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[Atypical picture of familial medullary thyroid carcinoma].

Two sisters are reported treated at various times for thyroid medullary carcinoma. In one of them, treated later, right adrenal tumour coexisted suggesting a classical picture of the syndrome of multiglandular endocrine adenomatosis type 2a. Histological examination of the adrenal tumour showed presence of metastasis of medullary thyroid carcinoma, that is atypical pattern of this neoplasm. In the light of these the authors stress the necessity of comprehensive diagnosis of thyroid tumours with particular stress laid on histological preoperative diagnosis. Indications are discussed to thyroid reoperation in patients in whom medullary thyroid carcinoma was diagnosed only in the resected goitre.

Adrenal Gland Neoplasms↗

Autotransplantation of diseased parathyroid glands into subcutaneous abdominal adipose tissue.

From October 1981 to the end of 1984, 13 patients with primary hyperparathyroidism (PHPT) and 17 with secondary hyperparathyroidism (SHPT) received fresh autografts of diseased parathyroid tissue into their subcutaneous abdominal adipose tissue. Because of previous surgery to treat hyperparathyroidism (HPT) (23%), concomitant thyroid surgery (26%), and a high proportion of multiglandular disease (73%), the patients were at high risk for HPT. During the follow-up period, hypercalcemia was diagnosed in five patients and successfully treated in four: by graft excision in two patients, by excision of a fourth gland from the neck in one patient, and with prednisolone in a patient with sarcoidosis. At follow-up (an average of 30 months after grafting), one patient had HPT and 29 others were euparathyroid. Parathyroid tissue can survive and function in adipose tissue, as was demonstrated by normocalcemia in 14 patients (whose only probable remaining parathyroid tissue had been transplanted into fat), by the demonstration that graft-dependent hypercalcemia could be eliminated by excision of the transplant, and by the demonstration of viable parathyroid tissue by microscopic examination of excised grafts. Autotransplantation of diseased parathyroid tissue into fat is simple and reliable. In cases of recurrent HPT, all or a portion of the graft can be removed while the patient is under local anaesthesia. If infiltrating growth occurs, broad excisions can be performed without sacrificing vital structures.

Abdomen↗

Observations on 334 patients operated on for primary hyperparathyroidism.

334 patients were operated on for PHPT during the years 1956-79. A follow-up study of the material was performed during the years 1980-82 and it included 289 patients. The material was divided into single adenoma (SA) and multiglandular disease (MGD) cases. The initial diagnosis of SA given by the primary operation was later changed in 12 patients (4%) by reoperations performed for persistent or recurrent hypercalcaemia; the follow-up study changed the diagnosis in four patients. The proportion of MGD in the follow-up material was 15.2%. Reoperations were performed in 4% in the SA group and in 29% in the MGD group. The primary results concerning the serum calcium level were good in both SA and MGD groups, but, at the follow-up study 1.6% of the patients in the SA group and 41% of the patients in the MGD group were hypercalcaemic (serum calcium over 2.65 mmol/l). The hypercalcaemia at the follow-up study was, however, slight in general (mean 2.75 +/- 0.09 mmol/l). In the whole material, permanent hypoparathyroidism occurred in 1.2%, permanent unilateral vocal cord paralysis in 0.9% and permanent bilateral vocal cord paralysis in 0.6%. Operative mortality was 0.9%. The "conservative" strategy used in the whole material gave good results in SA patients but considerably worse results in MGD patients. Accordingly, MGD patients should be operated more "radically".

Adenoma↗

Long-term effect of surgical treatment on the symptoms of primary hyperparathyroidism.

