Objective To analyze the clinical features of Von-Hippel-Lindau(VHL)syndrome and explore the diagnostic value of abdominal ultrasound for this disease.Methods The clinical features including age at first diagnosis,symptoms,signs,affected organs,number of operations,and diagnostic examinations of 35 patients with VHL syndrome admitted to our center from January 1994 to December 2017 were retrospectively analyzed.The diagnostic value of abdominal ultrasound for VHL syndrome was analyzed.Results Pheochromocytoma(=14)and nervous system hemangioblastoma(=13)were the common firstly-identified tumors.Nervous system hemangioblastoma(=21),pheochromocytoma(=19),renal carcinoma(=17),and pancreatic mass(=15)were common tumors.The main surgical reasons were nervous system hemangioblastoma(=22),pheochromocytoma(=23)and renal carcinoma(=13).Abdominal organ involvements were found in 33 patients,which were first detected by abdominal ultrasound in 20 patients and were found accidently during routine health checkups in 6 patients.The ultrasound results were accurate in 27 of 33 adrenal gland scans,13 of 16 pancreas scans,and 8 of 19 kidney scans.Conclusions When multiple tumors are detected in the kidney,adrenal gland,and pancreas by ultrasound,the possibility of VHL syndrome should be considered.When the clinical findings suggest the possibility of VHL syndrome,ultrasound can discover and diagnose the abdominal tumors and can also be used for the long-term follow-up of the tumors.Therefore,ultrasound is an important method in the screening and follow-up of patients with VHL syndrome.
Abdomen ; diagnostic imaging ; Adrenal Gland Neoplasms ; diagnostic imaging ; Hemangioblastoma ; diagnostic imaging ; Humans ; Pheochromocytoma ; diagnostic imaging ; Retrospective Studies ; Ultrasonography ; von Hippel-Lindau Disease ; complications ; diagnostic imaging

Adrenal Cortex Neoplasms ; complications ; Adrenal Gland Neoplasms ; complications ; Adrenal Glands ; pathology ; Adrenocortical Adenoma ; complications ; Humans ; Pheochromocytoma ; complications

Phaeochromocytomas are rare neuroendocrine tumours with variable clinical signs and symptoms. Hypertension, tachycardia, sweating and headaches are cardinal manifestations. Although nausea and abdominal pain are the more common gastrointestinal features, rare gastrointestinal spectrums have been reported that can mimic abdominal emergencies. Metabolic effects of hypercatecholaminaemia are vast and one such rare presentation is lactic acidosis. We describe a case of phaeochromocytoma presenting with both intestinal pseudo-obstruction as well as lactic acidosis. This case report highlights the importance of having a high index of suspicion for and early recognition of the gastrointestinal and metabolic manifestations of phaeochromocytomas.
Abdominal Pain ; Acidosis, Lactic ; complications ; Adrenal Gland Neoplasms ; complications ; diagnosis ; Diabetes Mellitus, Type 2 ; complications ; Dyslipidemias ; complications ; Female ; Humans ; Hypertension ; complications ; Intestinal Pseudo-Obstruction ; complications ; Middle Aged ; Neuroendocrine Tumors ; complications ; diagnosis ; Pheochromocytoma ; complications ; diagnosis ; Radiography, Abdominal

No abstract available.
Adrenal Cortex Function Tests ; *Adrenal Cortex Neoplasms/complications/diagnosis/metabolism/surgery ; *Adrenal Gland Neoplasms/complications/diagnosis/metabolism/surgery ; Adrenalectomy ; *Adrenocortical Adenoma/complications/diagnosis/metabolism/surgery ; Biopsy ; Cushing Syndrome/diagnosis/etiology ; Female ; Humans ; Immunohistochemistry ; *Incidental Findings ; Middle Aged ; *Neoplasms, Multiple Primary/complications/diagnosis/metabolism/surgery ; *Pheochromocytoma/complications/diagnosis/metabolism/surgery ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome ; Tumor Markers, Biological/metabolism

Adrenal gland tumours in pregnancy are very rare occurrences and have highly variable clinical presentations. The timely diagnosis of adrenal tumours in pregnancy is extremely important, as failure to do so may lead to fatality. As there is limited published literature on adrenal tumours in pregnancy and no consensus on its management, the management of such patients with regard to medical and surgical treatments, as well as timing of delivery, must therefore be individualised and carried out with multidisciplinary expertise. We present two cases of adrenal tumours in pregnancy, both with favourable outcomes and variable gestations. Our first and second cases discuss a large phaeochromocytoma and a cortisol-secreting adrenal cortical adenoma in pregnancy, respectively.
Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adrenalectomy ; Adult ; Biomarkers ; blood ; urine ; Diagnosis, Differential ; Female ; Humans ; Laparoscopy ; Magnetic Resonance Imaging ; Pheochromocytoma ; diagnosis ; surgery ; Pregnancy ; Pregnancy Complications, Neoplastic ; diagnosis

