Nelson's syndrome after bilateral adrenalectomy for Cushing's disease

BACKGROUND: Presentation and outcome of Nelson's syndrome after bilateral adrenalectomy is variable. METHODS: Clinical records of 39 patients who underwent bilateral adrenalectomy for primary or recurrent Cushing's disease during a 15-year period were analyzed for frequency and evolution of Nelson's syndrome. RESULTS: The study included 32 females and 7 males with a mean age of 31 years; 20 patients had a hypophysectomy as the initial procedure, and 19 had an adrenalectomy. Of the group, 17 patients received prophylactic radiation therapy to the pituitary gland. A total of 11 patients, none of whom had received prophylactic radiation therapy, developed Nelson's syndrome (determined by skin hyperpigmentation, elevated serum ACTH levels, and enlargement of a previous pituitary tumor or development of a new tumor in patients with no previous pituitary abnormality) over a mean follow-up period of 53 months. Treatment for Nelson's syndrome included valproic acid, radiation therapy, and hypophysectomy as monotherapy or combined therapy. Of the remaining 28 patients, 10 (7 without prophylactic radio therapy) developed skin hyperpigmentation and increased ACTH levels without a tumor. CONCLUSIONS: Nelson's syndrome is a frequent complication after bilateral adrenalectomy in the absence of prophylactic radiotherapy (28%). The syndrome can be successfully controlled by medical treatment and or radiotherapy; patients rarely require hypophysectomy.     

Prophylaktische Chirurgie des Pankreas

The goal of prophylactic surgery is to prevent malignant growth in patients with hereditary tumor predisposition. The pancreas presents as particularly challenging, due to the difficulty of operation and comparatively high risk of morbidity and even mortality. In addition, partial operative procedures and, more significantly, total resection lead to exocrine pancreas insufficiency and secondary diabetes, with grave consequences for the patient. Hereditary tumor predisposition syndromes that can result in pancreaticoduodenal endocrine tumors (PET) include multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau syndrome. As penetrance is maximally 70-80% and the 10-year survival rate over 80%, prophylactic pancreatic resection without evidence of a tumor is not indicated. However, prophylactic extension of a resection would be advised, should a PET be diagnosed. Patients predisposed to developing ductal pancreatic carcinoma (PC) are at risk of familial pancreatic cancer syndrome (FPC), hereditary pancreatitis, and other hereditary tumor predisposition syndromes such as Peutz-Jeghers syndrome and familial atypical multiple mole-melanoma syndrome. As the gene defect responsible for FPC has yet to be identified and the penetrance of PC in the other tumor predisposition syndromes is low or unknown, a prophylactic pancreatectomy based on today's knowledge is not indicated. Prophylactic extension of the resection is advisable should PC or high-grade PanIN lesions be diagnosed, as these patients often present with multifocal dysplasia and even carcinoma.     

Treatment of the Zollinger-Ellison syndrome. A 25 year assessment

An assessment of treatment methods for the Zollinger-Ellison syndrome over 25 years suggests that the dual surgical approach of excision of resectable tumor and total gastrectomy provides the greatest possibility of attaining tumor and serum gastrin control. Normal serum gastrin levels and reduction of tumor status, not observed with nonoperative management, were attained by surgical treatment, particularly when the tumor (or hyperplasia) was limited to the pancreas, stomach, duodenum and regional lymph nodes. Eleven of 16 such operations resulted in tumor control, and 10 of these patients developed normal serum gastrin concentrations. In elective clinical situations combined medical and surgical therapy is a rational approach allowing confirmation of diagnosis and safe, definitive surgical control of the syndrome before metastases occur.     

