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Colorectal cancer is the third most common neoplasm and the second most lethal worldwide. The most common histological type is adenocarcinoma, characterized by its glandular pattern. Medullary colon carcinoma is a rare histological variant of colorectal cancer, characterized by a predominantly solid architecture, poorly di?erentiated or undifferentiated morphology, often associated with an anomalous immunophenotype and microsatellite instability. The present study reports a case in an academic service of general surgery of a 74-year-old patient who presented with a tumor of the ascending colon, histologically with an exuberant lymphocytic in?ltrate, suggestive of large cell lymphoma, but which was revealed by subsequent immunohistochemistry to be medullary carcinoma of the colon with microsatellite instability.
O câncer colorretal é a terceira neoplasia mais comum e a segunda mais letal no mundo. O adenocarcinoma é o tipo histológico mais comum, caracterizado pelo seu padrão glandular. O carcinoma medular do cólon é uma variante histológica rara do câncer colorretal, caracterizada por uma arquitetura predominantemente sólida, morfologia pouco diferenciada ou indiferenciada, frequentemente associada a um imunofenótipo anômalo e instabilidade de microssatélites. O presente estudo relata um caso em um serviço acadêmico de cirurgia geral de um paciente de 74 anos que apresentou tumor de cólon ascendente, histologicamente com infiltrado linfocitário exuberante, sugestivo de linfoma de grandes células, mas que foi revelado através de exame subsequente imunohistoquímico como carcinoma medular do cólon com instabilidade de microssatélites.
Subject(s)
Male , Aged , Carcinoma, Medullary , Colon, Ascending , Surgical Oncology , Colonic NeoplasmsABSTRACT
In patients with medullary thyroid carcinoma (MTC), calcitonin (Ctn) and carcinoembryonic antigen (CEA) were the most important serum biomarkers for diagnosis, evaluation and follow-up. Approximately 0.3% to 5.9% of the thyroid nodule population could present with elevated Ctn on screening, and a diagnosis of MTC can be basically confirmed when serum Ctn > 100 pg/ml.. Ctn and CEA levels could reflect tumor burden and help determine the extent and timing of surgery. When preoperative serum Ctn >200 pg/mL or CEA >30 ng/mL, nearly more than one-third of patients had lateral neck lymph node metastasis. Few patients developed distant metastasis when Ctn<500 pg/mL, however the proportion of distant metastasis could reach 75% when CEA>100 ng/mL. In inherited MTC patients, tumors with Ctn<30 pg/mL were usually localized in the gland without metastasis. The time to normalization of serum Ctn and CEA postoperatively was one month in most patients. According to the response to initial therapy, patients with undetectable Ctn and normal CEA had a low risk of relapse and death in the follow-up period. The risk of local recurrence, LNM and distant metastasis increased as Ctn levels rose. The Ctn/CEA doubling time could predict the disease prognosis, and when it was less than 0.5 years, most patients would die.
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Objective:To summarize the prognosis of medullary thyroid carcinoma (MTC) patients with biochemical recurrence (the increase of postoperative calcitonin and no abnormal imaging) and to investigate the optimal cut-off value of calcitonin for postoperative structural recurrence (with imaging abnormality).Methods:Literature retrieval was conducted for PubMed, CNKI, EMbase, Web of Science, Cochrane and other databases, and literatures related to the increase of calcitonin after MTC surgery were included. Review Manager 5.4 software was used for Meta-analysis of the recurrence and death. SPSS 23.0 software was used and receiving operating characteristic (ROC) curve was used to analyze the rising folds of postoperative calcitonin level in comparison with the maximum value of experiment detection, and to predict the outcome of biochemical recurrence transforming to structural recurrence, and then the optimal cut-off value could be worked out.Results:A total of 7 studies including 1 005 MTC patients (276 cases of biochemical recurrence and 542 cases of biochemical cure). Meta-analysis showed that structural recurrence rate in postoperative biochemical recurrence group [40.6% (112/276) vs. 2.2% (12/542); OR = 27.99, 95% CI 14.57-53.78, P < 0.001] and mortality [10.0% (19/190) vs. 0.96% (3/312); OR = 7.26, 95% CI 2.42-21.84, P < 0.001] were higher than those in the biochemical cure group (normal postoperative calcitonin level and no disease state). The data of 89 MTC patients with biochemical recurrence were collected in another 4 studies. ROC curve analysis showed that area under the curve of the rising folds of postoperative calcitonin level in predicting structural recurrence was 0.825; according to the cut-off value at all sections, the optimal cut-off value of the increased postoperative calcitonin was 50 times, the sensitivity was 66. 7%, the specificity was 88.6%. Conclusions:MTC patients with postoperative biochemical recurrence have higher structural recurrence rate and mortality compared with patients with normal postoperative calcitonin. The postoperative elevation of calcitonin more than 50 times the maximum value of the laboratory detection can be taken as the critical diagnostic value, when more than 50 times is prone to structural recurrence.
