Primary mucoepidermoid carcinoma of the thyroid: diagnosis by fine-needle aspiration biopsy

Fine-needle aspiration biopsy (FNAB) has been recognized as a safe and reliable procedure in the evaluation of thyroid nodules. We herein report a case of a low-grade mucoepidermoid carcinoma of the thyroid that was diagnosed with this technique. Examination of an intra-operative FNAB showed cohesive clusters of polygonal squamoid cells with distinct cellular borders, uniform round nuclei, distinct nucleoli, and homogeneous amphophilic to cyanophilic cytoplasm. Focal keratin "pearl" formation was apparent, along with extracellular, lightly basophilic mucin deposits mantled by squamoid cells. These cytologic features are characteristic of low-grade mucoepidermoid carcinoma, as seen in other anatomic sites. This impression was confirmed by examination of paraffin sections. Previous reports have indicated that mucoepidermoid thyroid carcinoma is an indolent, locally recurring lesion. However, in spite of low-grade histology in our case, the neoplasm presented with distant metastases to bones, pleura, and lung.     

Management of the neck in thyroid cancer

The incidence of nodal metastasis in differentiated thyroid cancer ranges between 40% to 75%. Elective neck dissection is generally not advised in patients with differentiated thyroid cancer; however, if clinically apparent nodal disease is noted in the tracheoesophageal groove during surgery, central compartment clearance is advised. If clinically apparent nodal disease is present in the lateral compartment of the neck, modified neck dissection preserving the sternomastoid, accessory nerve, and jugular vein is advised. The "berry picking procedure" is generally not recommended because of the higher incidence of regional recurrence. Due consideration should be given for parathyroidal transplantation if the blood supply to the parathyroids is damaged during central compartment clearance. The incidence of lymph node metastasis is highest in young patients, however, lymph node metastasis has no bearing on long-term survival. There seems to be a higher incidence of regional recurrence in elderly individuals. If patients present with bulky nodal disease, consideration may be given for postoperative radioactive iodine dosimetry and ablation if necessary. Differentiated thyroid cancer represents a unique disease in the human body, where lymph node metastasis has no prognostic implication. Aggressive surgical clearance is advised in patients with medullary thyroid cancer in the central compartment and the jugular chain lymph nodes.     

Non-thyroid illness" or changed thyroid hormone parameter syndrome with non-thyroid illnesses

BACKGROUND: The multiple effects of systemic illness on thyroid economy are commonly referred to "non-thyroidal illness" (NTI) or "sick euthyroid syndrome". The various aspects of this common syndrome are summarized in this article. STUDIES: Results of the relevant studies published during the past 25 years were evaluated. The influence of the underlying illness and of drug administration was especially emphasized. RESULTS: The most common abnormalities in NTI are 1. the "low-T3 syndrome" due to a decreased T3 generation from T4 by a reduced activity of 5'-deiodinase (a selenoprotein); 2. the "low-T3 low-T4 state", which is associated with a poor prognosis. The low T4-levels are related to a binding inhibitor that displaces T4 from its binding proteins. However, there exists some controversy regarding the character of this binding inhibitor. 3. The high-T4 state is often found in acute psychiatric and liver diseases. The nutritional status of the patients and drugs known to influence thyroid hormone parameters have to be considered when patients with NTI are evaluated. Some difficulties may arise, when there is evidence of coexisting thyroid disease. Here aside from further biochemical evaluation such as thyroid antibodies, thyroid ultrasound and a thyroid scan have to be performed. CONCLUSION: NTI is associated with various alterations in thyroid hormone parameters when no intrinsic thyroid hormone disease exists. The severity of NTI reflects clinical outcome and clinical amelioration is associated with normalization of thyroid hormone parameters. There is no need for specific therapeutic intervention such as the administration of thyroid hormones in patients with the various forms of the NTI-syndrome.     

