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Anaplastic thyroid carcinoma, accounting for 5% to 15% of primary 

malignant thyroid neoplasms, is one of the most aggressive

solid tumours in humans.

Generally, it is rapidly fatal, with a mean survival of six months

after  diagnosis.

Multimodality treatment with surgery and/or external beam 

radiotherapy and chemotherapy are of fundamental

importance for  local control of disease and to enhance survival.

The differential diagnosis of thyroid anaplastic carcinoma includes 

lymphoma (some cases were previously regarded as the "small

cell  variant" of anaplastic carcinoma), thyroiditis,

metastases, and sarcomas.

Immunohistochemistry for cytokeratins is useful, but less so

for  thyroglobulin due to non-specific uptake by some other lesions.

Anaplastic carcinomas are thought to arise by differentiation

of  follicular neoplasms as evidenced by areas of poorly

differentiated  follicular carcinoma which may sometimes be

demonstrated by taking  large numbers of blocks.

It is important to be aware of the poorly cellular variant and

 some  extensively necrotic and hence inflamed examples which

may  sometimes be mistaken for benign lesions.

                         

Anaplastic thyroid carcinoma. Treatment outcome and prognostic factors. Cancer. 2005 Apr 1;103(7):1330-5.

BACKGROUND: Anaplastic thyroid carcinoma (ATC) is rare but is one of the most aggressive human malignancies. Several prognostic factors have been observed in patients with ATC, and some experts advocate aggressive multimodal therapy in selected patients. However, it is unclear whether such an approach significantly improves survival. The authors analyzed prognostic factors and treatment outcomes in patients with ATC reported in the National Cancer Institute's Surveillance, Epidemiology, and End Results data base. METHODS: The cohort consisted of 516 patients with ATC reported to 12 population-based cancer registries between 1973 and 2000. Demographic, pathologic, and treatment data were used for univariate and multivariate survival analyses. RESULTS: The mean patient age at diagnosis was 71.3 years, and there were 171 men and 345 women. Eight percent of patients had intrathyroidal tumors, 38% had extrathyroidal tumors and/or lymph node invasion, and 43% of patients had distant metastasis. The average tumor size was 6.4 cm (range, 1-15 cm). Sixty-four percent of patients underwent surgical resection of their primary tumor, and 63% received external beam radiotherapy. The overall cause-specific mortality rate was 68.4% at 6 months and 80.7% at 12 months. Univariate analysis showed that age < 60 years, female gender, intrathyroidal tumor, external beam radiotherapy, surgical resection, and combined surgical resection of tumor and radiotherapy were associated with a lower cause-specific mortality. On multivariate analysis, only age < 60 years, an intrathyroidal tumor, and the combined use of surgical and external beam radiation therapy were identified as independent predictors of lower cause-specific mortality. CONCLUSIONS: Although most patients with ATC had an extremely poor prognosis, patients < 60 years old with intrathyroidal tumors survived longer. Surgical resection with external beam radiotherapy for ATC was associated with lower cause-specific mortality.

Prognostic factors of anaplastic thyroid carcinoma. Surg Today. 2004; 34(5): 394-8.

PURPOSE: Anaplastic thyroid carcinoma (ATC) is a relatively rare but very aggressive malignancy, which usually results in death a few months after diagnosis. However, some patients survive for a long time, and the purpose of this study was to identify the prognostic factors of ATC. METHODS: We retrospectively analyzed the records of 19 patients diagnosed with ATC in our institution between 1984 and 2002. RESULTS: The median survival was 9.4 months, with a range of 0.6-76.3 months, and the 1-, 2-, and 5-year survival rates were 21%, 11%, and 5%, respectively. The mean tumor size was 5.7 cm, with a range of 3.6-10.5 cm, and complete resection was achieved in 4 patients. The prognostic factors according to univariate analysis were complete resection and small tumor size of less than 5 cm ( P = 0.005 and 0.047). However, according to multivariate analysis, complete resection was the only prognostic factor for ATC ( P = 0.015). No association was found between survival and acute symptoms, chemotherapy, radiation therapy, extrathyroidal invasion, distant metastasis, age, or leukocytosis. CONCLUSION: At present, while there is no successful treatment regimen for ATC, only patients who undergo complete resection at a very early stage have any chance of long-term survival.

Anaplastic thyroid carcinoma: a 50-year experience at a single institution. Surgery. 2001 Dec;130(6):1028-34

BACKGROUND: Anaplastic thyroid carcinoma (ATC) is among the most aggressive of human malignancies. However, there have been few large studies of histologically well-defined ATC. We report the results of a 50-year experience of this lethal malignancy. METHODS: We reviewed all cases of ATC managed in this institution between 1949 and 1999. One pathologist (J.R.G.) reviewed all pathologic material. Clinical details were obtained from medical records, and current status of all patients was determined. RESULTS: There were 134 cases, with a female-to-male ratio of 1.5:1 and a mean age of 67 years. Benign thyroid disease was present in 27 cases (20%) and well-differentiated thyroid carcinoma in 31 (23%). Sixty-two patients (46%) had distant metastases at diagnosis, and 98% of the tumors were locally invasive. Primary treatment was surgical for 96 patients (72%). Complete resection was achieved in 29 cases (30%), with "minimal residual disease" in 25. Neither extent of operation nor completeness of resection affected survival (P > .4). Postoperative radiotherapy gave slightly longer median survival (5 vs 3 months), which was not significant (P < .08). Multimodal therapy, including operation, chemotherapy, and radiotherapy, did not improve survival. CONCLUSIONS: The outlook for patients with ATC remains grim. Novel treatments for ATC are desperately needed.

