Adrenal Gland Neoplasms ; surgery ; Adrenalectomy ; methods ; Adult ; Female ; Humans ; Kidney ; abnormalities ; Laparoscopy ; Multiple Endocrine Neoplasia Type 2a ; surgery ; Pheochromocytoma ; surgery

Adrenal Gland Neoplasms ; surgery ; Adult ; Cystectomy ; methods ; Humans ; Laparoscopy ; methods ; Male ; Pheochromocytoma ; surgery ; Urinary Bladder Neoplasms ; surgery

Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition, or unusual symptoms such as diabetes mellitus develops in a cyanotic patient with congenital heart disease, pheochromocytoma must be ruled out. We report two patients presenting with cyanotic single-ventricle heart disease with pheochromocytoma.
Anoxia ; Diabetes Mellitus ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Hypertension ; Pheochromocytoma ; Thoracic Surgery

Adrenalectomy ; Female ; Humans ; Pheochromocytoma ; diagnosis ; surgery ; Tachycardia, Ventricular ; diagnosis ; Tomography, X-Ray Computed

In recent years, cardiopulmonary bypass(CPB), a standard adjunct for open heart surgery, has been used more frequently to treat diseases that are not primarily of cardiac origin. We report one case of major left adrenal pheochromocytoma surgery and another of surgery for localized renal cell carcinoma with a tumor thrombus extending up the vena cava to the right atrium using adjunctive cardiopulmonary bypass.
Carcinoma, Renal Cell ; Cardiopulmonary Bypass* ; Heart Atria ; Pheochromocytoma ; Thoracic Surgery ; Thrombosis

OBJECTIVETo study the diagnosis and treatment of pheochromocytoma in urinary bladder.

METHODSSix cases of bladder pheochromocytoma were studied. Four cases showed hypertension, 3 of which were paroxysmal hypertension during urination. Catecholamine (CA) was increased in a case, and vanillymandelic acid (VMA) was increased in 2 cases. Bladder submucosal mass was detected by B-ultrasound in 5 cases (5/5), computerized tomography (CT) in 3 cases (3/3), cystoscopy in 5 cases (5/6). Four cases took alpha-receptor blocker for 2 weeks, 1 case took beta-receptor blocker to decrease heart rate. All patients were treated with surgical operation including 4 partial cystectomies, 2 excavations.

RESULTSThree cases had manifestations including headache, excessive perspiration and hypertension during cystoscopy. Four cases were confirmed before operation. Two cases showed hypertension during operation. All patients were pathologically diagnosed as pheochromocytoma postoperatively. In five cases followed up, blood pressure returned to normal. No patient had relapse and malignancy.

CONCLUSIONSTypical hypertension during urination comprised the main symptoms. We should highly suspect bladder pheochromocytoma if a submucosal mass was discovered with B-ultrasound, CT, (131)I-MIBG (methyliodobenzylguanidine) and cystoscopy. The determination of CA in urine is valuable for qualitative diagnosis. The preoperative management of controlling blood pressure and expansion of the blood volume are very important. Surgical operation is a good method for effective treatment. Postoperative long-time followed up is necessary.

Adult ; Blood Pressure ; Female ; Humans ; Male ; Middle Aged ; Pheochromocytoma ; diagnosis ; physiopathology ; surgery ; Urinary Bladder Neoplasms ; diagnosis ; physiopathology ; surgery

BACKGROUNDAdrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions.

METHODSClinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and (131)I-metaiodobenzylguanidine (MIBG) were performed. Neither (131)I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance.

RESULTSTe mean age of patients in the adrenal ganglioneuroma group was 41.2 years (16 - 67 year) and in the adrenal pheochromocytoma patients 38 years (17 - 74 year). Contrast enhanced CT showed that the foci were intensified in 5 cases (27.8%) of adrenal ganglioneuroma and there were obvious contrast indications in 30 (83.3%) of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 (100%) cases with adrenal pheochromocytoma. (131)I-MIBG nuclear scan showed negative results in 4 patients (100%) with adrenal ganglioneuroma and positive results in 25 (96.2%) with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of (43 +/- 6) months, and all cases survived well.

