Functional tumors of the organ of Zuckerkandl.

Chromaffin-reacting pheochromocytomas of the adrenal medulla are the most frequently encountered functional paraganglionic neoplasms. However, extra-adrenal pheochromocytomas as well as non-chromaffin paragangliomas, including those of the carotid body and glomus jugulare, may produce symptoms from catecholamine secretion. One of the extra-adrenal sites from which these tumors arise is from a collection of para-aortic, paraganglion cells around the origin of the inferior mesenteric artery. This collection of paraganglia was described in fetuses by Zuckerkandl in 1901 and has subsequently been referred to as the organ of Zuckerkandl. The diagnosis and management of these neoplasms differ somewhat from that of adrenal pheochromocytomas, but excellent results are often obtained by excision of these lesions. Four patients with functional tumors of the organ of Zuckerkandl are reviewed together with the other reported cases in the literature.     

Variable hemodynamic fluctuations during resection of multicentric extraadrenal pheochromocytomas

PURPOSE: To report the perioperative management and the serious hemodynamic fluctuations during manipulation of an organ of Zuckerkandl tumour in a patient undergoing resection of multicentric extraadrenal pheochromocytomas. CLINICAL FINDINGS: A 28-yr-old man who had undergone at age 12 a laparotomy for excision of an extraadrenal pheochromocytoma complained of paroxysmal headache, occasional sweating and palpitations. The arterial blood pressure (BP) was 200/100 mmHg. A 24-hr-urine collection showed catecholamines 5076 microg x 24 hr(-1) (normal < 25 microg x 24 hr(-1)). Computed tomography of the abdomen revealed two retroperitoneal masses, one adjacent to the lower pole of the right kidney and a second larger mass located at the aortic bifurcation in the region of the organ of Zuckerkandl. The patient was scheduled for excision of multiple extraadrenal pheochromocytomas. He was prepared preoperatively for two weeks with prazosin 1 mg po q six hours and propranolol 10 mg tid. Manipulation of the infrarenal tumour was uneventful but manipulation of the Zuckerkandl tumour resulted in severe hypertensive episodes with BP ranging from 200/100 to 320/120 mmHg. Surgery was interrupted temporarily; the hypertensive crisis was controlled by the infusion of sodium nitroprusside and by iv phentolamine and esmolol. CONCLUSION: In a patient undergoing resection of recurrent multicentric extraadrenal pheochromoctyomas, severe hypertensive episodes occurred during manipulation of one tumour but not during manipulation of the other. This may be attributed to inadequate preparation of the patient, difficult surgical dissection of the large Zuckerkandl pheochromocytoma, and/or secondary to an excessive and different pattern of release of catecholamines during manipulation of the Zuckerkandl tumour.     

Twenty-five-year surgical experience with pheochromocytoma in children

Our objective was to analyze the presentation, diagnostic localization, operative management, histology, and long-term outcome of a single center's experience with pheochromocytomas in children. A chart review was done to identify all operatively managed pheochromocytomas in patients age 18 years or younger. Open and laparoscopic cases were included. We reviewed the presentation, diagnostic imaging, localization, operative management, pathology, and postoperative outcome of these patients. Clinic visits, contact with the tumor registry, and telephone interviews were used for follow-up. From 1973 through 1999, there were 11 children (four males and seven females) with 14 pheochromocytomas. Two (18.2%) patients had bilateral adrenal lesions and one patient had both adrenal and extra-adrenal tumors. Six (54.5%) patients had extra-adrenal lesions. The average age at operation was 14.7 years (range 9-18 years). Nine (82%) patients had significant hypertension at presentation. CT was used to localize the tumor in eight patients and urine catecholamine levels were used to confirm the diagnosis. Two of the cases were associated with inherited syndromes (multiple endocrine neoplasia 2A and von Hippel-Lindau). Ten patients underwent an open operation and one patient had a laparoscopic resection. The average patient follow-up was 9.2 years (range 9 months to 25 years). There were no operative complications and all patients were alive and well at the time of last follow-up. Three patients (27.2%) had tumors with microscopic malignant features. No tumors recurred or had evidence for metastatic spread. We conclude that peak incidence of pheochromocytomas in children is in early adolescence. Resection can be carried out safely with minimal morbidity and mortality. Current best management of this entity includes establishment of a biochemical diagnosis, adequate preoperative blockade, appropriate imaging, and an individualized operative approach based on tumor location and size.     

