甲状腺髓样癌

目的探讨甲状腺髓样癌的诊断要点及临床治疗原则。方法对我院１９７６～１９９６年收治的８８例甲状腺髓样癌的临床资料进行回顾性分析。结果患者均以颈部肿块就诊,可伴发腹泻。病理证实淋巴结转移率７３２４％。散发型甲状腺髓样癌８６例,家族型甲状腺髓样癌２例。误诊率２３８６％（２１／８８）。治疗方法均以手术切除原发灶或合并颈淋巴结清除为主。术后随访局部广泛切除术的５,１０,１５年存活率分别为４５４５％,２５００％,１００％;合并行颈部淋巴清扫术的５,１０,１５年存活率分别为７２４１％,６３１６％,６６６７％。结论甲状腺髓样癌术前确诊困难,但有下列情况应考虑本病：不论甲状腺是否触及肿块,但有淋巴结肿大并伴有顽固性非炎性腹泻者;有家族史者;血清降钙素明显高于或低于正常者。作者主张不论是否触及肿大淋巴结,对甲状腺髓样癌均应行原发灶根治性切除加同侧颈部淋巴清扫术。     

甲状腺髓样癌

目的 评价手术治疗甲状腺髓样癌的疗效和术后监测血清降钙素的意义。方法 回顾性分析１９９２年１月～１９９８年１２月的１４例甲状腺髓样癌的诊治资料。结果 全组均经病理证实为甲状腺髓样癌,６４．３％并颈淋巴结转移。据ＡＪＣＣ临床分期,Ⅰ期１例,Ⅱ期７例,Ⅲ期５例,Ⅳ期１例。术后监测血清降钙素的９例中４例持续增高,经Ｂ超扫描证实有残留甲状腺癌病灶和淋巴结肿大而再次手术治疗。再次术后３例血清降钙素恢复正常,     

Medullary thyroid carcinoma.

Medullary thyroid carcinoma (MTC) is subdivided into sporadic (75 %) and hereditary (25 %) forms. Several germline mutations in the RET proto-oncogene are the source of distinct clinical phenotypes in hereditary MTC including familial MTC (FMTC) and multiple endocrine neoplasia 2A (MEN 2A) and 2B (MEN 2B). The higher the penetrance of the MEN 2 phenotype the earlier the progression of MTC which forms the basis for the currently recommended codon-related concept of prophylactic thyroidectomy. In patients with sporadic MTC, routine calcitonin (CT) measurement in nodular goiter patients has been shown to reduce the frequency of advanced tumor stages. Patients with CT levels over 100 pg/ml after pentagastrin stimulation are recommended for total thyroidectomy. In patients with unexpected sporadic MTC after histological examination, completion thyroidectomy is currently only recommended when CT levels remain elevated. The extent of lymph node dissection in patients with MTC is controversial. However, with respect to lymphonodal micrometastases, systematic compartment-oriented microdissection has been shown to reduce the frequency of lymphonodal recurrence. On the other hand, to avoid unnecessary lymph node dissection, a more individualized concept is required in the future. New chemotherapeutic agents (tyrosine kinase inhibitors), therapeutic nuclids (90Yttrium-labeled octreotide), and chemoembolization of liver metastases are currently the most promising therapeutical concepts in patients with distant metastases.     

Medullary thyroid carcinoma]

The authors report a case of medullary carcinoma of the thyroid diagnosed at the 2nd Surgery Clinic of IRCCS Policlinico "San Matteo" in Pavia in a series of 600 cases of thyroid pathology. Having emphasised the rarity of this pathology, the authors report the characteristic pathological aspects. They also outline the clinical signs leading to suspected diagnosis, illustrate the diagnostic techniques used to achieve an early diagnosis and underline the importance of the tumour markers CT and CEA. They identify radical surgery, total thyroidectomy with MND lymphadenectomy, as the therapy of choice, followed by accurate and careful monitoring with a view to ensuring an early diagnosis of renewed disease or recidivation.     

Medullary carcinoma of the thyroid

Medullary carcinoma of the thyroid (MCT) is a rare tumor which, since its identification by Hazard et al. [2] in 1959, has attracted interest for 2 reasons. First, the secretion of the tumor marker, thyrocalcitonin, provides a means of making the diagnosis of MCT before the tumor is clinically evident. Second, MCT may be associated with pheochromocytoma and parathyroid hyperplasia as the autosomal dominant familial syndrome of multiple endocrine neoplasia, type II (MEN II). MCT may occur sporadically. When MCT is encountered in the familial form the natural history varies from family to family and also in individuals within a specific kindred. This variation creates difficulties in management and in reaching the decision for an aggressive approach to surgical treatment.     

