Medullary colon carcinoma with microsatellite instability: case report / Carcinoma medular do cólon com instabilidade de microssatélites: relato de caso

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.

Correlation of 18F-sodium fluoride uptake and radiodensity in extraosseous metastases of medullary thyroid carcinoma

ABSTRACT Objective: Although 18F-sodium fluoride (18F-NaF) uptake is frequently observed in extraosseous metastases of medullary thyroid carcinoma (MTC) with calcification, it can also occur in metastatic sites without visible calcium deposition, leading to the hypothesis that visually undetectable calcium accumulation may be responsible for this uptake. The aim of this study was to indirectly support this hypothesis by analyzing the correlation between the degree of 18F-NaF uptake and radiodensity in extraosseous MTC metastases, since calcium deposition can increase attenuation even when not visually detectable. Subjects and methods: Extraosseous metastatic lesions of 15 patients with MTC were evaluated using 18F-NaF positron-emission tomography (PET)/computed tomography (CT) and segmented by levels of standardized uptake value (SUV). The correlation between mean SUV and mean Hounsfield unit (HU) values was assessed for the entire group of segments and for two subgroups with different mean HU values. Results: Very high correlations were observed between mean SUV and mean HU values for both the entire group of segments and the subgroup with a mean HU value greater than 130 (p = 0.92 and p = 0.95, respectively; p < 0.01). High correlation (p = 0.71) was also observed in the subgroup with mean HU values ranging from 20 to 130 (p < 0.01). Conclusion: The findings of the present study suggest that there is an association between 18F-NaF uptake and calcium deposition in extraosseous metastases of MTC, supporting the hypothesis that visually undetectable calcium accumulation may be responsible for 18F-NaF uptake in regions without visible calcium deposition.

Consensus of the Brazilian association of hematology, hemotherapy and cellular therapy on patient blood management: Assessment and management of postoperative anemia

Abstract Postoperative anemia is a complex clinical issue that requires attention due to its ramifications on the patient's recovery and prognosis. Originating from multiple determinants, such as intraoperative blood loss, hemolysis, nutritional deficiencies, systemic inflammation and impact on the bone marrow, postoperative anemia has varied and often challenging presentations. Patients undergoing major surgical procedures, in particular, are susceptible to developing anemia due to the considerable associated blood loss. Accurate diagnosis plays a crucial role in the approach, requiring meticulous hematological analysis, including hemoglobin, hematocrit and reticulocyte count, as well as an in-depth investigation of the underlying causes. An additional challenge arises in the form of the excessive practice of phlebotomy during hospitalization for clinical monitoring. Although it is essential to assess the progression of anemia, frequent removal of blood may contribute to iatrogenic anemia, further delaying recovery and possibly increasing susceptibility to infection.

Clinicopathological analysis of superior mediastinal lymph node metastases in medullary thyroid carcinoma / 中华肿瘤杂志