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Objective:To compare the prognosis of patients with unilateral sporadic medullary thyroid carcinoma treated by different surgical selection, and analyze the independent risk factors affecting the prognosis.Methods:One hundred and twenty-six patients at Tianjin Medical University Cancer Institute and Hospital from Feb 2011 to Oct 2018 were retrospectively divided into group A (total thyroiclectomy) and group B (unilateral lobectomy).Results:There were no significant differences in postoperative recurrence rate ( χ2=0.394, P=0.530), mortality ( χ2=3.175, P=0.146), biochemical cure rate ( χ2=0.613, P=0.434), progression free survival and overall survival ( P=0.278, 0.175) between group A and group B; Tumor diameter ≥4 cm and lateral cervical lymph node metastasis were independent risk factors affecting the overall survival. The incidence of postoperative temporary hypocalcemia ( χ2=5.068, P=0.024) and permanent hypocalcemia ( χ2=6.590, P=0.010) in group A was higher than that in group B. Conclusions:Ipsolateral thyroidectomy can be applied to patients with unilateral sporadic medullary thyroid carcinoma with similar long term prognosis and tower incidence of temporary hypocalcemia and permanent hypocalcemia compared to total thyroidectomy.
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Objective:To evaluate the clinical and ultrasonographic features for early diagnosis and prediction of lateral cervical lymph node metastasis of medullary thyroid microcarcinoma.Methods:From Jan 2010 to Jan 2020 233 patients undergoing primary surgery were categorized as "medullary thyroid microcarcinoma" and "medullary thyroid macrocarcinoma". The preoperative clinical and ultrasonographic characteristics, the diagnostic positive rate of preoperative serum calcitonin and fine needle aspiration (FNA) were investigated between two groups. All patients with medullary thyroid microcarcinoma were divided on the basis of wether there was lateral cervical lymph node metastasis.Results:There were statistically significant differences in initial diagnostic method(χ 2=32.290, P=0.000), TNM staging(χ 2=50.300, P=0.000) between medullary thyroid microcarcinoma and medullary thyroid macrocarcinoma. Medullary thyroid microcarcinoma showed more malignant ultrasonic features. The diagnostic accuracy of preoperative serum calcitonin was higher than FNA for medullary thyroid micro carcinoma(χ 2=47.933, P=0.000). Multivariate regression analysis demonstrated that the abutment/perimeter ≥1/4( OR=25.475, 95%CI: 2.320-279.771), preoperative serum calcitonin >65 ng/L( OR=32.663, 95%CI:2.433-438.409) were the independent factor for lateral cervical lymph node metastases of medullary thyroid microcarcinoma. Conclusions:The combination of ultrasonography and serum calcitonin helps establish early diagnosis of medullary thyroid microcarcinoma. Medullary thyroid microcarcinoma with the abutment/perimeter ≥1/4, serum calcitonin >65 ng/L predicts lateral cervical lymph node metastases.
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Objective:To investigate the difference of clinicopathological characteristics between mixed medullary and papillary carcinoma of thyroid and medullary carcinoma coexistent with papillary carcinoma.Method:The clinicopathological data of 3 MMPTC cases and 9 MTC-PTC cases treated at Tianjin Medical University Cancer Institute & Hospital during the past ten years were retrospectively analyzed. The differences in clinical characteristics, pathological characteristics, immunohistochemistry results, treatment and prognosis of the two groups were compared.Results:In the MMPTC group, the median onset-age was 59 years old. 3 patients were all medullary carcinoma colliding with micropapillary carcinoma. The immunohistochemistry results showed that medullary carcinoma and papillary carcinoma showed their distinctive immunohistochemical characteristics. The lymph node metastasis rate was 66.7% (2/3). In MTC-PTC group, the median onset-age was 55; 8 out of 9 patients had an increased preoperative calcitonin level. Medullary carcinoma and papillary carcinoma showed their distinctive immunohistochemical characteristics. Four out of the 9 cases had lymph node metastasis.Conclusion:Compared with MTC-PTC, MMPTC is more common in middle-aged and elder patients, with higher lymph node metastasis rate. The pathogenesis of MTC-PTC is similar to papillary thyroid carcinoma, and the treatment should be individualized. The prognosis of these two groups of patients is fair.