CONCLUSIONSCT, urinary catecholamine and (131)I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.

Adolescent ; Adrenal Gland Neoplasms ; diagnosis ; surgery ; Adult ; Aged ; Female ; Ganglioneuroma ; diagnosis ; surgery ; Humans ; Laparoscopy ; methods ; Male ; Middle Aged ; Pheochromocytoma ; diagnosis ; surgery ; Retrospective Studies ; Young Adult

OBJECTIVETo evaluate the safety and efficacy of retroperitoneal laparoscopic resection for pheochromocytoma.

METHODSThe clinical data of 107 cases of pheochromocytoma in PUMCH from 2003 to 2008 were analyzed retrospectively. There were 58 males and 49 females with an age range from 8 to 77 years (mean 44 years) in this cohort. One hundred and two cases were intra-adrenal and 5 extra-adrenal. Of the 102 intra-adrenal tumors, 43 tumors were located in left adrenal, 51 in right adrenal and 8 in both sides. All of the 5 extra-adrenal tumors were at para-abdominal aorta. Retroperitoneal laparoscopic resection was performed for the 107 cases. This period was separated to 3 stages based on the degrees of the practical ability for retroperitoneal laparoscopic resection, such as tentative and exploratory stage, accumulative stage, and mature stage.

RESULTSAt tentative and exploratory stage from June 2003 to December 2003, 10 cases underwent retroperitoneal laparoscopic surgery, of which 3 cases were converted to open surgery. The mean diameter was 4.2 cm (range in diameter from 2.5 cm to 6.0 cm). The mean operation time was 105 min (range from 60 min to 230 min). The mean volume of blood loss during operation was 620 ml (range from 150 ml to 1800 ml). At accumulative stage from January 2004 to December 2006, 66 cases underwent retroperitoneal laparoscopic surgery with none converted to open surgery. The mean diameter was 5.7 cm (range in diameter from 2.1 cm to 8.7 cm), and the diameter was above 6.0 cm in 19 cases. The mean operation time was 95 min (range from 40 min to 210 min). The mean volume of blood loss during operation was 350 ml (range from 50 ml to 1800 ml). At mature stage from January 2007 to June 2008, 31 cases, including 5 extra-adrenal pheochromocytomas, underwent retroperitoneal laparoscopic surgery. The mean diameter was 6.5 (range in diameter from 1.5 cm to 12.3 cm). The mean operation time was 75 min (range from 40 min to 160 min). The mean volume of blood loss during operation was 180 ml (range from 50 ml to 800 ml). No peri-operative death occurred. Follow up period was ranging from 1 to 62 months (the mean was 34 months), and 7 failed to be followed up, 3 cases recurred. And there was no distant metastases and death case.

CONCLUSIONSRetroperitoneal laparoscopic surgery for pheochromocytoma is feasible and safe. This procedure will be more and more performed as the advancement of the skill and accumulation of experience. The dimension, recurrence and location of tumor are not the absolute contraindication of retroperitoneal laparoscopic surgery for pheochromocytoma.

Adolescent ; Adrenal Gland Neoplasms ; surgery ; Adult ; Aged ; Child ; Female ; Follow-Up Studies ; Humans ; Laparoscopy ; Male ; Middle Aged ; Pheochromocytoma ; surgery ; Retroperitoneal Neoplasms ; surgery ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo investigate clinical features and anesthetic management of multiple endocrine neoplasia (MEN) associated with pheochromocytoma.

METHODSMedical records of patients who were diagnosed as multiple endocrine neoplasia associated with pheochromocytoma in our hospital from April 1977 to April 2001 were reviewed retrospectively. The demographic data, clinical presentations, family history, biochemical examinations, type of MEN, sequence of different surgical procedures, anesthetic methods and hemodynamics during surgery were analyzed.