Objective: To investigate the clinicopathological characteristics of superior mediastinal lymph node metastases (sMLNM) in medullary thyroid carcinoma (MTC). Methods: This retrospective analysis enrolled the patients who were treated for sMLNM of MTC in our hospital from May 2012 to January 2021. All patients were suspected of sMLNM due to preoperative imaging. According to the pathological results, the patients were divided into two groups named sMLNM group and the negative superior-mediastinal-lymph-node group. We collected and analyzed the clinical features, pathological features, pre- and post-operative calcitonin (Ctn), and carcinoembryonic antigen (CEA) levels of the two groups. Logistic regression analysis was used to analyze risk factors, and receiver operation characteristic (ROC) curves were drawn to determine the optimal cut-off values of preoperative Ctn and preoperative CEA for predicting sMLNM. Results: Among the 94 patients, 69 cases were in the sMLNM group and 25 cases were in the non-SMLNM group. Preoperative Ctn level (P=0.003), preoperative CEA level (P=0.010), distant metastasis (P=0.022), extracapsular lymph node invasion (P=0.013), the number of central lymph node metastases (P=0.002) were related to sMLNM, but the multivariate analysis did not find any independent risk factors. The optimal threshold for predicting sMLNM by pre-operative Ctn is 1500 pg/ml and AUC is 0.759 (95% CI: 0.646, 0.872). The sensitivity, specificity, positive predictive value, and negative predictive value of diagnosis are 61.2%, 77.3%, 89.1%, 39.5%, respectively. In patients who underwent mediastinal lymph node dissection through transsternal approach, the metastatic possibility of different levels from high to low were level 2R (82.3%, 28/34), level 2L (58.8%, 20/34), level 4R (58.8%, 20/34), level 3 (23.5%, 8/34), level 4L (11.8%, 4/34). Postoperative complications occurred in 41 cases (43.6%), and there was no perioperative death in all cases. 14.8% (12/81) of the patients achieved biochemical complete response (Ctn≤12 pg/ml) one month after surgery, 5 of these patients were in sMLNM group. Conclusions: For patients who have highly suspicious sMLNM through imaging, combining with preoperative Ctn diagnosis can improve the accuracy of diagnosis, especially for patients with preoperative Ctn over 1 500 pg/ml. The superior mediastinal lymph node dissection for the primary sternotomy should include at least the superior mediastinal levels 2-4 to avoid residual lesions. The strategy of surgery needs to be cautiously performed. Although the probability of biochemical cure in sMLNM cases is low, nearly 40% of patients can still benefit from the operation at the biochemical level.

Clinical analysis of different parts of medullary infarction / 中华神经科杂志

Objective:To investigate the clinical, imaging, etiological and prognostic features of patients with infarctions in different locations of the medulla oblongata.Methods:Patients with acute medullary infarction hospitalized at Tianjin Huanhu Hospital from July 2017 to July 2022 were included. The risk factors, clinical manifestation, stroke mechanism and 90-day prognosis of these patients were analyzed retrospectively.Results:Among the 256 patients enrolled, 150 (58.6%) had lateral medullary infarction (LMI), 106 (41.4%) had medial medullary infarction (MMI). The most frequent clinical manifestation of patients with LMI was dizziness (84.7%,127/150). And motor disorders (83.0%,88/106) was the most frequent clinical manifestation of patients with MMI. LMI lesions were mostly located in the middle (42.7%,64/150) and MMI lesions were mostly located in the upper (60.4%,64/106) medulla oblongata, with statistically significant difference (χ 2=47.53, P<0.001). Large artery atherosclerosis (LAA) was the main stroke mechanism in LMI and MMI [57.3%(86/150) vs 56.6%(60/106)]. Early neurological deterioration was more common in MMI (25.5%,27/106) and less common in LMI (7.3%,11/150), with statistically significant difference (χ 2=16.17, P<0.001). At discharge, more patients with MMI showed poor prognosis in short term [45.3% (48/106) vs 24.0% (36/150), with statistically significant difference (χ 2=12.76, P<0.001)] and even long term at 90-day follow-up [33.0% (35/106) vs 12.7% (19/150), also with statistically significant difference (χ 2=15.48, P<0.001)] than those with LMI. A total of 10 patients (4.0%, 10/256) developed respiratory failure during hospitalization, including 7 patients with LMI (4.7%, 7/150) and 3 patients with bilateral MMI (2.8%,3/106). Early neurological deterioration ( OR=3.38, 95% CI 1.25-9.10, P=0.016) and LAA (compared with small artery occlusion) ( OR=3.08, 95% CI 1.13-8.37, P=0.028) were independent risk factors for poor prognosis in MMI. Age ( OR=1.01, 95% CI 1.01-1.17, P=0.026) and early neurological deterioration ( OR=20.19, 95% CI=2.63-155.06, P=0.004) were independently correlated with poor outcome in LMI. Conclusions:LMI and MMI had similar etiology and significant differences in clinical manifestations, early neurological deterioration and prognosis. Further classification of medullary infarction was of great significance for diagnosis, treatment and prognosis evaluation.

