Prognostic scoring systems in patients with follicular thyroid cancer: a comparison of different staging systems in predicting the patient outcome.

BACKGROUND: The use of prognostic scoring systems is important for predicting the survival of individuals with thyroid carcinoma. Relatively few studies have addressed this issue for patients with follicular thyroid cancer. The goal of this retrospective study was to establish the best and most pertinent prognostic scoring system to predict survival in patients with follicular thyroid cancer. METHODS: We selected 86 patients with follicular thyroid cancer treated at University of California, San Francisco (UCSF) hospitals from January 1954 to April 1998. The mean follow-up time was 11.5 years. There were 60 women (70%) and 26 men (30%), with a mean age if 48.6 years. Prognostic scoring systems included tumor, node, metastases (TNM), European Organization for Research and Treatment of Cancer (EORTC), Age, Grade, Extent, Size (AGES), Age, Metastases, Extent, Size (AMES), and the Metastases, Age, Completeness of resection, Invasion, Size (MACIS). Survival time was calculated using the Kaplan-Meier method. Using Cox proportional hazards analysis, the relative importance of each scoring method was determined by calculating the proportion of variation in survival time explained (PVE). RESULTS: Kaplan-Meier analysis indicated that all scoring systems were significant predictors of survival time (p < 0.0001). The PVE associated with each system was (from highest to lowest) 0.48 for MACIS, 0.46 for AGES, 0.44 for EORTC, 0.40 for AMES, and 0.33 for TNM. These results indicate that the MACIS scoring system accounted for a great proportion of explained variance in survival and is a more precise predictor of survival compared to the other scoring systems. CONCLUSIONS: TNM, EORTC, AGES, AMES, and MACIS, all provided useful prognostic information about the survival in our 86 patients with follicular thyroid cancers. The MACIS classification, however, was the most accurate predictor using PVE as a method of evaluation. Future scoring systems considering additional prognostic factors, may obtain a higher PVE.

Low-protein diet and progression of retinal degeneration in gyrate atrophy of the choroid and retina: a twenty-six-year follow-up.

Gyrate atrophy of the choroid and retina is an autosomal recessive chorioretinal dystrophy which leads to a slowly progressive loss of vision. The primary defect is due to a deficiency of the enzyme ornithine delta-aminotransferase, which is responsible for markedly elevated levels of ornithine in plasma and other body fluids. Although several therapeutic regimens have been proposed, the reduction in ornithine accumulation obtained by reducing the intake of its precursor arginine (semisynthetic low-arginine diet) is the one most practised. In this clinical and molecular study we report a patient with hyperornithinaemia and gyrate atrophy of the choroid and retina who had been diagnosed when she was 3 years 9 months old. She also presented mild mental retardation, delayed language development and speech defects. The patient has recently been found to be homozygous for the new Gly91Arg amino acid substitution of the enzyme ornithine delta-aminotransferase. This mutation lies in a region of the mature protein that is considered crucial for the mitochondrial targeting activity. In this patient, a 28-year treatment with a completely natural low-protein diet (0.8 g/kg per day of natural protein) has been able to significantly reduce ornithine plasma levels, and to greatly delay the natural progression of the chorioretinal changes. This study suggests that, in the long-term treatment of gyrate atrophy, the efficacy in slowing the progression of chorioretinal changes and the palatability of a completely natural low-protein diet make this treatment a potentially viable alternative in patients refusing the semisynthetic diet.

Laparoscopic antireflux surgery: preoperative lower esophageal sphincter pressure does not affect outcome.