RESULTSThirteen cases of MEN associated with pheochromocytoma were investigated, accounting for 6% (13/213) of the pheochromocytoma patients admitted into our hospital. Nine of the 13 patients presented as type IIa MEN (Sipple syndrome), one as type IIb MEN, and three as mixed MEN. Four patients with type IIa MEN had a family history of similar disease. Five patients with other coexisting endocrine disorders first underwent excision of the pheochromocytomas, although only two had hypertensive symptoms at the time of admittance. Seven patients without histories of hypertension received surgical treatment for pheochromocytoma secondly. The excision of pheochromocytoma was performed under general anesthesia in 8 patients and epidural block in 4 patients. Marked hemodynamic fluctuation was recorded in 8 patients. No perioperative death was recorded.

CONCLUSIONPheochromocytoma may be linked to other endocrine disorders during MEN, either as the main clinical presentation or most frequently as an occult tumor. Recognition of this feature of pheochromocytoma is of importance to the improvement of diagnosis and treatment both for pheochromocytoma and MEN.

Adrenal Gland Neoplasms ; diagnosis ; physiopathology ; surgery ; Adrenalectomy ; Adult ; Anesthesia ; methods ; Female ; Hemodynamics ; Humans ; Male ; Middle Aged ; Multiple Endocrine Neoplasia ; physiopathology ; surgery ; Pheochromocytoma ; diagnosis ; physiopathology ; surgery ; Retrospective Studies

OBJECTIVE: To explore the surgical strategy and experience of reoperation for pheochromocytoma and paraganglioma which is very challenging. METHODS: The clinical data of 7 patients with pheochromocytoma and paraganglioma who underwent reoperation in Department of Urology, Peking University Third Hospital from August 2016 to February 2021 were analyzed retrospectively. There were 4 males and 3 females, with an average age of (44.1±11.5) years (28-60 years), 6 cases on the right side and 1 case on the left side. The causes of the operations included: (1) 2 cases of tumor recurrence after resection; (2) The primary operations failed to completely remove the tumors in 3 cases, because the tumors were large and closely related to blood vessels. (3) Pheochromocytoma and paraganglioma wasn't diagnosed before primary operation, therefore, drug preparation wasn't prepared. Two cases were interrupted by severe blood pressure fluctuations during the primary operations. Imaging evaluation, catecholamine biochemical examination and adequate adrenergic α receptor blockers were administrated in all the cases. The surgical approaches included open transperitoneal surgery in 4 cases, robot-assisted laparoscopy in 1 case and retroperitoneal laparoscopy in 2 cases. The innovative techniques included mobilization of the liver, inferior vena cava transection and anastomosis, and transection of left renal vein. RESULTS: The average tumor size was (8.0±3.2) cm (3.6-13.9 cm). The median interval between the reoperation and the primary operation was 9 months (IQR: 6, 19 months). The median operation time was 407 min (IQR: 114, 430 min) and the median blood loss was 1 500 mL (IQR: 20, 3 800 mL). Operations of 5 cases were performed successfully, and 1 case failed only by exploration during the operation. One case died perioperatively. There were 5 cases of intraoperative blood transfusion, the median transfusion volume of red blood cells was 800 mL (IQR: 0, 2 000 mL). One case experienced postoperative lymphorrhagia, and recovered after conservative treatment. The renal function was normal in 2 cases after resection and anastomosis of inferior vena cava or transection of left renal vein. The average postoperative hospital stay was (7.2±3.3) d (4-13 d). The median follow-up time of 6 patients was 33.5 months (IQR: 4.8, 48.0 months). The case who failed in the reoperation still survived with tumor and there was no recurrence in the rest of the patients. CONCLUSION The reoperation of pheochromocytoma and paraganglioma, which can not be resected in the primary operation or recurred postoperatively, is difficult with high risk of hemorrhage, and there is a risk of failure and perioperative death. Different surgical approaches and strategies need to be adopted based on the different situation.
Adrenal Gland Neoplasms/surgery* ; Adult ; Female ; Humans ; Laparoscopy ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Paraganglioma/surgery* ; Pheochromocytoma/surgery* ; Reoperation ; Retrospective Studies