CLINICAL DIAGNOSIS AND TREATMENT OF NONFUNCTIONING PHEOCHROMOCYTOMAS IN 14 PATIENTS

Objective To analyze the clinical characteristics of nonfunctioning pheochromocytoma, and to evaluate the efficacy of 131I-metaiodobenzylguanidine (MIBG) scan in the diagnosis and perioperative treatment of nonfunctioning pheochromocy- toma. Methods The clinical data of 14 patients with nonfunctioning pheochromocytoma were analyzed retrospectively. Plasma free corticoid, renin, aldosterone, and urine catecholamines levels were estimated. B-mode ultrasonography, computed tomo- graphy scan, thoracic X-ray and 131I-MIBG were used. Results All patients with nonfunctioning pheochromocytoma had no hypertension and the tumors were found inciden- tally. The 24 hours urine catecholamines levels in 80% (8/10) patients were normal. The positive rate of 131I-MIBG was 80% (8/10) and the specificity was 100%. All patients underwent surgical operation of tumor resection. No preoperative volume expansion was given to all patients. All tumors were resected completely, and no death accident happened. There was no recurrence and metastasis after operation by long-term follow-up. Conclusion 131I-MIBG scan is the first choice technique for the diagnosis of nonfunctioning pheochromocytoma. Blood volume expansion is unnecessary before resection of pheochromocytoma.no recurrence and metastasis})

CLINICOPATHOLOGIC FEATURES AND SURGICAL TREATMENT OF NONFUNCTIONING ISLET CELL TUMORS

Objective.To in vestigate clinicopathologic characteristics and surgical results of nonfunctiona l islet cell tumors.Methods.We performed retrospective analysis of50patient s with nonfunctional islet cell tumor treat-ed at Peking Union Medical College Hospital from July1968to July1999,and summarized clinical symptoms and signs,primary diagnosis before surgery,surgical treatments,pathologic and im munohisto-logical characteristics.Results.Of the50cases examined from July 1968to July1999,12were found during physical examinations. The most common symptoms were upper abdominal pain and upper abdominal dis-com fort,which appeared in20casesand17casesrespectively,an d the most common sign was abdominal masses.B-ultrasound and CT are the most commonly used means for preoperative examination,with posit ive rates of97.8%and100%respectively.Op-e rative mortality was2%.Five-year survival rate of radical surgery of maligna nt cases was75%.Immuno-histological examinations showed that tumors rich i n multi-peptide linked hormones,neuron-specific eno-lase and chromaffin were in65%cases,90.6%and73.3% respectively.The positive rates of insulin,glucagons,somatostatin,pancreat ic polypeptide,gastrin and vasoactive intestinal peptide were68.2% ,51.2%,42.9%,40% ,25%and15.8%respectively.Conclusion.Nonfuncti onal islet cell tumors lack diagnostic specificity.Ultrasound and CT are primar y examination methods.Immunohistological analysis indicates different hormones ,but lacks the presence of related clinical symptoms.Surgery is an effective treatment for nonfunctional islet cell tumors,and even for those with distal m etastasis,immediate surgical removal and treatment can improve prognosis.

Malignant nonfunctioning islet cell tumor of the pancreas with intrasplenic growth: a case report

BACKGROUND: We reported a case of malignant nonfunction islet cell tumor (10.0 cm in diameter) of the pancreas, with malignant histological features and splenic infiltration. The case is rare, and few reports have been published. METHODS: A 46-year-old woman with a vague pain in the left upper quadrant for 3 months was found to have a tumor in the spleen. Ultrasonography and computed tomography demonstrated a well-defined pancreatic tumor of 8.2×10.0 cm in size, her serum levels of pancreatic hormones were within normal limits. RESULTS: Splenectomy combined with pancreatectomy was performed for the tail of the pancreas. Resected specimens showed a malignant nonfunctioning islet cell tumor invading the spleen. CONCLUSIONS: The growth pattern of the tumor causes malignant features. Resection of the tumor should be performed by enucleation, pancreaticoduodenectomy or distal pancreatectomy.

Clinicopathological features of small nonfunctioning pancreatic neuroendocrine tumors

AIM: To present our experiences in studying the clinicopathological features of small nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs).