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Medullary carcinoma of breast is a rare variant of invasive ductal carcinoma of breast and its incidence is less than 5% of invasive breast carcinomas. These tumours tend to occur in younger women, with the average age reported to range from 42 to 52 years. Authors are presenting this case in a 27 years old female having single, large, well circumscribed mass in right breast for 6 months. Fine needle aspiration cytology report was proliferative lesion with atypia Histopathology report was given as carcinoma with medullary features. Immunohistochemistry showed Estrogen Receptor (ER), Progesterone Receptor (PR) and Her-2 neu negative. Authors are presenting this case of Medullary carcinoma of breast for being a specific histopathological subtype.
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Objective@#To study the clinical significance of serum calcitonin in the diagnosis and treatment of medullary thyroid carcinoma and to analyze its cost-benefit.@*Methods@#One hundred and forty one patients with medullary thyroid carcinoma who undertook calcitonin test and frozen pathological examination were enrolled in this study from Oct 2012 to Mar 2018. Using the method of χ2 test, the positive rate of calcitonin test and frozen pathological examination in diagnosis of medullary thyroid carcinoma(MTC) were compared. Firstly, we compared the correct checkout cost of calcitonin test and that of frozen pathological examination (total number of patients×cost of examination/the correctly detected number of patients) . Secondly, we calculated whether calcitonin test help patients save money(average cost of treatment in hospital for MTC×number of patients who were evaluated to be candidate for surgery-cost of calcitonin test×total number of patients)/total number of patients.@*Results@#139 patients were positive in calcitonin test among 141 patients, and the positive rate was 98.58%. 91 patients were positive in frozen pathological examination, and the positive rate was 64.54% (χ2=97.821, P<0.000 1) . Cost-benefit analysis showed that the correct checkout cost of calcitonin test and frozen pathological examination were 71.01 yuan and 426.10 yuan, also,1 371 938.64 yuan could be saved totally and 9 730.06 yuan could be saved per patient because of calcitonin test.@*Conclusion@#Serum calcitonin test had a significant effect on the diagnosis and treatment of medullary thyroid carcinoma and was economical and practical.
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Objective@#There is no effective therapy for patients with advanced medullary thyroid carcinoma (MTC). Vandetanib,a novel multitargeted receptor tyrosine kinase inhibitor, has previously shown antitumor activity in phase Ⅱ studies of patients with advanced MTC. This study was to evaluate the efficacy and the safety of vandetanib on advanced MTC.@*Methods@#This study was an open, international multi-center phase Ⅲ clinical trial and the study number was NCT01298323. The single-center study was a sub-group analysis of the international study, which was conducted on 9 pathologically confirmed advanced MTC patients by Cancer Hospital Chinese Academy of Medical Sciences between March 2012 and October 2017. Vandetanib (300 mg) was orally administered daily till death or withdrawal. The efficacy was evaluated according to RECIST criteria and the adverse events were evaluated according to NCI criteria.@*Results@#The objective response rate was 3/9,and the disease control rate was 4/9. The median progression-free survival was 44 months. All patients who had the elevated levels of calcitonin (CTN) and carcino-embryonic antigen (CEA) before treatment began to show the decreases in the level of CTN and CEA after 3 months and later showed again the increases in the levels of both tumor markers with tumor progression. By ROC curve analysis, CTN was of statistically significance(P<0.05, 95%CI 0.558-0.834), but CEA was not(P>0.05). Adverse events were generally mild (grade 1 or 2),including hypertension (9 cases),skin rash (9 cases), and diarrhea (6 cases). Two patients developed grade 3 elevation of serum glutamate pyruvate transaminase and one patient developed grade 3 elevation of drug-related bowel disease. No grade 4 drug-related adverse event occurred.@*Conclusions@#Vandetanib is effective and well tolerated for patients with locally advanced or metastatic MTC who have no chance for surgery. This indicates the increase of CTN is clinically relevant to disease progression, but the number of patients are extremely low, and, therefore further research is needed. Long-term use of vandetanib may cause resistance.
