甲状腺髓样癌20例临床分析

目的 探讨甲状腺髓样癌临床特点、诊断要点及治疗原则.方法 回顾性分析2005年1月至2012年12月20例甲状腺髓样癌患者的临床资料,全组病例均经病理证实为甲状腺髓样癌,颈淋巴结转移9例,远处转移1例,总颈部淋巴结转移率达50％.20例均行手术治疗,至少行患侧腺叶及峡部切除16例,占80％ （16/20）,其中行甲状腺全切除9例.结果 术后常规监测血清降钙素,复发2例.术后随访17例,超过5年者9例,5年生存率达77.8％.结论 甲状腺髓样癌术前诊断困难,多数依赖组织病理学检查确诊,强调细针穿刺细胞学检查的重要性,治疗以根治性手术为主,术后监测降钙素能及早发现局部复发和转移.     

甲状腺髓样癌的超声特征分析

目的:总结甲状腺髓样癌（medullary thyroid cancer, MTC）的超声特征并分析其对MTC诊断的临床价值。方法:回顾性分析2013年4月至2018年11月郑州大学第一附属医院甲状腺外科收治并经手术确诊的91例MTC患者（共103个结节）,其中男36例,女55例,收集其术前超声影像学特征及血清降钙素水...     

甲状腺髓样癌15例诊治分析

目的 提高甲状腺髓样癌的术前诊断率.方法 回顾性分析15例甲状腺髓样癌的临床资料.结果 髓样癌除合并内分泌综合征者外,绝大多数与其他类型甲状腺癌相似,多以颈部肿块就诊,术前确诊困难,误诊率较高;多数系术中冰冻病理或术后病理确诊.结论 甲状腺髓样癌术前确诊困难,因此我们应采取以测定血清降钙素为主等一系列检测手段来作为甲状腺髓样癌的术前诊断程序.     

甲状腺髓样癌的诊治现状

甲状腺髓样癌（medullary thyroid carcinoma,MTC）是起源于甲状腺滤泡旁细胞（C细胞）的少见特殊甲状腺癌,属于侵袭性神经内分泌肿瘤。降钙素和癌胚抗原仍是目前常用的标志物,其升高能可靠诊断MTC。其术前测定值,术后倍增时间对手术淋巴结清扫范围、预后判断有重要意义。手术治疗建议甲状腺全切除和中央区淋巴结清扫,必要时侧区清扫。推荐常规进行RET基因检测。对无法手术或远处转移病人,考虑靶向治疗。仍需加强MTC病理机制研究。     

甲状腺髓样癌15例

甲状腺髓样癌（medullary thyroid carcinoma,MTC）是甲状腺恶性肿瘤中较少见的病理类型,起源于甲状腺滤泡旁细胞（C细胞）,或是RET原癌基因种系突变所致,多散发,具有独特的临床特征。MTC占全部甲状腺恶性病的5%～8%[1],     

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目的 探讨甲状腺髓样癌(MTC)的病理学特点，诊断和治疗选择。方法 对1982-2001年经治的32例MTC的临床和病理资料进行分析总结。结果 32例均予手术切除原发灶或加行颈淋巴结清扫术。均经病理证实，淋巴结转移率为65．4％。术后5年、10年生存率分别为77．8％，62．5％。结论 MTC具有多分化肿瘤的特点，可产生多种神经内分泌物质，少数病人可出现类癌综合征或顽固性腹泻。MTC的治疗以根治性切除为原则，而又要比其他类型的甲状腺癌更为积极，决不可任意缩小手术范围。     

甲状腺髓样癌的诊断与治疗

甲状腺髓样癌(MTC)来源于滤泡旁C细胞,占甲状腺癌的5%～10%,其中25%具有遗传性.MTC能够较早的出现淋巴结及远处转移,故早期诊断及治疗尤为重要.本文描述了MTC实验室及影像学等方面的主要表现,并对最新的治疗手段及预防性措施进行了阐述.     