Failure of triiodothyronine to inhibit TSH-mediated thyroid hormone release in man

We studied the effect of short-term triiodothyronine administration on thyroid gland responsivity to exogenous thyrotropin in four euthyroid human subjects. Thyroidal iodine release and serum thyroxine during daily im injections of bovine TSH were not significantly inhibited, despite a four-fold elevation in serum T3 concentrations. This negative finding contrasts with earlier positive reports of a regulatory "short-loop" effect of elevated circulating T3 on the thyroid gland. This difference may be due either to the use in previous murine or in vitro studies of non-physiologic, high doses of exogenous T3, or failure to control the withdrawal of the trophic effect of endogenous TSH in man on the subsequent glandular response.     

The inferior thyroid veins--the ultimate guardians of the trachea

The inferior thyroid veins and their multiple tributaries are the ultimate guardians of the cervical trachea. Deeply embedded in the pretracheal fat pad, this plexus of veins is consistently encountered during low tracheostomy that accompanies conservation laryngral procedures as well as in tracheal reconstruction. In a high tracheotomy, the handling of the thyroid isthmus is simplified by an appreciation of these veins. Even cricothyrotomy is potentially complicated by hemorrhage sebsequent to a tear in a tributary of the inferior thyroid venous system. A cadaver study, employing 10 embalmed head and neck specimens, was performed to elucidate the tributary patterns of these veins. In every dissection there was at least one and as many as five veins overlying the trachea just below the thyroid isthmus. In 7 of 10 dissections a confluence of right and left inferior thyroid veins formed a large thyroid ima vein draining into the left innominate vein, and in 1 of 10 cases the thyroid ima vein drained into the right innominate. This confluence was present at a level which would be encountered in low tracheostomy or tracheal repair procedures. Six of 10 dissections presented large tributaries of the inferior thyroid veins overlying the cricothyroid membrane. An awareness of such anatomical considerations should result in safer surgical procedure performed in a dry operative field.     

The Janeway laparoscopic gastrostomy in palliative surgery

For the creation of a gastrostomy percutaneous endoscopic technique should be the method of first choice. If impossible a laparoscopic procedure should be preferred to a conventional one. Laparoscopic Janeway-gastrostomy might have some advantages compared to Kader-Stamm-Fistulas. We present the results with this method which we have used since 1993 in 12 patients. The postoperative course of these patients with mainly malignant diseases of ENT (n = 4), esophagus (n = 3) and thyroid gland (n = 3) as well as two non-malignant underlying diseases is presented and discussed in the light of recent publications. The Janeway-gastrostomy is an effective, easily feasable and safe procedure that ensures a good palliation also due to easy handling and care.     

Chronic evaluation of orthotopically implanted bileaflet mechanical aortic valves in adult domestic sheep

Papillary carcinoma is the most common form of thyroid carcinoma and, generally, it has a more favorable prognosis than other carcinoma types, although diverse variants or subtypes have been described, some of which appear to have a less favorable prognosis. Recently, a new variant, the so-called "Warthin-like tumor" or "tall-cell variant with extensive lymphocytic infiltration of papillary thyroid carcinoma" with behavior similar to the usual papillary carcinoma, has been described. We present a case with the cytohistological pattern of "Warthin-like tumor" of the thyroid. Immunohistochemistry revealed reactivity at the epithelium lining papillae for antimitochondrial 113-I antibody and also membranous positivity for CD15 (Leu M1). Lymphoid stroma showed an immunoprofile similar to chronic lymphocytic thyroiditis. These findings point to the consideration of "Warthin-like tumor" as being a hybrid neoplasm between the tall-cell variant and oxyphilic papillary carcinoma associated to a lymphoid-rich stroma.     