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Objective@#To construct 131I-the fifth generation polyamidoamine (PAMAM(G5.0)) with targeting peptide Ser-Arg-Glu-Ser-Pro-His-Pro (SRESPHP; SR) or Gly-Pro-Leu-Pro-Leu-Arg (GPLPLR; GP) and double targeting peptide SR/GP, and evaluate the targeting ability in medullary thyroid carcinoma (MTC) model.@*Methods@#PAMAM(G5.0), PAMAM(G5.0)-SR, PAMAM(G5.0)-GP and PAMAM(G5.0)-SR/GP were radiolabeled with 131I by chloramine T method. The radiolabeled yield and radiochemical purity were determined by thin layer chromatography. MTC xenografts were developed and the percentage radio-activity of injection dose per gram of tissue (%ID/g) in tumor and organs was measured at 24 h post-injection. Region of interest (ROI) was drawn and the tumor/non-tumor (T/NT) ratios at 4, 8 and 24 h post-injection were calculated and compared among different groups. One-way analysis of variance, repetitive measurement analysis of variance and Dunnett-t test were used to compare the data of different groups. The relationship between %ID/g and T/NT was analyzed with Pearson correlation.@*Results@#The radiolabeled yield was more than 75% and radiochemistry purity was more than 90%. The difference of %ID/g at 24 h post-injection was significant (F=14.400, P<0.001) in tumors of all groups. The radioactive uptake in tumor of 131I-PAMAM(G5.0)-SR group was the highest at 24 h post-injection((1.80±0.18) %ID/g). There were significant differences of T/NT ratios among different groups(F=4.776, P<0.05)and between different time points(F=8.630, P<0.05). Compared with negative control group (Na131I), the T/NT ratios significantly increased in 131I-PAMAM(G5.0)-SR group at 4, 8 and 24 h post-injection (t=4.169, 7.123 and 4.032, all P<0.05) and in 131I-PAMAM(G5.0)-GP group at 4 h post-injection (t=5.893, P<0.05). The T/NT ratio in 131I-PAMAM(G5.0)-SR group was higher than that in 131I-PAMAM(G5.0)-GP group at 24 h post-injection (t=2.871, P<0.05).@*Conclusions@#PAMAM(G5.0)-SR, PAMAM(G5.0)-GP and PAMAM(G5.0)-SR/GP can target the MTC models. 131I-PAMAM(G5.0)-SR has the best biological properties and may provide a new precision method for MTC diagnosis, treatment and prognosis evaluation.
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Objective@#To review and summarize recent update on preoperative diagnostic criteria, treatment and postoperative follow-up for medullary thyroid carcinoma.@*Methods@#The relevant literatures and guidelines about medullary thyroid carcinoma were analyzed and summarized.@*Results@#In the early stages of the disease radical surgery still dominated. Ultrasound results suggested that prophylactic lateral neck dissection was required for patients with high risk factors or high levels of carcinoembryonic antigen and calcitonin need prophylactic. Early hereditary medullary carcinoma could receive prophylactic thyroidectomy based on RET gene test results. Advanced progressive medullary thyroid carcinoma could be treated with palliative surgery,molecular targeted drugs and chemotherapy.@*Conclusions@#The prognosis of medullary thyroid carcinoma is poor and lymph node metastasis is easy to occur early. The extent of initial operation should be enough. Locally advanced or distant metastatic medullary thyroid carcinoma can be treated with palliative surgery,molecular targeted drugs and chemotherapy.