Two hundred and eighty-nine patients operated on for primary hyperparathyroidism (PHPT) in the years 1956-79 have been followed up for a mean period of 5 years. The aim of the study was to investigate the symptomatology of PHPT and the disappearance of the symptoms after operative treatment. Of the presenting symptoms hypercalcaemic crisis and cystic bone changes were cured, and none of the patients with pancreatitis as presenting symptom had a recurrence. In the renal stone group, 10% of the patients had recurring stones during the follow-up period. The presenting symptom disappeared in 84% of the patients. Thirty-five% of the patients had no presenting symptom and were classified as "asymptomatic", though, on questioning, most of them had various symptoms which disappeared postoperatively. Malaise, fatigue and muscular weakness disappeared in 79% of the patients, upper abdominal pains in 66%, constipation in 63%, pains in the extremities in 51% depression in 65%. Hypertension increased by 28% during the follow-up period; only three of the 90 patients with hypertension has discontinued antihypertensive treatment postoperatively. During the follow-up study, 6% of the patients were hypercalcaemic, though the serum calcium was only slightly elevated in almost all of these patients (mean +/- SD 2.75 +/- 0.09 mmol/l) and most of them had the multiglandular form of PHPT. The renal function did not deteriorate as much as was expected on the basis of earlier reports; only two patients had a serum creatinine over 500 mumol/l.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Premature ovarian failure and hypothyroidism associated with sicca syndrome.

Two cases of premature ovarian failure, hypothyroidism, and sicca syndrome are described. To our knowledge, this association of sicca syndrome and multiglandular deficiency has not been previously reported. The finding of specific organ antibodies, as well as antibodies to nonorgan antigens in the 2 cases studied, supports the hypothesis that these rare clinical conditions may share a common autoimmune pathogenesis.

Adult↗

[Recurrent familial hyperparathyroidism. A propos of 7 adenomas in 3 members of the same family. Review of the literature].

Over a period of 23 years, 3 members of a family of 5 presented with 7 parathyroid adenomas (4 in the first case, 2 in the second case, 1 in the third case). Excision of each adenoma, with systematic pre-operative assessment of the remaining parathyroid tissue, led to complete clinical and laboratory cure of each episode. The delay in the appearance of recurrence was between 3 and 9 years. After reviewing the literature, the authors stress the importance, in any case of hyperparathyroidism, of routinely investigating the serum calcium levels in members of the patient's family, especially if the patient is young and if he has had several episodes or multiglandular involvement in one episode. The authors discuss the literature concerning recurrent familial hyperparathyroidism with endocrine polyadenomatosis and the "hypercalcaemia-hypocalciuria" syndrome.

Adenoma↗

The changing picture of primary hyperparathyroidism in the years 1956-1979.

The aim of this study was to examine the development of the clinical appearance of primary hyperparathyroidism in a material of 334 patients operated on for PHPT in the years 1956-79. The material was divided into three parts: 72 patients from the years 1956-70, 102 patients from the years 1971-75 and 160 patients from the years 1976-79. A marked change was observed in the structure of the material: the mean age of both men and women increased, and the group of women over 50 years became dominating. Symptoms such as renal stones, cystic bone changes and hypercalcaemic crisis proportionally decreased, and nonspecific symptoms as malaise, fatigue and various pains increased. The number of asymptomatic patients also steadily increased. The preoperative serum calcium values were lower in the consecutive groups. The number of small adenomas increased but, on the other hand, the number of big adenomas remained the same. The ratio between single adenomas and multiglandular disease remained unchanged in the three periods. In our material, the general development of the clinical appearance of PHPT seems to have taken only one decade. In the latest period PHPT has been diagnosed considerably more often than before, and the disease has usually been treated at an earlier stage. However, the severe forms of the disease have been diagnosed as frequently in all of the periods.

Adenoma↗

Hyperparathyroidism unmasked by the treatment of hypothyroidism secondary to Hashimoto's thyroiditis.