Health learning materials support in South Africa compared with other developing countries

An investigation was carried out in to thyroid hormones (TSH, T3, T4) and lipid parameters (total cholesterol, LDL cholesterol, HDL cholesterol, triglyceride) in 136 adolescents (94 femals, average age 13 years). An iodine deficiency (grade II-II) with respect to the daily urine excretion per 1,73 m2 BSA was found in 75%. With few exceptions the serum levels of TSH and T4 were in the normal range. In 36% of the patients we noticed compensatory elevated T3 levels. Correlations between thyroid hormones TSH, T4, renal iodine excretion and the volume of thyroid glands were not detectable, only T3 showed a dignificant positive correlation to the thyroid gland volume. The average values of lipids in patients were found to be higher than in normals. We consider the changed lipids as a sign of a disturbed efficacy of thyroid hormones. The regional insufficient iodine supply causes goiters and to a high degree the observed hyperchole-sterolemia, too. Our results underline the necessity of a common iodine salt prophylaxis as well as the treatment of "harmless" goiters in puberty.     

Anatomo-clinical study of a case of "ophthalmoplegia plus" with analysis of muscular, central nervous, ocular, myocardial, and thyroid lesions

This study deals with a case of "ophtalmoplegia plus" with histochemistry and electron microscopy of a muscle biopsy and full pathological examination in a 29 year old woman. Ragged-red fibers with abnormal mitochondria, cerebral spongiosis mostly involving white matter, perimacular pigmentary retinopathy and scattered myocardial fibrosis interrupting the Hiss'bundle were found. A goiter with hypersecretion of thyroid hormones and thyroid and ocular muscles changes characteristics of Graves' disease without any hypermetabolism occurred in her late twenties. This could be related to the absence of response from the abnormal mitochondria to the thyroid hormones.     

Utility of fine-needle aspiration cytology and frozen-section examination in the operative management of thyroid nodules

Fine-needle aspiration cytology has a high sensitivity for the diagnosis of solitary thyroid nodules. Certain diagnoses involving follicular histologies often cannot be made with needle biopsy alone. The utility of frozen-section examination of thyroid nodules, with particular regard to those lesions with follicular histologies, is also limited. We examined the correlation of fine-needle aspiration cytology and frozen-section examination in solitary thyroid nodules to determine the contribution of frozen-section examination to the operation. We reviewed the fine-needle aspiration cytology, frozen-section examination, and final pathology of 100 consecutive patients undergoing thyroidectomy for a solitary solid thyroid nodule in an 4-year period. The diagnoses were classified as indeterminant, benign, or malignant. The utility and impact of the diagnosis from fine-needle aspiration or frozen section on the operative procedure performed was analyzed. Fine-needle aspiration cytology as a diagnostic test for thyroid nodules demonstrated an indeterminant rate of 23 per cent, with a diagnostic accuracy of 77 and 92 per cent for benign and malignant disease, respectively. In all patients with inaccurate benign diagnosis on fine-needle aspiration cytology, follicular neoplasm was misinterpreted for follicular adenoma or multinodular goiter. In comparing frozen-section results, the indeterminant, benign, and malignant rates were 7, 96, and 64 per cent, respectively. Of the 23 patients with indeterminant results on fine-needle aspiration cytology, the intraoperative frozen-section diagnosis on 4 patients was deferred to permanent section; 18 received accurate cytological diagnosis; and in 1 patient, carcinoma was missed. Overall, the decision about the extent of surgical thyroid resection was changed in only 2 patients based on the frozen-section results. Preoperative evaluation with fine-needle aspiration cytology can accurately and appropriately define the extent of thyroid surgery in most patients with a diagnosis of malignant neoplasm or benign disease. Intraoperative frozen-section examination may be helpful if fine-needle aspiration cytology results are inderminant and in cases of follicular histology as an adjunct for evaluation of the thyroid nodule, but overall, frozen section does not contribute to the management of the thyroid lesion at the time of surgery.     

Cerebral lesions of mature newborn due to perinatal hypoxia. I. Placental and umbilical cord pathology

We report the case of a patient with multiple endocrine neoplasia type three, which was first diagnosed by members of the department of Ophthalmology. He was found to have multiple mucosal neuromata and medullary thyroid carcinoma. The ocular components of this syndrome include visible corneal nerves, conjunctival neuromas, thickened lids, anterior displacement of the cilia and a "dry eye" syndrome. The major systemic components are medullary thyroid carcinoma and pheochromocytoma. It is important that the Ophthalmologist recognize the ophthalmic manifestations of this syndrome so that early diagnosis and treatment may be possible.     

