastases. CONCLUSION: MFPTC comprises a more aggressive form of papillary thyroid cancer since it is associated with more frequent N1a/ N1b disease and occurs more frequently in T3/T4 patients. MFPTC foci number correlates with male gender and LN metastases.

Posterior retroperitoneoscopic versus laparoscopic adrenalectomy in sporadic and MENIIA pheochromocytomas.

INTRODUCTION: Retroperitoneal adrenalectomy (PRA) comprises an alternative approach in the management of adrenal tumors that has been set as the treatment of choice in our Institution. We assess the impact of PRA the management of hereditary and sporadic pheochromocytomas comparing its outcomes to the laparoscopic technique, in a case-controlled setting. PATIENTS AND METHODS: From May 2008 to January 2013, 17 patients [5 males and 12 females, mean age: 51 yrs (range 26-73)] with pheochromocytomas underwent PRA. Demographics, tumor characteristics, operative time, complications, hospital stay, and postoperative pain (based on VAS score at days 1 and 3) were compared to 17 selected laparoscopic patient controls [7 males and 10 females, mean age 49 yrs (range 25-64)]. RESULTS: 17 patients, 11 with the sporadic form and 6 with MENIIA associated pheochromocytomas, comprised the retroperitoneoscopic group. 19 pheochromocytomas with a mean size 3.7 cm (range 1.7-7.0) at a mean operative time: 105.6 min (range 60-180) were accordingly excised. In the laparoscopic group, 13 patients had sporadic pheochromocytomas, whereas 4 patients had MENIIA syndrome. Mean tumor size of the laparoscopic series was 5.1 cm (range 1.7-8.5) at a mean operative time of 137 min (range 75-195). No mortality or conversions were encountered in both groups. No blood transfusions were needed. Mean visual analog scale pain scores were significantly lower for the retroperitoneoscopic group both on days 1 and 3 [0.94 (0-3) vs 4.15 (3-6), p < 0.001 and 0.06 (0-1) vs 3.5 (2-6) p < 0.001] respectively. Mean hospital stay for the patients of the retroperitoneoscopic group was significantly better than the laparoscopic group [(2.1 ± 0.24 days vs 40 ± 0.70 days) p < 0.001]. CONCLUSIONS: Retroperitoneoscopic adrenalectomy is associated with excellent clinical results in the management of sporadic and hereditary pheochromocytomas. Moreover, it appears to be superior to the laparoscopic approach, because it is faster and affords the patient with less pain and shorter hospital stay.

Patient attitudes toward transaxillary robot-assisted thyroidectomy.

BACKGROUND: The transaxillary robot-assisted technique constitutes an acceptable treatment option for patients requiring thyroidectomy. However, patients' attitudes toward this new technique have not yet been analyzed. METHODS: A sample of 596 randomly selected patients who underwent thyroidectomy between January 2000 and March 2010 was assessed. We evaluated patients' attitudes toward transaxillary robot-assisted thyroidectomy, taking into account the validated Patient Scar Assessment Questionnaire, the SF-36 Health Survey Questionnaire, and 11 sociodemographic and surgical patient characteristics. RESULTS: Only 11.6 % of the patients would prefer to have been treated with the transaxillary method. Most patients had concerns that it would be a more painful procedure (39.2 %), and they expressed satisfaction with the existing esthetic outcome (29.1 %); other concerns were that the robotic approach would be of longer duration (25.4 %) and at higher cost (15.5 %). Nevertheless, the worse the appearance of the neck scar the more preferable is the new method (p = 0.025), a result that holds true irrespective of patients' physical health, the invasive procedure attained (conventional or minimal), and the presence of postoperative complications, among other characteristics. Patients diagnosed with a benign or uncertain neoplasm (p = 0.022) and younger patients (p = 0.003) held a more positive view of the new method. CONCLUSIONS: Patients who have undergone conventional thyroidectomy via the usual neck incision do not express a preference for the transaxillary method. The reasons given include various perceived disadvantages of the robotic procedure (increased pain, longer operative times, and higher cost). Younger patients, patients with poor appearance of their neck scar, and patients with benign thyroid pathology seem to hold a more positive attitude toward the robotic approach.