Acute renal failure from xanthine nephropathy during management of acute leukemia

Tumor lysis syndrome is a potentially life-threatening complication of induction chemotherapy for treatment of lymphoproliferative malignancies. Serious complications of tumor lysis syndrome are rare with the preemptive use of allopurinol, rasburicase, and urine alkalinization. We report a case of oliguric acute renal failure due to bilateral xanthine nephropathy in an 11-year-old girl as a complication of tumor lysis syndrome during the treatment of T-cell acute lymphoblastic leukemia. Xanthine nephrolithiasis results from the inhibition of uric acid synthesis via allopurinol which increases plasma and urinary xanthine and hypoxanthine levels. Reports of xanthine nephrolithiasis as a cause of tumor lysis syndrome are rare in the absence of defects in the hypoxanthine-guanine phosphoribosyl transferase (HGPRT) enzyme. Xanthine nephropathy should be considered in patients who develop acute renal failure following aggressive chemotherapy with appropriate tumor lysis syndrome prophylaxis. Urine measurements for xanthine could aid in the diagnosis of patients with nephrolithiasis complicating tumor lysis syndrome. Allopurinal dosage should be reduced or discontinued if xanthine nephropathy is suspected.     

Locked-in syndrome due to metastatic pontomedullary tumor--case report

A 21-year-old man presented with an extremely rare case of locked-in syndrome caused by a metastatic brainstem tumor manifesting as quadriplegia, lower cranial nerve pareses, and irregular respiration. Cranial magnetic resonance imaging revealed a large pontomedullary tumor. An emergency operation was performed via a posterior fossa approach and the tumor was grossly totally removed. The histological diagnosis was malignant melanoma. The clinical status of the patient remained unchanged and he died on postoperative day 34 of diffuse bronchopneumonia. Locked-in syndrome is characterized by quadriplegia, lower cranial nerve paralysis, and mutism but with maintenance of consciousness, as well as vertical eye movements and eyelid blinking. This case suggests that locked-in syndrome should be considered in any patient seemingly comatose or stuporous, this syndrome may be due to a pontomedullary tumor, and malignant melanoma metastasis should be considered in the differential diagnosis of patients who present with brainstem tumor.     

Association between neuroepithelial tumor and multiple intestinal polyposis (Turcot's syndrome): report of a case and critical analysis of the literature.

We report a case of association of a brain tumor with multiple intestinal polyposis (Turcot's syndrome) and offer a critical analysis of the relevant literature with a view to revising the classification of the syndrome in relation to familial multiple polyposis and Gardner's syndrome. For this purpose, we considered only cases of intestinal polyposis associated with a primary neuroepithelial tumor (medulloblastoma, glioma, or glioblastoma) as originally described by Turcot. Differences emerged, depending on the central nervous system tumor type, which suggests that this neoplastic association may be classified as two distinct syndromes.     

Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome

PURPOSE: Testicular tumor of the adrenogenital syndrome is a rare clinical entity found in young men with endocrine disorders. Histologically it resembles Leydig cell tumor. We 1) reviewed the clinical features of testicular tumor of the adrenogenital syndrome and 2) determined if special histopathological features of the tumor and synaptophysin reactivity could distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor. MATERIALS AND METHODS: We reviewed the medical and pathological records for all patients with testicular tumor of the adrenogenital syndrome seen at our institution from 1978 to 2004. These tumors were examined by histological and immunophenotypic methods for comparison to Leydig cell tumor. RESULTS: A total of 14 males with an endocrine disorder had pathological evidence of testicular tumor of the adrenogenital syndrome. These tumors were often bilateral (93% or 13 of 14 cases), associated with pain (92% or 12 of 13) and refractory to medical management with high dose exogenous steroids (93% or 13 of 14). Testicular tumor of the adrenogenital syndrome was managed by tumor enucleation in 7 patients (54%) and by radical orchiectomy in 6 (46%). All patients had resolution of pain at 3-month followup. Upon histological review features found to be more common to testicular tumor of the adrenogenital syndrome compared with Leydig cell tumor were nuclear pleiomorphism, low mitotic activity, extensive fibrosis, lymphoid aggregates, adipose metaplasia and prominent lipochrome pigment. Synaptophysin (ICN, Costa Mesa, California) reactivity was strong in testicular tumor of the adrenogenital syndrome but rarely observed in Leydig cell tumor. CONCLUSIONS: In our series medical treatment failed in patients with testicular tumor of the adrenogenital syndrome and conservative surgical therapy was possible in select individuals. We identified special histopathological and immunophenotypic features, including synaptophysin staining, which distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor.     