Primary hyperparathyroidism masked by untreated hypothyroidism was first noted by Kissin and Bakst in 1947; since then there has been only a single case reported. As a result, it has been thought to be a rare complex of parathyroid disease. During the past 17 years we observed 17 patients who developed primary hyperparathyroidism after their treatment for hypothyroidism (secondary to Hashimoto's thyroiditis) with thyroxine had been well underway. The patients were all women whose ages ranged between 16 and 76 years. The clinical and biochemical manifestations of hyperparathyroidism were noted 2 months to 30 years after thyroxine therapy. All patients underwent standard subtotal parathyroidectomy. Fourteen patients had a single parathyroid adenoma and three had multiglandular adenomatous hyperplasia. It is of interest that all 17 patients were found to have Hashimoto's thyroiditis, based on antimicrosomal antibody titers or histopathologic criteria. Thus we present a series of patients who developed primary hyperparathyroidism in a background of Hashimoto's thyroiditis and hypothyroidism treated with thyroxine. We are intrigued by the association of hyperparathyroidism and Hashimoto's chronic thyroiditis. From this experience it seems appropriate to evaluate parathyroid function in patients with hypothyroidism secondary to Hashimoto's thyroiditis before the initiation of treatment with thyroxine and at intervals thereafter.

Adenoma↗

Hyperparathyroidism in the old age.

In a well-defined geriatric population the prevalence of primary hyperparathyroidism was 1.5%. From a clinical material consisting of 400 patients treated surgically for primary hyperparathyroidism during a 10-year period 158 patients (38%) above the age of 64 were studied. Neuromuscular symptoms were present in 80%, renal insufficiency or kidney stones in each 16%, constipation and/or anorexia in 38%. 10% were considered clinically asymptomatic. In 80% the primary hyperparathyroidism was caused by a solitary adenoma. Normocalcaemia was achieved in 94%. Hypocalcaemia requiring vitamin D treatment occurred in 3%, and in 2% hypercalcaemia persisted or recurred. All of these had multiglandular parathyroid disease. The therapeutic effect of the surgical treatment was considered good or fair in most of the cases. No mortality occurred. Primary hyperparathyroidism occurs with a high prevalence in the elderly. It can be treated successfully by surgical therapy with a low cost of morbidity, mortality and medical care.

Adenoma↗

Primary hyperparathyroidism. Changing trends over fifty years.

Two groups of patients with PHPT, one (early group) operated on between 1930 and 1960, and the other (late group) from 1972 to 1981 were compared, in order to find out the changing trends in this disease during these years. The number of operated PHPT increased when serum calcium and phosphate were added to routine blood screening. The mean age of patients was lower in the early than in the late group. There was a decrease in the occurrence and severity of bone and renal disease and an increase in the number of asymptomatic patients in the late group. In the early group the serum calcium levels were higher, serum phosphate levels lower and parathyroid adenomas larger. The ratio between uni- and multiglandular parathyroid disease was similar in both groups of patients.

Adenoma↗

Clinical, biochemical, and pathologic features of radiation-associated hyperparathyroidism.

The clinical, biochemical, and pathologic findings in 43 patients with primary hyperparathyroidism (HPT) and a history of irradiation to the head or neck were compared with those found in a group of 162 patients with HPT without prior irradiation. There were no differences between the groups with regard to mean age at the time of diagnosis, male to female ratio, serum calcium, phosphorus, and parathyroid hormone concentrations; in the frequency of single parathyroid adenomas v multiglandular disease; or in associated nonthyroidal neoplasms. Asymptomatic HPT was found with greater frequency in the irradiated patients. Almost 80% of the patients with a history of irradiation had concomitant thyroid disease, primarily adenomas and carcinomas, while less than 50% of the patients without a history of irradiation had thyroid disease, mainly colloid nodules, at surgery.

Adenoma↗

[Familial and recurrent hyperparathyroidism. Apropos of 7 adenomas in 3 members of the same family. Review of the literature].

Over a period of 23 years, 3 members of a family of 5 presented with 7 parathyroid adenomas (4 in the first case, 2 in the second case, 1 in the third case). Excision of each adenoma, with systematic pre-operative assessment of the remaining parathyroid tissue, led to complete clinical and laboratory cure of each episode. The delay in the appearance of recurrence was between 3 and 9 years. After reviewing the literature, the authors stress the importance, in any case of hyperparathyroidism, of routinely investigating the serum calcium levels in members of the patient's family, especially if the patient is young and if he has had several episodes or a multiglandular involvement in the one episode. The authors discuss the literature concerning recurrent familial hyperparathyroidism with endocrine polyadenomatosis and the "hypercalcaemia - hypocalciuria" syndrome.

