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Familial thyroid cancers of follicular origin are rare and include syndromic and non-syndromic tumours. In familial adenomatous polyposis, the prevalence of papillary thyroid cancer is 2-12% and in 20-40% of cases it is a cribriform-morular papillary thyroid carcinoma. Morules and cribriform pattern are the two main typical criteria, associated with a nuclear and cytoplasmic immunopositivity for beta catenin. DICER1 syndrome is associated with pleuropneumoblastoma, ovarian tumors and thyroid pathology (multinodular goiter and less frequently a well-differentiated thyroid cancer without microscopic particularity). Cowden syndrome is characterized by multiple hamartomas and two-thirds of patients develop thyroid pathology, including multinodular goiter (50-67%) and cancer (35%), the latter being one of the major diagnostic criteria of the syndrome. Classic triad of Carney complex associates lentiginosis, myxoid tumors, and various endocrine abnormalities; thyroid pathology occurs in 10% of cases and may be benign or malignant. In Werner's syndrome, thyroid cancer is present in 18% of cases. McCune-Albright syndrome is characterized by fibrous dysplasia, café-au-lait spots and various endocrinopathies including hyperthyroidism and nodular hyperplasia. Non-syndromic thyroid cancers, which represent the majority of familial cancers, are most often papillary carcinomas. In daily practice, in the presence of multiple benign thyroid nodules and/or thyroid cancer in a young person, or with family thyroid diseases, the pathologist should be aware about hereditary predispositions to propose an oncogenetic consultation.

Fine needle aspiration cytopathology is currently the best approach for evaluating thyroid nodules. Follicular lesions represent a morphological limit which may be overcome using immunocytochemistry. We report here a series of known markers useful in cytology and helping the cytopathologist in distinguishing benign from malignant follicular neoplasms of the thyroid.

The management of diagnosis, treatment and outcome of malignant neoplasm arising in a lingual thyroid.We report a case of papillary-follicular carcinoma arising in a lingual thyroid.Lingual carcinoma causes dysphagia and pain due to oropharyngeal obstruction. Surgical pharyngotomy with a trans-hyoid approach provides good access to the lesions. Therefore, the eutopic thyroid was present, in pretracheal position, without neoplastic proliferation.Carcinoma arising in lingual thyroid is an extreme rare entity. Its diagnosis is histologic. Its treatment is surgical associated with radio-iodine therapy.

Cutaneous metastasis of thyroid cancer is rare. When it occurs, it usually affects the scalp and neck. We report here a case where follicular thyroid carcinoma metastasized to unusual skin locations.An 82-year-old woman was followed after a total thyroidectomy for follicular thyroid carcinoma. Assessment after 2 years showed the presence of a small cervical residue, pulmonary metastases, and a high thyroglobulin level. Two sets of internal radiation therapy with iodine 131 (100 mCi each) followed. Response to treatment was poor, and cutaneous and subcutaneous nodules appeared on the abdomen, back, and front left thigh. Biopsy of these lesions showed that they were malignant, and testing with anti-thyroglobulin antibodies confirmed the diagnosis of cutaneous metastasis of follicular thyroid carcinoma.Although the rare cutaneous metastases of follicular thyroid carcinoma usually occur on the scalp, firm nodular cutaneous lesions that are painless and resist standard treatment in patients with a history of thyroid cancer should suggest the possibility of metastasis.