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Objective To construct 131 I-the fifth generation polyamidoamine (PAMAM(G5.0)) with targeting peptide Ser-Arg-Glu-Ser-Pro-His-Pro (SRESPHP;SR) or Gly-Pro-Leu-Pro-Leu-Arg (GPLPLR;GP) and double targeting peptide SR/GP,and evaluate the targeting ability in medullary thyroid carcinoma (MTC) model.Methods PAMAM(GS.0),PAMAM(GS.0)-SR,PAMAM(GS.0)-GP and PAMAM(GS.0)-SR/GP were radiolabeled with 131I by chloramine T method.The radiolabeled yield and radiochemical purity were determined by thin layer chromatography.MTC xenografts were developed and the percentage radio-activity of injection dose per gram of tissue (％ID/g) in tumor and organs was measured at 24 h post-injection.Region of interest (ROI) was drawn and the tumor/non-tumor (T/NT) ratios at 4,8 and 24 h post-injection were calculated and compared among different groups.One-way analysis of variance,repetitive measurement analysis of variance and Dunnett-t test were used to compare the data of different groups.The relationship between ％ID/g and T/NT was analyzed with Pearson correlation.Results The radiolabeled yield was more than 75％ and radiochemistry purity was more than 90％.The difference of ％lD/g at 24 h post-injection was significant (F=14.400,P＜0.001) in tumors of all groups.The radioactive uptake in tumor of 131I-PAMAM (G5.0)-SR group was the highest at 24 h post-injection ((1.80± 0.18) ％ID/g).There were significant differences of T/NT ratios among different groups (F =4.776,P＜ 0.05)and between different time points (F =8.630,P＜0.05).Compared with negative control group (Na131 I),the T/NT ratios significantly increased in 131I-PAMAM(G5.0)-SR group at 4,8 and 24 h post-injection (t=4.169,7.123 and 4.032,all P＜0.05) and in 131I-PAMAM(G5.0)-GP group at 4 h post-injection (t =5.893,P＜0.05).The T/NT ratio in 131I-PAMAM (G5.0)-SR group was higher than that in 131 I-PAMAM (G5.0)-GP group at 24 h post-injection (t=2.871,P＜0.05).Conclusions PAMAM(G5.0)-SR,PAMAM(G5.0)-GP and PAMAM(G5.0)-SR/GP can target the MTC models.131I-PAMAM(G5.0)-SR has the best biological properties and may provide a new precision method for MTC diagnosis,treatment and prognosis evaluation.
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Objective To investigate the value of fine-needle aspiration cytology (FNAC) combined with serum calcitonin in the diagnosis of medullary thyroid carcinoma (MTC).Methods Retrospective analysis of the serum calcitonin and FANC results in 17 cases of MTC confirmed by pathological examination.The diagnostic value of the two methods alone and in combination for MTC were compared.Results 13 of the 17 MTC patients (13/17,76.5％) underwent fine needle aspiration biopsy before surgery,11 cases (11/13,84.6％) diagnosed as thyroid malignant tumor,7 of whom were diagnosed or suspected of MTC (7/13,53.8％).Serum calcitonin levels increased in different degrees in 16 patients.The sensitivity of the two methods combined in the diagnosis of MTC was 92.3％ (12/13).Conclusions FNAC is an important diagnostic method for the evaluation of thyroid nodules,but it is not sensitive to the diagnosis of MTC.Serum calcitonin is highly sensitive but not universally available.Compared with FNAC alone,FNAC combined with the serum calcitonin examination may significantly improve the detection rate of MTC preoperative and reduce missed diagnosis.