Adenoma↗

Hyperparathyroid crisis. Successful treatment of ten comatose patients.

Hyperparathyroid crisis is a rare disease manifested by elevated serum calcium, weakness, nausea and vomiting, altered states of consciousness, and elevated circulating parathormone. This hypercalcemic state is noted for a frequently acute presentation and associated high mortality rate, approaching 60% in some series. Ten patients in parathyroid crisis were observed in a consecutive personal series of 325 cases of operatively proved hyperparthyroidism. All 10 patients were successfully treated. Each patient remained or lapsed into persistent coma despite extensive medical management and normalization of serum calcium in some instances. An emergency parathyroidectomy was performed in all cases. Reversal of the comatose state was noted in all patients within 24 hours, followed by gradual normalization of serum calcium. Serum calcium ranged from 15 to 19.6 mg/dl. The blood urea nitrogen level was elevated in six patients. A single adenoma was found in nine patients and multiglandular disease involving the neck and the mediastinum in one. All patients survived. The successful treatment of this disease demands prompt and accurate diagnosis coupled with vigorous medical therapy and emergency parathyroidectomy if the patient's status continues to deteriorate.

Acute Disease↗

Persistent and recurrent sporadic primary hyperparathyroidism: histopathology, complications, and results of reoperation.

BACKGROUND: Our purpose was to analyze the causes of persistent and recurrent sporadic primary hyperparathyroidism (PD and RD). METHODS: The histopathology, complications, and results of reoperation were studied. Five hundred sixty-eight patients with primary hyperparathyroidism were operated on initially by one surgeon and underwent follow-up examination for 3.7 +/- 3.8 years. During the operation, all parathyroids were sought and confirmed by biopsy. Enlarged glands were resected, and subtotal parathyroidectomy was done for multiglandular disease (hyperplasia). RESULTS: The cure rate after the initial surgical procedure was 96.4%, PD = 2.8% (16 of 568). At reoperation (10 of 16), nine of 10 were cured (90%) (two adenomas, six hyperplasias, one lung carcinoma). RD was documented (at years 4, 4, 10, 15, 16) in five (0.9%) patients, one with parathyroid carcinoma and four with hyperplasia. Thirty-five patients with PD and two patients with RD were referred for reoperation: 17 with adenomas (eight mediastinal) and 18 with hyperplasias (one mediastinal gland). Preoperative calcium level was higher for PD (12.57 mg/dl) and RD (13.89 mg/dl) versus all cases (12.19 mg/dl) (p < 0.03 and p < 0.0005, respectively). After reoperation, normocalcemia was achieved in 47 (92.%) of 51 patients with PD or RD. Transient hypocalcemia occurred in 22% of patients (permanent, 2.0%) and transient hoarseness in 2.0% of patients (no permanent nerve damage). Permanent hypocalcemia and nerve damage after 568 initial operations were 0% and 0%, respectively. Two perioperative deaths occurred. CONCLUSIONS: We conclude that inadequate neck exploration or resection of hyperplastic tissue accounts for most cases of PD and RD. Optimal results necessitate intraoperative identification of all parathyroids whenever possible, with minimal morbidity.

Adult↗

Results and complications of the surgical treatment of primary hyperparathyroidism.

Between the late 1960's and 1980, the operation for primary hyperparathyroidism at The University of Chicago changed from subtotal parathyroidectomy for all patients to removal of an adenoma with performance of biopsies of all other normal glands. After 1980, our technique was again modified to bilateral neck exploration, resection of the adenoma when it was present, and performance of fewer biopsies of normal glands (usually one and sometimes two). Between 1980 and 1990, 308 parathyroidectomies were performed; 288 patients underwent first operations. Two hundred forty-five (85.1%) of these patients had an adenoma and forty-three (14.9%) had hyperplasia (multiglandular disease); none had a carcinoma. Resolution of hypercalcemia with the first operation was achieved in 281 patients (97.5%); seven patients experienced failed explorations. The early cure was the same whether or not preoperative localization studies were performed. Biopsy of fewer normal parathyroid glands when an adenoma was present resulted in a decreased incidence of transient postoperative hypocalcemia. Nineteen patients underwent 20 reoperative parathyroidectomies during this period. Preoperative localization studies; done in 16 (80%) of 20 cases, were very helpful. Ninety percent of patients with abnormal parathyroid glands in their neck or mediastinum were cured with their initial reoperation.

