Localization of neuroendocrine tumors utilizing linear-array endoscopic ultrasonography.
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Duodenal Neoplasms↗
Biomedical subjects
N W Thompson
Publications and source records attributed to N W Thompson.
Undescended superior parathyroid gland: a potential cause of failed cervical exploration for hyperparathyroidism.
BACKGROUND: The undescended inferior parathyroid gland is a well-established although infrequent embryologic abnormality resulting in an ectopically located gland usually associated with thymic tissue and is found most commonly within the carotid sheath at the level of the carotid bifurcation. Embryologically undescended superior glands (either normal or enlarged), clearly above the level of the upper pole of the thyroid gland and found within or in approximation to the lateral pharyngeal wall, have not been previously described. METHODS: The locations of all parathyroid glands at the time of cervical exploration were carefully examined as to embryologic origin in a combined experience of more than 3000 patients with primary or secondary hyperparathyroidism. RESULTS: Five patients had enlarged abnormal glands located within or in close approximation to the pharyngeal wall. All were above the upper pole of the thyroid gland. Two were identified at reoperation on the basis of localization studies. One gland was found after resection of a massive concomitant cervical goiter. The other two were found after an extensive search for a superior gland. CONCLUSIONS: Undescended superior glands are extremely rare (0.08%). They are located within or in approximation to the pharynx above the level of the thyroid gland with an unusual blood supply, which supports an embryologic rather than acquired cause for their ectopic location.
Adolescent↗
Operative experience of U.S. general surgery residents in thyroid and parathyroid disease.
BACKGROUND: We wanted to determine whether the experience of general surgery residents is adequate and effective. METHODS: The Resident Statistic Summaries (Report C) of the Residency Review Committee (Surgery) for 8 academic years from 1986 through 1994 were analyzed. The main outcome measurements were total number of residents and programs, average number of operations performed, maximum number of operations performed, standard deviation, and the most common number of operations performed. RESULTS: For thyroidectomy the average per resident ranged from 10.3 to 12.6. The maximum ranged from 52 to 102. The standard deviations ranged from 6.96 to 8. The most common number of thyroidectomies performed ranged from 7 to 10 per graduating resident. For parathyroidectomy the average ranged from 4.1 to 5.1, the standard deviations were 3.44 to 4, the maximum ranged from 25 to 60, and the most common number performed was 2. CONCLUSIONS: U.S. graduates have highly variable experience in thyroid and parathyroid surgery. Most residents have inadequate experience in parathyroid surgery and marginal experience in thyroid surgery.
Humans↗
Progress in genetic screening of multiple endocrine neoplasia type 2A: is calcitonin testing obsolete?
BACKGROUND: Recent identification of RET mutations in multiple endocrine neoplasia type 2A (MEN 2A) allows a DNA-based approach to diagnosis in lieu of calcitonin sampling. To prospectively evaluate the efficacy of mutational analysis, genetic screening was performed in 124 patients (53 male, 71 female; age, 1 month to 80 years) at risk for MEN 2A referred over 3 months. METHODS: Analysis used genomic DNA and a polymerase chain reaction-based denaturing gradient gel electrophoresis strategy for mutation detection at RET codons 609, 611, 618, 620, and 634. Ninety-three of 124 patients were from established MEN 2A kindreds (group A), and screening replaced calcitonin testing. Twenty-one of 124 patients (group B) represented index cases of medullary thyroid carcinoma (MTC), and DNA analysis was performed to distinguish sporadic from hereditary disease. Ten patients (group C) had modest calcitonin elevations or had undergone thyroidectomy without confirming pathologic results, and testing was undertaken to clarify status. RESULTS: Group A: RET mutations occurred in 29 (median age, 10 years) of 93 patients, 14 of whom underwent thyroidectomy. No false-positive results were observed. Group B: five (24%) of 21 patients with seemingly sporadic MTC had RET mutations at codons 618 (one), 620 (one), or 634 (three). Group C: Nine of 10 patients with alleged MEN 2A had genetically negative results. CONCLUSIONS: Denaturing gradient gel electrophoresis reliably detects MEN 2A. Modest calcitonin elevations may lead to a false-positive diagnosis of MTC. DNA testing is the optimal approach to evaluating MEN 2A. Index cases of sporadic MTC should also undergo DNA analysis.
