These data suggest consideration of a more conservative approach for most patients with thyroid nodules that are cytologically indeterminate on fine-needle aspiration and benign according to gene-expression classifier results. (Funded by Veracyte.).

The National Cancer Institute (NCI) sponsored the NCI Thyroid Fine-needle Aspiration (FNA) State of the Science Conference on October 22-23, 2007 in Bethesda, MD. The two-day meeting was accompanied by a permanent informational website and several on-line discussion periods between May 1 and December 15, 2007 (http://thyroidfna.cancer.gov). This document summarizes matters regarding diagnostic terminology/classification scheme for thyroid FNA interpretation and cytomorphologic criteria for the diagnosis of various benign and malignant thyroid lesions. (http://thyroidfna.cancer.gov/pages/info/agenda/).

Purpose NUT midline carcinoma (NMC) is a poorly differentiated squamous cancer characterized by rearrangement of the NUT gene. Research advances have provided opportunities for targeted therapy in NMC, yet the clinical features of this rare disease have not been systematically characterized. We report on a large population of such patients to identify the disease characteristics and treatments, correlate them with outcome, and to consider clinical recommendations. Experimental Design A clinical database was established using retrospective demographic and outcomes data available on all known cases of NMC. Questionnaires were completed by treating physicians. Pathologic, demographic, and clinical variables were assessed for 63 patients, the largest cohort of NMC patients studied to date. Outcome data from 54 patients were available for survival analyses. Results The diagnosis of NMC has increased annually since 2007. Since 2009, there has been an observed increase in the age at diagnosis (p<0.05). Geographic distribution of NMC patients has been concentrated in the United States (n=41, 65%). The median overall survival for patients with NMC was 6.7 months. The 2-year progression-free survival (PFS) was 9% with a 95% CI of 1%–17% (1-year PFS 15% (5%–24%)) and 2-year overall survival (OS) was 19% with a 95% CI of 7%–31% (1-year OS: 30% (27%–34%). Multivariate analysis suggested that extent of surgical resection and initial radiotherapy were independent predictors of PFS and OS. Notably, no chemotherapeutic regimen was associated with improved outcome. Conclusions NMC portends a poor prognosis among all squamous cell neoplasms and appears to be frequently unrecognized. The finding that conventional chemotherapy has been inadequate indicates a pressing need for the development of targeted therapeutics. Intensive local therapies such as gross total resection and radiotherapy might be associated with enhanced survival.

Poorly differentiated (PD) thyroid carcinomas lie both morphologically and behaviorally between well-differentiated and undifferentiated (anaplastic) carcinomas. Following the original description of this entity, different diagnostic criteria have been employed, resulting in wide discrepancies and confusion among pathologists and clinicians worldwide. To compare lesions occurring in different geographic areas and the diagnostic criteria applied in those countries, we designed a study with a panel of internationally recognized thyroid pathologists to develop consensus diagnostic criteria for PD carcinomas. Eighty-three cases were collected from Europe, Japan, and the United States, and circulated among 12 thyroid pathologists. Diagnoses were made without any knowledge of the clinical parameters, which were subsequently used for survival analysis. A consensus meeting was then held in Turin, Italy, where an agreement was reached concerning the diagnostic criteria for PD carcinoma. These include (1) presence of a solid/trabecular/insular pattern of growth, (2) absence of the conventional nuclear features of papillary carcinoma, and (3) presence of at least one of the following features: convoluted nuclei; mitotic activity >or=3 x 10 HPF; and tumor necrosis. An algorithmic approach was devised for practical use in the diagnosis of this tumor.

A clinicopathologic study of 241 cases of papillary thyroid carcinoma treated at the University of Florence Medical School, Florence, Italy is presented. The features of greatest prognostic value were patient's age at presentation, small tumor size, total encapsulation, extrathyroid extension, multicentricity, and presence of distant metastases. The prognosis of the disease was not influenced by the pattern of tumor growth, presence of solid areas, initial presence or subsequent development of cervical lymph node metastases, type of initial thyroid operation, performance of neck dissection, or prophylactic administration of radioactive iodine. It is concluded that conservative thyroid surgery in the form of lobectomy, without neck dissection or prophylactic administration of iodine 131 (131I), constitutes adequate therapy for most cases of papillary thyroid carcinoma. More extensive therapy should be considered for older patients and for those in whom the tumor exhibits extrathyroid extension or easily detectable multicentricity.

Thirty‐four cases of sinus histiocytosis with massive lymphadenopathy (SHML) are analyzed. Most of the cases involved Negro children and were characterized by cervical lymphadenopathy, which was often bilateral, painless, and of massive proportions. Other lymph node groups were sometimes involved. Fever, leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia were common features. The involved lymph nodes showed pericapsular fibrosis; dilation of sinuses; presence of numerous intrasinusal histiocytes with abundant clear cytoplasm, occasionally multinucleated, foamy or atypical; and large collectiions of plasma cells. A striking and constant finding was the presence of many lymphocytes and other hematopoietic cells within the cytoplasm of the sinus histiocytes. The diease characteristically followed a protracted clinicl course, with eventual spontaneous regression of the lymphadenopathy and total recovery in most cases. The etiology and pathogenesis are unknown. In this regard, the most likely possibilities include a specfic infectious process and a status resulting from an immunologic deficit.

No abstract

This large review identifies a greater degree of clinical, pathologic, and molecular variation than originally appreciated for tumors associated with t(11;22)(p13;q12). Translocation and functional fusion of the EWS and WT1 genes appears to be a consistent feature of this unique tumor.