Phaeochromocytoma.
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3-Iodobenzylguanidine↗
Biomedical subjects
N W Thompson
Publications and source records attributed to N W Thompson.
The value to the surgeon of parathyroid hormone assays in primary hyperparathyroidism.
The role of various parathyroid hormone (PTH) radio-immunoassays in the diagnosis of primary hyperparathyroidism (PHP) is controversial. A series of 204 patients with surgically proven PHP was studied. Serum total calcium, serum ionized calcium, amino (N)-terminal PTH and carboxyl(C)-terminal PTH were assessed in relation to the volume and weight of adenomatous or hyperplastic parathyroid tissue excised at operation. N-terminal PTH was elevated above the normal laboratory range in only 24% of patients and correlated relatively poorly with the volume of abnormal parathyroid tissue (r = 0.20, P = 0.05). C-terminal PTH was elevated above the normal range in 91% of patients and had a strong correlation with the volume of abnormal parathyroid tissue (r = 0.63, P less than 0.001). The correlation coefficients between C-terminal PTH and serum total calcium and serum ionized calcium were both 0.63 (P less than 0.001). In contrast, there was no correlation between N-terminal PTH and serum total calcium (r = -0.02), serum ionized calcium (r = -0.04) or C-terminal PTH (r = 0.09). A combination of hypercalcaemia and elevated C-terminal PTH can be regarded as strong diagnostic evidence of PHP. Furthermore, the level of C-terminal PTH can assist the surgeon by approximately predicting the amount of adenomatous or hyperplastic parathyroid tissue that may be expected at surgical exploration.
Adolescent↗
Sarcoidosis of the thyroid presenting as a painful nodule.
A patient, known to have sarcoidosis, developed a painful, enlarging right thyroid mass. Thyroid lobectomy was performed revealing sarcoidosis involving the thyroid. A review of the literature shows sporadic reports of involvement of the thyroid with sarcoidosis. There were no previous reports of this entity presenting as painful thyroid enlargement. Thyroid lobectomy was curative.
Adult↗
Antrectomy for multicentric, argyrophil gastric carcinoids: a preliminary report.
Multicentric gastric carcinoids develop infrequently in association with atrophic gastritis, achlorhydria, and hypergastrinemia. These unusual tumors, thought to arise from proliferation of enterochromaffin-like (ECL) cells, have not been shown to secrete any measurable biogenic amines and usually grow slowly. Hypergastrinemia, which results from antral G cell stimulation secondary to atrophic gastritis, is believed to be the trophic stimulus, but alternative explanations include production of gastrin-releasing factor (GRF) or gastrin per se by the tumor. We recently encountered two patients with pentagastrin-resistant achlorhydria and multiple gastric carcinoids. Neither had symptoms of carcinoid syndrome. Urinary 5-hydroxyindoleacetic acid and serum human pancreatic polypeptide, vasoactive intestinal peptide, and motilin values were normal. Fasting gastrin values were nearly 1800 pg/ml. Antrectomy and regional lymphadenectomy was performed in each patient. The tumors were locally invasive with penetration through the submucosa. One patient had regional lymph node involvement, and one had an isolated hepatic metastasis. Immunohistochemical stain tests were positive in both patients for neuron-specific enolase and chromogranin, with focal positive staining for gastrin and serotonin. Serum gastrin levels decreased to less than 25 pg/ml after antrectomy. Evaluation with upper gastrointestinal endoscopy and biopsy examination 4 to 6 months after antrectomy showed complete regression of disease in one patient and residual neoplasm in one patient, despite normal serum gastrin levels. Additional studies with careful long-term follow-up will be needed to determine whether antrectomy eliminates the hypergastrinemia associated with enterochromaffin-like hyperplasia and leads to regression of disease.
Adult↗
Therapeutic dilemmas in patients with symptomatic polycystic liver disease.