Medullary carcinoma of the thyroid: Prognostic factors and treatment recommendations

Background: Because medullary thyroid carcinoma accounts for only 7% of all thyroid malignancies, data to support treatment strategies are scarce. Methods: We retrospectively reviewed treatment and outcome in 34 patients with MTC treated at Roswell Park between 1961 and 1995. Univariate analysis was performed using the variables age, sex, tumor size, N stage, and M stage. Results: Median survival was 4.7 years, with 51% and 32% of patients alive at 5 and 15 years, respectively. Nodal metastases were seen in 76% and distant metastases in 67% of all patients. More than 60% of the patients with nodal metastases survived longer than 10 years. Once diagnosed with distant metastases, 90% of the patients died within 5 years. Local failure rate with lobectomy was 44%, compared to 10% after total thyroidectomy (P<.02). Age, extrathyroid extension, and M stage portend a poor outcome. Nodal status had no statistically significant impact on survival. Conclusion: Survival with tumors confined to the thyroid gland is independent of nodal status. Long-term survival in patients with distant metastases is rare. This study underscores the role of total thyroidectomy in the initial treatment and the need to develop effective adjuvant therapy for MTC.Presented at the 50th Annual Cancer Symposium of The Society of Surgical Oncology, Chicago, Illinois, March 20–23, 1997.     

甲状腺结节患者常规行血清降钙素检查对早期发现甲状腺髓样癌的临床价值

目的:探讨甲状腺结节患者血清降钙素(Ct)检测对甲状腺髓样癌(MTC)的诊断价值。方法:回顾性分析2016年10月—2017年8月1922例因甲状腺结节就诊于郑州大学第一附属医院甲状腺外科并行手术治疗的患者资料。所有患者术前均常规行血清Ct检测,术后均有明确的常规病理结果。分析Ct水平与MTC的关系、用受试者工作特征曲线分析Ct对MTC的诊断效能。结果:在1922例甲状腺结节患者中,新发现的MTC患者有12例(0.62%)。在术前血清Ct值为10~100pg/mL的155例、100~500pg/mL的12例和500pg/mL的10例中,术后确诊为MTC的分别为1例(0.65%)、1例(8.33%)和9例(90.00%)。在血清Ct500pg/mL的9例MTC患者中,有8例(88.89%)出现了不同程度的颈部淋巴结转移。血清Ct诊断MTC的截断值为258.5pg/mL,敏感性为90.91%,特异性为96.99%。结论:术前常规检测血清Ct有助于MTC的早期诊断,对患者接受彻底的手术治疗,避免二次手术有十分重要的意义。     

Synchronous multifocal medullary and papillary thyroid microcarcinoma detected by elastography

INTRODUCTION

A few cases of concomitant medullary and papillary carcinoma in the same thyroid nodule have been described in the literature. However, the presence of multiple foci of both types of malignancy in the same gland is very rare.

PRESENTATION OF CASE

A 39 year-old female with multiple thyroid nodules, elevated serum calcitonin levels and elastographic findings suggestive of thyroid malignancy, underwent total thyroidectomy and central neck dissection. Histology revealed the presence of one focus of medullary and one focus of papillary carcinoma on each thyroid lobe. Subsequently, the patient underwent treatment with radioactive iodine.

DISCUSSION

This is the third case of synchronous multifocal medullary and papillary thyroid carcinoma reported in the literature. Several theories for the simultaneous development of these malignant entities have been proposed.

CONCLUSION

Ultrasound elastography can be a useful, noninvasive tool in the assessment of thyroid nodules.     

Hyalinizing trabecular adenoma--an uncommon thyroid tumor frequently misdiagnosed as papillary or medullary thyroid carcinoma

BACKGROUND: Hyalinizing trabecular adenoma (HTA) is an uncommon benign thyroid tumor that can present as a solitary thyroid nodule, a prominent nodule in a multinodular goiter, or as an incidental finding in a thyroidectomy specimen. The clinical significance of the lesion is that it is frequently misdiagnosed as papillary carcinoma on fine-needle aspiration cytology or as papillary or medullary carcinoma on histopathological section. We reviewed our recent experience with 7 patients diagnosed with HTA. METHODS: Fine-needle aspiration biopsy was performed in 7 patients presenting with a solitary thyroid nodule (n = 4) or a multinodular goiter (n = 3). The patients underwent total thyroidectomy (n = 6) or hemithyroidectomy (n = 1). RESULTS: In 4 patients, the preoperative cytology was suggestive of papillary carcinoma, in 2 patients suspicious, and in 1 patient positive for papillary carcinoma. On histopathological section, 2 patients had a microscopic HTA, 2 patients had HTA in 1 or 2 nodules of a multinodular goiter, and 3 patients had HTA in a solitary nodule. Except in 1 patient, who had a microscopic focus (3.2 mm) of papillary carcinoma, there was no evidence of malignancy in the surgical specimens on permanent histopathological section. CONCLUSIONS: Although HTA is a rare condition of the thyroid, the surgeon needs to be aware of this entity to be able to better discuss the pathological findings with the patient, particularly since some pathologists and endocrinologists believe that HTA may represent a malignant neoplasm of low metastatic potential.     