Periprostatic pheochromocytoma

In adults, 10% of pheochromocytomas are extra-adrenal. Most extra-adrenal pheochromocytomas occur in the organ of Zuckerkandl. Occasionally, pheochromocytomas can arise from paraganglion chromaffin cells in other locations. We present the case of a 63-year-old hypertensive man with a periprostatic pheochromocytoma. Our patient underwent surgical extirpation of the pheochromocytoma, while sparing the prostate from resection. His blood pressure has remained well controlled postoperatively without the addition of antihypertensive medications.     

Recurrent pheochromocytomas in children

Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 6 years (1980 to 1986). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 6 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences, there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension.     

Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

Introduction The rule of “tens” is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant. The objective of this study was to review the ectopic pheochromocytomas in a tertiary endocrine center and to establish whether the rule of tens holds true. Methods Retrospective data were collected on all adrenalectomies and ectopic pheochromocytoma resections performed between 1993 and 2005 at our institution. Results In total, 77 patients had pheochromocytomas: 75%; (58/77) adrenal and 25%; (19/77) ectopic. Of the adrenal pheochromocytomas, 10%; (6/58) were bilateral. The anatomic locations of the ectopic pheochromocytomas were as follows: 26%; (5/19) adjacent to the adrenals, 53%; (10/19) in the organ of Zuckerkandl, 11%; (2/19) in the bladder, 5%; (1/19) in the mediastinum, and 5%;(1/19) in the neck. Conclusions Our series demonstrates an incidence of 10%; for bilateral pheochromocytoma, which is similar to that in the published reports. However, 25%; of the pheochromocytomas were ectopic. Zuckerkandl pheochromocytomas were the most common among the ectopic lesions. Rarely, these tumors present outside the abdominal cavity An erratum to this article is available at .     

Two boys with four pheochromocytomas each

Two teenage boys were treated at separate pediatric institutions for four separate pheochromocytomas over the last 15 years. The first operation in each boy was performed between 9 and 11 years of age, after they presented with severe hypertensive encephalopathy. One boy had separate adrenal tumors excised in 1968, and 3 and 11 years later, two more separate new left adrenal pheochromocytomas were again resected. They were not recurrent left adrenal tumors from residual pheochromocytoma-secreting tissue, because no visible tumor tissue was left behind at the completion of each previous operation, and he was clinically well for years between each tumor resection. The pathology was benign pheochromocytoma. He remains well since the last operation in 1979 but has a residual hemiplegia from the first tumor. The family history is negative. The second boy was first operated on in 1976 at age 11 years when two benign pheochromocytomas were removed from around the right renal artery and the left para-aortic area. He remained well for 6 years when he became hypertensive again (this time without encephalopathy), and had a right chest paravertebral pheochromocytoma removed and several weeks later a left adrenal tumor was also removed. He remains well and followed closely. His family history is negative.     