Adolescent↗
The surgical management of hyperparathyroidism and endocrine disease of the pancreas in the multiple endocrine neoplasia type 1 patient.
The surgical management of multiple endocrine neoplasia type 1 (MEN1) parathyroid disease and involvement of the endocrine pancreas remains controversial. Hyperparathyroidism, usually the first clinical manifestation of the syndrome, requires surgical treatment in nearly all patients. We favour a subtotal parathyroidectomy and cervical thymectomy rather than a total parathyroidectomy and autotransplant because of good long-term results and the absence of permanent hypoparathyroidism. The results of treating 34 MEN1 patients during a 20-year period are reported. The most common functional pancreatic or duodenal tumours in MEN1 patients are gastrinomas and insulinomas. In addition to the management of functional syndromes, another major concern is the malignant potential of the neuroendocrine tumours that frequently develop. Our surgical management of gastrinomas and the ZES has evolved over a period of 15 years. We have found that distal pancreatectomy, enucleation of any neoplasms in the head, and duodenotomy and excision of any neuroendocrine tumours (gastrinomas) combined with a regional node dissection are effective in the majority of patients. The results of treating 21 MEN1 patients with ZES are reported.
Adult↗
Characterization of adrenal masses with chemical shift and gadolinium-enhanced MR imaging.
PURPOSE: To assess the potential role of chemical shift and dynamic gadolinium-enhanced magnetic resonance (MR) imaging in the characterization of adrenal masses. MATERIALS AND METHODS: Fifty-one adrenal masses (35 adenomas and 16 nonadenomas) in 43 patients were evaluated with chemical shift MR imaging, dynamic gadolinium-enhanced MR imaging, or both. The relative change in the signal intensity (SI) ratio of the adrenal mass to liver and paraspinal muscles was quantitatively and qualitatively assessed. Opposed-phase gradient-echo (GRE) images were compared with in-phase images. RESULTS: With qualitative visual inspection, only adenomas showed a decrease in relative SI ratio on opposed-phase chemical shift images (specificity, 100%; sensitivity, 81%). Quantitative ratios corresponding to 100% specificity were also observed, with similar sensitivities. Adenomas could not be differentiated from nonadenomas with visual assessment of maximum SI after contrast material administration or washout. CONCLUSION: Characterization of an adrenal mass as an adenoma can be made with high specificity and acceptable sensitivity by visually comparing opposed-phase with in-phase GRE images.
Adenoma↗
Use of 2-[F-18]-fluoro-2-deoxy-D-glucose PET to locate parathyroid adenomas in primary hyperparathyroidism.
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Adenoma↗
Role of endoscopic ultrasonography in the localization of insulinomas and gastrinomas.
BACKGROUND: We have previously found selective venous sampling to be the most sensitive method to localize otherwise occult functioning endocrine tumors. However, recently we have used endoscopic ultrasonography (EUS) as the initial and in some cases the only localization study in the preoperative evaluation of proven insulinomas and of selected cases of gastrinoma. METHODS: All patients referred between April 1993 and April 1994 with a subsequently confirmed diagnosis of organic hyperinsulinism or Zollinger-Ellison syndrome (ZES) underwent EUS. Ten patients with insulinomas and six with gastrinomas were studied. Only one patient with ZES had multiple endocrine neoplasia type I. Patients with negative EUS findings had additional localization procedures including angiography and arterial stimulation tests. All but one patient underwent surgical exploration. RESULTS: Solitary insulinomas were found in all 10 patients. EUS correctly identified and localized the insulinoma in seven (70%) of 10 patients but failed to identify two pedunculated insulinomas that were easily found at exploration. Because of an incomplete examination, a single insulinoma was not detected within the parenchyma. The EUS examination correctly excluded the pancreatic gastrinomas in five patients. The sixth patient, who had multiple endocrine neoplasia type I, had two 0.5 cm tumors in the head. CONCLUSIONS: EUS is a sensitive and cost-effective technique for localization of insulinomas and may be the only study needed. In patients with ZES a negative pancreatic result suggests the likelihood of a duodenal or other extrapancreatic tumor.