A gene predisposing to familial thyroid tumors with cell oxyphilia maps to chromosome 19p13.2

Familial nonmedullary thyroid cancer (FNMTC) is a clinical entity characterized by a phenotype more aggressive than that of its sporadic counterpart. Families with recurrence of nonmedullary thyroid cancer (NMTC) have been repeatedly reported in the literature, and epidemiological data show a very high relative risk for first-degree relatives of probands with thyroid cancer. The transmission of susceptibility to FNMTC is compatible with autosomal dominant inheritance with reduced penetrance, or with complex inheritance. Cases of benign thyroid disease are often found in FNMTC kindreds. We report both the identification of a new entity of FNMTC and the mapping of the responsible gene, named "TCO" (thyroid tumors with cell oxyphilia), in a French pedigree with multiple cases of multinodular goiter and NMTC. TCO was mapped to chromosome 19p13.2 by linkage analysis with a whole-genome panel of microsatellite markers. Interestingly, both the benign and malignant thyroid tumors in this family exhibit some extent of cell oxyphilia, which, until now, had not been described in the FNMTC. These findings suggest that the relatives of patients affected with sporadic NMTC with cell oxyphilia should be carefully investigated.     

Transition from cold to hot thyroid nodules

We report three cases of autonomously functioning thyroid nodules (AFTNs) that appeared hypofunctioning at radioactive iodine (131I) thyroid scan carried out at initial observation. Since at that time thyroid hormones and thyrotropin (TSH) were also normal, they were initially classified as "cold" nodules and treated with levothyroxine (LT4). The correct diagnosis of AFTN was made years later when a thyroid scintigraphy was repeated. In two of these patients, re-evaluation of the nodule was done because of the development of LT4 intolerance. A possible explanation is that these AFTNs had undergone hemorrhagic/cystic degeneration when they were first studied, but in subsequent years, proliferation of residual AFTN tissue caused the recurrence of a typical functioning nodule.     

The role of thyroid resection during reoperation for persistent or recurrent hyperparathyroidism

BACKGROUND: The role of "blind" thyroid lobectomy in the surgical management of patients with persistent or recurrent primary hyperparathyroidism is not known. We reviewed our experience with reoperation for hyperparathyroidism to determine the utility of blind thyroid resection in this setting. METHODS: From 1982 to 1995, 269 patients underwent reoperation for hyperparathyroidism at our institution. All patients had biochemical confirmation of hyperparathyroidism and underwent noninvasive and if necessary invasive localization studies. Patients who underwent thyroid lobectomy in an attempt to extirpate the hyperfunctioning parathyroid gland form the basis of this report. RESULTS: Thirty-two of 269 patients (12%) underwent thyroid lobectomy to remove a parathyroid gland. Intrathyroidal parathyroids were confirmed in 19 of 32 patients (59%). In 18 of 19 patients (94%), preoperative or intraoperative ultrasonography correctly identified an intrathyroidal lesion suspicious or a parathyroid. Only 1 of 6 patients (17%) undergoing a blind thyroidectomy had an intrathyroidal gland identified. Ultrasonography had a sensitivity of 95% and a negative predictive value of 99.5% in detecting an intrathyroidal parathyroid gland. CONCLUSIONS: The prevalence of an intrathyroidal parathyroid gland in our series is low (19 of 269, 7%). Ultrasonography can be used reliably to select patients for thyroid resection, reducing the need to perform a blind thyroid lobectomy and avoiding the potential morbidity of thyroid resection in this clinical setting.     