Advances in diagnosis and treatment of medullary thyroid carcinoma complicated with papillary carcinoma / 中华内分泌外科杂志

Thyroid cancer is the most common malignant tumor of the endocrine system, and its incidence is increasing year by year showing younger trend. The pathological types of thyroid cancer are papillary carcinoma, follicular carcinoma, medullary carcinoma and undifferentiated carcinoma. Medullary thyroid carcinoma (MTC) and papillary thyroid carcinoma (PTC) are the two common types of thyroid cancer. Since MTC and PTC have different biological origins, invasiveness and prognosis. Therefore, the occurrence of both medullary carcinoma and papillary carcinoma is very rare and rarely reported in China and abroad and the treatment is challenging. In this paper, we review the pathogenesis, ancillary tests, diagnosis and treatment of this disease, with the aim of providing reference for clinical treatment of patients with medullary thyroid carcinoma complicated by papillary carcinoma.

Reassess the value of calcitonin and carcinoembryonic antigen in the diagnosis and treatment of medullary thyroid cancer / 国际外科学杂志

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.

Lesión ovárica compleja. Localización infrecuente de metástasis de cáncer medular de tiroides. Reporte de caso y revisión de la bibliografía / Complex ovarian lesion. Infrequent localization of metastasis of medullary thyroid cancer. Case report and review of the literature

Resumen ANTECEDENTES: El carcinoma medular de tiroides es un tumor maligno poco frecuente que se origina en las células parafoliculares tiroideas productoras de calcitonina. Si bien puede aparecer espontáneamente, la asociación familiar pocas veces es excepcional. Aunque la supervivencia en estadios iniciales es considerable, es frecuente la posibilidad de metástasis al momento del diagnóstico. CASO CLÍNICO: Paciente de 72 años, con un abultamiento en la región anterior del cuello y una adenopatía cervical izquierda de 15 días de evolución. La TAC, la ecografía y la biopsia reportaron: carcinoma bilateral de ovario poco diferenciado. Se procedió a la cirugía radical (histerectomía, doble anexectomía, linfadenectomía pélvica y para aórtica, omentectomía y apendicectomía) con la que se consiguió una citorreducción completa. En el estudio definitivo se encontraron metástasis de carcinoma medular de tiroides en ambos ovarios, miometrio y peritoneo. Enseguida se inició la quimioterapia con vandetanib. Después de 25 ciclos de tratamiento, la paciente continuó con el ácido zoledrónico y asintomática. Un mes después de finalizarlo comenzó con crisis comiciales que se trataron con quetiapina, con respuesta satisfactoria. Posteriormente, la paciente tuvo una celulitis facial que se trató con antibiótico y corticosteroide; tres semanas después falleció debido a insuficiencia multiorgánica. CONCLUSIONES: El carcinoma medular de tiroides es una rara localización de metástasis que aún requiere investigación que permita establecer protocolos de diagnóstico, tratamiento y estimación de la supervivencia.

Abstract BACKGROUND: Medullary thyroid carcinoma is a rare malignant tumor originating in the calcitonin-producing parafollicular thyroid cells. Although it can occur spontaneously, familial association is rarely exceptional. Although survival in early stages is considerable, the possibility of metastasis at the time of diagnosis is frequent. CLINICAL CASE: 72-year-old patient with a lump in the anterior neck region and left cervical adenopathy of 15 days of evolution. CT, ultrasound, and biopsy reported: bilateral poorly differentiated ovarian carcinoma. Radical surgery (hysterectomy, double adnexectomy, pelvic and para-aortic lymphadenectomy, omentectomy and appendectomy) was performed and complete cytoreduction was achieved. In the definitive study, metastases of medullary thyroid carcinoma were found in both ovaries, myometrium and peritoneum. Chemotherapy with vandetanib was then started. After 25 cycles of treatment, the patient continued with zoledronic acid and was asymptomatic. One month after the end of the treatment, she began to have comic crises that were treated with quetiapine, with satisfactory response. Subsequently, the patient had a facial cellulitis that was treated with antibiotics and corticosteroids; three weeks later she died due to multiorgan failure. CONCLUSIONS: Medullary thyroid carcinoma is a rare site of metastasis that still requires research to establish diagnostic and treatment protocols and to estimate survival.