Endoscopic Endonasal Surgery for Clinically Nonfunctioning Pituitary Adenomas

Background: Clinically nonfunctioning pituitary adenomas (NFPAs) are the most frequent pituitary macroadenomas, and represent approximately one-third of all pituitary adenomas. Patients often present with symptoms of mass effects, such as visual field defects, chronic headache, and hypopituitarism. Objective: The objective of this study was to retrospectively analyze the surgical results of 35 patients with non-functioning pituitary adenomas (NFPAs) operated by the endoscopic endonasal approach (EEA). Surgical outcomes including postoperative complications, recurrence and the postoperative visual and endocrine profile were assessed. Results: This retrospective study included 35 patients operated for clinically nonfunctioning pituitary adenomas (NFPAs) in Al Azhar university hospitals treated by endoscopic endonasal approach (EEA) in the last 6 years. 65.7% (n = 23) were male and 34.3% (n = 12) were female. The mean age was 41.5 (range 18 - 65) years. The most common presenting symptoms were headache, with 60% of the patients having headache. Visual disturbances were the presenting symptom in 82.9% patients and endocrinological problems (panhypopituitarism) were present in 6 (17.1%) patients. The number of patients suffering from cranial nerve deficit was 2 (5.7%). Visual acuity and visual field improved in 23 (79.3%) of 29 patients. The rate of tumor recurrence/regrowth was higher in the case of giant adenomas and cavernous sinus invasion represented 11 patients (31.4%). Conclusions: Early endoscopic endonasal approach surgery of NFPAs and effective surgical decompression reduces morbidity. Visual deficit improves in two third of cases. NFPAs represent high rate of recurrence due to invasion or incomplete resection especially with giant adenomas. The recurrence rate with growth total resection (GTR) is lower than subtotal resection (STR).

Intrapericardial Paraganglioma, an Exceedingly Rare Finding: A Case Report

Paragangliomas are rare tumors that arise from the sympathetic or the parasympathetic ganglia. Parasympathetic paragangliomas are usually nonfunctional. They are often found in the pre aortic and paravertebral sympathetic plexus or at the base of the skull. 80% of these are Glomus jugulare and carotid body paragangliomas. Intrapericardial paragangliomas are exceedingly rare. Less than 2% of paragangliomas are found in the chest and most of them are situated in the posterior mediastinum. As such, there are no clear guidelines on how to treat intrapericardial paragangliomas. We present here the case of a patient with an intrapericardial paraganglioma who was evaluated by cardiology, cardiac surgery, and endocrinology. In this case cardiac surgery opted for a conservative approach and did not offer surgery to the patient. There is some literature supporting a surgical approach but no specific guidelines about this have been written. The patient is currently being monitored since she did not have metastatic disease and her paraganglioma was nonfunctioning. With this case we want to add to the body of evidence that this type of cases does not need an urgent surgical approach.

Medium-Term Follow-Up of Children with Unilateral Non or Poorly Functioning Kidney: A Single-Center Experience

Purpose: The aim of the study was to assess the safety of conservative management of nonfunctioning kidney in children. Material and Methods: The study group consisted of 29 children with a unilateral or poorly functioning kidney who were followed at the outpatient clinic of the Urology Unit of a tertiary pediatric medical center for at least two years. Pain, hypertension, urinary tract infection, and renal calculi were assessed regularly during follow up. Results: Mean duration of follow-up was 62 months. The main causes for non-functioning kidney were high grade vesicoureteral reflux (55%) and ureteropelvic junction obstruction (34%). Urinary tract infection (24%) occurred mostly during the first 18 months of follow up. UTI was observed in all children who had bilateral vesicoureteral reflux or an ectopic ureter. None of the children developed pain, hypertension or renal calculi during follow-up period. Conclusions: In children, preserving a nonfunctioning kidney due to causes other than vesicoureteral reflux does not increase the risk of complications on medium term follow up. Those with non-functioning kidney due to high grade vesicoureteral reflux should be offered nephrectomy to decrease the risk of UTI.