Pheochromocytoma is a rare cause of hypertension in children. Hypertension is one of the common reasons of posterior reversible encephalopathy. Intracerebral hemorrhage is a serious and unexpected complication of hypertensive encephalopathy due to pheochromocytoma, and very rarely seen in the childhood. Intracerebral hemorrhages should be searched if there are hypertensive reversible signal changes on the brain. Susceptibility weighted imaging (SWI) is a more sensitive method than conventional MRI when demonstrating cerebral microhemorrhagic foci. This is the first report of SWI findings on intracerebral hemorrhages in basal ganglia, brain stem and periventricular white matter due to hypertensive encephalopathy in a child with pheochromocytoma.
Adolescent ; Adrenal Gland Neoplasms/*complications/diagnosis ; Brain/*pathology ; Diagnosis, Differential ; Female ; Humans ; Hypertensive Encephalopathy/*diagnosis/etiology ; Magnetic Resonance Imaging/*methods ; Pheochromocytoma/*complications/diagnosis

PURPOSE: During laparoscopic partial cystectomy (LPC), lesion identification is essential to help to determine the appropriate bladder incisions required to maintain adequate resection margins. The inability to use tactile senses makes it difficult for surgeons to locate lesions during laparoscopic surgery. Endoscopic India ink marking techniques are often used in laparoscopic gastroenterological surgery. We present our experience with performing LPC with India ink during the surgical resection of various bladder lesions. MATERIALS AND METHODS: LPC with cystoscopic fine needle tattooing was performed on 10 patients at our institute. Tattooing was performed at 1- to 2-cm intervals approximately 1 cm away from the outer margin of the lesion with enough depth (the deep muscle layer) under cystoscopic guidance. LPC was performed by the transperitoneal approach. The clinical courses and pathologic results were analyzed. RESULTS: All LPC with cystoscopic tattooing cases were performed successfully. The mean patient age was 39.1 years. The mean operative time was 130.5 minutes, and the mean estimated blood loss was 93 ml. The mean hospital stay was 13.1 days, and the mean duration of indwelling Foley catheterization was 10.7 days. There were no significant intraoperative or postoperative complications except 1 case of delayed urinary leak and 1 case of delayed wound healing. The pathological diagnosis included 1 urachal cancer, 1 urachal remnant, 4 urachal cysts, 2 pheochromocytomas, and 2 inflammatory masses. All specimens showed adequate surgical margins. CONCLUSIONS: Cystoscopic tattooing in LPC is a simple and effective technique to assist in locating pathological bladder lesions intraoperatively. This technique can help to determine appropriate resection margins during LPC without incurring additional complicated procedures.
Carbon ; Cystectomy ; Cystoscopy ; Humans ; India ; Ink ; Laparoscopy ; Length of Stay ; Muscles ; Needles ; Operative Time ; Pheochromocytoma ; Postoperative Complications ; Tattooing ; Urachal Cyst ; Urinary Bladder ; Urinary Bladder Neoplasms ; Urinary Catheterization ; Wound Healing

OBJECTIVETo analyze the change in the incidence of pheochromocytomas in adrenal medulla or extra-adrenal and multiple endocrine neoplasm type 2 (MEN2), to summarize the clinical characteristics of benign, potentially malignant and malignant pheochromocytomas and to investigate the correlation between clinical manifestations and pathological changes.

METHODSStatistic analysis was performed to detect the incidence, constituent ratio, mean diagnostic age, sex proportion and correlation between clinical manifestions and pathologic changes in pheochromocytomas in adrenal medulla or extra-adrenal gland and MEN2 from 1993 to 2008 in the Department of Pathology, the General Hospital of Tianjin Medical University with Runs test, ANOVA, t test and chi-square test.

