Carcinoma medular esporádico de tiroides / Sporadic Medullary Thyroid Carcinoma

Introducción: El carcinoma medular de tiroides, representa aproximadamente entre el 5 - 10 por ciento de todos los carcinomas tiroideos, aparece con más frecuencia entre los 25 y 60 años y en el sexo femenino. Se distinguen dos tipos: el esporádico (no hereditario) y el familiar (hereditario). La localización más frecuente es la unión del tercio superior de lóbulo tiroideo con el tercio medio, que es la zona de mayor concentración de células C. Objetivo: Presentar el caso de paciente masculino operado de carcinoma medular de tiroides, tipo esporádico, en la provincia de Cienfuegos. Caso clínico: Paciente masculino de 60 años de edad, piel blanca, que acudió a la consulta; refiere aumento de volumen del cuello acompañado de disfonía y disfagia a los alimentos sólidos. Al examen físico se constató la presencia del nódulo tiroideo. Se le realizó ultrasonido que corrobora la presencia de un nódulo en el lóbulo derecho del tiroides. La biopsia por aspiración con aguja fina informó el nódulo como sospechoso de malignidad. Se le realizó tiroidectomía total, informándose por la biopsia por parafina de la pieza como un carcinoma medular del tiroides. En la actualidad lleva 6 meses de operado con evolución favorable. Conclusiones: El carcinoma medular de tiroides constituye una entidad rara y agresiva más frecuente en los pacientes mayores de 45 años, cuyo tratamiento de elección es la cirugía(AU)

Introduction: Medullary thyroid carcinoma accounts for approximately 5-10 percent of all thyroid carcinomas. It appears more frequently at ages 25-60 years and in females. Two types are distinguished: sporadic (nonhereditary) and familial (hereditary). The most common location is the union of the upper third of the thyroid lobe to the middle third, the area with the highest concentration of C cells. Objective: To present the case of a male patient operated on for medullary thyroid carcinoma, of sporadic type, in the Cienfuegos Province. Clinical case: A 60-year-old male patient of white skin attended consultation. He reported an increase in neck volume accompanied by dysphonia and dysphagia for solid food. The physical examination revealed the presence of the thyroid nodule. Ultrasound was performed, which confirmed the presence of a nodule in the right lobe of the thyroid. Fine needle aspiration biopsy reported the nodule as suspicious for malignancy. A total thyroidectomy was performed, after which, paraffin biopsy of the specimen permitted to report a medullary carcinoma of the thyroid. At present, he has been operating for six months, with favorable evolution. Conclusions: Medullary thyroid carcinoma is a rare and aggressive entity, more frequent in patients over 45 years of age, whose treatment of choice is surgery(AU)

Carcinoma medular de tiroides: experiencia de 20 años / Medullary thyroid carcinoma: 20 years experience

Background: Medullary thyroid carcinoma (MTC) is a rare malignant tumor that arise from C cells. Surgical treatment and its results are controversial, so we decided to study it. Aim: To describe clinically MTC, treatment and outcomes in the long term. Material and Method: We retrospectively reviewed medical records of patients with MTC operated in our hospital between the years 1987 and 2007. We analyzed the cli-nical characteristics, treatment, morbidity and long-term follow up. Results: There were 24 patients operated with a mean age of 46.1 +/- 16.6 years. The main form of presentation was painless increased cervical volume (56.2 percent). In 15 percent this pathology was part of a MEN 2b. All of them have had a total thyroidectomy, which was extended in 50 percent of cases. The 35.2 percent were multifocal, 29.4 percent bilateral and 62.5 percent had metastatic lymph node involvement. Five patients remained higher calcitonin levéis in the postoperative period and 9 patients recurred clinically on average 4.5 years after surgery. The presence of persistent disease was significantly associated with hereditary MTC (p = 0.0088) and the clinical recurrence was significantly determined by the presence of not expanded total thyroidectomy (p = 0.0196). The probability of surviving more than 19 years was 66.6 percent (95 percent CI = 0.24 to 0.89). Conclusions: The MTC is a rare tumour and treatment of choice is surgery. The persistent disease is associated with hereditary MTC form, and the clinical recurrence is associated with not expanded total thyroidectomy. We recommend total thyroidectomy with central voiding and radical modified jugular dissection.

