Thyroid-like follicular carcinoma of the kidney presenting on a ten year-old prepubertal girl

ABSTRACT The very rare thyroid-like carcinoma of the kidney (TLCK) is microscopically similar to thyroid follicular cell carcinoma (TFCC). Differential diagnosis with secondary thyroid tumors depends on non-reactivity to immunohistochemical (IHC) markers for TFCC (thyroglobulin - TG and TTF1). We herein describe the fourth Pediatric case in literature and extensively review the subject. Only 29 cases were published to the moment. Most cases were asymptomatic and incidentally detected. Most tumors are hyperechoic and hyperdense with low grade heterogenous enhancement on CT and MRI. Most patients were treated with radical nephrectomy, but partial nephrectomy was used in some cases, apparently with the same results. Metastases are uncommon and apparently do not change prognosis, but follow-ups are limited. Up to the moment, TLCK presents as a low grade malignancy that may be treated exclusively with surgery and frequently with partial kidney renal preservation. A preoperative percutaneous biopsy is a common procedure to investigate atypical tumors in childhood and adult tumors. To recognize the possibility of TLCK is fundamental to avoid unnecessary thyroidectomies in those patients, supposing a primary thyroid tumor.

Hallazgos anatomopatológicos definitivos en pacientes tiroidectomizados con diagnóstico preoperatorio de neoplasia folicular / Definitive anatomopathological findings in thyroidectomized patients with preoperative diagnosis of follicular neoplasm

Background: The finding of follicular neoplasm, using a FNAP, is an indication for partial or total thyroidectomy, to obtain the definitive malignant or benign histology. Frequently, it is possible to identify significant additional histological diagnosis. Aim: To obtain the definitive histological findings in patients with follicular neoplasm by FNAP. Patients and Method: Transversal analysis of 133 patients that underwent to total thyroidectomy between 2003 and 2009, that filled de requirements for adequate histological assessment. Results: In 33.1 percent of the treated patients the final diagnosis was indeed a follicular neoplasm (adenoma in 26.3 percent and cancer in 6.8 percent). In the 51.9 percent the finding was follicular colloidal hyperplasia and other thyroid cancer in 8.3 percent. The total malignant prevalence in the whole gland was 29.3 percent. Conclusions: The thyroidec-tomy is the treatment of choice and the final diagnostic procedure for these patients. The histological findings of cancer different from follicular not only in the punctioned nodule are a secondary and an additional argument for reinforcing the surgical indication.

Introducción: El hallazgo de una neoplasia folicular por PAAF, obliga a realizar una tiroidectomía parcial o total, para definir la naturaleza maligna o benigna definitiva de la lesión tiroidea. Junto a este diagnóstico preoperatorio se identifican finalmente con alta frecuencia lesiones histológicas adicionales. Objetivo: Conocer y describir los hallazgos anatomopatológicos definitivos que se encuentran en tiroidectomías por neoplasias foliculares diagnosticadas por PAAF. Materiales y Métodos: Revisión transversal de las biopsias definitivas de 133 pacientes sometidos a tiroidectomía total entre 2003 y 2009, que cumplieron los requisitos establecidos para evaluar la histología definitiva del nódulo puncionado y de la glándula tiroides completa. Resultados: En el 33,1 por ciento de los pacientes el diagnóstico definitivo del nódulo puncionado fue efectivamente una neoplasia folicular (adenoma en el 26,3 por ciento y cáncer en el 6,8 por ciento). El 51,9 por ciento correspondió a hiperplasia folicular y el 8,3 por ciento otro cáncer. La prevalencia de malignidad final en la glándula completa fue de un 29,3 por ciento. Conclusiones: Siendo la indicación de tiroidectomía en estos pacientes un tratamiento y procedimiento diagnóstico aceptado y necesario, se concluye que la alta prevalencia de lesiones malignas (29,3 por ciento) tanto en el nódulo puncionado como, adicionalmente, en el resto de la glándula, reforzaría la necesidad de este tratamiento quirúrgico.