Humans↗

Success of unilateral neck exploration for sporadic primary hyperparathyroidism.

BACKGROUND: Unilateral neck exploration for primary hyperparathyroidism (PHPTH) is controversial because of concern about missed multiglandular disease. METHODS: In patients with sporadic PHPTH our approach has been arbitrarily to surgically explore the right neck first unless preoperative or intraoperative evidence suggests left-sided parathyroid pathologic condition. When an adenoma is identified and a normal ipsilateral gland is confirmed on histologic examination, the contralateral side is not explored. A 15-year series of 371 patients with sporadic PHPTH was reviewed retrospectively to determine the efficacy of this strategy. RESULTS: At operation unilateral exploration was possible in 125 patients (34%). Of these 122 had a single adenoma and two patients had parathyroid carcinoma. One patient with unrecognized hyperplasia required subsequent subtotal parathyroidectomy. There were no major complications. Bilateral surgical exploration was required in 246 patients including 18 with hyperplasia, 4 with double adenomas, and 4 with parathyroid cancer. Bilateral surgical exploration failed in 18 patients (14 persistent and 4 recurrent PHPTH) and was complicated by one recurrent nerve palsy and two cases of permanent hypocalcemia. Operative time was significantly shorter in the unilaterally explored group. CONCLUSIONS: When possible, unilateral exploration for PHPTH is safe and effective, avoids obliterative scarring of the contralateral neck, and may reduce morbidity and operative time.

Adenoma↗

Preoperative ultrasound in patients undergoing initial neck exploration for primary hyperparathyroidism.

92 patients with primary hyperparathyroidism were subjected prospectively to preoperative ultrasound to identify enlarged parathyroid glands and detect simultaneous thyroid lesions of surgical significance. The overall sensitivity and specificity rates were 62.7% and 95.7%, 85.2% and 96.8% for patients with a single adenoma, 44.3% and 88% for those with multiglandular disease, 56.8% and 95.6% for those with simultaneous thyroid disease and 20% and 100% for mediastinal glands. Ultrasound revealed a pathological thyroid gland in 26 patients (28%). The sensitivity and specificity rates were 96% and 100%. The thyroid lesion was considered clinically significant in 15 of the 92 patients (16.3%) and only in three patients (3.3%) was the diagnosis not established preoperatively. Operating room times and costs were significantly lower for patients with an accurate ultrasound scan than for those with an inaccurate scan (P < 0.001). We conclude that both the sensitivity of ultrasound for locating enlarged parathyroid glands and its yield of previously unknown thyroid lesions of surgical significance are too low to warrant preoperative ultrasound evaluation. Although our operating room expenses were significantly higher for patients with an inaccurate scan, the cost effectiveness of preoperative ultrasound cannot be proved until a prospective, randomised investigation has been carried out.

Cost-Benefit Analysis↗

[Reoperations in parathyroid surgery].

In a continuous series of 439 parathyroidectomies for primary or secondary hyperparathyroidism, 36 cases were reoperations. The initial operation had been a prior parathyroid exploration (PTx) in 17 cases, a thyroidectomy (Tx) in 17 cases, both operations in two cases. The incidence of parathyroid glands discovered at exploration was lower in both types of reoperations (67% after Tx, 72% after PTx) than in patients who were explored for the first time (94%). Anatomical variations and parathyroid pathologies were comparable in patients reoperated after Tx and in those explored for the first time. In patients reoperated after PTx, anatomical abnormalities (46% of glands in ectopic locations, 12% of supernumeray glands) and multiglandular pathologies (56% of hyperplasias) were much more frequently encountered. Long term results were satisfactory in patients explored for the first time (98% cures) and in reoperations after Tx (94% cures), but were less favourable in reoperations after PTx (78% cures).

Adult↗