Scar perceptions after thyroid and parathyroid surgery: comparison of minimal and conventional approaches.

BACKGROUND: An incision less than 3 cm in length in the neck is the main feature that discriminates the minimally invasive thyroidectomy and parathyroidectomy from traditional procedures. Smaller neck scars are assumed to yield better patient satisfaction, although no established data support this. In this analysis, we evaluated the satisfaction of patients who had undergone both procedures, while examining the effects of sociodemographic and surgical characteristics. METHODS: We analyzed data from 691 patients who underwent a thyroidectomy or parathyroidectomy between January 2000 and March 2010. We assessed the satisfaction of patients who underwent conventional compared to minimally invasive procedures, using the validated Patient Scar Assessment Questionnaire (PSAQ). We included both the appearance and the consciousness subscales. RESULTS: Overall, patients were satisfied with their neck scars, as indicated by the low scores in appearance (13.3; range, 9 to 31) and consciousness (8.5; range, 6 to 24) subscales. The degree of satisfaction improved with increased time since surgery (P < .001). Patient satisfaction was similar regardless of the procedure used, implying that smaller scars do not provide better patient satisfaction. Most patients (81.2%) reported that they would not have preferred a transaxillary procedure over the procedure they received. CONCLUSION: A smaller incision in the neck was not associated with better patient satisfaction. New surgical approaches aimed at maximizing cosmesis while minimizing scar size should be evaluated for cost-effectiveness and clinical outcomes, as well as patient satisfaction, before becoming the standard of care.

Impact of posterior retroperitoneoscopic adrenalectomy in a tertiary care center: a paradigm shift.

BACKGROUND: Posterior retroperitoneoscopic adrenalectomy has substituted its anterior laparoscopic counterpart as the treatment of choice in the management of adrenal tumors at the authors' institution. The authors present their comparative results between these operative techniques, demonstrating the reasons for this change. METHODS: From May 2008 to September 2010, 30 patients underwent posterior retroperitoneoscopic adrenalectomy. Operative time, complications, hospital stay, postoperative pain, and cost were compared with those of 30 selected laparoscopic control subjects treated from 2005 to 2010. Statistical analysis was based on Chi-square, the Mann-Whitney U test, the independent-samples t-test, and the Wilcoxon matched pairs test, as appropriate. RESULTS: The median tumor size was 3.8 cm (range, 1.5-8.0 cm) in the retroperitoneoscopic group and 4.9 cm (range, 2.4-8.0 cm) in the laparoscopic group. The median operative time was similar between the two groups (90.0 min; range, 60-165 min vs. 77.5 min; range, 55-120 min; P = 0.138). It was, however, significantly reduced after the 20th case (97.5 min; range, 80-165 min vs. 70 min; range, 60-110 min; P < 0.001) in the retroperitoneoscopic group. The median visual analog pain scores were significantly lower in the retroperitoneoscopic group on both the first and the third postoperative days, respectively (1; range, 0-1 vs. 4; range, 3-6; P < 0.001 and 0; range, 0-1 vs. 3; range, 2-6; P < 0.001). The median postoperative hospital stay also was shorter in the retroperitoneoscopic group (2 days; range, 2-3 days vs. 4 days; range, 3-6 days; P < 0.001). The cost of the posterior approach was significantly less than that of the laparoscopic technique (P < 0.001). CONCLUSIONS: Posterior retroperitoneoscopic adrenalectomy compared with laparoscopic adrenalectomy was safe, fast, and vastly superior in terms of postoperative pain and hospital stay in this series. Because of the ability to reproduce such excellent operative results, the impressive patient recovery, and the significantly reduced operative cost, the authors suggest that the retroperitoneoscopic approach should become the method of choice in minimally invasive adrenal surgery.

Adrenal cortical oncocytoma mimicking pheochromocytoma.