Nephropathy associated with Wilms' tumor. A case of a 13-year-old girl

The association of nephropathy with Wilms' tumor and male pseudohermaphroditism is known as Drash's syndrome. The absence of either pseudohermaphroditism or Wilms' tumor has also been reported. The nephropathy, characterized by proteinuria or nephrotic syndrome, leads rapidly and inevitably to renal failure. The renal findings are those of a chronic glomerulopathy with mesangial hypercellularity, glomerular sclerosis, interstitial infiltration and marked tubular dilation. This infrequent syndrome usually appears in early life, between 1 and 3 years of age. The case of nephropathy associated with Wilms' tumor we present here is characterized by age and onset: an abrupt onset of renal insufficiency without previous signs of nephropathy in a 13-year-old girl.     

椎管内肿瘤疼痛特点分析

目的：通过对椎管内肿瘤疼痛特点的分析,建议将椎管内肿瘤患者出现的腰穿痛、造影痛、牵引痛、卧眠痛称为椎管内肿瘤楔压症。方法：对２４例椎管内肿瘤患者的临床症状进行了分析。结果：腰穿痛占３５３％,造影痛占７０６％,卧眠痛占７５％,牵引痛占１００％。结论：当肿瘤上、下方脑脊液的压力差增大时或椎管长度有变化时,肿瘤会发生移位,犹如木楔卡压一样,加重对神经组织的压迫,疼痛加重甚至出现瘫痪     

Recurrence of desmoid tumor in a multivisceral transplant patient with Gardner's syndrome

BACKGROUND: Desmoid tumors are locally invasive fibromatous tumors, which, in patients with Gardner's syndrome, usually occur in the abdominal wall or intra-abdominally. After excision, they tend to recur, often leading to multiple bowel resections. METHODS: This is a report of the clinical course of a patient with Gardner's syndrome and desmoid tumor who had multiple enterectomies and gradually developed short-gut syndrome. He required prolonged parenteral nutrition, which damaged the liver. The patient underwent a multivisceral transplantation as a life-saving procedure. RESULTS: After the transplant, the desmoid tumor recurred in the thoracic wall twice and was successfully resected. It also recurred in the abdominal cavity, compressing the intestinal loops; the tumor was excised uneventfully, leaving the graft intact. The recurrent tumors were all of recipient origin. CONCLUSIONS: Intestinal and multivisceral transplantation could be considered in patients with short-gut syndrome caused by recurrent desmoid tumor. In the case of posttransplant tumor recurrence, resection is the only option recommended.     

Islet cell tumors of the pancreas and the alimentary tract.

Functioning tumors of the pancreatic islets are now recognized as the source of clinical syndromes affecting the gastrointestinal tract which have a wide variety of catastrophic symptoms. Experiences with thirty-six cases suggest at least four separate diagnostic categories in the ulcerogenic tumor syndrome. These include: a typical history, gastric analysis, and roentgenographic findings with boderline fasting serum gastrin levels; ulcerogenic tumor with evidence of hyperparathyroidism; iatrogenic ulcerogenic syndrome associated with failure of a previous operation for duodenal ulcer; and the classic ulcerogenic syndrome associated with a fulminating ulcer diathesis or diarrhea and high serum gastrin levels. The problems presented at operation include: decisions to be make in the presence of a negative exploration; the finding of a solitary tumor in the wall of the duodenum; solitary pancreatic tumors particularly in the body and tail; ulcerogenic tumors in the very young; liver metastases in the elderly; and the wisdom of removing gross metastases in combination with total gastrectomy. The long-term survival in the ulcerogenic tumor syndrome approximated 50 per cent, with 40 per cent of those having proved malignancy living five years. Evidence of hyperparathyroidism is relatively common in association with both the ulcerogenic and the diarrheogenic tumor syndromes. The association may by a result of a congenital abnormality, metabolic alkalosis, or a direct effect of the islet cell tumor. Parathyroidectomy may be indicated when both the serum calcium and parathormone levels are elevated in the presence of borderline fasting gastrin levels. The latter may return to normal after parathyroidectomy. The evidence of hyperparathyroidism closely parallels the episodes of diarrhea in the diarrheogenic syndrome, and hyperparathyroidism may regress spontaneously after total removal of the pancreatic tumor. Just as routine calcium determinations made the diagnosis of hyperparathyroidism more commonplace, it is suggested that the gastrointestinal syndromes associated with islet cell tumor would receive wider recognition if radioimmunoassays for gastrin as well as secretin, and the other secretin-like polypeptides, were carried out routinely.     