Neuroendoscopic and microscopic transsphenoidal approach for resection of nonfunctional pituitary adenomas

BACKGROUND Nonfunctional pituitary adenoma is a common type of pituitary adenoma, which can lead to headache, visual field disturbance, and cranial nerve damage due to increased tumor volume. Neuroendoscopic and microscopic transsphenoidal approaches have been widely used in the resection of nonfunctional pituitary adenomas. However, the clinical efficacy in neuroendoscopic and microscopic surgery is still controversial. AIM To explore the clinical efficacy of neuroendoscopic and microscopic transsphenoidal approach for resection of nonfunctional pituitary adenomas. METHODS We retrospectively analyzed 251 patients with nonfunctional pituitary adenomas;138 underwent neuroendoscopic surgery via transsphenoidal approach, and 113 underwent microscopic surgery via transsphenoidal approach between July 2010 and September 2015. All patients were followed up for > 6 mo. Gender, age, course of disease, tumor diameter, tumor location, and percentage of patients with headache, visual impairment, sexual dysfunction, and menstrual disorders were contrasted between the two groups to compare the difference of preoperative data. Cure rate, symptom improvement rate, recurrence rate, the postoperative hospital stay, operating time, intraoperative blood loss, and the incidence of postoperative complications were compared in order to evaluate the advantages and disadvantages of neuroendoscopic and microscopic surgery.RESULTS There was no significant difference in cure rate, symptom improvement rate, and recurrence rate between neuroendoscopy group and microscopy group (82.6% vs 85.8%, P > 0.05;90.6% vs 93.8%, P > 0.05;5.1% vs 9.7%, P > 0.05). In the neuroendoscopy group, the postoperative hospital stay was 8.4 ± 0.6 d;operating time was 167.2 ± 9.6 min;intraoperative blood loss was 83.4 ± 9.3 mL, and the rates of diabetes insipidus and electrolyte imbalance were 4.3% and 8.0%, respectively. The corresponding results in the microscopic group were 11.2 ± 0.6 d, 199.7 ± 9.3 min, 138.8 ± 13.6 mL, and 32.7% and 20.4%, respectively. There were significant differences in postoperative hospital stay, operating time, intraoperative blood loss, and the rates of diabetes insipidus and electrolyte imbalance between the two groups (P < 0.05). CONCLUSION Neuroendoscopic and microscopic transsphenoidal approaches have similar clinical efficacy for the resection of nonfunctional pituitary adenomas. Neuroendoscopic surgery reduces operating time, intraoperative bleeding, postoperative recovery, and complications.

Ectopic adrenal cortical adenoma in the gastric wall:Case report

Ectopic adrenal cortical neoplasms are extremely rare. Ectopic adrenocortical tissue can be found in locations such as the celiac axis,the broad ligament,the adnexa of the testes,and the spermatic cord;however,they rarely involve the stomach.We report an unusual case of a patient with an ectopic adrenal cortical adenoma in the gastric wall.The patient was a 72-year old female admitted to our hospital with upper abdominal discomfort.Physical examination revealed tenderness below the xiphoid process.Both computed tomography and fibergastroscopy revealed a mass on the lesser curvature side of the gastric antrum;it was initially diagnosed as a gastric stromal tumor.After adequate preparation,the patient underwent surgery.During the procedure,we found a 30 mm×30 mm mass with medium density in the lesser curvature near the gastric antrum within the serosa.Following immunohistochemistry examination, we corrected the diagnosis to an ectopic adrenal cortical adenoma;the tumor was nonfunctional.

Non-functioning pancreatic neuroendocrine tumors: Surgery or observation?

Incidentally detected, sporadic, nonfunctional pancreatic neuroendocrine tumors are increasingly diagnosed on imaging studies performed for unrelated purposes. Although their resection is usually recommended, controversy still exists regarding their optimal management, due to their highly variable and difficult to predict biologic behavior. Recently, several studies and guidelines advocated an expectant management approach in small size, low grade, incidentally diagnosed nonfunctional pancreatic neuroendocrine tumors. The aim of this study is to review and summarize the available literature addressing nonfunctional pancreatic neuroendocrine tumors, with an emphasis on surgical management controversies.

Effect of remote ischemic preconditioning among donors and recipients following pediatric liver transplantation:A randomized clinical trial