BACKGROUND: Concern has been raised about operating on patients with gastroesophageal reflux disease (GERD) and normal lower esophageal sphincter (LES) pressure for the fear that a fundoplication may fail to control reflux and result in a high rate of postoperative dysphagia. We hypothesized that fundoplication is effective in patients with GERD irrespective of the preoperative LES pressure, and that in patients with normal LES pressure, a total fundoplication does not result in a high incidence of dysphagia. METHODS: We studied 280 unselected patients with GERD who underwent laparoscopic fundoplication. They were divided in three groups based on the preoperative LES pressure (normal, 14-24 mmHg): group A (LES pressure, 0-6 mmHg; 61 patients; 22%); group B (LES pressure, 7-13 mmHg; 178 patients; 64%); group C (LES pressure, >or=14 mmHg; 41 patients; 14%). De novo dysphagia was defined as new onset of postoperative dysphagia lasting more than 10 weeks. The average follow-up period was 17 +/- 22 months. RESULTS: There was no difference in resolution of symptoms among the three groups. Heartburn and regurgitation resolved or improved respectively in 96% of group A, 90% of group B, and 91% of group C patients. In addition, there was no difference in the incidence of de novo dysphagia, which occurred in 8% of group A, 7% of group B, and 2% of group C. CONCLUSIONS: We conclude that fundoplication controlled GERD irrespective of preoperative LES pressure, and that a normal LES pressure before surgery was not associated with a higher rate of postoperative dysphagia.

Death from thyroid cancer of follicular cell origin.

BACKGROUND: Although patients with differentiated thyroid cancer (DTC) of follicular cell origin usually have an excellent prognosis, some patients die from progressive tumor. Numerous postoperative criteria have been used to predict prognosis in patients with DTC. The purpose of this investigation was to determine whether the TNM and metastases, age, completeness of resection, invasion, size (MACIS) classifications predicted survival time and why patients died from DTC. The extent of initial treatment and causes of death were also evaluated in these patients who died from thyroid cancer. STUDY DESIGN: Between 1965 and 1995, 102 of 1,224 patients with DTC treated at the University of California at San Francisco (UCSF) and UCSF/Mount Zion Medical Centers died from DTC. Risk factors including age at diagnosis, gender, histologic characteristics, TNM and MACIS classifications, the intervals among initial treatment, recurrence, and death, and the initial and subsequent treatments were documented in these 102 patients. RESULTS: Among the 102 patients who died of DTC 50% were men and 50% were women. The mean age of patients with DTC at diagnosis was 58 years at recurrence, 62 and 65 years at death. Thirty percent of these patients initially had unilateral thyroid operations and 70% had a bilateral operation. Tumors at presentation ranged from 0.6 to 13.0 cm (mean 4.4 cm); 46% of patients presented with late-stage tumors (TNM stage III, IV; MACIS score > 8). At presentation 46% of the patients had locally recurrent disease or regional metastases and 18% had distant metastases. Patients with persistent disease had a significantly shorter survival time than those with recurrent disease (p < 0.001). Both TNM and MACIS classifications were good predictors of survival time. Reoperations were performed in 51% of papillary, 26% of follicular, and 67% of Hürthle cell thyroid cancer patients. Fifty percent of patients with papillary thyroid cancer, 50% of patients with Hürthle cell thyroid cancer, and 11% of patients with follicular cell thyroid cancer died of locally advanced disease. CONCLUSIONS: As expected, patients with local or regional recurrence and those with TNM stage I or MACIS score < 6 survived longer than patients with distant metastasis and TNM stage III or IV, MACIS score > 6, but some patients thought to be at low risk (TNM stage I; MACIS < 6) also died from thyroid cancer.

Hyperparathyroidism after thyroid surgery and autotransplantation of histologically normal parathyroid glands.

BACKGROUND: Parathyroid autotransplantation is a well-established method to prevent hypoparathyroidism during parathyroid and thyroid operations. The reported success rate of parathyroid autotransplantation ranges from 75% to 100%. Recurrent hyperparathyroidism may develop after parathyroid autotransplantation, especially after the transplantation of hyperplastic or adenomatous parathyroid tissue. Hyperparathyroidism recurs most frequently after subtotal parathyroidectomy or total parathyroidectomy and autotransplantation, in patients with renal failure and secondary hyperparathyroidism, and in patients with familial primary hyperparathyroidism or MEN I or MEN II syndrome. We report three patients who experienced primary hyperparathyroidism after autotransplantation of normal parathyroid tissue during thyroid operations (two patients) or after a long period of hypoparathyroidism. STUDY DESIGN: We reviewed our records from 1983 to May 1998 and identified three patients in whom hyperparathyroidism developed after thyroid operations. RESULTS: One patient had a thyroidectomy with left modified radical neck dissection for papillary thyroid cancer, followed by radioiodine ablative therapy. Two patients had thyroid operations for benign thyroid disease. One of these patients had a history of radiation exposure for acne, and in the other one secondary hyperparathyroidism arose 6 years after a thyroidectomy for hyperthyroidism. CONCLUSIONS: Our study documents that hyperparathyroidism may develop after autotransplantation of histologically normal parathyroid tissue and after a period of hypoparathyroidism after thyroid operations. For this reason, it is important to mark the site of the parathyroid transplantation.