Adult↗
Enucleation combined with hepatic vascular exclusion is a safe and effective alternative to hepatic resection for liver cell adenoma.
Liver cell adenomas are benign, rare tumors that occur primarily in women with a history of oral contraceptive use. Surgical treatment is recommended to reduce the risk of sudden, unpredictable hemorrhage or malignant transformation, but mortality rates of 5 to 8 per cent are reported for major hepatic resections. This report summarizes an experience of eight patients with liver cell adenoma treated by enucleation or resection, alone or combined with hepatic vascular exclusion (HVE). The latter technique, originally described in the mid-1960s, markedly reduces operative blood loss associated with hepatic resection. All eight patients were women of child-bearing age, and seven reported previous oral contraceptive use. The majority of patients were asymptomatic at the time of presentation. Six patients (75%) had a single adenoma and the remaining two had multiple lesions. HVE was used successfully in four patients with no perioperative mortality and reduced by nearly 50 per cent the intraoperative blood loss compared to conventional hepatic resection (1635 mL versus 3875 mL, respectively). Six evaluable patients were followed for an average of 65 months, with no evidence of liver dysfunction or adenoma recurrence. Enucleation of liver cell adenoma appears to be a safe alternative to formal hepatic resection and provides excellent long-term results. HVE markedly reduces operative blood loss, has no adverse effect on metabolic function, decreases the potential health risk associated with transfusion, and should be used whenever possible.
Adenoma, Liver Cell↗
Scintigraphic evaluation of clinically silent adrenal masses.
UNLABELLED: We studied 229 patients with abnormal adrenal anatomy depicted by CT who were without biochemical evidence of endocrine dysfunction using the presence of 131I-6 beta-iodomethyl-nor-cholesterol (NP-59) adrenal gland uptake as an index of differential adrenal function in the evaluation of the clinically "silent" adrenal mass lesion. METHODS: NP-59 (1 mCi) was injected intravenously with posterior and lateral abdominal images obtained 5-7 days postinjection. RESULTS: One-hundred and fifty-nine of 185 patients with unilateral adrenal enlargement on CT had scintigraphic evidence that the mass represented a functioning (NP-59 avid) but not hypersecretory, (biochemically normal) adrenal cortical adenoma (concordant imaging pattern). Forty-one of 44 patients with intra-adrenal neoplasms were depicted on scintigraphy as decreased or absent NP-59 accumulation on the side of the adrenal mass (discordant imaging pattern). In this study, sensitivity was 71% (41 of 58 patients; 95% confidence interval (CI), 58% to 88%); specificity was 100% (171 of 171 patients; 95% CI, 95% to 100%) and accuracy was 93% (212 of 229 patients; 95% CI, 88% to 96%). CONCLUSIONS: These data confirm our earlier observations that the functional information depicted by scintigraphy complements the morphological evaluation by CT and in the absence of hormonal dysfunction, the presence of concordant CT and 131I-NP-59 scans are characteristic of functioning, but not hypersecretory, benign adrenocortical adenomas. Conversely, discordant CT and 131I-NP-59 scans are suggestive of nonfunctioning, space-occupying, adrenal lesions.
Adenoma↗
Traumatic rupture of a cervical parathyroid adenoma.
BACKGROUND: Spontaneous rupture of a hyperplastic parathyroid gland or adenoma resulting in extracapsular hemorrhage is extremely rare. METHODS: We report a case of traumatic rupture of a cervical parathyroid gland adenoma. RESULTS: The patient had progressive stridor, neck swelling, and chest and back pain with evidence of ongoing blood loss resulting in airway compromise. Exploration of the neck and superior mediastinum revealed hypercellular parathyroid tissue consistent with adenoma or hyperplasia. CONCLUSIONS: Traumatic rupture of an enlarged parathyroid gland is a distinct and potentially lethal cause of unexplained cervical or mediastinal hemorrhage after blunt neck trauma. Failure to consider the diagnosis may result in delayed operative intervention with persistent hemorrhage resulting in airway compromise.
