Prognostic influence of clinical and pathological factors in medullary thyroid carcinoma: a study of 53 cases.

UNLABELLED: OBJECTIVES AND INTRODUCTION: Medullary thyroid carcinoma, a neoplasia of intermediate prognosis and differentiation, does not always respond predictably to known treatments. This study aimed to correlate the clinical progression of surgically treated patients with clinical and pathological data. METHODS: A total of 53 patients were followed for 75 months (mean average) in tertiary-care hospital. The clinical status of patients at the end of the study period was characterized to determine correlations with a range of disease aspects. A value of p < 0.05 was considered statistically significant. RESULTS: Twenty-two patients (41.5%) were alive and disease-free at the end of the follow-up period; twenty-three patients (43.4%) had persistent disease; and eight patients (15.1%) had recurrent disease. Four patients (7.6%) died from medullary thyroid carcinoma with clinical and/or imaging evidence of neoplasia. The following aspects demonstrated statistically significant correlations with the final medical condition: positive initial cervical examination (p = 0.002); neoplastic extensions to the thyroid capsule (p = 0.004) and adjacent tissues (p = 0.034); cervical lymph node metastases (p < 0.001); diameter of neoplasia (p = 0.018); TNM (tumor, node and metastasis) Stage (p = 0.001) and evidence of distant and/or cervical diseases in the absence of a cure (p = 0.011). Through logistic regression, the presence of cervical lymph node metastases was considered an independent variable (p < 0.001). CONCLUSIONS: Clinical and pathological aspects of patients with surgically treated medullary thyroid carcinomas are predictors of disease progression. Specifically, even treated cervical lymph node metastases are significantly correlated with disease progression.

Cáncer de tiroides pediátrico: serie de casos / Retrospective review of pediatric patients with thyroid cancer

Background: In childhood, thyroid cancer is uncommon and has a different biological behaviour than in adults. Aim: To analyze the surgical experience in thyroid cancer in children. Material and Methods: Review of medical records of patients aged 15 years or less with a thyroid cancer, operated in a Regional hospital between 1980 and 2007. Results: Six females and 2 males, aged 9 to 15 years, were operated in the study period. Five had a family history of thyroid diseases. Four had involvement of cervical lymph nodes at the moment of operation but none had systemic dissemination. All were euthyroid. A total thyroidectomy was performed in four patients. Cervical lymph node dissection was also performed in four patients. No immediate complications were recorded. One patient had a local relapse. Five patients were also treated with radioiodine. At the end of the follow up ranging from 108 to 320 months, all patients are asymptomatic. The pathological study disclosed a papillary carcinoma in seven and medullary carcinoma in one patient. Conclusions: Thyroid cancer in childhood has low mortality rates and surgical treatment is safe and effective.

El cáncer de tiroides en edad pediátrica es extremadamente infrecuente y tiene un comportamiento biológico diferente al adulto. El objetivo de este estudio es analizar la experiencia quirúrgica local en los últimos 27 años. Estudio de serie de casos describiendo las características anatomoclínicas, quirúrgicas, resultados del tratamiento y condición actual de los pacientes analizados mediante estadística descriptiva, realizando curva de sobrevida con método de Kaplan-Meier. Se intervinieron quirúrgicamente 8 pacientes por cáncer tiroideo, 6 del sexo femenino y 2 del masculino, con edad promedio de 13 años (9 a 15 años). Cinco tenían antecedentes familiares de patología tiroidea. Cuatro presentaron al momento del diagnóstico linfoadenopatías metastáticas cervicales, ninguno evidenció diseminación sistémica. Todos presentaban estado eutiroideo. El diagnóstico histopatológico concluyó carcinoma papilar en 7 casos y medular en 1. Se realizó tiroidectomía total en 4 pacientes. En 4 se practicó linfadenectomía cervical. No hubo complicaciones postoperatorias inmediatas. En 5 se complementó con radioyodoterapia. Una paciente desarrolló recidiva regional. Al término del estudio 7 llevan vida normal. Una paciente con carcinoma medular esporádico falleció siete años después de la cirugía, a causa de metástasis pulmonares y hepáticas. El seguimiento promedio de los cánceres papilares fue de 165 meses (108 a 320 meses). El cáncer de tiroides en edad pediátrica se asocia a una supervivencia prolongada, incluso en etapas avanzadas. La extensión de cirugía en los carcinomas papilares debe considerar factores pronósticos como tamaño y extensión del tumor primario, presencia o ausencia de metástasis y ciertamente, experiencia y buen juicio del equipo quirúrgico.