Skip metastases in medullary thyroid carcinoma: A single-center experience

PURPOSE: Total thyroidectomy (TT) with level VI and VII central neck dissection is the initial treatment for medullary thyroid carcinoma (MTC) without identifiable neck metastasis. Level II to V lateral neck dissection is performed if neck metastasis is present or suspected. We conducted this study to identify the frequency and clinical determinants of skip neck metastasis in MTC. METHODS: We reviewed the medical records of 32 patients who underwent TT and bilateral neck dissection for MTC. The clinical features were correlated with pN status in the central versus lateral compartments of the neck. RESULTS: Neck lymph node metastasis (pN+) was found in 20 patients (62.5%) and skip metastases were found in 7 (35%) patients. The sensitivity of the pN status of the central compartment of the neck to predict the pN status of the lateral compartment of the neck was 53.8% and specificity was 63.2%. We found pN+ in 90% of the patients with lymph nodes >15 mm in diameter versus 50% in those with lymph nodes <15 mm in diameter. CONCLUSIONS: There is skip metastasis in MTC. It is unsafe to use the lymph node status of the central compartment of the neck to define the pN status of the lateral neck. A lymph node greater than 15 mm in diameter is related to pN status.     

Influence of lymph node metastases on survival in pediatric medullary thyroid cancer

Purpose

Lymph node metastases (LNM) from medullary thyroid cancer (MTC) are common in adults and are a poor prognostic factor. Less is known about LNM in children, who often have hereditary forms of MTC. Guidelines recommend prophylactic thyroidectomy in early childhood, but randomized prospective trials are not feasible. We hypothesized that LNM is associated with poor prognosis in children.

Methods

Patients with MTC 21 years or younger from the National Cancer Data Base from 1985 to 2007 were studied. Multivariable logistic regression was used to identify factors associated with lymph node evaluation. Survival was estimated using the Kaplan-Meier method.

Results

Of 430 patients, 276 (64.2%) had nodal evaluation with LNM present in 121 (28.1%). Older patients, those with tumors larger than 2 cm, and those with involved margins were more likely to have LNM (all P < .05). Patients undergoing total thyroidectomy, those with involved margins, and older patients were more likely to undergo lymph node evaluation after controlling for patient, tumor, and hospital factors (all P < .05). Over time an increasing number of patients with MTC have undergone total thyroidectomy. Patients 16 to 21 years of age had lower 10-year overall survival compared to patients 0 to 15 years old (88.7% vs 98.1%, P = .005). Lymph node metastases were also associated with decreased 10-year overall survival (84.4% vs 100%, P < .001).

Conclusions

In pediatric MTC, LNM predict poorer overall survival. Adequate lymph node assessment can provide valuable prognostic information for the pediatric MTC patient. Lymph node assessment should be considered for older pediatric patients undergoing surgery for hereditary MTC or biopsy confirmed MTC.     

Increased incidence of medullary thyroid carcinoma in patients treated for Hirschsprung's disease

Background/Purpose

Mutations of the RET proto-oncogene are responsible for the development of inherited multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma (MTC). RET mutations are encountered in patients with Hirschsprung's disease (HD). We hypothesized that the incidence of MTC is increased in patients with HD.

Methods

Patients treated for HD at the Children's Hospital, University of Helsinki, during 1939 and 1986 were surveyed for cancer using the population-based countrywide Finnish Cancer Registry from 1967 to 2000. The number of observed cancer cases and that of person-years at risk were counted. The expected number of cancer cases was extrapolated from national cancer incidence rates. To calculate the standardized incidence ratios (SIRs), the observed number of cancer cases was divided by the expected number of cancer cases.

Results

One hundred fifty-six patients (132 males) with HD were identified. The mean length of patient follow-up was 30.9 years. Seven cases of cancer were observed (SIR, 3.5; 95% CI, 1.4-7.3). Two patients developed MTC (SIR, 550; 95% CI, 67-2000). The cases of MTC occurred in male patients at the ages of 34 and 37 years. No patient developed pheochromocytoma.

Conclusions

In this study, we report for the first time an increased risk of MTC occurring in patients treated for HD. The increased risk may be attributed to mutations of the RET proto-oncogene shared by MTC and HD. These findings warrant further studies concerning screening for MTC-type RET mutations in patients with HD.