Most reports of the operative treatment of symptomatic polycystic liver disease (PCLD) are anecdotal or consist of only a small subset of patients in an institution's overall experience treating hepatic cysts. We have reviewed our experience with nine consecutive patients with symptomatic PCLD undergoing operative treatment from 1981 to 1987. Indications for operation include chronic abdominal pain (4 patients), cyst infection (2 patients), biliary obstruction (2 patients), inferior vena cava obstruction (2 patients), and symptomatic abdominal distention (2 patients). The average duration of symptoms leading to operation was 7.8 months. Three types of cystic disease were identified based on gross morphology: dominant cystic disease (3 patients), diffuse cystic disease (4 patients), and mixed cystic disease (2 patients). Operations to treat symptomatic PCLD included unroofing and external drainage of infected cysts (2 operations), simple unroofing (1 operation), cyst fenestration alone (4 operations) and fenestration combined with resection (3 operations). Treatment directed at principally dominant cysts (five patients) was associated with resolution of symptoms and low morbidity and mortality. Treatment directed at diffusely cystic disease (four patients) resulted in significant morbidity and mortality including three deaths. Successful surgical treatment of symptomatic patients with PCLD depends on accurate preoperative identification of patients with symptoms related to one or more dominant cysts. In this setting fenestration or simple unroofing of the dominant cyst is safe and effective treatment. By comparison, extensive fenestration with or without hepatic resection in patients with symptoms attributed to a diffusely cystic liver may be associated with unacceptable morbidity and mortality.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Hurthle cell thyroid tumors. An immunohistochemical study.
Twenty-three Hurthle cell neoplasms of the thyroid were analyzed immunohistochemically for thyroglobulin, carcinoembryonic antigen (CEA), and for immunoreactivity with a monoclonal antibody to p21 ras oncogene product. Both benign and malignant tumors defined by the presence or absence of invasion and metastasis stained positively for thyroglobulin. Most tumors expressed both CEA and p21 ras oncogene product. Clinicopathologic analysis showed that malignant tumors were larger, occurred in slightly older patients (mean age, 52.8 years), and had an almost equal sex distribution, whereas benign tumors were more common in women and occurred in younger patients (mean age, 44.7 years). These results indicate that both benign and malignant Hurthle cell thyroid tumors produce thyroglobulin and express both CEA and p21 ras oncogene product, whereas adjacent normal thyroid tissues showed weak to absent immunoreactivity for p21 ras oncogene product. Because there are no specific immunohistochemical markers to distinguish between benign and malignant Hurthle cell tumors, one needs to rely on traditional histologic features such as invasion and metastasis to distinguish between benign and malignant Hurthle cell neoplasms.
Adult↗
Diagnosis and treatment of functioning and nonfunctioning adrenocortical neoplasms including incidentalomas.
The most important functional tumors of the adrenal cortex are those that secrete cortisol or aldosterone in excess. Biochemical testing when appropriately utilized can diagnose and differentiate the cause of Cushing's syndrome, and when an adrenal adenoma is found, surgical excision is curative. The diagnosis and surgical treatment of primary aldosteronism are straightforward today, and localization of the usual small cortical tumor producing the syndrome can be achieved by CT and NP-59 scanning or selective venous assays. Adrenocortical carcinomas are relatively rare, are usually incurable when diagnosed, and are an important consideration in the incidentally discovered adrenal mass found by CT scanning.
Adenoma↗
Iodine-131 MIBG scintigraphy of neuroendocrine tumors other than pheochromocytoma and neuroblastoma.
Metaiodobenzylguanidine (MIBG) locates most pheochromocytomas and neuroblastomas. The tracer is concentrated in intracellular storage vesicles by an active process. Many other neuroendocrine tumors of the amine precursor uptake and decarboxylation (APUD) series have hormonal storage vesicles and, thus, the potential to take up [131I]MIBG. A variety of neuroendocrine tumors in 57 patients were studied 1, 2, and 3 days after 0.5 mCi [131I]MIBG. Views from skull to pelvis were obtained. Results of MIBG scans were compared with all available imaging modalities (including plain radiography, liver scan, ultrasound, computed tomography, and angiography) and surgical exploration. The neuroendocrine nature of the tumor was determined by histology, immunohistochemistry, electron microscopy, and the assay of appropriate biogenic amines and peptide hormones. Results were (positive/total cases): carcinoids (four of ten), nonsecreting paragangliomas (three of three), sporadic medullary carcinomas of the thyroid (MCT) (one of five), familial MCT (one of 26), chemodectomas (two of five), oat cell carcinomas (zero of four), choriocarcinoma (one of one), atypical schwannoma (with storage granules) (one of one), Merkel cell skin cancer (one of one), islet cell carcinoma (zero of one). We conclude that a wide range of neuroendocrine tumors show [131I]MIBG uptake; tumors other than pheochromocytomas and neuroblastomas are less often seen scintigraphically, but in certain cases (e.g., carcinoid and nonsecreting paragangliomas) scintigraphy may be useful in depicting the extent and location of disease and may indicate therapeutic potential. Iodine-131 MIBG shows promise in the diagnosis and staging of tumors of varied types.