Evolución y factores de pronóstico en cáncer diferenciado de tiroides / Evolution and prognosis of differentiated thyroid carcinoma

Differentiated thyroid carcinoma has a good prognosis with a 90 percent survival at 10 years. Follicular carcinoma is more aggressive than papillary carcinoma Aim: To study the clinical presentation, evolution and prognosis of differentiated thyroid carcinoma. Material and Methods: The pathological registries of differentiated thyroid cancer processed in a pathology service of a general hospital, between 1984 and 2007, were searched. The clinical records of patients were reviewed. Results: One hundred six pathology registries were identified but only 89 patients had complete medical records. Seventy six patients aged 44 +/- 16 years had a papillary carcinoma and 13 patients aged 42 +/- 15 years had a follicular carcinoma. After a mean follow up of 53 months, 65 patients with papillary and 12 patients with follicular thyroid cancer were alive. Five patients with papillary carcinoma and one with follicular carcinoma had a local or distant relapse. Calculated actuarial survival at 10 years was 84 and 100 percent for papillary and follicular carcinoma, respectively. Ten year survival for stage I, II, III and IV tumors was 100, 92, 68 and 0 percent, respectively. Mortality was associated with the presence of distant metastases, a stage IV tumor, vascular or lymphatic tumor infiltration, tumor size, lymph node involvement, extra capsular invasion and a multi focal lesions. The relative risk for mortality increases between 2.7 and 9 times with the presence of lymph node infiltration and distant metastases. Conclusions: The prognosis of differentiated thyroid cancer is related to lymph node infiltration and the presence of distant metastases.

Los objetivos del presente estudio son identificar la forma de presentación clínica y evolución del cáncer diferenciado de tiroides (CDT); definir la curva de sobrevivencia para ambos tipos histológicos e identificar los factores clínicos e histológicos de mal pronóstico. Se presentan 89 pacientes portadores de cáncer diferenciado (papilar y folicular) de tiroides (75 CP Y 13 CF) tratados en los Hospitales de Coquimbo y La Serena, entre 1984 y 2007. La forma de presentación de ambos tumores fue similar. La mediana de seguimiento fue 53 meses. El 75 por ciento de los pacientes tuvo un seguimiento mínimo de 7 años y el 86,4 por ciento de los pacientes (64 cáncer papilar y 12 cáncer folicular) se encuentran vivos y libres de enfermedad; el intervalo libre de enfermedad fue de 52,7 meses con rango entre 3 meses y 16 años. La sobrevida actuarial a 10 a±os para cáncer papilar es 84 por ciento y, para cáncer folicular 100 por ciento. Por estadios, a los 10 años la sobrevida es de 100 por ciento en estadio I, 92 por ciento en estadio II, 68 por ciento en estadio III y 0 por ciento en estadio IV. El riesgo de muerte se asoció a la presencia de metástasis distantes, T4 e infiltración histológica vascular y/o linfática, p = 0,02. Otros factores no mostraron asociación: tamaño del tumor < T4, compromiso de linfonodos, invasión extracapsular y multifocalidad histológica. Sin embargo, el RR se incrementa 2,7 y 9 veces con invasión linfática o con presencia de metástasis, 1,6 veces con el aumento del tamaño tumoral, y 1,33 con multifocalidad neoplásica en la glándula. La presencia de metástasis linfáticas en cáncer papilar representa un RR de 1,22. Aunque el seguimiento de esta casuística es reducido (mediana 5 años), los resultados sugieren que el mal pronóstico se asocia fundamentalmente a compromiso linfático y metástasis a distancia y, por otra parte, a que el planteamiento terapéutico ha resultado efectivo para el control de la enfermedad en estadio I y II.

Microcarcinoma da tireóide: experiência e conduta em clínica privada / Thyroid microcarcinoma: experience and management in private clinic

OBJETIVO: Estudar o microcarcinoma papilífero observado nas tireoidectomias realizadas em nossa clínica privada. MATERIAL E MÉTODOS: Revisamos clínica e histologicamente 1.930 pacientes submetidos a tireoidectomias no período de 2002 a 2006, tendo 606 carcinomas e, desses, 332 como microcarcinomas papilíferos. Avaliamos sexo, idade, tipo histológico, tamanho da neoplasia, multifocalidade, freqüência do esvaziamento cervical, resultados da PCI e tireoglobulina sérica. RESULTADOS: Dos 332 pacientes, 48 eram do sexo masculino e 146 tinham idade superior a 45 anos. Todos foram submetidos a tireoidectomia total, 19 concomitantemente a esvaziamento cervical de necessidade (5,72 por cento), e 313 a iodoterapia (94,27 por cento). Tivemos metástase a distância em 5 pacientes (1,5 por cento). Após 1 ano, de 170 pacientes submetidos à PCI, temos 141 considerados livres de doença, 21 com anti-tireoglobulina positivo, 6 com tireoglobulina superior a 2 ng/ml, sendo 1 com metástase pulmonar e outro com recidiva linfonodal (0,3 por cento). CONCLUSÃO: Recomendamos que seja realizada a tireoidectomia total, esvaziamento cervical de necessidade e iodoterapia ablativa na presença de fatores clínicos e anátomo-patológicos de risco.