Adenoma↗
Nonfunctioning cystic neuroendocrine neoplasms of the pancreas.
BACKGROUND: Three types (functioning solid, nonfunctioning solid, and functioning cystic) of neuroendocrine neoplasms (benign and malignant) of the pancreas have been reported. PATIENTS: We report three patients with nonfunctioning neuroendocrine neoplasms of the pancreas with cystic components, a type of neoplasm not previously reported. One of the neoplasms we report was benign; the other two were malignant. The patients were admitted with abdominal pain. In each case an abdominal computed tomography scan defined a large multicystic pancreatic mass. RESULTS: In all instances, immunochemical staining was positive for neuroendocrine markers, confirming the neuroendocrine nature of the tumors. The nonfunctional nature of the neoplasms was confirmed by (1) the absence of clinical symptoms traditionally attributed to neuroendocrine secretagogues and (2) the lack of gastroenteropancreatic peptide serum levels. CONCLUSIONS: Two of these three cases represent another subset of pancreatic neuroendocrine pathology: a nonfunctional cystic neoplasm. Increased clinical awareness, appropriate operation, and better immunohistochemical staining techniques may yield more, heretofore unrecognized, subtypes. These cases reemphasize the need for proper surgical exploration and biopsy of all unexplained pancreatic cystic lesions, particularly if they are multicystic.
Adenoma, Islet Cell↗
Pheochromocytomas that do not accumulate metaiodobenzylguanidine: localization with PET and administration of FDG.
Many imaging methods can be used to detect pheochromocytoma, but some tumors are not detected with conventional modalities. To explore the possible usefulness of positron emission tomography (PET) after administration of 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG) to localize pheochromocytoma in patients with false-negative scintigrams obtained after administration of metaiodobenzylguanidine (MIBG), FDG was administered and PET was performed in two adult patients with pheochromocytomas that had never been localized despite administration of MIBG. In both patients, images were obtained dynamically for 50 minutes; then a limited truncal sequence was performed. PET enabled correct localization of the tumors. In patient 1, a tumor that had not been detected for 21 years was localized in the middle mediastinum; in patient 2, a pheochromocytoma was detected in the right adrenal gland. PET performed after administration of FDG may be useful for localization of pheochromocytomas that do not accumulate MIBG.
3-Iodobenzylguanidine↗
Pheochromocytoma and pregnancy: the epitome of high risk.
BACKGROUND: Past review has shown that one half of the fetuses and nearly that many mothers died of pheochromocytoma if the tumor was not detected in the mother before delivery. The rarity of the occurrence adds to the potential danger because experience with diagnosis and management is unusual. However, the diagnosis is straightforward when considered because pregnancy does not alter diagnostic levels of catecholamines. Management must be individualized and can be done safely. METHODS: The cases of five patients with pheochromocytomas during pregnancy are presented here. Diagnosis was made by urinary catecholamine measurement. Localization methods included caval catheterization and ultrasonography. Blood pressure control was done with alpha- and beta-adrenergic blockers. Treatment strategy was planned by a team of internists, obstetricians, surgeons, and anesthesiologists. RESULTS: All five mothers and three fetuses survived. The two fetal deaths were planned terminations. Two mothers and one fetus had multiple endocrine neoplasia type 2B syndrome. One mother had two recurrences, one of which was involved with the pregnancy. CONCLUSIONS: Pregnancy does not alter urinary catecholamine levels to confuse the diagnosis of pheochromocytoma. Localization by ultrasonography and magnetic resonance imaging is safe to the fetus. alpha- And beta-adrenergic blockers are well tolerated. Every effort should be made to save a normal fetus when the tumor is first discovered during the third trimester. Timing of excision is a decision best done by team planning.
Abortion, Induced↗
High-output cardiac failure: an unusual manifestation of intravenous leiomyomatosis.