3-Iodobenzylguanidine↗
Surgical treatment of primary hyperparathyroidism in elderly patients.
Surgical treatment of hyperparathyroidism is often withheld from elderly patients out of concern about operative morbidity and mortality. We reviewed the case records of 81 patients over the age of 65 who underwent neck exploration for primary hyperparathyroidism at the University of Michigan Medical Center during the past 10 years. The most common presenting symptoms were lethargy (62%), neurological complaints (44%), and constipation (42%). Less common were histories of peptic ulcer disease (30%) and renal lithiasis (25%). Delay from time of diagnosis to operation averaged 33 weeks. Seventy-seven percent had adenomas, of which 9% were multiple. Average postoperative hospital stay was 7.1 days, ranging from 3 to 22 days. There was no mortality. We believe that neck exploration for primary hyperparathyroidism can be safely performed in elderly patients and should not be delayed or withheld merely on the basis of advanced age.
Age Factors↗
Total thyroidectomy: complications and technique.
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Humans↗
Plasma gut hormone levels in 37 patients with pheochromocytomas.
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Adrenal Gland Neoplasms↗
Primary hyperaldosteronism caused by adrenocortical carcinoma.
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Adrenal Cortex Neoplasms↗
Fibrolamellar hepatocarcinoma: clinical, radiologic, and pathologic features.
Three new cases of an unusual subtype of hepatocellular carcinoma (HCC) referred to as fibrolamellar hepatocarcinoma (FLHC) recently seen at our institution are described. This report focuses on the clinical, radiologic, and pathologic features of this rare subset of HCC. All three patients were under 30 years of age with no previous history of hepatitis or cirrhosis. Each had had subacute symptoms for 5 months to 1 year before medical attention was sought and/or diagnosis of FLHC was established. There was no reliable correlation with oral contraceptive use in the 2 female patients. Serum alpha-1-fetoprotein levels were normal with only mild elevation of liver enzymes. The CT features, although not specific, were suggestive of an aggressive tumor with amorphous calcification in 2 of the 3 cases. Angiographically all tumors were hypervascular without any evidence of arterioportal shunting or venous invasion of major vessels. The clinical and radiologic recognition of these tumors is important since the surgical resectibility rate and 2- and 5-year survival rates are higher than those applicable to conventional HCC.
Adult↗
Helium-oxygen mixtures in airway obstruction due to thyroid carcinoma.
The management of a patient with severe airway obstruction secondary to a thyroid mass is reported. When breathing room air the patient appeared in severe respiratory distress but when inspiring 22 per cent oxygen in helium she reported almost instantaneous relief and there was a marked decrease in respiratory rate, and increase in tidal volume and arterial oxygen tension. This improvement was to be expected because in situations where turbulent flow predominates a decrease in the density of inspired gases will result in an increase in flow rates. Contrary to established dogma a marked improvement was sustained when the patient was breathing 50 per cent oxygen in helium. The concentration of oxygen in helium was adjusted to obtain subjective relief for the patient in conjunction with adequate oxygenation.
Aged↗
The scintigraphic localization of mineralocorticoid-producing adrenocortical carcinoma.