OBJECTIVE: Adrenal tumors present with clinical features and signs unique to their specific hormonal hypersecretion. However, there have been cases in which the clinical expression has been in conflict with the histologic features of the tumor. In this communication we report an unusual clinical presentation of an adrenal cortical tumor with histologic features of an oncocytoma that clinically mimicked a pheochromocytoma. DESIGN: A 49-year old man was referred to our Unit due to type B aortic dissection and a mass of the left adrenal gland. Computed tomography and magnetic resonance imaging confirmed the presence of aortic dissection extending from the left subclavian artery to both iliac arteries and also revealed a 6 cm tumor on the left adrenal gland. Preoperative endocrine evaluation showed a near tenfold increase of urinary vanillylmandelic acid (VMA) and metanephrine values. RESULTS: Transperitoneal laparoscopic adrenalectomy was successfully performed. The adrenal tumor proved to be an adrenal cortical neoplasm with histologic features of oncocytoma. CONCLUSION: Although the case of an adrenal cortical adenoma clinically mimicking a pheochromocytoma has been described in the literature, to the best of our knowledge, there has been no previous report of an adrenal cortical neoplasm with predominant features of oncocytoma.

Adrenal ganglioneuromas: incidentalomas with misleading clinical and imaging features.

BACKGROUND: Ganglioneuromas are benign neoplasms of the neural crest, occurring rarely in the adrenal glands. This study presents our experience regarding diagnostic and therapeutic management of these neoplasms and a review of the relevant literature. METHODS: Among 150 patients with of incidentalomas, we had 7 primary ganglioneuromas. Their clinical, imaging, and operative data were collected retrospectively, and the literature was reviewed using MEDLINE. There were 4 females and 3 males, with mean age of 50 years (range, 39-64). All neoplasms were discovered incidentally with ultrasonography and were evaluated subsequently with computed tomography (CT). One patient was studied further with (131)I-MIBG due to asymptomatic increased in urine vanillylmandelic acid, and 1 patient with history of breast cancer underwent additional FDG-PET/CT. RESULTS: All but 2 patients were asymptomatic. Two patients complained of epigastric pain and hypertension, respectively. The preoperative mean size on CT was 6.8 cm, whereas the postoperative true mean histologic size was 7.7 cm. Both patients who were evaluated with radionuclide studies had false positive results, suggestive of pheochromocytoma and adrenal metastasis, respectively. Three patients underwent open adrenalectomy due to preoperative suspicion of carcinoma, and the remaining 4 underwent laparoscopic anterior adrenalectomy. Histologically, all 7 neoplasms were completely differentiated, mature ganglioneuromas. We had no mortality or significant morbidity. No recurrence occurred during a mean follow-up of 6 years (range, 1-18). CONCLUSION: Adrenal ganglioneuromas are rare incidentalomas that can mimic primary or secondary adrenal malignancies as well as pheochromocytomas. Despite their usually large size, resection via laparoscopic approach is safe and effective.

Laparoscopic sleeve gastrectomy in morbidly obese patients. Technique and short term results.

OBJECTIVE: To evaluate the efficacy of Laparoscopic Sleeve Gastrectomy (LSG) as a definitive procedure for morbidly obese patients. DESIGN: This constitutes a prospective study carried out in a tertiary care private hospital and included 15 morbidly obese patients who underwent LSG. The operation was performed through two 12 mm and two 5 mm ports, using the Endo-GIA stapler to create a lesser curve gastric tube over a 36-Fr bougie. RESULTS: Operative time, complication rates, hospital length of stay, Body Mass Index (BMI), % of Excess Weight Loss (EWL) and appetite were evaluated. There were six females and nine males, aged (mean+/-SD) 40.5+/-10.5 yrs and preoperative BMI 47.8+/-7.5 kg/m2. The operative time was 147.7+/-43.2 min. There was one conversion to open surgery and one gastric leak with haemorrhage that led to gastric tube stenosis, ultimately requiring revision surgery. All patients, except these two, were discharged on the 2nd postoperative day after an upper GI series and the initiation of a clear liquid diet. At the follow-up (7.5+/-4.4 months post operatively), the % EWL was 35.7+/-10.1. Eight patients who received regular postoperative dietician counselling at follow-up did better than the others who did not (% EWL 40.4+/-3.8 vs 30.2+/-4.1, respectively). All patients reported significant loss of appetite. CONCLUSIONS: Although the number of patients is relatively small, the data of this study indicate that laparoscopic sleeve gastrectomy is effective in weight reduction, being an acceptable surgical option for morbidly obese patients. A higher number of patients and longer follow-up period will be necessary to evaluate long-term efficacy.