Guidelines from the Brazilian society of surgical oncology regarding indications and technical aspects of neck dissection in papillary, follicular, and medullary thyroid cancers

ABSTRACT Objective: The purpose of these guidelines is to provide specific recommendations for the surgical treatment of neck metastases in patients with papillary, follicular, and medullary thyroid carcinomas. Materials and methods: Recommendations were developed based on research of scientific articles (preferentially meta-analyses) and guidelines issued by international medical specialty societies. The American College of Physicians' Guideline Grading System was used to determine the levels of evidence and grades of recommendations. The following questions were answered: A) Is elective neck dissection indicated in the treatment of papillary, follicular, and medullary thyroid carcinoma? B) When should central, lateral, and modified radical neck dissection be performed? C) Could molecular tests guide the extent of the neck dissection? Results/conclusion: Recommendation 1: Elective central neck dissection is not indicated in patients with cN0 well-differentiated thyroid carcinoma or in those with noninvasive T1 and T2 tumors but may be considered in T3-T4 tumors or in the presence of metastases in the lateral neck compartments. Recommendation 2: Elective central neck dissection is recommended in medullary thyroid carcinoma. Recommendation 3: Selective neck dissection of levels II-V should be indicated to treat neck metastases in papillary thyroid cancer, an approach that decreases the risk of recurrence and mortality. Recommendation 4: Compartmental neck dissection is indicated in the treatment of lymph node recurrence after elective or therapeutic neck dissection; "berry node picking" is not recommended. Recommendation 5: There are currently no recommendations regarding the use of molecular tests in guiding the extent of neck dissection in thyroid cancer.

Histogram analysis of based on two-dimensional ultrasound images to differentiate medullary thyroid carcinoma and thyroid adenoma / 中华肿瘤杂志

Objective: To investigate the feasibility and value of histogram analysis based on two-dimensional gray-scale ultrasonography in the differential diagnosis of medullary thyroid carcinoma (MTC) and thyroid adenoma (TA). Methods: The preoperative ultrasound images of 86 newly diagnosed MTC patients and 100 TA patients treated in the Cancer Hospital of Chinese Academy of Medical Sciences from January 2015 to October 2021 were collected. Histograms were performed based on the regions of interest (ROIs) delineated manually by two radiologists, thereafter, mean, variance, skewness, kurtosis, percentiles (1st, 10th, 50th, 90th, 99th) were generated. The histogram parameters between the MTC group and the TA group were compared, and the independent predictors were screened by multivariate logistic regression analysis. Receiver operating characteristic (ROC) analysis was used to compare the individual diagnostic efficacy and joint diagnostic efficacy of independent predictors. Results: Multivariate regression analysis showed that mean, skewness, kurtosis and 50th percentile were independent factors. The skewness and kurtosis in the MTC group were significantly higher than those in the TA group, and the mean and 50th percentile were significantly lower than those in the TA group. The area under the individual ROC curve of mean, skewness, kurtosis and 50th percentile is 0.654-0.778. The area under the combined ROC curve is 0.826. Conclusion: Histogram analysis based on two-dimensional gray-scale ultrasonography is a promising tool to distinguish MTC from TA, in which the joint diagnosis value of mean, skewness, kurtosis and 50th percentile is the highest.