Limitations and hazards of palliative renal tumor embolization

Today the indication for palliative embolization of inoperable renal carcinoma is more restricted than several years ago. Reviewing 31 own palliative occlusions of the renal artery in 29 patients over a period of 5 1/2 years two main reasons for this attitude are presented: 1. Because of collateral or parasitic vascular supply of kidney tumors the occlusion of the renal arteries only results in a retarded tumor growth rate and does not seem to prolong patient survival. 2. the "postembolization syndrome" after tumor occlusion has a relatively high complication rate and lethality (20% serious side effects, 3% deaths directly related to embolization). Therefore embolization of inoperable renal carcinomas is justified only in patients whose remaining lifetime can be alleviated by this measure. Certain indications are: massive hematuria, severe local pain due to the tumor and life endangering endocrine tumor activity, e.g. hypercalcemia. Uncertain indications such as recurring but not perilous hematuria causing progressive anemia and refusal of tumor surgery should be carefully balanced against the hazards of embolization.     

Surgical treatment of hormone-induced recurrent and peptic ulcers of anastomosis

The authors operated on 20 patients with the Zollinger-Ellison syndrome and on 3 patients with Wermer's syndrome. The clinical picture of the disease and the findings of laboratory tests and special methods of examination allow the Zollinger-Ellison syndrome to be diagnosed in most cases and a niveau diagnosis of gastrinoma to be established in some cases. Intraoperative ultrasonic examination is significant in making the niveau diagnosis. The authors claim that the operation of choice in the Zollinger-Ellison syndrome is total gastrectomy (extirpation of the gastric stump) with esophagojejunoduodenoplasty as well as one-stage removal of the revealed tumor by resection of the pancreas or enucleation of the tumor. The application of the group of H2-receptor blocking agents cannot be a substitute for operative treatment, but is expedient as a measure of preoperative management.     

Gastrin-producing ovarian mucinous cystadenomas: a cause of the Zollinger-Ellison syndrome

An ovarian gastrinoma would appear to be an extremely rare cause of the Zollinger-Ellison syndrome. Nevertheless, two consecutive cases of Zollinger-Ellison syndrome seen at our institution each proved to be caused by a gastrin-producing ovarian mucinous cystadenoma. That the ovarian tumor was the source of gastrin production was established by preoperative and postoperative gastrin and gastric secretory studies and by specific immunocytochemistry of the excised tissue. After oophorectomy, each patient was apparently cured of the disease. Ovarian gastrinomas may not be as uncommon as is generally believed and should be considered as a possible cause of the Zollinger-Ellison syndrome in any female patient in whom a primary gastrointestinal or pancreatic tumor cannot be located.     

Testicular thecoma in an 11-year-old boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome)

We report a case of testicular thecoma in an 11-year-old Japanese boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome). He presented with left testicular swelling and underwent a radical orchiectomy on suspicion of a malignant paratesticular tumor. The tumor arose from the testis exophytically and was diagnosed as a thecoma histopathologically. Ovarian thecoma-fibroma group tumors are closely associated with Gorlin syndrome or with abnormalities in PTCH, a candidate gene for the syndrome. The occurrence of an extremely rare testicular thecoma in this case (the second in the literature) suggests that such an etiological association may also exist in the pathogenesis of testicular tumors.     