Antecedentes: El carcinoma medular de tiroides (CMT) es un tumor maligno poco frecuente, originado a partir de las células C. Su tratamiento quirúrgico y resultados son controvertidos, por lo que hemos decidido estudiarlo. Objetivo: Describir clínicamente el CMT, tratamiento y resultados a largo plazo. Material y método: Se revisaron retrospectivamente las fichas clínicas de pacientes con CMT operados en nuestro hospital entre 1987 y el 2007. Se analizaron las características clínicas, tratamiento, morbilidad y seguimiento a largo plazo. Resultados: Se operaron 24 pacientes, cuya edad media fue 46,1 +/- 16,6 años. La principal forma de presentación fue aumento de volumen cervical (56,2 por ciento). Un 15 por ciento formaba parte de una NEM 2b. A todos se les realizó una tiroidectomía total, ampliada en el 50 por ciento de los casos. El 35,2 por ciento eran multifocales, el 29,4 por ciento bilaterales y el 62,5 por ciento tenía metástasis ganglionar. Cinco pacientes mantuvieron niveles de calcitonina elevados en el postoperatorio y nueve pacientes recurrieron clínicamente, en promedio, a los 4,5 años. La enfermedad persistente se asoció significativamente con CMT hereditario (p = 0,0088) y la recurrencia clínica a tiroidectomía total no ampliada (p = 0,0196). La probabilidad de sobrevivir más de 19 años fue 66,6 por ciento (IC 95 por ciento = 0,24 a 0,89). Conclusiones: EL CMT es un tumor raro cuyo tratamiento de elección es la cirugía. La persistencia de enfermedad se asocia con la forma hereditaria, y la recurrencia clínica con la tiroidectomía total no ampliada, lo que nos hace recomendar una tiroidectomía total asociada a vaciamiento central y disección yugular radical modificada.

Endocrine cancer in Iran: based on cancer registry system.

BACKGROUND: A population-based registry of endocrine cancer cases in four Iranian provinces, was performed for the years 1996-2000. MATERIALS AND METHODS: Patients in each province were grouped according to age, gender and tumor specifics (site, morphology, behavior) and the data was coded according to the international classification of diseases for oncology. STATISTICAL ANALYSIS USED: Person-years of population at risk were calculated and the results were presented as incidence rates by sex, age, age specific rates and age standard rate (ASR) per 100,000 person-years, using direct method of standardization to the world population. RESULTS: A total of 319 cases of primary endocrine cancer were found and registered, including 313 cases of thyroid carcinoma and 6 cases of adrenal cancer. The thyroid carcinoma group cases consisted of papillary (82.7%), follicular (8.6%), medullary (7.0%) and anaplastic (1.6%) carcinomas. The ASR for thyroid carcinoma was 1.289 (0.627 for men, 1.59 for women), with the highest incidence rate in Kerman (ASR 1.643) and the lowest incidence rate in Golestan (ASR 0.735). For the 6 cases of adrenal cancer, 4 were neuroblastoma and 2 were pheochromocytoma. CONCLUSIONS: Iran was considered as an endemic, iodine-deficient area, until fairly recently. Iodinization of salt has been started about 12 years ago, in the nation. Considering the effect of improvement in the iodine intake in previously deficient communities, which is associated with an increase in the incidence of papillary carcinoma compared to other histologic types, the frequency and distribution of histologic types of thyroid carcinoma was closer to what can be seen in iodine-rich areas.

A study of histopathological features of medullary carcinoma of the thyroid: cases from a single institute in India.