Medullary carcinoma of jejunum presenting as perforation peritonitis: A case report

Small intestinal medullary carcinoma (MC) is a newly recognized subclass of small intestinal carcinomas and is an exceptional entity for this site. A search of the literature for similar cases arising in the small intestine revealed only six previously reported cases. Here we present a case of MC arising in the jejunum of a 65-year-old male. The patient presented to the emergency with features of perforation peritonitis with liver metastasis and no known predisposing factors like inflammatory bowel disease and celiac disease. Studies conducted on this tumor's colonic counterpart have shown microsatellite instability (MSI) and B-type Raf kinase (BRAF) mutations; however, few exceptions are known. Also, this subtype of carcinoma is known to have a better prognosis than its other histological subtypes.

Revisão de anatomia e correlações clínicas do tronco encefálico, parte i: bulbo / Review of brain anatomy and clinical correlations, part i: medulla

O Tronco encefálico (TE) é uma estrutura singular do sistema nervoso central, pois nele passam tratos sensoriais ascendentes da medula espinal, tratos sensoriais da cabeça e do pescoço, os tratos descendentes motores originados no prosencéfalo (divisão mais rostral do encéfalo), e as vias ligadas aos centros de movimento dos olhos. Contém ainda os núcleos dos nervos cranianos e está envolvido na regulação do nível de consciência através de projeções ao prosencéfalo oriundas da formação reticular. Todas essas estruturas coexistem em um espaço muito exíguo, o que faz com que o TE seja um local muito sensível às alterações patológicas, sendo que os pacientes apresentam muitos sinais neurológicos mesmo com lesões muito pequenas nesse local. Compreender a anatomia interna do TE é essencial para o diagnóstico neurológico e a prática da medicina clínica. Outros profissionais da saúde também se beneficiam desse conhecimento para melhor manejo dos seus pacientes neurológicos. Essa revisão apresenta detalhes da anatomia macroscópica e microscópica do bulbo, bem como seus correlatos clínicos frente às lesões mais comuns dessa divisão particular do TE, conhecidas como síndromes bulbares.

The brainstem is a unique structure in the central nervous system, since it gives way to ascending sensory tracts from the spinal cord, sensory tracts from the head and neck, motor descending tracts originating from the forebrain, and the pathways connected to the eye movement centers. It also contains the cranial nerve nuclei and is involved in the regulation of consciousness levels through projections to the forebrain originating in the reticular formation. All these structures coexist in a very small space, which makes the brainstem very sensitive to pathological changes, with patients presenting several neurological symptoms even with very small brainstem lesions. Understanding the internal anatomy of the brainstem is essential for neurological diagnosis and the practice of clinical medicine. Other health professionals also benefit from this knowledge to better manage their neurological patients. This review presents detailed information on the macroscopic and microscopic anatomy of the medulla, as well as its clinical correlates in the face of the most common lesions of this particular division of the brainstem, known as medullary syndromes.

Unusual Presentation of a Thyroid Malignancy: A Case Report

Medullary thyroid carcinoma is a rare malignancy. We report a case of Medullary thyroid carcinoma in a 56-year-old male patient. Patient presented with lower limb paresis and severe hypokalemia. Cushing’s syndrome is a rare complication of Medullary thyroid carcinoma [MTC] and is due to ectopic Adrenocorticotropic [ACTH] secretion by tumor cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Entire clinical history, laboratory investigations, microscopic pictures are discussed in detail.

Effect of Yiqi Jiedu Prescription-containing Serum on Proliferation of mTEC and Regulatory T Cells in Myasthenia Gravis Patients with Thymus Hyperplasia / 中国实验方剂学杂志