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Objective: The aim of this study is to depict the clinical and epidemiological profile of patients treated for invasive lobular carcinoma (ILC) at Hospital das Clínicas of Universidade Federal do Paraná (HC-UFPR) over the course of ten years and to evaluate the variation of ILC dimensions on imaging exams by comparing them to real-size lesions identified in surgical specimens. Methods: Patients undergoing breast surgical procedures at HC-UFPR from 2005 to 2014 were selected. Out of these, 36 were diagnosed with ILC and had their medical files sought after clinical, epidemiological, therapeutic and prognosis characteristics. The variance of tumor sizes in imaging methods and anatomopathological descriptions were also studied. Results: Patients' mean age at diagnosis was 59.6 years. Most of them were classified as clinical stages II (40%) and III (26.7%) by the time they were diagnosed. The majority of tumors were HER2 negative (77.2%) and estrogen-receptor positive (90%). The surgical treatment was radical in 74.2% of the cases. 31.4% of the patients underwent both mammography and ultrasonography screening and 45.7% underwent only one of them. None of the patients were submitted to magnetic resonance imaging (MRI). Conclusion: Data found about patients with invasive lobular carcinoma at HC-UFPR is in accordance with the medical literature, including incidence rates and tumor characteristics. The variance of tumor sizes in imaging exams and surgical specimen was not statistically significant
Objetivo: O estudo busca caracterizar o perfil clínico epidemiológico referente às pacientes tratadas por carcinoma lobular invasor de mama (CLI) no Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR) em um período de dez anos e avaliar as variações das dimensões dos CLI nos exames de imagem quando comparadas ao real tamanho das lesões identificadas nas peças de anatomia patológica. Métodos: Foram selecionadas pacientes submetidas a procedimentos cirúrgicos de mama no HC-UFPR entre os anos de 2005 e 2014, dentre as quais 36 apresentaram diagnóstico de CLI. Seus prontuários foram analisados para avaliação de características clínicas, epidemiológicas, terapêuticas e prognósticas. Também foi avaliada a discrepância dos valores de tamanho do tumor em métodos de imagem em relação ao descrito nos laudos anatomopatológicos. Resultados: A s p acientes c om d iagnóstico d e C LI tinham média de idade no diagnóstico de 59,6 anos. O diagnóstico foi feito, em sua maioria, nos estádios clínicos II (40%) e III (26,7%). Houve maior negatividade (77,2%) para HER2 e positividade (90%) para receptor de estrógeno. O tratamento cirúrgico foi radical em 74,2% das pacientes. Em exames de imagem, 31,4% das pacientes realizaram mamografia e ultrassonografia em conjunto, 45,7% fizeram apenas um dos exames e nenhuma realizou ressonância magnética. Conclusão: Observou-se que a casuística de patologias mamárias do HC-UFPR está de acordo com a literatura em relação à incidência e às características próprias dos CLI. A análise da discrepância dos tamanhos dos tumores em exames de imagem em relação às peças cirúrgicas não obteve resultados significativos estatisticamente
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Objective To analyze the characteristics of breast medullary carcinoma in CEUS and to compare with pathologic features.Methods Morphologic characteristics of 13 breast medullary carcinomas in CEUS were analyzed.The diameter of mass before and after CEUS were compared.Parameters from time-intensity curves of masses were analyzed in contrast with peripheral breast parenchyma.All the results from CEUS analysis were compared with pathological manifestations.Results Breast medullary carcinoma was characterized as irregular shape (n=10),clear margins (n=11) and uniform enhancement (n=11) in CEUS.These characteristics were in accordance with their morphologic characters in pathology.The diameter of mass before and after CEUS had no significant defference (P=0.61),which was in accordance with expansive growth in pathology.In contrast with peripheral breast parenchyma,the arrival time and time to peak of breast medullary carcinoma were significantly shorter (P=0.034,0.021),and peak enhancement intensity was significantly stronger (P=0.005),which were in accordance with the increased vascular density and their uniform distribution,big arteries at the margin of masses in pathology.Conclusion Breast medullary carcinoma has distinguished characteristics in CEUS,which are in accordance with characters in pathology,and can be used as the basis in clinical diagnosis and differential diagnosis of breast medullary carcinoma.
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El carcinoma medular de la mama ha sido reconocido como una variedad de carcinoma ductal con un pronóstico relativamente favorable a pesar de su alto grado nuclear y su elevado índice mitótico. Se presenta el caso de una paciente de 87 años con antecedentes de haber sido operada de carcinoma medular de la mama derecha hace 41 años y la cual se mantiene libre de enfermedad metastásica actualmente; por lo cual se considera de interés para el personal médico dedicado al tratamiento de esta enfermedad, pues es la paciente operada de cáncer de mama de mayor supervivencia en la provincia de Cienfuegos.
Medullary breast carcinoma has been regarded as a type of ductal carcinoma with a relatively favorable prognosis despite its high nuclear grade and mitotic index. The case of 87 year-old patient operated on for medullary carcinoma of the right breast 41 years ago is presented. Currently, she remains free of metastatic disease. This case is interesting for the medical personnel dedicated to the treatment of this disease since she is the patient operated on for breast cancer with the longest survival in the province of Cienfuegos.