To study the expression of cytokeratin 19 (CK-19), HBME-1 and Ret in follicular-patterned thyroid tumors, and their significance for the diagnosis of the follicular variant of papillary thyroid carcinoma.111 well-differentiated follicular tumors were examined by immunohistochemistry: 59 papillary carcinomas (43 of the follicular variant), 40 follicular adenomas (among which 11 atypical adenomas), 10 oxyphil cell tumors and 2 follicular carcinomas.CK-19 was diffusely expressed in all the papillary carcinomas, and was also expressed in 2/4 oxyphil cell carcinomas and 4/11 atypical adenomas. 90% of the follicular adenomas (26/29), the six oxyphil cell adenomas and the two follicular carcinomas showed at best a focal staining of dystrophic areas. 78% of the papillary carcinomas and 3/11 atypical adenomas were stained with HBME-1, whereas 26/29 adenomas (90%) and the 10 oxyphil cell tumors were negative. 34% of the papillary carcinomas expressed Ret (most of them were of the usual type (14/20)). The staining was often weak and focal (13/20). The other tumors were all negative for Ret.CK-19 is a sensitive (100%) and specific (82.5%) marker of papillary carcinomas, which can be helpful in the diagnosis of its follicular variant. HBME-1 can improve this specificity when associated with CK-19. The Ret protein expression is of limited practical interest.

The aim of this retrospective study was to assess the role of thyroid peroxidase immunodetection in the cytological diagnosis of follicular variants of thyroid papillary cancer (FVTPC) which are difficult to identify by standard cytology.Between 1991 and 1998, 3,505 thyroid fine needle aspiration biopsies were performed by thyroid peroxidase immunocytochemistry and 1,576 patients were operated on. Out of a total of 227 thyroid papillary cancers (TPC), 42 (18.5%) were diagnosed as FVTPC. The results of standard cytology and thyroid peroxidase immunodetection were compared with the histological findings.The rate of false negatives for TPC in standard cytology was 11% (25/227 cases), with 40% of these false negatives being FVTPC; ten out of 42 (23.8%) cases of FVTPC were not identified by standard cytology. However, cytology with thyroid peroxidase immunodetection diagnosed 224 out of the 227 TPC (99%), and all the FVTPC were correctly identified (100%).FVTCP are the most frequent source of false negatives in standard cytology. Thyroid peroxidase immunodetection allows most of these errors to be avoided, and correctly identifies 99% of TPC including FVTPC.

MANAGEMENT STRATEGIES: Management of papillary and follicular cancer of the thyroid varies somewhat between centers because of the generally good prognosis and the absence of well-controlled therapeutic trials. The internationally recognized TNM system is widely used to modulate treatment and follow-up to the individual situation.Surgery is indicated in well-differentiated thyroid cancer. Total thyroidectomy is required for clinically patent tumors (or = 1 cm) and small tumors (1 cm) recognized prior to surgery. For small tumors found at histology examination, reoperation is discussed in terms of prognosis. Post-operative 131-iodine is indicated when surgical resection is incomplete or in case of unfavorable prognosis. External radiotherapy is currently reserved for exceptional cases with unremoved tumoral tissue unresponsive to 131-iodine.All operated patients are given L-thyroxine to achieve euthyroidism and low TSH levels (0.1 microU/ml). Early detection of relapse is based on combined thyroglobulin assay and whole body 131-iodine scintigraphy. Both are performed during the first year of follow-up after a period of thyroid hormone withdrawal. Human recombinant TSH will soon be available allowing selection of patients with a detectable thyroglobulin level after stimulation; these patients should have a 131-iodine scintigram. If thyroglobulin remains undetectable during L-thyroxine treatment, an annual dosage is indicated and other exams are unwarranted.Surgery is indicated in case of small areas of active recurrent tumoral tissue in a cervical location. If a high-sensitivity scintigram does not show iodine uptake, the surgical procedure is completed by radiotherapy or possibly chemotherapy with doxorubicin. Small recurrent tumors in other areas respond to 131-iodine (3.7 GBq). Surgery, 131-iodine and radiotherapy are usually indicated for large ectopic recurrences. Chemotherapy is ineffective.Standard primary therapy generally provides cure and most patients are followed by annual thyroglobulin and TSH assays. Other explorations beginning with a whole-body 131-scintigram may be indicated in selected patients.

