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Biomedical subjects

J A Van Heerden

Publications and source records attributed to J A Van Heerden.

At least 19 recordsLinked to original sources

Cushing's syndrome due to medullary thyroid carcinoma: diagnosis by proopiomelanocortin messenger ribonucleic acid in situ hybridization.

Medullary thyroid carcinoma (MTC) rarely causes ectopic ACTH syndrome. We describe a 38-yr-old man with renal stones who had a 5-cm MTC removed in 1992. He was RET-protooncogene positive (codon 618). Serum calcitonin was 1597 pg/ml postoperatively. In 1996 he had rib fractures, bruising, weakness, and three to four stools per day. Laboratory studies revealed an elevated 24-h urine-free cortisol (780 micro g/d), epinephrine (66 micro g/d), and calcium (558 mg/d). Baseline serum cortisol was 23.9 micro g/dl and decreased to 12.9 and 4.5 micro g/dl after 2 mg and 8 mg dexamethasone suppression, respectively. Plasma ACTH was 170 pg/ml and decreased to 75 and 24 pg/ml after dexamethasone. Bone density t-score was -4.3 (trochanter). Computed tomography scans showed multiple cervical nodes and 2-cm right adrenal nodule. Magnetic resonance imaging (MRI) scan showed a prominent, homogeneous pituitary; the adrenal MRI scan was not typical for a pheochromocytoma. Serum CRH was less than 6.6 pg/ml. Bilateral adrenalectomy revealed two adjacent right adrenal pheochromocytomas and corrected the elevated urine cortisol (30 micro g/d), epinephrine (0 micro g/d), and calcium (281 mg/d) but not plasma ACTH (125 pg/ml). Neck dissection reduced calcitonin by 96% (5300 to 120 pg/ml) and ACTH by 91% (125 to 11 pg/ml). Carcinoembryonic antigen was reduced from 32.0 to 2.3 ng/ml. Immunohistochemical stain was negative for ACTH in the MTC-positive lymph nodes and the pheochromocytoma. Proopiomelanocortin mRNA by in situ hybridization was positive in the MTC but not in the pheochromocytoma. A repeat pituitary MRI scan was normal. The differential diagnosis of ACTH-dependent Cushing's syndrome in this case included pituitary disease or ectopic ACTH, either from medullary thyroid carcinoma or pheochromocytoma. ACTH stains were unrevealing, but proopiomelanocortin mRNA in situ hybridization in MTC tissue and plasma ACTH response to neck dissection confirmed MTC as the source of ectopic ACTH.

Adrenal Glands↗

Intrahepatic cholangiocarcinoma: clinical aspects, pathology and treatment.

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary tumor of the liver. To further define its clinicopathology and surgical management, we reviewed our experience. Clinical presentations of 32 patients with ICC was similar to that with hepatocellular carcinoma. Jaundice occurred in only 27 percent. ICC was unresectable due to advanced disease stage in 81 percent. Six patients had curative resections with two 5 year disease free survivors. Underlying liver disease was associated with ICC in 34 percent of patients.

Adenoma, Bile Duct↗

Multiple endocrine adenopathy.

The syndromes of multiple endocrine adenopathy (MEA) have associated endocrine abnormalities that are similar to the clinical syndromes based in the same hyperfunctions of parathyroid, pancreatic islets, thyroid C-cell and chromaffin tissue seen in sporadic cases. The natural history, histopathology and management of these problems when they occur in the hereditary MEA syndromes differ in significant and instructive ways over the isolated disorders. These differences and similarities of MEA adenopathies were discussed at an international conference, with the suggestion that the MEA syndromes afford opportunity to investigate regulation and control of pathogenesis in surgical endocrinological syndromes.

Adult↗

Pseudohyperparathyroidism secondary to gigantic mammary hypertrophy.

Pseudohyperparathyroidism encompasses both ectopic hyperparathyroidism and the more commonly encountered nonparathyroid humoral hypercalcemia of malignancy--metastatic breast cancer being the classic example. Benign breast disease is a rare cause of hypercalcemia. We recently managed a patient with gigantic mammary hypertrophy that occurred during pregnancy who concomitantly manifested marked nonparathyroid hypercalcemia. This report strongly suggests that a casual relationship exists between benign breast disease and hypercalcemia.