Somatic deletions and mutations in the Cowden disease gene, PTEN, in sporadic thyroid tumors

The majority of familial medullary thyroid neoplasms are associated with germ-line mutations of the RET proto-oncogene, yet very little is known about the mechanisms involved in the pathogenesis of familial and sporadic nonmedullary thyroid tumors. A subset of thyroid tumors have loss of heterozygosity of chromosome 10q22-23, a region harboring the gene responsible for Cowden disease, an autosomal dominant hamartoma syndrome associated with thyroid and breast tumors. PTEN/MMAC1/TEP1 codes for a dual-specificity phosphatase and is likely a tumor suppressor gene. We sought to determine the PTEN status in a series of epithelial thyroid neoplasms. We studied 95 sporadic thyroid tumors, of which 39 were papillary thyroid carcinomas (PTCs), 12 were follicular carcinomas, 9 were anaplastic carcinomas, 5 were Hürthle cell carcinomas, 21 were nonfunctioning follicular adenomas, and 9 were Hürthle cell adenomas. Direct sequencing of PCR-amplified products was performed for all nine exons of PTEN. Two polymorphic markers, one located in intron 8 and another, a dinucleotide repeat marker, AFMa086wg9, located within intron 2, were analyzed in paired blood-tumor DNA samples to assess hemizygous deletions of PTEN. We found a somatic frameshift mutation in one PTC, which was expected to generate a premature stop codon 2 amino acids downstream. Twenty-six % of informative benign tumors (four follicular adenomas and three Hürthle cell adenomas) and only 3 of 49 (6.1%) informative malignant tumors (one PTC, one follicular carcinoma, and one anaplastic carcinoma) showed evidence of hemizygous deletion of PTEN (P = 0.046). We conclude that a subset of thyroid tumors have somatic deletions of the PTEN gene, predominantly the benign forms, and that small intragenic mutations of PTEN are infrequent in thyroid tumors. We speculate that other mechanisms of PTEN inactivation, rather than small intragenic mutations, might occur in the hemizygously deleted samples and act as the "Knudson second hit." Alternatively, other tumor suppressor genes mapping to chromosome 10q22-23 could be the actual targets for such deletions and thus represent the various hits in the pathway of multistep carcinogenesis.     

The relationship of head and neck irradiation to the subsequent development of thyroid neoplasms

Individuals who have received head and neck radiation for benign conditions have a markedly increased risk of developing thyroid, salivary, and perhaps breast cancer as compared to the general population. Although the relative risk is very high, the absolute risk that any one individual who has had head or neck irradiation will develop a subsequent malignancy is low. Identification of these patients through some type of screening procedure may be beneficial in terms of prevention of subsequent morbidity and perhaps mortality from cancer, especially thyroid and salivary cancer. The risks of any detection or prophylaxis program must be carefully weighed against the probable, but unproved benefits of early detection. A major unresolved question is the natural history of microscopic thyroid carcinoma in the 25 yr-40 yr old radiation exposed population.     

Insular carcinoma of the thyroid. Apropos of a case and review of the literature

We present a new case report of "Insular" thyroid carcinoma. The first series was published, in 1984, by Carcangiu et al. The propensity for local recurrence and early distant metastases explains the poor prognosis of this carcinoma and demands aggressive therapy at the time of diagnosis. This includes radical surgery, radiotherapy with possible chemotherapy. Clinical and pathological arguments suggest that this carcinoma could be an intermediate stage in the process of loss of differentiation of some thyroid neoplasms.     

Surgery of differentiated cancer of the thyroid

A retrospective study on patients with differentiated thyroid carcinoma operated on at the 3rd Department of General Surgery of the University "La Sapienza" of Rome from 1970 to 1996 was performed. In 709 patients total thyroidectomy was performed as the minimal procedure acceptable, while 19 patients had subtotal thyroidectomy out of necessity. A functional ipsilateral or bilateral lymphnectomy of the neck was performed in 256 cases. This wider operation is indicated in the presence of metastatic lymph nodes and on principle in patients older than forty-five years in which at least another risk factor is present. Long term follow-up (12 years) was assured in 302 patients and the survival rate was 92% independently from the histotype (papillary or follicular). The survival rate of a group of 120 patients (80 with papillary and 40 with follicular carcinoma) was analyzed in relation to the risk factors. This group analysis demonstrated a very low mortality rate in patients with low risk index and an increased rate in patients with a high risk index.