Prognostic influence of clinical and pathological factors in medullary thyroid carcinoma: a study of 53 cases

OBJECTIVES AND INTRODUCTION: Medullary thyroid carcinoma, a neoplasia of intermediate prognosis and differentiation, does not always respond predictably to known treatments. This study aimed to correlate the clinical progression of surgically treated patients with clinical and pathological data. METHODS: A total of 53 patients were followed for 75 months (mean average) in tertiary-care hospital. The clinical status of patients at the end of the study period was characterized to determine correlations with a range of disease aspects. A value of p < 0.05 was considered statistically significant. RESULTS: Twenty-two patients (41.5 percent) were alive and disease-free at the end of the follow-up period; twenty-three patients (43.4 percent) had persistent disease; and eight patients (15.1 percent) had recurrent disease. Four patients (7.6 percent) died from medullary thyroid carcinoma with clinical and/or imaging evidence of neoplasia. The following aspects demonstrated statistically significant correlations with the final medical condition: positive initial cervical examination (p = 0.002); neoplastic extensions to the thyroid capsule (p = 0.004) and adjacent tissues (p = 0.034); cervical lymph node metastases (p < 0.001); diameter of neoplasia (p = 0.018); TNM (tumor, node and metastasis) Stage (p = 0.001) and evidence of distant and/or cervical diseases in the absence of a cure (p = 0.011). Through logistic regression, the presence of cervical lymph node metastases was considered an independent variable (p < 0.001). CONCLUSIONS: Clinical and pathological aspects of patients with surgically treated medullary thyroid carcinomas are predictors of disease progression. Specifically, even treated cervical lymph node metastases are significantly correlated with disease progression.

Renal medullary carcinoma: report of seven cases from Brazil.

We report seven cases of renal medullary carcinoma collected from several institutions in Brazil. In spite of a relatively high incidence of sickle cell trait in Brazil, this is a rare tumor. All patients were males between the ages of 8 and 69 years (mean 22 years). From the collected information, the most frequent presenting symptoms were gross hematuria and flank or abdominal pain. The duration of symptoms ranged from 1 week to 5 months. Most of the tumors were poorly circumscribed arising centrally in the renal medulla. Size ranged from 4 to 12 cm (mean 7 cm) and hemorrhage and necrosis were common findings. All seven cases described showed sickled red blood cells in the tissue and six patients were confirmed to have sickle cell trait. All cases disclosed the characteristic reticular pattern consisting of tumor cell aggregates forming spaces of varied size, reminiscent of yolk sac testicular tumors of reticular type. Other findings included microcystic, tubular, trabecular, solid and adenoid-cystic patterns, rhabdoid-like cells and stromal desmoplasia. A peculiar feature was suppurative necrosis typically resembling microabscesses within epithelial aggregates. The medullary carcinoma of the 69-year-old patient was associated with a conventional clear cell carcinoma. To our knowledge, this association has not been previously reported and the patient is the oldest in the literature. The survival after diagnosis or admission ranged from 4 days to 9 months. The 8-year-old African-Brazilian patient with a circumscribed mass is alive and free of recurrence 8 years after diagnosis. This case raises the question whether a periodic search for renal medullary carcinoma in young patients who have known abnormalities of the hemoglobin gene and hematuria could result in an early diagnosis and a better survival.

Medullary thyroid carcinoma: multivariate analysis of prognostic factors influencing survival.

BACKGROUND: Medullary thyroid carcinoma (MTC) is a rare development of thyroid cancer with a no negligible mortality rate. Our aim was to determine factors that predict outcome in patients with MTC. METHODS: We reviewed the records of all patients with MTC (n=56) who underwent treatment at our institution between January 1990 and December 2000. Univariate and multivariate analysis of clinicopathologic predictors of MTC outcome were performed to identify subsets of patients with different probabilities in terms of overall survival, local recurrence, and distant metastases. RESULTS: Multivariate analysis demonstrated that a statistically significant decrease in overall survival is associated with T4b tumours (p=0.06), the presence of distant metastases at the time of presentation (p=0.033), lymphatic invasion (p=0.099), and postoperative treatment (p=0.045). CONCLUSIONS: The analysis of survival curves of patients with MTC shows that the occurrence of locoregional and distant metastases occurs preferentially within the first 5 years, which identifies this as a crucial period for follow-up. In this series of patients with MTC, the tumours classified as T4b, metastases at presentation, the presence of lymphovascular invasion, and postoperative treatment were the most important prognostic features. At present, there is no available beneficial adjuvant therapy. However, as the development of molecular therapy progresses, it should be tested in clinical trials with the purpose of achievement of novel targeted therapies for selected MTC patients with risk factors.

Long-term survival rates in young patients with thyroid carcinoma.