Medullary carcinoma of the thyroid

A clinical study was conducted using histological slides of all cases diagnosed as carcinoma of the thyroid in the United Birmingham (England) Hospitals since 1940 and in the Regional Histological Collection from 1953-67. A total of 23 patients, or 8% were found to have medullary carcinonma. This type is histologically distinct, being the only type to contain amyloid in the stroma. The clinical features of the patients, treatment, pathological findings, histological findings, and course of the disease are discussed and tabulated. Microscopic photographs from 6 of the cases are presented. Medullary carcinomas are of intermediate malignancy but tend to metastasize to cervical and mediastinal glands at an early stage. They do not respond to external irradiation or radio-iodine. Prognosis following surgery, however, is good. Total thyroidectomy and regional clearance of any involved glands is the treatment of choice.     

Medullary carcinoma of the thyroid gland

Nine cases of medullary carcinoma of the thyroid gland (MTC) are reported. Four of the carcinomas were of the familial type. Five of the patients were men and four were women. Patient age ranged from 23 to 66 years, with a mean age of 40 years. The median age of the four patients with the familial MTC was 32 years. A total or a subtotal thyroidectomy was performed in four and five patients, respectively, associated with a modified neck dissection in six patients with involved cervical lymph nodules. An underlying pheochromocytoma of the left adrenal was excised in one patient prior to thyroidectomy. In all cases the parathyroid glands were identified, and in two cases of familial MTC, in which they were grossly enlarged, the parathyroid glands were removed. Four patients died as a result of their disease within 3 years, whereas patients are well 4 to 12 years after surgery. The best chance of cure lies in early diagnosis and an aggressive surgical removal of the primary tumor and any cervical metastases.     

Medullary carcinoma of the thyroid gland.

Out of the discovery of concurrent multiple endocrine neoplasms has evolved the concept of multiple endocrine adenomatosis (MEA1 and MEA2). Medullary carcinoma of the thyroid gland is the most constant facet of MEA2 and is derived from C-cells of the neural crest. These cells, resembling parafollicular cells of lower animals, elaborate calcitonin which acts as a sensitive signal of the presence of the tumor. Ninety per cent of MCT occurs sporadically; in 10% the tumor presents as an atuosomal dominant trait. Other endocrinopathies, especially pheochromocytomas, are present in 70% of cases. The lesions are "cold" on iodine radioisotope scan. On microscopic examination, the appearance of amyloid is characteristic. Regional lymph node metastasis occurs early. The tumor deserves appropriate aggressive management. Surgical therapy should begin early and vigorously with the minimum procedure being total thyroidectomy. Frequent lymph node metastasis speaks for the need for regional neck dissection extended into the superior mediastinum. The search for, and the treatment of, the frequently associated endocrinopathies is essential. Pheochromocytoma must be suspected and eradicated before treatment of the thyroid tumor. A genetic workup should be included.     

Medullary thyroid carcinoma: state of the art

Medullary thyroid carcinoma (MTC) constitutes about 3-10% of all thyroid cancers. It arises from the parafollicular C cells that produce calcitonin (CT) and occurs as a sporadic form. or less commonly, as a hereditary form, as part of multiple endocrine neoplasia syndromes types 2A (MEN 2A) and 2B (MEN 2B). The hereditary forms are autosomal dominant traits associated with germline mutations of RET proto-oncogene. Progresses in genetics have permitted an improvement of management, screening and treatment. Surgery is the only successful treatment for MTC, as there is no effective adjuvant therapy for residual disease. A total thyroidectomy and vigilant management and surveillance of the neck are recommended. Interdisciplinary management including surgeons, endocrinologists, pathologists, radiotherapists, radiologists, and oncologists should be considered.     

Medullary carcinoma of the thyroid gland (sporadic, familial)