Iodine-131 whole body imaging and serum thyroglobulin are used in the follow-up evaluation of differentiated thyroid carcinomas. Iodine 131 is used to detect and treat functioning lesions. Iodine 131 activity detected outside the normal thyroid bed is usually attributed to metastatic disease. False-positive localization of radioiodine has been described, and may be a potential pitfall. We report here six personal cases of false-positive localisations of radioiodine du to body secretions, pathologic exudate, activity in nonlactating breast, salivary gland inflammation, ovarian teratoma. Some of these false-positive can be ruled out easily by removing of clothes, removing of nozzle in patients with tracheostomy, or by ingestion of water in subjects with abnormal mediastinal hyperfixation. Recognition of false-positive radioiodine images is very important to prevent costly additional investigation and possibly inappropriate therapy.

In order to review the experience of a single institution with thyroid surgery and identify prognostic factors, we did a retrospective chart review of all patients who underwent thyroid surgery between 1980 and 1995. Fifty patients, 32 girls and 18 boys, underwent thyroid surgery. Clinical presentation include a thyroid nodule (36 patients), a family history of MEN syndrome with a positive pentagastrin stimulation test (10), diffuse nodular goitre (3) and a neonatal cervical mass (1). Radiologic investigation include ultrasonography in 58% of cases, radionucleide imaging in 70% of cases. The mean age at surgery was 13.3 years and the delay between onset of symptoms and diagnostic was 13 months. Twenty-one patients had a lobectomy +/- isthmectomy, 19 had a total thyroidectomy and 10 had a subtotal thyroidectomy. Nine patients required a second surgery to complete the surgical treatment, and perform a total or near total thyroidectomy when the final pathology showed a carcinoma. A well differentiated carcinoma was found in 52% of the patients, a medullary carcinoma in 20% and a benign lesion in 28% of cases. Nine patients had local or distant metastases at initial surgery. Post-op treatment consisted of suppressive hormonotherapy in all cases of cancer and radioactive iodine when recurrence occurred in 24% of patients and when metastasis where present. Survival remained at 100%.Thyroid nodules should be rapidly investigated in children because of a high potential of cancerous lesions. With an aggressive surgical treatment and post-op I131 for recurrent lesions and metastasis the prognostic remains excellent.

The aim of this study was to evaluate the ability of MRI to detect recurrent differentiated thyroid carcinomas developed in the neck or the upper mediastinum.MRI was performed in 25 patients, and was compared in 5 cases with surgery. In 20 cases it was compared with I-131 scintigraphy (100 mCi in 14 cases and 5 mCi in 6 cases). The sensibility, specificity and overall accuracy of MRI was respectively: 100%, 66.6%, 82.6%.MRI is a good technique to detect recurrent thyroid carcinomas. It is specially interesting to investigate patients with a biological suspicion of recurrence and a negative scintigraphy. Mediastinal localisations that cannot be detected by US can be detected by MRI.

This article reviews the pathology and classification of differentiated thyroid carcinomas. Diagnostic criteria are reviewed. Follicular and papillary carcinomas and their variants are described. New entities are detailed. Atypical adenomas and difficult follicular diseases are discussed. The need for a consensual terminology is assessed.

From 1966 throughout September 1990, 753 patients underwent surgery for thyroid carcinoma, in the same institution, covering all pathological types. Complete follow-up was achieved in 96% of them, being at least 7 years in 50% of cases. 599 (80%) are currently alive. Neck dissection was not routinely done, except for medullary thyroid carcinoma, but rather selectively, if nodes were palpable either pre or intraoperatively, and also (since oct. 1983 i.e. the last 400 cases) if, after routine sampling of mid jugular nodes, frozen sections assessed nodal invasion. On the grounds of this policy, 205 patients underwent unilateral or bilateral neck dissection; 17% of them died during follow-up whereas 5.9% (12 cases) exhibited a cervical nodal recurrence, 6 of them occurring less than two years post-operatively, including 3 medullary thyroid carcinomas. 548 had no neck dissection; 9% died during follow-up whereas 0.9% (5 cases) exhibited a cervical nodal recurrence, 3 of them occurring less than two years post-operatively. Routine neck dissection seems not to be justified in the surgery of non medullary differentiated thyroid carcinoma, in as much as late occurrence of cervical node metastases is uncommon and does not obviously impair life-expectancy.