Anaplastic thyroid carcinoma: current diagnosis and treatment.Ann Oncol. 2000 Sep;11(9):1083-9.

BACKGROUND: Anaplastic thyroid carcinoma (ATC), accounting for 5% to 15% of primary malignant thyroid neoplasms, is one of the most aggressive solid tumors in humans. Generally, it is rapidly fatal, with a mean survival of six months after diagnosis. Multimodality treatment with surgery and/or external beam radiotherapy and chemotherapy are of fundamental importance for local control of disease and to enhance survival. DESIGN: We evaluated consecutive patients with ATC observed at the Mayo Clinic from 1971 to 1993 and reviewed relevant articles published in major English-language medical journals. We used the MEDLINE database, selected bibliographies, and articles available in our personal files. RESULTS: ATC usually does not concentrate radioiodine or express thyroglobulin. It is essential to verify the diagnosis histologically because insular thyroid cancer, lymphomas, and medullary thyroid cancer are occasionally confused with undifferentiated neoplasms. Immunohistochemical study is helpful in establishing the diagnosis. Multimodal therapy and the development of effective systemic chemotherapeutic agents should result in improvements in survival, although no single agent has yet been identified. CONCLUSIONS: Aggressive multimodality treatment regimens show promise in improving local control in patients with ATC. However, survival rates remain low. Despite intense application of such therapy, no standardized successful treatment protocol has been established.

Anaplastic thyroid carcinoma: risk factors and outcome. Surgery. 1991 Dec;110(6):956-61.

Anaplastic thyroid carcinoma, in contrast to well-differentiated thyroid carcinoma, has a dismal prognosis, and little progress has been made in improving survival for this disease. We reviewed our experience during a 23-year period to identify risk factors and possible methods to improve outcome. Between 1966 and 1989, 340 patients with thyroid carcinoma underwent operation. Of these, 17 (5%) were undergoing operative treatment of anaplastic or undifferentiated thyroid carcinoma. The female/male ratio was 3.5:1, and mean age at presentation was 63 years. The most common presenting symptoms included neck mass, voice change, or dysphagia. Unusual presentations included symptomatic bradycardia from compression of the vagus nerve and superior vena cava syndrome. Four patients had a history of well-differentiated thyroid carcinoma. Nine patients had been diagnosed or treated in the past for "goiter" or a neck mass, and four patients had concurrent differentiated thyroid carcinoma associated with the anaplastic tumor. Thus 13 (76%) of 17 patients had a previous thyroid disorder, benign or differentiated malignant, and eight (47%) of 17 patients had previous or concurrent differentiated thyroid carcinoma. At the time of presentation, six patients had unilateral true vocal cord paralysis. At operation, 14 patients had local extension of the tumor and four required tracheostomy. Only five of 12 patients showed response to postoperative radiation therapy. Overall median survival was 12 months, and 13 (76%) of 17 patients died. The two patients alive longer than 12 months had only small foci of anaplastic carcinoma in association with well-differentiated carcinoma. Anaplastic thyroid carcinoma is a locally and systemically aggressive disease, with long-term survival seen only in those with well-localized anaplastic tumor. The major risk factor in this series is a history of previous benign or malignant thyroid disease. Because of this, a more aggressive approach to thyroid masses may be warranted. Long-standing goiters or benign nodules should be followed carefully and considered for resection if they grow or do not respond to medical therapy, and total thyroidectomy for malignant disease may obviate the subsequent development of anaplastic carcinoma. This method of early diagnosis and resection of abnormal thyroid tissue seems to be the only method currently available to improve the nearly uniform fatality of this disease.

 
May 2009

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Anaplastic carcinoma of the thyroid. Am J Surg.1999 Apr;177(4):337-9.

BACKGROUND: Anaplastic thyroid carcinoma, albeit rare, is one of the most aggressive human tumors, with a dismal prognosis. METHODS: Twenty-eight patients with anaplastic thyroid carcinoma were identified during the past 30 years to evaluate its clinicopathologic features and to document our experience in management. Potential risk factors for survival time were analyzed. RESULTS: The usual presentation was that of a rapidly enlarging neck mass. Distant metastases were present in 50% of patients on presentation. Palliative resection was performed in 16 patients. The median survival was 38 days, and the 2-year survival rate was 4%. Among factors analyzed, patients selected for surgical resection, absence of distant metastases at presentation, young age, and tumor size <6 cm were associated with an increased survival time. Concomitant well-differentiated thyroid carcinoma and p53 overexpression were present in 12 of the 22 and 13 of the 19 specimens, respectively. CONCLUSIONS: Patients with anaplastic carcinoma of thyroid have a dismal prognosis heralding imminent death. Surgical ablation followed by adjuvant therapy can provide palliation for selected patients only.

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