Medullary thyroid cancer (MTC) is uncommon thyroid tumor with specific characteristics which undoubtedly divide this tumor from other thyroid malignancies. Patients with sporadic or hereditary form of MTC differ in clinical presentation, recurrence of the disease and outcome. The aim of study was to establish surgical characteristics of MTC as well as clinical factors that influence surgical treatment. The study group consisted of 68 patients with MTC managed at the Center for Endocrine Surgery between 1987 and 1999. Retrospective analysis included clinical form of the disease, general data, histological and other tumor characteristics. Mean age of the patients were 47.3 years (female/male ratio: 1.5:1). Mean size of tumor was 80.5 cm3, 72.1% patients had tumor greater than 4 cm. in diameter or extrathyroid spread. The majority of patients were in II and III stadium of the disease. Primary operation (at least total thyroidectomy) was performed in 57 (84%) patients. 2(3%) had postoperatively temporally nerve palsy and 7(10.29%) temporally hypoparathyroidism. The overall survival was 46.8 +/- 9.9% after 9 years and 63.6 +/- 7.2% at 5 years. Postoperative calcitonin value is significant predictor of survival/Spearman's coefficient (R = 0.7048)/, worse prognosis is in correlation with high postoperative calcitonin values. The treatment of choice is at least total thyroidectomy and central lymph nodes resection if enlarged lymph nodes are found. Precise operative technique lowers the risk of postoperative complications. Complex approach to the patient with MTC includes all available methods in pre and postoperative evaluation as well as surgeon's knowledge and skill.     

Medullary carcinoma of the thyroid. A clinical case

A case of medullary thyroid carcinoma is reported. Moreover, the main peculiarity, the correct diagnostic approach and the therapeutic indication of this rare pathology are described.     

Medullary thyroid carcinoma presenting with an initial CEA elevation

Background: Carcinoembryonic antigen (CEA) is a tumour marker commonly associated with gastrointestinal malignancy. Patients presenting with an elevated CEA will therefore often undergo extensive investigations in order to elucidate an underlying gastrointestinal malignancy that may not be clinically apparent. However the GI tract is not the only source of CEA elevation. Methods: We present a series of patients presenting with raised CEA levels that were initially investigated for a gastrointestinal cause, but after work up were detected to have medullary thyroid cancer. Results: Four patients with raised CEA were evaluated for a gastrointestinal cause for the elevation. We discuss the non gastrointestinal causes for an elevated CEA. Conclusion: The paper highlights that in patients presenting with an elevated CEA, in whom a gastrointestinal cause has been ruled out, a tumour of neuroendocrine origin needs to be considered as a cause for the elevated CEA.     

Medullary thyroid carcinoma. Genetic screening and prophylactic thyroidectomies

Medullary thyroid cancer is a rare, neuroendocrine, tumor. It arises from parafollicular or C-cells with the ability to produce and secrete different bioactive substances like calcitonin (TC) and CEA (1-5) TC is ideal tumor marker in early diagnosis, in patents' follow up and in evaluation of their treatment. TC determinations after ca/pentagastrine stimulation test give us even more accurate results and the procedure is used for biochemical family screening. MTC occurs as a sporadic tumor or in hereditary settings MEN 2A, MEN 2B and FMCT. Germ/line point mutations in RET proto/onkogene are responsible for tumor arise and inheritance of settings. Genetic screening provides information of these RET mutations in family members even before pathologic changes occur. These individuals with MEN 2A, 2B and FMCT characteristic RET mutations are almost certain to acquire MTC (95% penetrance) in their lives and are candidates for preventive total thyroidectomy (TT), with or without central neck dissection (CND). Surgery is still the treatment of choice for MTC and only C-cell hyperplasia and early stage of MTC can be cured. Prophylactic thyroid surgery eliminates the possibility of MTC but doesn't influence appearance of other diseases (PHEO, HPTH) of MEN 2 syndromes.     

Medullary carcinoma of the thyroid: a clinical contribution

The authors report 20 cases of medullary thyroid carcinoma over the period from 1980 to 2000. Two patients turned out to be inoperable and 18 patients underwent total thyroidectomy, associated with dissection of the central lymphatic compartment in 5 patients and with dissection of the central and lateral lymphatic compartments in 10 patients with clinical or instrumental evidence of cervical lymphadenomegaly. Serum calcitonin levels proved to be a reliable marker for the diagnosis of persistence or recurrence of the disease. The follow-up, lasting from 1 to 208 months, demonstrated that in 7 cases in which serum levels of calcitonin underwent normalization there was no recurrence of disease. Among 11 cases with persistence of high calcitonin levels, 6 died and only 2 presented no evidence of metastases. On the basis of our analysis of the cases reported, total thyroidectomy associated with dissection of the central lymphatic compartment is an adequate treatment for patients in stages I and II. The authors regard routine dissection of the lateral lymphatic compartment as unadvisable.     

Medullary thyroid carcinoma: a study about 28 operated patients

In this study on 28 operated patients, the Authors show that total thyroidectomy with elective central neck dissection and ipsilateral neck dissection is the most appropriate surgical treatment for medullary thyroid carcinoma. Pathologic study on lymph nodes removed in central and in latero-cervical compartments showed malignancy respectively in 75% and in 70% of the cases. However the role of elective ipsilateral lateral neck dissection remains controversial.     

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.