OBJECTIVE: The study of papillary microcarcinoma observed during the thyroidectomies in our private clinic. MATERIAL AND METHODS: We reviewed clinical and histopathological results of 1,930 consecutive patients submitted for thyroidectomies in the period from 2002 to 2006, 606 had thyroid carcinomas but only 332 were papillary microcarcinoma. We studied gender, age, histologic type, size, multifocality, frequency of neck dissection, and results of Whole Body Scan and thyroglobulin. RESULTS: Overall, we found 48 males and 146 older than 45 years old. Therapy included total thyroidectomy for all, therapeutic neck dissection in 19 (5.72 percent), and radioiodine for 313 (94.27 percent). We found 5 patients with distant metastases (1.5 percent). After 1 year, from 170 submitted to WBS, we consider 141 free of disease, 21 have positive thyroglobulin antibody, 6 have thyroglobulin level above 2 ng/ml, being 1 with lung metastases and other with lymph node recurrence (0.3 percent). CONCLUSION: We recommend total thyroidectomy, therapeutic neck dissection and radioiodine ablation when present clinical and pathological risk factors.

Repercussão clínica da reclassificação dos carcinomas diferenciados de tireóide de acordo com a 6ª edição do TNM / Clinical repercussion of the 6th edition TNM staging system classification on differentiated thyroid carcinoma

A classificação TNM segundo a UICC é usada para avaliar os resultados do acompanhamento do carcinoma de tireóide. A 6ª edição modificou a descrição do tumor primário (T), dos linfonodos regionais (N) e dos grupos de estadiamento. O objetivo deste estudo foi comparar a habilidade das 5ª e 6ª edições em predizer resultados. As duas classificações foram aplicadas em uma análise retrospectiva de 90 pacientes do HUCFF. Sessenta e nove pacientes apresentavam carcinoma papilífero, 14 folicular, 4 células de Hürthle e 3 misto. Os pacientes foram acompanhados por um período médio de 58,3 meses. Ao final do acompanhamento, 49 pacientes estavam em remissão, 23 com doença persistente, 4 com recorrência tumoral, 11 com metástases e 3 evoluíram para o óbito. De acordo com a 6ª edição, 19 pacientes foram classificados como T1, comparado com 7 pela 5ª edição; 19 pacientes T2 comparado com 30; 14 classificados como T3 comparado com 10; 22 como T4 comparado com 27, e 16 pacientes como Tx. Ambas as edições mostraram remissões comparáveis para os estágios I, II e III. Para o estágio IV houve uma mudança significativa na remissão, entretanto não houve diferença comparando IV e IV C.

The TNM classification of UICC is used for predicting the outcome of thyroid cancer. The 6th edition changed the description of primary tumor (T), regional lymph node (N) and the staging group. The aim of this study was to compare the ability of the 5th and the 6th editions to predict outcome. The two classifications were applied in a retrospective analysis of 90 patients from HUCFF. Sixty-nine patients had papillary carcinoma, 14 follicular, 4 Hürthle cell, and 3 mixed. Patients were followed for a mean period of 58.3 months. At the end of follow-up, 49 patients were disease-free, 23 persisted with disease, 4 had cervical recurrence, 11 had metastases and 3 died. According to the 6th edition, 19 patients were classified as T1, compared to 7 based on the 5th edition; 19 patients were T2 compared to 30; 14 were T3 compared to 10; 22 were T4 compared to 27, and 16 patients were Tx. Both editions showed comparable remissions for stages I, II, and III. For the stage IV there was a significant change in remission, however there was no difference comparing IV and IV C.

Seguimento de pacientes de alto risco com carcinoma diferenciado de tireóide sem doença persistente após a terapia inicial / Follow-up of high-risk patients with differentiated thyroid cancer without persistent disease after initial therapy / Follow-up of high-risk patients with differentiated thyroid cancer without persistent disease after initial therapy