The role of positron emission tomography in selecting patients with metastatic cancer for adrenalectomy

Metastases to the adrenal glands usually signal disseminated disease. However, isolated metastases do occur that may be curable with adrenalectomy. Functional imaging with positron emission tomography (PET) can differentiate benign from malignant pathology and isolated from disseminated metastases. The purpose of this study was to determine whether PET scanning can influence the outcome of adrenalectomy for metastatic disease. We conducted a retrospective review of eight patients undergoing adrenalectomy for presumed isolated metastatic disease from 1985 through 1997. The patients included six women and two men with an average age of 58 (range, 36-74). Their primary tumors were six lung carcinomas, one renal cell carcinoma, and one colon carcinoma. The adrenal masses were located on the right in six patients, on the left in one, and bilaterally in one. Before operation, all patients were evaluated by chest and abdominal CT. Four patients were also evaluated by PET scan. Six right, one left, and one bilateral adrenalectomies were performed. Associated organ resections included two right partial nephrectomies and one right total nephrectomy, one left partial nephrectomy, two distal pancreatectomies, one splenectomy, and two partial hepatic resections. All eight patients survived operation. There were no major perioperative complications, but one patient required readmission for congestive heart failure. Three of the four patients who did not have PET scanning died from 4 to 48 months after operation with disseminated disease from lung, colon, and renal carcinoma respectively. The remaining patient who did not have PET scanning is alive and well 11 years later. Two of the four patients who had PET scans showing isolated disease are alive at 28 and 43 months after operation, whereas the other two died of disseminated disease at 29 and 36 months after operation. We conclude that 1) adrenalectomy can provide survival benefit in patients with isolated metastases, and 2) PET scanning is useful in confirming isolated metastatic disease and selecting patients for adrenalectomy.     

Multiple digital glomus tumors.

In 28 patients with glomus tumors of the digits, 21 were relieved of symptoms by excision of a solitary tumor. Seven other patients had multiple tumors, four of which were the cause of recurrent symptoms. Three patients had multiple tumors--five in one patient, four in another, and two in a third, which had been found at the time of the first operation. A 25% incidence of multiple tumors and a 15% incidence of recurrent symptoms indicates that a careful search should be made for additional tumors.     

单纯家族性嗜铬细胞瘤的一家系调查与临床分析

目的:探讨单纯家族性嗜铬细胞瘤的临床特征。方法:调查一个单纯家族性嗜铬细胞瘤家系,绘制该家系发病图谱,并进行遗传学分析。结果:该家系4代56人中7人患嗜铬细胞瘤,其中2例拟诊,5例经手术证实。发病部位均为肾上腺,6例为双侧多发,其中术后对侧复发肿瘤3例。病理检查均为良性。术后患者症状消失,随访6月~23年患侧无复发。结论:该家系嗜铬细胞瘤符合常染色体显性遗传特点。单纯家族性嗜铬细胞瘤发病急骤,常见双侧多发,肿瘤极少恶变,不并发各种遗传综合征。切除单侧肿瘤者术后应定期随访。     

Primary Pulmonary Artery Sarcoma: Surgical Management and Differential Diagnosis with Pulmonary Embolism and Pulmonary Valve Stenosis

Abstract   Background and aim of the study: Primary pulmonary artery sarcomas are rare and lethal tumors that are usually diagnosed during surgery or autopsy. We present six cases of primary pulmonary artery sarcomas and discuss clinical features, differential diagnosis, surgical treatment, and outcome of the tumors. Methods: Between January 1994 and July 2008, six patients were identified with the disease during operation. Three patients were initially diagnosed with pulmonary valve stenosis, and two patients had a presumptive diagnosis of chronic pulmonary embolism. Two patients had simple or partial tumor resection. Four patients had radical tumor resection and homograft reconstruction of the pulmonary arteries. Results: Histological examinations showed five malignant mesenchymomas and one fibrosarcoma. One patient died of refractory pulmonary hypertension during operation. Two patients died 4 months postoperatively because of brain metastases. Two patients were alive for 3 and 9 months, respectively after the operation with recurrent tumor. One patient is alive even 2 years after resection with no signs of recurrence or metastasis. Conclusions: Because of similar clinical features, pulmonary artery sarcomas are often confused with other pulmonary vascular obstructive diseases. Computed tomography scanning and gadolinium-enhanced magnetic resonance imaging could be useful methods for differential diagnosis. The prognosis is very poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis. Early diagnosis and radical surgical resection presents the only opportunity for a potential cure.     