Somatostatin analogue (SMS 201-995) in patients with gastrinomas

We have examined the effects of the somatostatin analogue (SMS 201-995) in 10 patients with gastrinoma syndrome. Four had hepatic metastases, one had a tumor in a peripancreatic lymph node, two had resectable intrahepatic and intraduodenal gastrinomas, and in three the primary tumor was not found. Acutely, SMS 201-995 decreased acid secretion and restored the BAO/MAO ratio to normal in eight of eight patients. Basal and secretin-stimulated gastrin responses were suppressed but not normalized in eight of eight patients. Suppression of endogenous gastrin restored responsiveness to exogenous gastrin. Treatment for up to 12 months with SMS 201-995 controlled symptoms in six of eight patients, suppressed serum gastrin in three of five, and suppressed acid secretion in three of three patients. Treatment with SMS 201-995 in three patients for 5 months decreased tumor secretion of gastrin and diminished basal acid secretion, an effect that persisted in two of three patients 48 hours after withdrawal of SMS. In patients with metastatic disease who had high levels of gastrin, SMS treatment for 5 to 12 months did not inhibit tumor growth or decrease gastrin levels. SMS treatment arrested progression of tumor growth only in patients who had a reduction in gastrin and gastric acid secretion. We conclude that SMS may be useful in the management of gastrinoma patients by decreasing hypersecretion of gastrin and gastric acid and, over a longer term, may even change tumor capacity to release gastrin and gastric acid secretion. SMS may thus be useful as a palliative agent and as an adjunct to conventional treatment of the gastrinoma syndrome. SMS does not appear to shrink tumor mass in patients with very high basal gastrin levels.     

A case of Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement

Foster Kennedy syndrome is a very rare syndrome which includes ipsilateral optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis. A large frontal lobe, olfactory groove, or medial third sphenoidal wing tumor, usually a meningioma, creates this syndrome. In this report, the author presents a case of metastastic cerebral tumor with Foster Kennedy syndrome but without frontal lobe or anterior cranial fossa involvement.     

Pleural mesothelioma and membranous nephropathy

Underlying malignancy has been thought to be responsible for 5-10% of the cases of membranous nephropathy in adults, with the risk being highest in patients over the age of 60 years. Solid tumors such as carcinomas of lung or colon, are most often involved. It is presumed that tumor antigens are deposited in the glomeruli; this is followed by antibody deposition and complement activation, leading to epithelial cell and basement membrane injury and proteinuria due to the associated increase in glomerular permeability. We describe a patient with a resistant nephrotic syndrome and massive proteinuria due to membranous nephropathy associated with pleural mesothelioma.     

Parathyroid cancer

The study of one case of parathyroid cancer and a literary review permitted to state the following characteristics: it is a rare but not exceptional tumor. The endocrine syndrome represented by a hypercalcemia is frequently severe present. The tumor syndrome is often associated with locoregional invasion or metastasis. Clinical signs of malignity are as important as the anatomopathologic data. The surgical treatment is essential after a mecal hypocalcemic preparation. Thus, the prognosis may be good if the carcinologic rules are respected.     

Zollinger-Ellison syndrome: special considerations.

Twelve patients with Zollinger-Ellison syndrome and one patient with WDHA syndrome are reviewed. Three of the Z-E patients exhibited MEA, two having hyperinsulinism and one hyperparathyroidism. Ages ranged from nine to 71 years. Diagnosis of Z-E syndrome was established from history, gastric acid secretion, radiologic studies, serum gastrin measurements and from actual tissue biopsy in 10 of the 12 patients. Total gastrectomy was performed in 8 of the 12 Z-E patients, with abolition of the ulcer diathesis in all. However, in none of our patients was there objective evidence of subsequent tumor regression. Three patients remain alive. Four died of tumor, one from post-total gastrectomy complications, one from post-subtotal gastrectomy in another hospital, two from ulcer hemorrhage, and one from electrolyte imbalance with autopsy diagnosis of Z-E tumor. A patient is recorded in detail who exhibited both hyperinsulinemia and hypergastrinemia from a malignant islet cell tumor, had the tumor "debulked" four times over a 14 year period and whose hepatic metastases were temporarily abolished by streptozotocin infusion. The question is raised regarding relationships between chronic organic hyperinsulinism and subsequent hypergastrinemia.