RESULTSThe total number of biopsies within the 16 years was 167 702 cases (average 10 481 cases per year). The numbers (detectable rate) of total adrenal diseases, pheochromocytomas in adrenal medulla and extra-adrenal glands were 910 (0.54%), 139 (0.08%), and 42 (0.03%) cases, respectively. The numbers (constituent ratio) of benign, potentially malignant and malignant of pheochromocytomas in adrenal medulla were 102 cases (73.4%), 29 cases (20.9%) and 8 cases (5.7%), respectively; in the 102 cases of benign tumors, patients with MEN2 were 8 (7.8%); the three groups of the tumors in extra-adrenal sites were 18 (42.8%) cases, 12 (28.6%) cases and 12 (28.6%) cases. There were no changes in the detectable rate and constituent ratio of adrenal diseases, benign, potential malignant and malignant pheochromocytomas in adrenal medulla or extra-adrenal glands and patients with MEN2 during the past 16 years (P > 0.05), but there was a tendency that malignant transformation was gradually increased with age, which was more commonly found in male patients than females. The mean age at diagnosis of patients with benign and potentially malignant pheochromocytomas was 42.7 years (ranged from 10 - 74 years), and 40.1 years (13 - 66 years), respectively, which were younger than patients with malignant pheochromocytomas (51.6 years, P < 0.05); the mean age of patients with benign and potentially malignant pheochromocytomas in extra-adrenal sites was 43.1 years (ranged from 20 - 75 years) and 45.2 years (28 - 65 years) that were older than those with malignant (37.8 years, ranged from 14 - 58 years, P < 0.05). It was spectacular that patients with malignant pheochromocytoma in adrenal medulla (51.6 years) were older than that in extra-adrenal sites (37.8 years); all the patients with MEN2 were female benign pheochromocytoma in adrenal medulla, whose age (38.9 years) was younger than that of benign lesions (42.7 years, P < 0.05), in which thyroid medullary carcinoma appeared early than pheochromocytomas in adrenal medulla. The detectable rate of hypertension in patients with malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites were less than that in benign and potentially malignant ones (P < 0.05). The bilateral lesions more commonly found in malignant pheochromocytoma (25.0%) than benign (15.7%) and potentially malignant pheochromocytomas (6.9%) only in adrenal medulla. Relapse rates in both adrenal and extra-adrenal tumors were rising from benign (11.8%, 0), potentially malignant (13.8%, 25.0%), to malignant (33.3%, 37.5%) groups; the average diameter of pheochromocytomas in both adrenal and extra-adrenal sites was increasing from benign (4.2 cm, 4.0 cm), potentially malignant (5.3 cm, 5.6 cm) to malignant (7.3 cm, 6.9 cm) groups (P < 0.05).

CONCLUSIONSThe diagnostic criteria of benign, potentially malignant and malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites are well established according to the WHO classification of endocrine tumors (2004). The closer relationship is found between clinical manifestations and pathologic changes. The definite type and nature of pheochromocytomas are mainly rested upon the pathologic examination.

Adolescent ; Adrenal Gland Diseases ; pathology ; Adrenal Gland Neoplasms ; complications ; pathology ; Adrenal Medulla ; pathology ; Adult ; Age Factors ; Aged ; Carcinoma, Neuroendocrine ; Child ; Female ; Humans ; Hypertension ; etiology ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a ; pathology ; Neoplasm Recurrence, Local ; Pheochromocytoma ; complications ; pathology ; Retroperitoneal Neoplasms ; pathology ; Thyroid Neoplasms ; pathology ; Urinary Bladder Neoplasms ; pathology ; Young Adult

Adrenal Gland Neoplasms ; complications ; physiopathology ; Hemorrhage ; etiology ; Humans ; Male ; Middle Aged ; Peritoneal Diseases ; etiology ; Pheochromocytoma ; complications ; physiopathology

PURPOSE: Laparoscopic surgery on the adrenal gland is a highly specific procedure that requires mastery of laparoscopic surgery. From January 2000 to October 2008, 66 laparoscopic adrenalectomies on 65 patients were performed by a single surgeon. This study was done to summarize our experience and to evaluate the safety and effectiveness of laparoscopic adrenalectomy. METHODS: A retrospective review of the patient records was carried out. The patients' demographics, the tumor characteristics, the open conversion rate, the operating time, the length of the hospital stay and other clinical outcomes were studied. RESULTS: There were 8 conversions out of 65 patients. The indications for surgery included functional tumors in 42 patients (19 pheochromocytomas in 18 patients, 14 patients of primary aldosteronism and 10 patients of Cushing syndrome), and 23 nonfunctional tumors. The average tumor size was 3.5 cm. The mean operating time was 163 min. The average length of the hospital stay was 4.1 days. Postoperative complications occurred in 8 patients with no perioperative mortality, and most of the complications could be considered as minor. During follow-up, two patients had adrenal insufficiency and port site hernia, and none had recurrence of hormonal excess. CONCLUSION: Laparoscopic adrenalectomy has several advantages such as minimal postoperative pain, few surgical complications, a short hospital stay and an early return to work. The results confirm that laparoscopic adrenalectomy is the procedure of choice for resection of various benign adrenal neoplasms.
Adrenal Gland Neoplasms ; Adrenal Glands ; Adrenal Insufficiency ; Adrenalectomy ; Demography ; Follow-Up Studies ; Hernia ; Humans ; Hyperaldosteronism ; Laparoscopy ; Length of Stay ; Pain, Postoperative ; Pheochromocytoma ; Postoperative Complications ; Recurrence ; Retrospective Studies ; Return to Work