Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome.
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Biomedical subjects
Publications and source records attributed to W H ReMine.
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Forty-five patients with biochemically documented primary hyperparathyroidism as part of multiple endocrine neoplasia syndrome types 1 or 2 were surgically treated from 1960 through 1980. Hyperplasia occurred in 69% of the patients, single adenoma in 27%, and double adenomas in 4%. All but two patients with hyperplasia underwent subtotal parathyroidectomy. In this group, the cure rate was 93% and the incidence of permanent postoperative hypoparathyroidism 23%. In the adenoma group, treatment included excision of the adenoma and biopsy of at least one other gland. The cure rate was 76%, with no postoperative hypoparathyroidism. Analysis of patients with persistent hyperparathyroidism suggested that failure to recognize multiple gland disease was the principal cause of postoperative hypercalcemia. In view of the high incidence of hypocalcemia after subtotal parathyroidectomy, approximately 500 mg of tissue should be cryopreserved to allow transplantation should hypocalcemia ensue postoperatively.
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.
From 1971 through 1980, 106 patients underwent operations for pheochromocytoma. Twelve patients had a pheochromocytoma as a manifestation of the multiple endocrine neoplasia type 2 syndrome. Twenty patients were found to have extra-adrenal paragangliomas. Fifteen patients (14%) had malignant tumors; eight of these tumors were encountered in the group with extra-adrenal lesions. The cumulative survival probability at 5 years was 53%. Determination of urinary metanephrines and vanillylmandelic acid were the most sensitive diagnostic aids, with accuracy rates of 95% and 89%, respectively. In recent years, the measurement of fractionated urinary and plasma catecholamine levels has greatly increased the diagnostic accuracy. Preoperative localization of pheochromocytoma has improved during the last decade. Nephrotomography, selective angiography, and venous sampling have essentially been abandoned in favor of computed tomography. With an accuracy of more than 90%, computed tomography represents the major step forward in the overall management of pheochromocytoma during the past decade.
This review reveals that, once again, surgical therapy of ductal adenocarcinoma of the pancreas has been thwarted, probably by the inherent biologic aggressiveness of this particular malignancy. The operative mortality rate (greater than 10 percent) coupled with the 5 year survival rate of 2.3 percent is eloquent testimony to this. Multicentric disease occurred in one third of the patients, whereas extrapancreatic spread was found in half of the resected specimens. At 1 year, the only statistically favorable determinants were multicentricity and female sex. The main bonus of total pancreatectomy appears to be the elimination of pancreatojejunal anastomosis with its lethal complications.
Hyperfunctioning parathyroid carcinoma is a relatively rare endocrine tumor, accounting for approximately 1% of all cases of primary hyperparathyroidism. The diagnosis is suspected when the tumor is large, parathyroid hormone (iPTH) levels are high, and a palpable tumor is present in the neck. Patients who have recurrence of hyperparathyroidism several months after surgical treatment should be suspected of having a recurrent or persistent parathyroid carcinoma. At operation, a large invasive tumor is usually found. The fibrous, inflammatory-like reaction is the most characteristic indication of malignancy. Even in tumors with minimal invasiveness, the possibility of a carcinoma should be considered if the tumor has mitotic activity and a monotonous instead of a pleomorphic cellular population. If the surgeon can recognize the possibility of parathyroid malignancy and adequately treat the patient during the initial operation, more gratifying results should be obtained.
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Over the past 20 years, carcinoma of the pancreas has increased in incidence by 20 percent. In spite of this marked increase, there continues to be controversy in some areas regarding the proper surgical management of ductal lesions. To date, there have been 122 total pancreatectomies performed at the Mayo Clinic; 63 of these were performed for carcinoma of the pancreas. Among 21 patients treated by total pancreatectomy since 1975, there was one operative death for an overall operative mortality of 4.7 percent. During the same period of time, when 34 patients underwent partial pancreatectomy of the Whipple type, there were three postoperative deaths with an operative mortality of 8.8 percent. The three patients that expired following performance of the Whipple procedure did so as a result of leakage at the anastomosis between the pancreas and the jejunum. Endocrine and exocrine pancreatectomy management have not been a particular problem. Conclusions can be drawn that total pancreatectomy for carcinoma of the pancreas is an acceptable procedure.
Between 1960 and 1977, 50 patients with agnogenic myeloid metaplasia were splenectomized. Twenty-five of 26 patients with painful splenomegaly, 4 of 9 patients with refractory hemolytic anemia, 4 of 10 patients with refractory thrombocytopenia, and 4 of 4 patients with portal hypertension showed significant benefit from the procedure. There were five immediate postoperative deaths. Four of these deaths occurred early in our series of splectomies for myeloid metaplasia before 1970. Only one death has occurred in the last 21 patients operated on. Survival following splenectomy averaged 25.5 mo.
Forty-five patients with pancreatic cystadenoma were treated surgically at the Mayo Clinic between 1939 and 1975. Each lesion was classified as mucous or serous. The most frequent lesion site was the tail of the pancreas. The typical patient was a middle-aged woman with upper abdominal pain and a palpable mass on initial examination. Total extirpation of the cystadenoma was the treatment of choice; internal drainage or external drainage of these cysts should not be done. Occasionally, the nearness of the tumors to the mesenteric vessels precluded excision. The mucous type had malignant potential whereas the serous does not. None of the cystadenomas subsequently underwent malignant degeneration. Because of the slow-growing characteristics of the tumor, undue operative risks should not be taken.
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An unusual parathyroid adenoma containing abundant adipose tissue is described in four patients. In one patient, hyperparathyroidism was documented; in another, studies were incomplete but the adenoma was probably functioning. Prior reports of similar lesions have been called "parathyroid hamartoma or parathyroid adenoma with myxoid stroma," and some of these also have been shown to be functional. They may create diagnostic difficulties because of the presence of adipose tissue, a feature associated with normal parathyroid glands.
Cystadenocarcinomas are rare tumors of the pancreas. They are half as common as their benign counterpart--the cystadenoma. A review of 21 cases, including eight reported since 1963, revealed that most patients presented with upper abdominal pain and a large palpable upper abdominal mass. All patients had evidence of malignant degeneration occurring in a mucous cystadenoma. None had evidence of a serious cystadenoma. Grading revealed a predominance of low-grade tumors: 20 of the 21 lesions were grade 1 or 2. Cystadenocarcinomas were slightly more frequent in the head of the pancreas. Four patients had metastasis. Complete excision by distal or total pancreatectomy or Whipple pancreaticoduodenectomy is recommended. The five year survival after complete excision is 68%, and for patients with grade 1 lesions, and five year survival is 64%.
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An alternative to transcolonic polypectomy, transabdominally directed, transanal colonoscopic polypectomy, for patients who require polyp removal but in whose cases standard colonoscopic approach has failed is presented. The early results are excellent, and the method also can be applied to other patients who have colonic polyps who undergo procedures such as hysterectomy and cholecystectomy.
During the period of 1962 to 1972, 71 patients underwent surgical treatment of pancreatic pseudocysts. Internal drainage was performed in 73% of these patients in comparison to only 20% in a series during the previous decade. From an analysis of results, it would appear that the treatment of choice is internal drainage via either cystogastrostomy or cystojejunostomy. Postoperative bleeding and sepsis were of negligible consequence. An unexpected finding was that the long-term results of these patients seemed to be better than those of patients with pancreatitis in whom pseudocysts did not develop.