BACKGROUND: Thyroid carcinoma in patients younger than 18 years is rare. It is associated with a greater risk of metastases. However, the prognosis for these patients is better when compared with that of adults. OBJECTIVE: To present the experience of a single institution in the treatment of patients with thyroid carcinoma during childhood and adolescence. PATIENTS AND METHODS: Thirty-eight patients, ranging in age from 4 to 18 years, were diagnosed as having thyroid carcinoma. Pathologic types of carcinoma included 29 papillary, 4 follicular, 1 Hürthle cell, and 4 medullary cases. RESULTS: Hypocalcemia was the main complication, being transitory in 9 patients (24%) and permanent in 6 patients (16%). Vocal cord palsy occurred in 2 patients (5%). Two patients (5%) had a surgical site infection. After a mean follow-up of 9.5 years (range, 1-40 years), 28 patients (74%) were alive and had no evidence of disease, 3 (8%) were alive and had recurrent disease, 4 (11%) died (2 of the disease and 2 of non-cancer-related causes), and 3 (8%) were lost to follow-up. The survival rates at 10 years for the patients with papillary, follicular, and medullary carcinoma were 93%, 100%, and 50%, respectively. CONCLUSIONS: Thyroid carcinoma in patients younger than 18 years has a good prognosis even in the presence of neck or distant metastasis. Total thyroidectomy, associated with adjuvant radioactive iodine therapy and thyroidal suppression or not, is effective in patients with a well-differentiated thyroid carcinoma.

Multifactorial analysis of survival and recurrences in differentiated thyroid cancer. Comparative evaluation of usefulness of AGES, MACIS, and risk group scores in Mexican population.

Many risk factors have been identified in differentiated thyroid cancer, with them, some prognostic scores have been designed to asign the risk of recurrence and survival. In Mexican population, this type of study is scarce. This is a retrospective review of 180 patients with differentiated thyroid cancer completely treated at the Hospital de Oncologia, IMSS, in Mexico City from 1980 to 1990. All prognostic factors were analyzed and a score obtained either by the method of AGES, MACIS, or SKMH. Correlation of recurrences and survival was carried out according to score or risk assignment. There was a predominance of females (4.8:1), 48% had metastatic cervical nodes, median tumor size was 4 cm, 16% had multiple macroscopic thyroid tumors, in 12% resection was incomplete, 96% were papillary, and 4% follicular cancers. According to AGES, 46% were high risk patients, 49.4% with MACIS and 45.5% with SKMH, respectively. Median follow-up was 8.3 years. There were 67 (37%) recurrences. Ten-year overall survival was 89.4% and disease-free survival was 79.2%. There was no statistical significant difference of survival of AGES until the score reached 6 or more or the MACIS score reached 8 or more. Cox multivariate model showed that above the age of 45, tumor size of 5 cm or more, follicular histology, multiple macroscopic thyroid tumors, and extracapsular node invasion affected ten-year survival. In conclusions our patients are diagnosed at more advanced stages than patients in the U.S. or European countries. Nearly one half of our patients belonged to the high-risk group. This study confirms that patients over the age of 45, tumor size > 5 cm, and follicular histology are adverse prognostic factors and report that extracapsular node invasion and multiple macroscopic thyroid tumors are also adverse prognostic factors. In Mexican population, with 50% of high-risk patients, AGES and MACIS scores reached statistical differences with higher qualifications than observed in the U.S.

Allelic loss on distal chromosome 17p is associated with poor prognosis in a group of Brazilian breast cancer patients.

We examined loss of heterozygosity (LOH) for two loci on chromosome 17p (D17S5 and TP53), and erbB-2 gene amplification, in primary breast cancers from 67 Brazilian patients. We identified two distinct regions of LOH on chromosome 17p, one spanning TP53 and the other a more telomeric region (D17S5). Based on a short-term follow-up, Kaplan-Meier analyses of patients' disease-free survival showed that patients with LOH for D17S5, but retaining heterozygosity for TP53, were at higher risk of recurrence (P = 0.007) than those who retained heterozygosity for D17S5. Bivariate analyses indicated that patients with LOH for D17S5 alone had an increased risk of recurrence (hazard ratio = 7.2) over patients with erbB-2 amplification (hazard ratio = 3.7), when compared with patients with neither alteration (hazard ratio = 1.0). Further, lymph node-positive patients whose tumours had both LOH for D17S5 and erbB-2 gene amplification had a higher risk of recurrence than patients whose tumours had neither of these genetic alterations. Our data confirm previous reports of a putative tumour-suppressor gene, distinct from TP53, on distal chromosome 17p which is associated with breast cancer. They further suggest that LOH for loci in this region may provide an independent indicator to identify patients with poor prognosis.