Surgery of retroperitoneal pheochromocytoma

Retroperitoneal pheochromocytomas are very uncommon tumors. During the last 10 years only 3 cases have been operated on in our clinic. Two of them were primary retroperitoneal paragangliomas. These two patients are now well and without recurrence 1 and 10 years respectively after operation. The third patient had retroperitoneal metastases of an adrenal pheochromocytoma. Now, after 1 year he has disseminated metastases without response to therapy with (131I)metaiodobenzylguanidine (MIBG). The surgical procedure includes often extended resection and replacement of retroperitoneal blood vessels and requires therefore an experienced surgical team and optimal anesthetic management (Swan-Ganz-katheter). In two cases the aorta was resected and replaced with a Dacron-graft. Reimplantation of both renal arteries into the graft was necessary in one case. Further interventions were: nephrectomy (2x), resection and ligation of the inferior vena cava (1x), resection and replacement of the left renal vein (1x).     

Hemoperitoneum after spontaneous rupture of liver tumor: results of surgical treatment

Five cases of massive hemoperitoneum caused by spontaneous rupture of liver tumors, collected during a 27-year period, are reported. Four patients had a primary liver malignancy and one patient a liver cyst with hemangioma. Initial symptoms were obscure and hemoperitoneum was suspected pre-operatively in only one patient. At operation, a mean of 3100 ml of blood was found in the abdomen. Hemostatis was achieved by liver resection in four patients and by suture ligation in one. Two patients died during or shortly after operation. The three patients surviving the operation had primary liver cancer and lived for 6 months to 6.5 years. It is concluded that liver resection, whenever possible, is the treatment of choice and that pre-operative delay and mortality may be diminished by increased awareness of this condition.     

Causes of recurrence of patients with chromaffin tumors

From 1957 to 2001, 520 patients with pheochromocytomas were operated. Two hundred and sixty patients were followed up from 4 to 21 years (8.4 +/- 1.9 years on the average). Complaints of 83 (31.9%) patients were similar to those before removal of the primary tumor. There was no evidence of tumors in 34 patients. Chromaffin tumors were revealed in 49 (18.9%) patients with clinical symptoms of hypercatecholaminemia. In 27 (55.1%) cases contralateral tumors were detected, in 11 of 27--the disease was familial. Extraadrenal chromaffin tumors occurred in 6 (12.2%) patients. In 8 of 49 (16.3%) patients recurrent clinical symptoms resulted from metastasis, malignant tumor after the first operation was diagnosed morphologically only in 6 of them. True recurrence of tumor at the in site of primary operation was recorded in 8 (16.3%) patients, including in 2 patients with MEN-2 in multicentric but benign tumor. Thus, true recurrence was diagnosed in 6.15% of 260 patients. True recurrence is most probable in multicentric tumor, when the tumor involves adjacent organs and tissues, and in vascular emboli in great vessels.     

"Nonfunctioning" islet cell carcinoma of the pancreas

Although most pancreatic islet cell tumors are associated with clinically evident hormone hypersecretion, a small group have no obvious signs or symptoms of excess endocrine activity and are termed "nonfunctioning." The clinical course of eight patients with "nonfunctioning" islet cell carcinoma seen during an eight-year period was reviewed. The six men and two women ranged in age from 36 to 68 years (mean--52). The initial complaint in six was a palpable abdominal mass associated with pain, steatorrhea, or jaundice. Two patients presented with abdominal pain that was initially thought to be of biliary tract origin, and the tumor was discovered at operation. Two patients underwent radical distal pancreatectomy and have no gross evidence of residual or recurrent tumor one and two years later. Five had a biopsy and biliary diversion; three of these also had a gastrojejunostomy. Five were given postoperative 5-fluorouracil and streptozotocin chemotherapy. One developed renal dysfunction and was switched to dimethyltriazenoimidazole carboxamide (DTIC) chemotherapy. Three patients are alive four, six, and eight years, respectively, after diagnosis. Two expired two and a half and three years after diagnosis. One patient had only biopsy of peripancreatic nodes, and he expired in one year. "Nonfunctioning" islet cell carcinoma presents with symptoms related to the mass effects of the tumor. An aggressive therapeutic approach utilizing surgery and chemotherapy is advocated for these slow growing neoplasms.     