BACKGROUND Studies suggested that remote ischemic preconditioning(RIPC)may effectively lessen the harmful effects of ischemia reperfusion injury during organ transplantation surgery.AIM To investigate the protective effects of RIPC on living liver donors and recipients following pediatric liver transplantation.METHODS From January 2016 to January 2019 at Renji Hospital Affiliated with Shanghai Jiao Tong University School of Medicine,208 donors were recruited and randomly assigned to four groups:S-RIPC group(no intervention;n=55),D-RIPC group(donors received RIPC;n=51),R-RIPC group(recipients received RIPC,n=51)and DR-RIPC group(both donors and recipients received RIPC;n=51).We primarily evaluated postoperative liver function among donors and recipients and incidences of early allograft dysfunction,primary nonfunction and postoperative complications among recipients.RESULTS RIPC did not significantly improve alanine transaminase and aspartate aminotransferase levels among donors and recipients or decrease the incidences of early allograft dysfunction,primary nonfunction,and postoperative complications among recipients.Limited protective effects were observed,including a lower creatinine level in the D-RIPC group than in the S-RIPC group on postoperative day 0(P<0.05).However,no significant improvements were found in donors who received RIPC.Furthermore,RIPC had no effects on the overall survival of recipients.CONCLUSION The protective effects of RIPC were limited for recipients who received living liver transplantation,and no significant improvement of the prognosis was observed in recipients.

Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: A case report

BACKGROUND Adrenocortical cancer(ACC) is an infrequent and often aggressive malignancy with a very poor prognosis. It can be classified as functional or nonfunctional.Nonfunctional ACC is hampered by the absence of specific signs or symptoms;only abdominal pain with or without incidental adrenal occupation is typically present.CASE SUMMARY We report a rare case of a patient with a 30 cm × 15 cm × 8 cm ectopic ACC on the anterior abdominal wall without organ adhesion. A 77-year-old male was admitted to our hospital because of a huge abdominal mass, which, by ultrasonography, had an unclear border with the liver. Computed tomography showed that the mass was not associated with any organ but was adherent to the anterior abdominal wall. The patient underwent tumor resection, and a postoperative pathology examination showed a neuroendocrine tumor, which was diagnosed as ACC. The patient was disease-free at the 9-mo follow up.CONCLUSION The anterior abdominal wall is a rare site of ACC growth.

Nonfunctional pancreatic neuroendocrine tumours misdiagnosed as autoimmune pancreatitis:A case report and review of literature

BACKGROUND Nonfunctional pancreatic neuroendocrine tumours are difficult to diagnose in the early stage of disease due to a lack of clinical symptoms,but they can rarely manifest as autoimmune pancreatitis.Autoimmune pancreatitis is an uncommon disease that may cause recurrent acute pancreatitis and is therefore often regarded as a special type of chronic pancreatitis.CASE SUMMARY We report a case of a 42-year-old female who had nonspecific upper abdominal pain for 4 years and radiological abnormalities of the pancreas that mimicked autoimmune pancreatitis.The symptoms and pancreatic imaging did not improve following 1 year of steroid therapy.Finally,pancreatic biopsy was performed through endoscopic ultrasonography-guided fine-needle aspiration biopsy,and nonfunctional pancreatic neuroendocrine tumours were ultimately diagnosed.Pancreatectomy has resolved her symptoms.CONCLUSION Therefore,the differentiation of nonfunctional pancreatic neuroendocrine tumours from autoimmune pancreatitis is very important,although it is rare.We propose that endoscopic ultrasonography-guided fine-needle aspiration biopsy should be performed if imaging characteristics are equivocal or the diagnosis is in question.

The changes of preoperative bone mineral density of patients with nonfunctional pituitary adenoma

Objective To evaluate the bone mineral density (BMD) alteration of patients with nonfunctional pituitary adenoma in Chinese mainland and further to investigate the relevant factors of BMD changes. Methods 31 patients with nonfunctional adenoma and 255 healthy controls were enrolled in this study between December 2007 and May 2008.

Folate receptor-mediated boron-10 containing carbon nanoparticles as potential delivery vehicles for boron neutron capture therapy of nonfunctional pituitary adenomas

Invasive nonfunctional pituitary adenomas （NFPAs） are difficult to completely resect and often develop tumor recurrence after initial surgery. Currently, no medications are clinically effective in the control of NFPA. Although radiation therapy and radiosurgery are useful to prevent tumor regrowth, they are frequently withheld because of severe complications. Boron neutron capture therapy （BNCT） is a binary radiotherapy that selectively and maximally damages tumor cells without harming the surrounding normal tissue. Folate receptor （FR）-targeted boron-10 containing carbon nanoparticles is a novel boron delivery agent that can be selectively taken up by FR-expressing cells via FR-mediated endocytosis. In this study, FR-targeted boron-10 containing carbon nanoparticles were selectively taken up by NFPAs cells expressing FR but not other types of non-FR expressing pituitary adenomas. After incubation with boron-10 containing carbon nanoparticles and following irradiation with thermal neutrons, the cell viability of NFPAs was significantly decreased, while apoptotic cells were simultaneously increased. However, cells administered the same dose of FR-targeted boron-10 containing carbon nanoparticles without neutron irradiation or received the same neutron irradiation alone did not show significant decrease in cell viability or increase in apoptotic cells. The expression of Bcl-2 was down-regulated and the expression of Bax was up-regulated in NFPAs after treatment with FR-mediated BNCT. In conclusion, FR-targeted boron-10 containing carbon nanoparticles may be an ideal delivery system of boron to NFPAs ceils for BNCT. Furthermore, our study also provides a novel insight into therapeutic strategies for invasive NFPA refractory to conventional therapy, while exploring these new applications of BNCT for tumors, especially benign tumors.