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Neuroendocrine lesions of the thyroid are rare. The most common types are medullary thyroid carcinomas (MTCs) and C-cell hyperplasia. MTCs originate from thyroid parafollicular cells that secrete calcitonin which serves as a serum marker of MTCs. Here, the rare case of a calcitonin-negative neuroendocrine tumor (NET) derived from follicular lesions of the thyroid is described. A 34-year-old man presented at our hospital for the surgical management of an incidental thyroid nodule that was observed on an ultrasound sonography (USG) of the neck. Initially, USG-guided aspiration cytology was performed, and a MTC was suspected. The expressions of thyroglobulin and thyroid transcription factor-1, which are thyroid follicular cell markers, and synaptophysin and chromogranin A, which are neuroendocrine markers, was confirmed following surgical pathology. However, the staining of calcitonin, a marker of MTCs, was not observed. A nonmedullary NET of the thyroid is uncommon, and the distinction between calcitonin-negative NETs and MTCs of the thyroid may be important due to differences in their clinical courses and management.
Subject(s)
Adult , Humans , Calcitonin , Carcinoma, Medullary , Chromogranin A , Hyperplasia , Neck , Neuroendocrine Tumors , Pathology, Surgical , Synaptophysin , Thyroglobulin , Thyroid Gland , Thyroid Neoplasms , Thyroid Nodule , Ultrasonography , BiomarkersABSTRACT
En esta revisión de literatura, proponemos aclarar conceptos almomento de tomar decisiones en la consulta por carcinomamedular de tiroides (CMT). Lo hemos dividido en 4 etapas:diagnóstica, terapéutica, seguimiento, recurrencias òpersistencias. El CMT es un tumor originado en las células Cparafoliculares, capaz de secretar calcitonina y con característicasclínico-terapéuticas que lo diferencian de los tumoresdiferenciados y lo acercan a los anaplásicos (no responden a laTSH y no captan yodo131). Representa el 5 al 10% de los cancerestiroideos y es responsable del 14% de las muertes debido a cáncertiroideo. Se dividen en esporádico (80%) y familiar (20%), estosúltimos pueden ser MEN2a (70%) y MEN2b (28%) y no MEN (2%),todos de transmisión autosómica dominante. Por ahora la cirugíasigue siendo el único tratamiento que permite la curaciónbioquímica y clínica de la enfermedad. Tanto la quimioterapia comola radioterapia carecen de resultados beneficiosos para losenfermos.
In this literature review, we intended to clarify on concepts to makedecisions in the consultation for thyroid medullary carcinoma(CMT). We divided it in 4 stages: diagnostic, treatment, follow-up,relapsing or persistence. The CMT is a tumor originated in the "C"parafollicular cells, which is able to secrete calcitonin (CT) and withclinical-therapeutic characteristics that differentiate it of the welldifferentiated tumors and make them similar to the anaplastic type,as they do not respond to the TSH and nor capture Iodine131. It5epresents 5 to 10% of thyroid cancers and it is responsible for 14%of the deaths due to thyroid cancer. They are divided in sporadic(80%), and familiar (20%), these last ones being MEN2a (70%) andMEN2b (28%) and non-MEN (2%), all having dominant autosomictransmission. Up to now, surgery continues being the onlytreatment that allows the biochemical and clinic cure of the illness.Both chemotherapy and radiation therapy lack beneficial results.
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BACKGROUND: Medullary thyroid carcinoma (MTC) that originates from C cells comprises about 10% of all the malignant thyroid tumors. Activating mutations of the RET proto-oncogene have been found to be involved in the anti-apoptotic pathway of MTC that harbors the RET mutation. We investigated the correlation between the clinicopathologic parameters and the expressions of survivin, a novel anti-apoptotic molecule, and the other apoptosis-related proteins, and the known prognostic markers. METHODS: Immunohistochemical staining was performed using antibodies for survivin, Fas, Fas ligand (FasL), bcl-2, calcitonin, CEA and cyclin A in 19 case of MTC; 10 sporadic MTCs, eight multiple endocrine neoplasia (MEN) type 2A MTCs and one familial MTC (FMTC). RESULTS: Survivin protein expression was found in five cases (26%) and this was correlated with the presence of angiolymphatic tumor emboli (p=0.019). FasL was expressed in 14 cases (74%) and it had correlation with the presence of lymph node metastases (p=0.029). The cyclin A-labeling indices were correlated with local invasiveness (p=0.001). CONCLUSIONS: Survivin and FasL might be involved in the lymphatic tumor spread of MTC.