BACKGROUND: The microscopic features of medullary carcinoma have been described in world literature, together with its behavior and molecular biology. However, no large study has been reported from India. AIMS: This study aims to analyse the clinical, and especially the pathological features of medullary carcinoma of the thyroid, and the surrounding thyroid. MATERIALS AND METHODS: In this study a total of 234 cases of medullary thyroid carcinoma (MTC) were gathered over a period of 3 decades. The clinical presentation, the microscopic features and the clinical outcome were analyzed. RESULTS: MTC was found to be twice as common in men as in women and for some reason it occurred 10 years earlier in women. The histology revealed certain interesting features like the presence of apoptosis in over half of the tumors, in addition to the other common and not so common histological findings (encapsulated variant, small cell variants, follicular pattern, rosettes, oncocytic change, osteosarcoma-like pattern, and cribriform pattern). The adjacent thyroid in about 19% of the cases showed optically clear nuclei in the follicles that were close to the tumor cells. These features were similar to those seen in papillary thyroid carcinoma. CONCLUSIONS: The thyroid adjacent to MTC showed nuclear changes, which are also found in papillary carcinoma of the thyroid. The occasional concurrent occurrence of these two tumors and the involvement of the RET gene in both medullary and papillary carcinomas, makes this observation worth discussing and studying further.

Epidemiologic analysis of the Tehran Cancer Institute Data System Registry (TCIDSR).

OBJECTIVE: To review epidemiological data on thyroid cancer in Iran. METHODS: The Tehran Cancer Institute Data System Registry (TCIDSR) was used to identify patients with different histological types of thyroid cancer (TC) in Iran. Data were analysed from 438 thyroid cancer cases identified by the TCIDSR in 1998-99. Disease prevalence was calculated with reference to age, time and place. RESULTS: The TCIDSR recorded 438 primary malignancies of the thyroid gland: papillary, follicular, medullary, and anaplastic carcinomas accounted for 67.1%, 10.7%, 5.3% and 4.3% of cases, respectively. The remaining 12.6% were classified as OD (other diagnoses). The prevalence of TC was highest in ethnic Farsis. The age range of patients was 8-85 years. Mean patient age was 44.52+17.03 years (mean + SD) overall, 47.74+18.10 years in female patients and 43.04+16.34 years in male patients. Anaplastic (6.5% vs. 3.3%) and medullary (10.0% vs. 3.0%) cancers were more common in men than women. CONCLUSION: This study was undertaken to define the epidemiological aspects of thyroid carcinoma in Iran, an area of endemic iodine deficiency until fairly recently. Against expectation for an iodine-deficient area, the frequency distribution of tumours in our study was closer to that seen in iodine-rich areas. Additional research on the risk factors for thyroid cancer--genetic, ethnic, geographic and environmental--is needed to explain the high incidence of PTC overall, and among ethnic Farsis in particular, in Iran.

Abordagem clínica e laboratorial do bócio uninodular sólido: vantagens da determinaçäo da calcitonina sérica por métodos distintos no rastreamento do carcinoma medular de tireóide, forma esporádica / Clinical and laboratorial approach of solid uninodular goiter: advantage of serum calcitonin determination by different methods in medullar thyroid carcinoma screening, sporadic form

A apresentação clínica mais freqüente da forma esporádica do carcinoma medular da tireóide (CMT) é o bócio uninodular sólido (BUS), apresentação esta semelhante aos demais tumores que afetam a glândula. O estabelecimento da freqüência de CMT em BUS apresenta implicações importantes não só diagnósticas, como também terapêuticas, visto a abordagem cirúrgica do CMT diferir de outros tumores tireoideanos. Para investigar a prevalência de CMT em BUS, dosamos calcitonina (CT) sérica, marcador bioquímico do CMT, por métodos distintos (RIA e IRMA) em 60 casos (55 mulheres; com idades entre 22 e 75 anos). A análise citológica obtida através de punção biópsia (PAAF) revelou 100 por cento de especificidade e 67 por cento de sensibilidade na detecção de carcinoma de tireóide. Considerando-se o grau de suspeita clínica para neoplasia tireoideana e os achados anatomopatológicos, houve 60 por cento de correlação positiva. CMT foi diagnosticado através da elevação da CT sérica em um dos 59 casos (1,69 por cento) e confirmado posteriormente pela PAAF e anatomopatológico. A incidência de CMT entre os casos de neoplasias tireoideanas nesta amostra foi de 12,5 por cento (1/8). Concluímos que a dosagem rotineira da CT sérica em casos com BUS não só complementa o estudo desta doença, como auxilia fortemente no diagnóstico do CMT. Tanto o IRMA como o RIA mostraram-se métodos úteis no rastreamento do CMT. Entretanto, o RIA pode provavelmente detectar ainda mais precocemente a elevação de formas não monoméricas da molécula de CT, as quais nos casos de CMT são usualmente mais abundantes que as formas monoméricas.