Impact and Clinical Predictors of Lymph Node Metastases in Nonfunctional Pancreatic Neuroendocrine Tumors

Background： The optimal surgical management of nonfunctional pancreatic neuroendocrine tumors （NF-PNETs） is still controversial. Here, we evaluated the impact of lymph node status on postoperative recurrence in patients with NF-PNET and the potential of preoperative variables for predicting lymph node metastasis （LNM）. Methods： In this mono-institutional retrospective cohort study conducted in 100 consecutive patients who underwent NF-PNET resection between January 2004 and December 2014, we evaluated risk factors for survival using the Kaplan-Meier method and the Cox regression model. Predictors of LNM were evaluated using the logistic regression model, and the power of predictive models was evaluated using receiver operating characteristic curve analysis. Results： Five-year disease-free survival of resected NF-PNET was 64.1%. LNM was independently associated with postoperative recurrence （hazard ratio = 3.995, P = 0.003）. Multivariate analysis revealed tumor grade as an independent factor associated with LNM （G2 vs. G I ： odds ratio [OR] =6.287, P- 0.008; G3 vs. G 1： OR = 12.407, P = 0.001）. When tumor grade was excluded, radiological tumor diameter 〉2.5 cm （OR = 5.430, P = 0.013） and presence of symptoms （OR = 3.366, P = 0.039） were significantly associated with LNM. Compared to neoplasms with radiological diameter 〉2.5 cm （32.1%）, tumors ≤2.5 cm had an obviously lower risk of LNM （7.7%）, indicating the reliability of this parameter in predicting LNM （area under the curve, 0.693）. Incidentally discovered NF-PNETs ≤2.5 cm were associated with a low-risk of LNM and excellent survival. Conclusions： LNM is significantly associated with postoperative recurrence. Radiological tumor diameter is a reliable predictor of LNM in NF-PNETs. Our results indicate that lymphadenectomy in small （≤2.5 cm） NF-PNETs is not routinely necessary.

Twenty-six patients with nonfunctional pheochromocytomas

Nonfunctional pheochromocytoma is a particular type of pheochromocytoma without typical clinical features of catecholaminism. It is difficult to diagnose or differentially diagnose. This type of pheochromocytoma is rarely reported in the literature. From January 1, 1990 to June 1, 2004, we performed operations for 197 patients with pheochromocytomas, including 26 patients with nonfunctional pheochromocytoma. We analyzed the clinical data of the 26 patients.

Feature-Oriented Nonfunctional Requirement Analysis for Software Product Line

Domain analysis in software product line （SPL） development provides a basis for core assets design and imple- mentation by a systematic and comprehensive commonality/variability analysis. In feature-oriented SPL methods, products of the domain analysis are domain feature models and corresponding feature decision models to facilitate application-oriented customization. As in requirement analysis for a single system, the domain analysis in the SPL development should con- sider both flmctional and nonfunctional domain requirements. However, the nonfunctional requirements （NFRs） are often neglected in the existing domain analysis methods. In this paper, we propose a context-based method of the NFR analysis for the SPL development. In the method, NFRs are materialized by connecting nonfunctional goals with real-world context, thus NFR elicitation and variability analysis can be performed by context analysis for the whole domain with the assistance of NFR templates and NFR graphs. After the variability analysis, our method integrates both functional and nonfunc- tional perspectives by incorporating the nonfunctional goals and operationalizations into an initial functional feature model. NFR-related constraints are also elicited and integrated. Finally, a decision model with both functional and nonfunctional perspectives is constructed to facilitate application-oriented feature model customization. A computer-aided grading system （CAGS） product line is employed to demonstrate the method throughout the paper.