Adult↗

The natural history of hepatic metastases from colorectal cancer. A comparison with resective treatment.

Five-year survival after resection of hepatic metastases from colorectal cancer is 25%. Although resection palliates some patients who do not live that long, 50% of patients so treated are not helped at all. Until ignorance of a cancer's real stage is resolved by improved techniques, the evaluation and choice of therapy can be based only upon knowledge of the natural history of untreated metastases and determinants of prognosis derived from treated patients. Analysis of the survival rates of 252 patients who had biopsy proven, unresected hepatic metastases that were the only evidence of residual disease shows the extent to which natural history, rather than resection, may determine length of survival-- and indicates the need for critical analysis of 2- and 3-year survival rates reported after any therapy. Study of 141 patients who had hepatic metastases resected shows that the stage of the primary lesion, being female, and the absence of extrahepatic metastases are significant determinants of favorable prognosis after resection of hepatic metastases.

Colonic Neoplasms↗

Fine-needle aspiration biopsy of the thyroid. The problem of suspicious cytologic findings.

Cases in which thyroid nodules yielded suspicious cytologic findings on fine-needle aspiration biopsy were prospectively studied during a 3-year period. Of 1970 patients, 333 (17%) had suspicious cytologic findings; from this group, the cases of 253 patients were studied. The cytologic diagnoses were 96 (38%) H urthle cell neoplasms, 84 (33%) follicular cell neoplasms, and 73 (29%) nonspecific diagnoses. A thyroid scan was obtained in 123 of 203 (61%) patients with a solitary nodule, and the nodules were hypofunctional in 102 (83%). Malignant lesions were found in 60 (24%) of the 253 patients. Our findings are consistent with reports that 20% of patients who have a fine-needle aspiration biopsy will have suspicious cytologic findings and that of these, 20% may have a malignant lesion. Thyroid scanning will not distinguish between benign and malignant thyroid lesions that are suspicious on cytologic examination. Surgical excision of all suspicious lesions seems reasonable.

Adenocarcinoma↗

The surgical management of medullary thyroid carcinoma.

Medullary carcinoma of the thyroid may occur in three patient groups: multiple endocrine neoplasia, type 2b (MEN2b), MEN2a, and sporadic. The prognosis is best in MEN2a and worst in MEN2b. Multicentric disease occurs in approximately 90% of patients in the MEN groups and in 20% of the patients in the sporadic group. The minimal surgical procedure advocated is total thyroidectomy with dissection of the central compartment nodes. When neck dissection is performed, there appears to be no advantage in resecting the internal jugular vein or the sternomastoid muscle. Primary relatives of all patients with medullary carcinoma should be screened by measurement of plasma immunoreactive calcitonin to identify C-cell disease in a generally unsuspecting group/reservoir and because it results in earlier diagnosis, which leads to a less extensive surgical procedure and a higher percentage of patients with a disease-free state.

Adolescent↗

Proximal gastric vagotomy: update.

Experience with proximal gastric vagotomy at the Mayo Clinic from 1973 to Mayo 1980 is reported. Among 298 patients who had proximal gastric vagotomy for chronic duodenal, pyloric channel, or prepyloric ulcers, a recurrent ulcer rate of 7% was present, with a mean follow-up of 49 months. Three recurrences developed in six patients who had proximal gastric vagotomy for gastric ulceration. In 40 patients, proximal gastric vagotomy was combined with gastrojejunostomy, pyloroplasty, or pyloric dilatation for obstructing ulcers. There was a 15% incidence of reoperation in the gastrojejunostomy group. All nine patients who had proximal gastric vagotomy for active or recent bleeding ulcers were dismissed from the hospital without further hemorrhage, and only one developed a recurrent ulcer. It is concluded that proximal gastric vagotomy remains an acceptable operation for chronic duodenal and pyloric ulcers, but its efficacy in gastric ulcers is unproved.

Female↗

Surgical management of inflammatory bowel disease.