I-131-6 beta-iodomethylnorcholesterol (NP-59) was used to localize mineralocorticoid-secreting adrenocortical carcinomas in two patients and functioning metastases in a third patient studied after the removal of the primary tumor. The presence of sufficient NP-59 activity within these lesions for discernable imaging is unusual and would not have been expected based on previous experience with other functioning and nonfunctioning carcinomas of the adrenal cortex. These cases serve to illustrate the variable spectrum of iodocholesterol uptake into adrenocortical malignancies and suggest that scintigraphic studies, preoperatively for localization and postoperatively to confirm the presence of recurrence or metastases, might be useful to identify and characterize these rare neoplasms.
19-Iodocholesterol↗
Perioperative use of long-acting somatostatin analog (SMS 201-995) in patients with endocrine tumors of the gastroenteropancreatic axis.
The clinical manifestations of hormone excess caused by functioning neuroendocrine tumors of the gastroenteropancreatic (GEP) axis can be life threatening and frequently prove refractory to conventional antisecretory drugs. Administration of a long-acting somatostatin analog (SMS 201-995) proved effective in three patients with complex management problems related to GEP tumors. A patient with an insulinoma was maintained euglycemic intraoperatively with a single 100 micrograms dose of SMS given before surgery. Gastric suction in two patients with gastrinomas caused hypochlorhydric alkalosis that was preventable with preoperative SMS. Iatrogenic pancreatic fistula occurring after resection of a benign insulinoma healed within 4 days of SMS administration. This drug may be a useful adjunct in the perioperative management of patients with GEP endocrine tumors. Caution is advised regarding potential hazards related to malabsorption and gastric dysmotility.
Adenoma, Islet Cell↗
Nonfunctioning malignant neuroendocrine tumors of the pancreas.
The clinical features of eight women and three men with nonfunctioning islet cell carcinoma of the pancreas were reviewed. The mean patient age was 58 years (range 44 to 75 years). Weight loss and abdominal pain were the most frequent presenting symptoms. An abdominal mass was palpable in five patients. At operation regional or distant metastases were present in 82% of patients. Only 18% of patients underwent resection for potential cure. All tumors proved histologically to be neuroendocrine in origin. Immunohistochemical staining showed positive reactivity for neuron-specific enolase and chromogranin in all tumors studied but was negative for insulin, glucagon, and somatostatin. Focal positivity for pancreatic polypeptide was seen in one tumor. Nine patients with unresectable disease at operation were available for follow-up. Mean survival for the entire group was 23 +/- 7.2 months (range 4 to 72 months). Survival differences between women and men appeared to favor women but were not statistically significant. Postoperative regional or systemic chemotherapy also had no significant effect on patient survival although two of the longest survivors (36 and 72 months) had received adjunctive chemotherapy. Nonfunctioning islet cell neoplasms are locally aggressive, have a propensity for early metastases, and are rarely resectable for cure. Unlike pancreatic exocrine carcinomas, endocrine malignancies may respond favorably to adjunctive chemotherapy.
Adenoma, Islet Cell↗
Effect of hyperparathyroidism on the control of diabetes mellitus.
Hyperparathyroidism is associated with abnormalities in glucose tolerance and insulin secretion. To assess the effects of hyperparathyroidism on the control of diabetes mellitus, 56 patients with concomitant hyperparathyroidism and diabetes mellitus were studied before and after parathyroidectomy. Fifty patients (89.3%) had hypercalcemia, and six patients (10.7%) had normocalcemia associated with inappropriately elevated parathyroid hormone. After surgery, three of five patients with insulin-dependent diabetes mellitus showed more than a 50% reduction in insulin requirement. Thirty-nine of 49 patients with noninsulin-dependent diabetes mellitus were followed. Of these, three patients had restoration of normal blood glucose levels without any diabetic treatment including diet restriction. Diabetes control improved in eight parents, remained stable in 18, and deteriorated in 10 patients. In the remaining two patients, impaired glucose tolerance disappeared in one patient and progressed to frank diabetes in the other. Overall 60.7% of the patients improved or remained stable in their diabetes control after parathyroidectomy. We conclude that in patients with hyperparathyroidism, the coexistence of diabetes mellitus might be a further indication for parathyroidectomy. Physicians should be alerted to the possible change in diabetic regimen and the risk of hypoglycemia in patients with diabetes after parathyroidectomy.
Adult↗