Cáncer medular de tiroides: experiencia en el hospital de Valdivia / Thyroid medullar cancer: experience at Valdivia hospital

Medullary carcinoma of the thyroid is an infrequent type of thyroid tumor. To retrospectively study all cases of MCT diagnosed at a regional hospital. The charts and pathological studies of eight patients with MCT among 75 with thyroidal cancer were reviewed. Solitary nodules were the presenting gign in seven patients and cachexia in one. Five patients were subjected to total thyroidectomy, five to cervical lymph node dissection and 4 to complementary radiotherapy. Pathological diagnoses was made with the surgical piece in 7 patients and the aspiration cytology in 1. Five of the 8 patients are alive after 50 months of follow up as a mean, 1 patient with bone metastasis. The small sample size precludes conclusions on the prognosis and treatment of the disease

Carcinomas ocultos da tireóide: estudo anatomopatológico

O autor estuda 82 casos de tumores ocultos da tireóide ,totalizando, devido ao caráter multicêntrico, 110 lesões malignas, com predomínio quase absoluto do carcinoma papilífero. Analisa diversos aspectos anatomopatológicos, incluindo considerações macroscópicas, classificação histopatológica, peculiares particularidades microscópicas e o diagnóstico diferencial. Discute as razões que justificam a diferença entre as duas prevalências nas amostras do material de autópsia (Hospital Universitário Gaffrée e Guinle - 5%; Instituto Médico Legal Afrânio Peixoto - 2,5%) e aponta a alta incidência do carcinoma entre os casos cirúrgicos (34% dos tumores malignos, que corresponde a cerca de metade dos casos de carcinomas papilíferos de nossa série), associados com quase todas as condições patológicas da tireóide. Destaca a hiperplasia nodular de célula-C no contexto epidemiológico do carcinoma medular oculto, descrevendo dois casos e a possível relação entre a doença de Basedow-Graves, sob condições terapêuticas especiais e o carcinoma papilífero oculto. Comenta o comportamento biológico deste particular tipo tumoral, através de revisão clínica da maioria dos pacientes, incluindo três casos que apresentavam metástase ganglionar, os quais estão vivos, sem evidência atual de enfermidade maligna o que concorda com o conceito de neoplasia de baixa malignidade e evolução lenta.

The author has studied 82 cases of thyroid occult carcinoma, in a total of 110 malignant lesions, considering multicentric aspect, with the majority, almost plain, of papillary carcinoma. Several pathologic features, including macroscopic comments, histological classification, peculiar details, and the differential diagnosis have been analysed.Discusses the reason for the difference between ese two prevalences obtained from autopsy samples (Hospital Universitario Gaffree e Guinle - 5%; Instituto Medico Legal Afranio Peixoto - 2,5%) and is also showed the high incidence occult carcinoma among surgical cases (34% of malignant what means almost half of all papillary carcinomas in our samples' series), related to all pathologic conditions. The C-cell nodular hyperplasia is appraised in epidemiological context of occult medular carcinoma by o studied cases, and the relationship between Basedow-Graves sease (with the influence of special treatments, drugs) and papillary carcinoma that is also mentioned. The biologic behaviour of this particular tumoral ype is presented by clinical reviews, obtained from most tients, adding three cases with ganglial metastases. These tients, with ganglial metastases, are still alive, and there is no evidence of any tumoral disease, what makes we all agree with the sense of less malignancy and slow progressing of this neoplasm.