Classification and treatment of follicular thyroid neoplasms are discordant between and within medical specialties.

BACKGROUND: The histologic criteria to classify follicular thyroid neoplasms are controversial. Criteria used for diagnosis and treatment varies both within and between specialty groups. This discordance makes it difficult to compare disease and management practice. This is especially problematic in issues concerning reoperations and survival. To determine the degree of disparity, we surveyed 3 groups of specialists. METHODS: A questionnaire describing 10 histologic scenarios was sent to an equal number of thyroidologists, endocrine surgeons, and endocrine pathologists. Individuals were randomly selected from rosters of 3 corresponding societies. Each item asked for a rating of a diagnosis and treatment. Questionnaires were distributed and received by facsimile, and responses were kept confidential. The response rate was 60%. RESULTS: Responses were analyzed by nonparametric statistical tests. Two scenarios had significant disagreement among specialties in both diagnosis and treatment: one scenario involved the assessment of neoplasms with minimal capsular invasion; the other scenario involved Hürthle cell features. In both scenarios pathologists tended to be more conservative in assigning the term carcinoma and recommending total thyroidectomy. Significant disagreement within specialty groups was also noted. Two other scenarios dealt with the distinction between minimally and widely invasive carcinoma; significantly, pathologists viewed tumors as less invasive. CONCLUSIONS: This study indicates that much disparity exists among specialists in pathology, endocrinology, and surgery and among experts in each of these disciplines. It highlights that there is no uniform classification. If multicenter trials to evaluate treatment options are to occur, a universal classification must be accepted.

Surgical management of benign thyroid disease.

The most recent surgical approaches to benign uninodular, multinodular, normo and/or hyperfunctioning thyroid disease are considered.

[Widespread endemic goiter and iodine deficiency in the province of Avellino]. / Diffusione del gozzo endemico e della carenza iodica in provincia di Avellino.

1352 schoolchildren between 6-14 years old (699 males and 653 females) and 943 adults (176 males and 767 females) from eight villages of the province of Avellino were studied. All subjects were examined for thyroid size by at least two expert examiners. In most of them urine samples were collected for iodine determinations. 387 schoolchildren and 161 adults from Flumeri and Villanova were evaluated by thyroid echography. The prevalence of goiter was from 23.5 to 52.2% and the median urinary iodine excretion was from 42.3 to 66.2 micrograms/l in schoolchildren. In adults the prevalence of goiter was from 41.2 to 86.7% and the median urinary iodine excretion was from 37.1 to 53.7 micrograms/l. Our data showed a degree of iodine deficiency from low to moderate. The echography permitted to point out a greater prevalence of nodules than the thyroid palpation.

[Short- and long-term evaluation of treatment of critical ischemia of the lower lim with PGE1 (alprostadyl alpha-cyclodestrine)]. / Valutazione a breve e lungo termine del trattamento con PGE1 (alprostadil alpha-ciclodestrina) nell'ischemia critica degli arti inferiori.

Twenty-five patients affected by chronic lower limb obliterating arteriopathy with critical ischemia in one limb were treated with PGE1 for 4 weeks and then followed-up for one year. On day 14 of treatment three groups of patients were selected on the basis of clinical symptoms and instrumental tests; patients were subdivided into Responders, Partial Responders and Non-Responders. The results obtained were satisfactory in view of the fact that the selection into three groups enabled Partial Responders to be reclassified, in some cases by repeating the treatment cycle.