DTIC therapy in patients with malignant intra-abdominal neuroendocrine tumors

Malignant intra-abdominal neuroendocrine tumors are rare; consequently, a standard chemotherapeutic protocol for patients with unresectable disease has not been established. This prompted a review of our experience with dimethyltriazeno imidazole carboxamide (dacarbazine) (DTIC) treatment for these tumors. From 1976 to 1986, 14 patients were treated with DTIC for metastatic neuroendocrine tumors. There were seven men and seven women whose ages ranged from 19 to 76 years. Diagnoses included eight nonfunctioning islet-cell carcinomas, three retroperitoneal neuroendocrine tumors, two glucagonomas, and one ileal carcinoid. Before DTIC chemotherapy, four patients were treated with streptozotocin and 5-FU, and one was treated with cytoxan and methotrexate without response. Two patients who were initially treated with DTIC with no response were subsequently treated with streptozotocin and 5-FU without benefit. Standard treatment with DTIC consisted of monthly cycles of 250 mg/m2/day administered intravenously for 5 days. Seven patients had an objective response to DTIC with both improvement in quality of life and a decrease of more than 50% in tumor size on computerized tomography (CT) or liver scanning. Response duration ranged from 1 to 10 years. One patient with a glucagonoma was treated for two years and had no evidence of disease at laparotomy 7 years later. Four patients with nonfunctioning islet cell carcinoma had a positive response to DTIC, but three of these patients had tumor recurrence 3 to 6 years after treatment. Two patients with retroperitoneal neuroendocrine tumors had a positive response to DTIC treatment. One patient with a glucagonoma and one with a nonfunctioning islet-cell tumor had equivocal responses with transient clinical improvement but no objective changes in tumor size. Five patients did not respond; two were given DTIC therapy as a last resort and died 1 and 12 days later. Of the other three patients, two died 6 months and one 2 years after treatment. DTIC chemotherapy was effective in 50% of patients with intra-abdominal neuroendocrine tumors. Although DTIC therapy was associated with nausea, no major gastrointestinal, hematologic, or renal complications were noted. This favorable experience with DTIC chemotherapy for nonresectable intra-abdominal neuroendocrine tumors indicates that further clinical evaluation and use are warranted.     

Surgical treatment in familial visceral myopathy.

In a kindred with a familial visceral myopathy, seven patients had operations seeking relief of chronic abdominal pain and other symptoms of intestinal obstruction; one patient had an 80% cystectomy and a Y-V-plasty of the bladder neck for urinary retention. Five patients with megaduodenum had bypass operations; a side-to-side duodenojejunostomy was done in four and a retrocolic gastrojejunostomy in one. Two of these died of postoperative complications, and one developed symptomatic adhesions. Two other patients who had duodenojejunostomy have done well for 6 years and 1 1/2 years respectively. One patient with dilation of the distal jejunum and proximal ileum had relief of intestinal obstructive symptoms from jejunostomy to decompress the destal jejunum. One patient who had a resection of the descending and sigmoid colon for sigmoid volvulus has done well for four years. Three of these seven patients developed peritonitis postoperatively, and two had symptomatic adhesions after operations. Duodenal aspiration from a patient who developed postoperative peritonitis grew E. coli, 10(13) colonies per ml. After review of the results of operations in other families and in our kindred, we favor side-to-side duodenojejunostomy in megaduodenum. Duodenal aspirate must be cultured before operation. Evidence of bacterial overgrowth in the aspirate should prompt appropriate antibiotic treatment to reduce the likelihood of sepsis.     