Este estudo avaliou o seguimento de pacientes com câncer de tireóide de alto risco, após a terapia inicial. Foram selecionados 125 pacientes de alto risco (tumor >4 cm e/ou invasão extra-tireoidiana e/ou metástases linfonodais e idade >45 anos), com ressecção tumoral completa. Todos foram tratados com tireoidectomia total e ablação com 131I [3,7­5,5 GBq (100­150 mCi)] e foram excluídos 18 casos (14,8 por cento) com metástases na PCI pós-dose (t-PCI). O valor preditivo negativo da Tg estimulada <1 ng/ml combinada ao US cervical na primeira avaliação (6­12 meses após a terapia ablativa) foi de 96,2 por cento para ausência de recidivas em até 5 anos. Este valor aumentou para 98,7 por cento quando acrescentamos a PCI com 185 MBq (5 mCi) 131I (d-PCI). O valor preditivo positivo (VPP) da Tg estimulada >1 ng/ml foi de 52 por cento para presença de metástases detectadas até 5 anos depois, mas considerando apenas pacientes que apresentaram d-PCI e US negativos inicialmente, o VPP foi 19 por cento (9 por cento se Tg 1­10 ng/ml vs. 40 por cento se Tg >10 ng/ml). Tg reduziu espontaneamente nos pacientes com Tg estimulada >1 ng/ml na primeira avaliação, US e d-PCI negativos e sem recidiva no seguimento, sendo indetectável em metade destes ao final de 5 anos. No primeiro ano após a terapia ablativa, 20 pacientes tiveram captação em leito tireoidiano na d-PCI com Tg estimulada e US negativos e não foram tratados com 131I; estes evoluíram sem recidiva e 60 por cento apresentavam uma d-PCI sem nenhuma captação após 5 anos. Em pacientes sem doença aparente (no US e d-PCI) e Tg estimulada <1 ng/ml, a recidiva em 5 anos foi de apenas 1,3 por cento. Um algoritmo para o seguimento de pacientes de alto risco após a terapia inicial é apresentado por este estudo.

This study evaluated the follow-up of high-risk patients with thyroid cancer after initial therapy. A total of 125 high-risk patients (tumor >4 cm and/or extrathyroid invasion and/or lymph node metastases, and age >45 years), with complete resection of the tumor, were selected. All patients underwent total thyroidectomy and ablation with 131I [3.7­5.5 GBq (100-150 mCi)]. Eighteen patients (14.8 percent) presenting metastases on post-dose whole-body scan (RxWBS) were excluded. The negative predictive value of stimulated Tg <1 ng/ml in combination with neck US during first assessment (6­12 mo. after ablative therapy) was 96.2 percent for the absence of recurrence up to 5 years. This value increased to 98.7 percent when adding WBS performed with 185 MBq (5 mCi) 131I (DxWBS). The positive predictive value (PPV) of stimulated Tg >1 ng/ml was 52 percent for the detection of the presence of metastases up to 5 years; however, considering only patients with initially negative DxWBS and US, the PPV was 19 percent (9 percent if Tg of 1­10 ng/ml vs. 40 percent if Tg >10 ng/ml). Tg levels decreased spontaneously in patients with stimulated Tg >1 ng/ml during first assessment, negative US and DxWBS, and no recurrence during follow-up, with Tg being undetectable in half these patients at the end of 5 years. Twenty patients presented uptake in the thyroid bed upon DxWBS during the first year after ablative therapy, with stimulated Tg and US being negative, and were not treated with 131I; these patients did not relapse and no uptake on DxWBS was observed in 60 percent after 5 years. Recurrence after 5 years was only 1.3 percent in patients without apparent disease (negative US and DxWBS) and stimulated Tg <1 ng/ml. An algorithm for the follow-up of high-risk patients after initial therapy is presented in this study.

Follow up study of thyroidectomy.

One hundred thyroidectomized patients were followed up. The male:female ratio was 1:4. Majority (62%) of the patients were in the 4th and 5th decades. Multinodular goiter was the commonest lesion found in 74, diffuse colloid goiter in 12 and carcinoma in 14 cases. After operation, sixty-five patients were relieved of all preoperative symptoms of thyroid disease. Thirteen patients had some persistent symptoms. Rest 22 had developed different types of complication in addition to some preoperative symptoms. Three patients developed hoarseness of voice due to recurrent laryngeal nerve injury. Hypoparathyroidism occurred in 1, hypothyroidism in 4 and recurrent neck swelling in 14 patients. One patient died. It seems that a thorough knowledge of the surgical anatomy and meticulousness during the procedure would reduce the rates of such complications in future.

Hemiagenesia da glândula tireóide associada a hipertireoidismo: revisäo da literatura e apresentaçäo de dois casos / Hemiagenesis of the thyroid gland associated to hyperthyroidism: review of the literature and report of 2 cases

A hemiagenesia da glândula tireóide é bastante rara. Apresentamos dois casos de pacientes com quadro de hipertireoidismo, tratados inicialmente com metimazol e posteriormente com cirurgia, durante a qual foi realizado o diagnóstico, sendo que em um deles, ocorreu associaçao com carcinoma folicular. Discutimos estes casos revisando a literatura quanto aos estudos cirúrgicos, cintilográficos e necroscópicos.