ObjectiveTo investigate the effect of Yiqi Jiedu prescription-containing serum on the proliferation of medullary thymic epithelial cells （mTEC） and regulatory T （Treg） cells in myasthenia gravis （MG） patients with thymus hyperplasia. MethodAccording to serological methods，35 SD rats were adaptively fed for one week and randomized into the low-，medium-， and high-dose Yiqi Jiedu prescription groups，control group， and prednisone group，with seven rats in each group， which were then gavaged with the corresponding drugs for one week for preparing the drug-containing serum. The effect of Yiqi Jiedu prescription-containing serum at different concentrations on the proliferation of mTEC and Treg cells were determined by cell counting kit-8 （CCK-8） assay. Besides， the effect of mTEC and Yiqi Jiedu prescription-containing serum on Treg cell proliferation were observed through co-culture. ResultThymocytes were cultured for a period of time. Their mean positive rate revealed by flow cytometry using mTEC characteristic marker Ulex europaeus agglutinin Ⅰ （UEAI） was 92.54%. Treg cells were sorted by magnetic beads. The purity of Treg cells after repeated magnetic bead sorting was as high as 92%. mTEC and Treg cells showed high positive expression rates，and their cell purity met the requirements of subsequent experiments. When the concentration of Yiqi Jiedu prescription-containing serum was 2.5%-15%，it exhibited an inhibitory effect against mTEC and Treg cells. When the concentration was equal to or greater than 20%，it promoted cell proliferation，which was further enhanced with the extension of action time. The results after 48 h of culture showed that compared with the control group，prednisone and low-dose Yiqi Jiedu prescription had no significant effect on the proliferation of these two kinds of cells，but the medium- and high-dose Yiqi Jiedu prescription remarkably reduced their proliferation inhibition rate （P<0.01）. After co-culture with mTEC， the control group was not significantly different from the prednisone group and the low-dose Yiqi Jiedu prescription-containing serum group in the proliferation of Treg cells，while the medium- and high-dose Yiqi Jiedu prescription-containing serum groups significantly lowered the proliferation inhibition rate （P<0.01）. ConclusionYiqi Jiedu prescription-containing serum affects the proliferation of mTEC and Treg cells in MG patients with thymus hyperplasia. Compared with the solely cultured Treg cells isolated from MG patients，the Treg cells co-cultured with mTEC exhibit enhanced proliferation in MG patients，suggesting that mTEC can regulate the proliferation of Treg cells. This effect becomes more obvious after the intervention with Yiqi Jiedu prescription-containing serum，indicating that intervention effect of Yiqi Jiedu prescription on Treg cells can be produced during its treatment of mTEC， which may be one of the mechanisms of Yiqi Jiedu prescription-containing serum in alleviating MG.

Value of 18F-FDG PET/CT Imaging in Secondary Extramedullary Infiltration of Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To investigate the characteristics of 18F-FDG PET/CT images of multiple myeloma secondary extramedullary infiltration in order to improve recognition.@*METHODS@#Twenty-one patients with multiple myeloma secondary extramedullary infiltration confirmed by pathology or follow-up from January 2012 to October 2020 in the First Affiliated Hospital of University of Science and Technology of China were retrospectively analyzed. All the patients underwent 18F-FDG PET/CT imaging before treatment, and the PET/CT characteristics of extramedullary infiltration and bone marrow were analyzed.@*RESULTS@#Twenty-one patients included 12 males and 9 females, aged from 41 to 77 years old, with an average of 58.3±10.0; 9 cases of extramedullary infiltration involving lymph nodes; lung, stomach, spleen, and kidney were involved respectively in 2 cases; retroperitoneal, right auricle, subcutaneous nodule, and spinal meninges involvement were reported in each one case respectively. The maximum SUVmax value of extra-medullary lesions was 21.2, the minimum value was 2.1, and mean was 7.7±5.3. The maximum SUVmax value of bone marrow was 33.5, the minimum was 2.4, and mean was 6.6±3.6. There was no statistically significant difference in SUVmax value between extra-medullary lesions and bone marrow (Z=-1.195, P=0.232).@*CONCLUSION@#18F-FDG PET/CT not only has a good diagnostic value for multiple myeloma, but also a good evaluation value for secondary extramedullary infiltration, which provides reference for clinical treatment and prognosis.