“Tension-free” herniorrhaphy for groin hernias in patients with cirrhosis: Report of four cases

Tension-free herniorraphies were carried out using either the Mesh Plug repair (M-P) or Perfix plug technique (P-P) in four patients with cirrhosis. Three patients had a lateral inguinal hernia, and the other had bilateral inguinal and femoral hernias. The patients’ ages ranged from 55 to 80 years. The Child-Pugh classifications showed that one was A, two were B, and one was C. The main complaint in two patients was a difficult reduction and the others had discomfort. An M-P was performed in the three patients with inguinal hernias and an M-P was performed for the femoral hernias and a P-P for the inguinal hernias in the patient with both inguinal and femoral hernias. Two patients had fluid collection under the incision and one of them required a single puncture. However, the others had no related complications after the operation. One patient died due to liver failure without recurrence of the groin hernias 31 months after the operation. The others had no recurrence and no related symptoms from 5 to 52 months after the operation.     

Experience with extra-adrenal pheochromocytoma

From 1955 to 1985, 20 patients presented with a total of 22 extra-adrenal pheochromocytomas (2 had multiple tumors and 2 had a malignant extra-adrenal pheochromocytoma). There were 13 male and 7 female patients, and the highest incidence was in the second decade. Although most patients presented with symptoms typical of pheochromocytoma, several presented with unusual features related to the anatomical location, such as mediastinal mass (chest tumor), upper airway obstruction (neck tumor) or gross hematuria (bladder tumor). In 5 of 6 patients in whom plasma catecholamine levels were fractionated epinephrine levels were elevated. The most common tumor location was the superior para-aortic region (13 patients). In 16 patients the location of tumors was established before treatment. Computerized tomography (9 patients) was the most accurate imaging study for tumor localization. A total of 19 patients underwent complete excision of all pheochromocytomas. Postoperative followup information (median interval 120 months) was available for 15 of these patients. Three patients had recurrent pheochromocytoma that was treated successfully. One patient had essential hypertension. No patient had metastatic disease. The low incidence of malignancy suggests a benign course for extra-adrenal pheochromocytoma and represents a departure from the previously reported higher incidence of malignancy with extra-adrenal pheochromocytoma.     

Pancreatic VIPomas: Subject Review and One Institutional Experience

VIPomas are rare pancreatic endocrine tumors associated with a well-defined clinical syndrome characterized by watery diarrhea, hypokalemia, and metabolic acidosis. The objective of this study was to review a single institution’s experience with VIPomas, as well as to review the English literature. A retrospective review of the Johns Hopkins pancreatic database revealed four cases of VIPoma, with three patients being male. All patients presented with watery diarrhea, hypokalemia, hypercalcemia, and acidosis. All patients had no family history of multiple endocrine neoplasia. Computed tomography revealed the primary pancreatic tumor in all patients, with three tumors located in the tail of the pancreas. One tumor involved the entire pancreas. Computed tomography and/or octreotide radionuclide scans identified hepatic metastasis in three patients. Mean serum vasoactive intestinal polypeptide levels were 683 pg/ml (range 293 to 1,500 pg/ml). All patients underwent resection of the pancreatic primary tumor. Two patients also had simultaneous liver resections. All patients had evidence of malignancy as defined by the presence of metastatic lymph nodes and/or hepatic metastases. Two patients had complete resolution of symptoms after surgical resection. One patient required radioablation of liver metastases and adjuvant octreotide therapy for control of symptoms. One patient died of progressive metastatic disease 96 months after surgery, whereas the other three remain alive. Extended, meaningful survival can be achieved for VIPoma patients, combining an aggressive surgical approach with additional strategies for treatment of unresected disease.