Intravenous leiomyomatosis is a rare uterine neoplasm characterized by nodular or wormlike masses of histologically benign smooth muscle growing within myometrial veins and extending variable distances into the veins of contiguous organs and major pelvic veins. In some cases, there has been extension into the gonadal or iliac veins to the vena cava and right atrium, causing symptoms of congestive heart failure and an eventual fatal outcome. We present a report of the first case in which high-output cardiac failure was caused by the development of arteriovenous shunting within the intravenous component of the tumor that involved the pelvic organs and retroperitoneal and gonadal veins but not the vena cava. Treatment by total abdominal hysterectomy, bilateral oopherectomy, and tumor mass excision from the retroperitoneum was successful.
Adult↗
Surgical approach to insulinomas. Assessing the need for preoperative localization.
The purpose of this study was to examine our experience with the diagnosis, surgical approach, and outcomes of surgery for organic hyperinsulinemia in the era of transhepatic venous sampling. During the period from 1978 to 1991, 50 patients were evaluated and treated for hyperinsulinemia at the University of Michigan Medical Center, all of whom underwent preoperative localization. Forty-one patients (82%) had solitary, benign tumors; four (8%) had either multiple tumors or islet cell dysplasia; and five (10%) had metastatic disease. Forty-seven patients underwent laparotomy, and the source of the hyperinsulinemia was found in all patients. In three patients (6%) preoperative localization did not accurately locate the tumor due to technical difficulties with the completion of the studies. Overall, computed tomography localized nine (26%) of 35 tumors. Angiography accurately localized 18 (44%) of 41 tumors. Transhepatic venous sampling localized 34 (94%) of 36 tumors, and was essential to successful surgical treatment in 15 patients. Compared with angiography, transhepatic venous sampling was a more accurate method of localization and should be performed in all patients in whom computed tomography and visceral angiography do not clearly identify the site of disease. Preoperative localization plays a critical role in the surgical treatment of patients with organic hyperinsulinemia and eliminates the need for blind pancreatic resection.
Adult↗
Fine-needle aspiration biopsy causing peritoneal seeding of a carcinoid tumor.
A rare complication of fine-needle aspiration biopsy (FNA) is needle tract seeding. Peritoneal seeding from FNA in the clinical setting is rarely reported. To our knowledge, this is the first case of peritoneal seeding of a neuroendocrine carcinoid tumor as a result of FNA. Peritoneal contamination and tumor seeding can occur with FNA and they probably do occur more often than is recognized clinically. Therefore, we caution against the use of FNA in the assessment of potentially curable intra-abdominal tumors, and recommend that this technique be used only when a cytological diagnosis is required before initiation of palliative therapy.
Adenoma, Islet Cell↗
Analysis of gastrinomas by immunohistochemistry and in situ hybridization histochemistry.
Gastrinomas from 25 patients were examined by immunohistochemistry (IHC) and in situ hybridization histochemistry (ISH). Most patients (84%) presented with the Zollinger-Ellison syndrome. Six had multiple endocrine neoplasia type I (MEN-I). Twelve patients (48%) had duodenal primaries and 11 of 12 of these had metastases to regional lymph nodes and/or liver in spite of the small sizes of the primary tumors (mean size of 0.9 cm). Five patients had pancreatic gastrinomas and eight patients had metastatic tumor in regional lymph nodes or liver at surgery but a primary was not found. IHC and ISH analyses showed that all cases were positive for gastrin protein and 24 of 25 (96%) expressed gastrin mRNA that was easily detected in formalin-fixed, paraffin-embedded tissue sections. Both benign and malignant tumors expressed alpha subunit of human chorionic gonadotropin protein (alpha-HCG). However, only malignant gastrinomas (29%) expressed adrenocorticotropic hormone protein or proopiomelanocortin (POMC) mRNA. ISH and Northern hybridization analysis revealed that chromogranin A mRNA was the most common member of the chromogranin/secretogranin (Cg/Sg) family which was expressed in both benign and malignant gastrinomas. These results indicate that duodenal gastrinomas are common in both sporadic and MEN-1-associated cases, and small duodenal primaries may be associated with extensive regional lymph node and liver metastases. Expression of ACTH/POMC protein and mRNA was consistently associated only with malignant gastrinomas while gastrin protein, gastrin mRNA and Cgs/Sgs mRNAs were readily detected in both benign and malignant gastrinomas.
Adrenocorticotropic Hormone↗