Clinical investigation and ret proto / 西安交通大学学报(医学版)

【Objective】 To investigate the clinical features and gene analysis of one pedigree with multiple endocrine neoplasia type 2A （MEN2A） so as to clarify the diagnosis and classification of the disease， guide treatment and prevention， and improve prognosis. 【Methods】 The clinical data of a 36-member MEN2A family， including 6 probands， with medullary thyroid carcinoma， were investigated， and the peripheral blood genomic DNA of 28 family members （blood sample of one proband was not collected） was extracted. PCR amplification was performed on exons 8， 10， 11， 13， 14， 15 and 16 of the RET gene， and the products were directly sequenced. 【Results】 Review of the medical history showed that two probands with medullary thyroid carcinoma were accompanied with hyperparathyroidism， and one family member had pheochromocytoma. The RET gene mutation test confirmed that 13 family members， consisting of 5 probands and 8 family members， had the RET proto-oncogene exon 10 missense mutation. The heterozygous missense had mutation c.1852T>A， leading to the conversion of cysteine （TGC） at position 618 to serine （AGC） （Cys618Ser）. All subjects carrying RET gene Cys618Ser mutation had abnormal thyroid ultrasound change， accompanied with elevated calcitonin levels. Subjects carrying wild type of RET gene had normal calcitonin levels. The family was finally diagnosed with MEN2A by RET gene detection. 【Conclusion】 RET gene detection plays key role in the diagnosis and treatment of patients with MEN2A family and has guiding value in the follow-up and prognosis of asymptomatic carriers. There is a positive correlation between calcitonin level and the RET protooncogene mutation Cys618Ser. Patients suspected of MEN2A should be screened in time.

Meta-analysis of the prognosis of medullary thyroid carcinoma patients with biochemical recurrence and the determination of optimal cut-off value of calcitonin for predicting postoperative structural recurrence / 肿瘤研究与临床

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.

Risk Factors of lateral cervical lymph node metastases and survival analysis in patients with medullary thyroid carcinoma / 中华内分泌外科杂志

Objective:To investigate the risk factors of cervical lymph node metastasis (LNM) and survival analysis in patients with medullary thyroid carcinoma (MTC) .Methods:93 patients with MTC admitted to the Department of General Surgery and Department of Otorhinolaryngology, First Medical Center of PLA General Hospital from Sep. 2008 to Aug. 2020 were analyzed retrospectively, including 45 males and 48 females, with an average age of 47 years old. SPSS 26.0 statistical software was used for data processing of the initial surgical year and procedures, tumor pathological stages, preoperative calcitonin (Ctn) level, preoperative carcinoembryonic antigen (CEA) level, LNM status, recurrence free survival (RFS) , etc. The risk factors of LNM and prognosis of MTC patients were analyzed by COX univariate and multivariate regression. Kaplan Meier method was used to estimate the survival rates of independent risk factors affecting prognosis and draw their survival curves.Results:The median follow-up time of 93 patients was 53 months, ranging from 2 to 192 months. The 1-year, 3-year, 5-year and 10-year survival rates were 97.8%, 96.6%, 94.6% and 88.9% respectively. Multivariate COX regression analysis showed that Initial surgical procedures ( P=0.018) and preoperative Ctn level ( P=0.012) were independent risk factors of central cervical LNM. Preoperative Ctn level ( P=0.028) and Capsule invasion ( P=0.024) were the independent risk factors of lateral cervical LNM. Preoperative Ctn level≥180.30 pg/ml and ≥234.15pg/ml indicated central and lateral cervical LNM respectively (all P<0.001) . Distant metastasis was an independent risk factor of RFS ( P=0.037) of MTC. Conclusions:Standardized surgical procedures are recommended for initial treatment of MTC, which can reduce the possibility of residual occult metastasis and the risk of reoperation. Distant metastasis affects prognosis of MTC.

Total thyroidectomy versus lobectomy for unilateral sporadic medullary thyroid carcinoma / 中华普通外科杂志

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.

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)