Indications for operation in patients with inflammatory bowel disease are now standardized as a result of the vast surgicaL experience that has been accumulated during the past 40 years. The surgical indications in Crohn's disease and chronic ulcerative colitis vary minimally with the anatomic distribution of either disease, and can be recognized easily in a particular patient. Consequently, decision or judgment regarding the need for operation is rarely difficult. Delaying operation on the basis of fear of recurrence of Crohn's disease is unrealistic because (1) indications for operation are complications of the disease that have not responded or cannot be expected to respond to medical treatment, (2) conservative resection primarily removes diseased bowel that will never return to normal, (3) many patients, perhaps 50%, will never have recurrence of disease, and (4) those who have recurrence will have experienced varying periods when they were free of disease and relieved of the serious complications for which their operations were performed. The value of surgery in the treatment of patients with chronic ulcerative colitis can be stated even more positively, because recurrence of disease is never a concern after proctocolectomy.

Adult↗

Sexual dysfunction following proctocolectomy and abdominoperineal resection.

Sexual dysfunction after rectal excision was studied in 45 male patients who were less than 50 years of age. Of 25 patients having had proctocolectomy, one (4%) was impotent, while three (15%) of 20 patients having had abdominoperineal resection were impotent. Two patients in the abdominoperineal group reported no ejaculation with normal potency and sensation of orgasm. The age of the patient and the extent of dissection seemed to be the two main factors concerned with sexual dysfunction after rectal excision.

Abdomen↗

Major hepatic resections for metastatic colorectal cancer.

In 1973, our study of patients with resectable hepatic metastases from colorectal cancer compared survival of 60 patients who had metastases removed with survival of 60 patients with similar lesions that had been biopsied only. We concluded that excision of small, apparently solitary metastatic lesions could be justified on the basis of the low operative risk and prolonged survival. However, the risks and benefits of resection of larger metastatic lesions could not be determined by that earlier study, because only seven of those patients had lesions so large as to require major hepatic resection. Therefore, to evaluate size as a determinant of prognosis after resection, we added to those 7 patients 27 others who were managed since 1973 by major hepatic resection of larger metastases. There were two hospital deaths. Of the 32 surviving patients, 82% lived one year or more, 77% 18 months or more, 58% two years or more, and 41% three years or more postoperatively. Three patients are living 10--22 years after resection. We conclude from a critical analysis of the duration and quality of life of surviving patients that at least 20% and perhaps 30% of these patients were benefited by major hepatic resection of their large hepatic metastasis.

Aged↗

Rectal preservation in nonspecific inflammatory disease of the colon.

Sixty-three patients with chronic ulcerative colitis and 80 patients with Crohn's disease underwent abdominal colectomy and primary ileorectal or ileosigmoid anastoimosis and were followed up for between five and 17 years. No patient developed carcinoma of the rectal stump. The interval proctectomy rate was similar in both groups (24 and 29%, respectively), the rate being influenced by age in the group with chronic ulcerative colitis. The young patients with ulcerative colitis required proctectomy more often than did the patients with onset later in life. The proctectomy rate was not influenced by the level of the anastomosis. In patients with normal or moderately diseased rectal mucosa, the preoperative condition of the rectum did not influence the proctectomy rate. Functional results were satisfactory in 55% of the patients with chronic ulcerative colitis who survived and in 35% of the patients with Crohn's disease who survived. Because of the non-curative nature of surgery for Crohn's disease, a conservative posture should be maintained, with preservation of normal or moderately diseased rectal segments. If sigmoidoscopie examination and biopsy are done periodically, abdominal colectomy and ileorectal or ileosigmoid anastomosis appears to be a viable option to proctocolectomy in selected patients with chronic ulcerative colitis.

Adult↗

Anastomotic ulceration following subtotal and total pancreatectomy.

Ulcerogenic potential exists after subtotal gastrectomy, pancreatic resection and separation of the pancreatic and bile ducts from the gastric outlet. Collectively, as with radical pancreatoduodenectomy or total pancreatectomy, a substantial risk of an anastomotic ulcer developing can be expected. To elucidate this, we reviewed the records of 297 patients who had undergone either radical pancreatoduodenectomy (Whipple procedure) or total pancreatectomy. We found that 18 patients (6%) had developed an anastomotic ulcer and that total pancreatectomy was statistically more ulcerogenic than a Whipple procedure. An anastomotic ulcer a virulent complication; two-thirds of the patients had bleeding, and it was a contributing cause of death in 22% of patients in whom ulcer developed. In patients who have a favorable prognosis, the addition of vagectomy to either procedure should be considered.

Adult↗