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Background: An increase in thyroid cancers, predominantly papillary thyroid carcinoma (PTC), has been recently reported in children., Methods: The histopathology of 28 consecutive PTCs from the northeast United States was reviewed. None of the patients (ages 6-18 years; 20 females, 8 males) had significant exposure to radiation. Nucleic acid from tumors was tested for genetic abnormalities (n = 27). Negative results were reevaluated by targeted next-generation sequencing., Results: Seven of 27 PTCs (26%) had neurotrophic tyrosine kinase receptor (NTRK) fusion oncogenes (NTRK type 3/ets variant 6 [NTRK3/ETV6], n =5; NTRK3/unknown, n = 1; and NTRK type 1/translocated promoter region, nuclear basket protein [NTRK1/TPR], n = 1), including 5 tumors that measured >2 cm and 3 that diffusely involved the entire thyroid or lobe. All 7 tumors had lymphatic invasion, and 5 had vascular invasion. Six of 27 PTCs (22%) had ret proto-oncogene (RET) fusions (RET/PTC1, n = 5; RET/PTC3, n = 1); 2 tumors measured >2 cm and diffusely involved the thyroid, and 5 had lymphatic invasion, with vascular invasion in 2. Thirteen PTCs had the B-Raf proto-oncogene, serine/threonine kinase (BRAF) valine-to-glutamic acid mutation at position 600 (BRAF(V) (600E)) (13 of 27 tumors; 48%), 11 measured <2 cm, and 6 had lymphatic invasion (46%), with vascular invasion in 3. Fusion oncogene tumors, compared with BRAF(V) (600E) PTCs, were associated with large size (mean, 2.2 cm vs 1.5 cm, respectively; P = .05), solid and diffuse variants (11 of 13 vs 0 of 13 tumors, respectively; P < .001), and lymphovascular invasion (12 of 13 vs 6 of 13 tumors, respectively; P = .02); BRAF(V) (600E) PTCs were predominantly the classic variant (12 of 13 vs 1 of 13 tumors). Two tumors metastasized to the lung, and both had fusion oncogenes (NTRK1/TPR, n = 1; RET/PTC1, n = 1)., Conclusions: Fusion oncogene PTC presents with more extensive disease and aggressive pathology than BRAF(V) (600E) PTC in the pediatric population. The high prevalence of the NTRK1/NTRK3 fusion oncogene PTCs in the United States is unusual and needs further investigation., (© 2016 American Cancer Society.)

Background: There is continuous debate regarding the optimal classification, prognosis, and treatment of the follicular variant of papillary thyroid carcinoma (FVPTC). The objective of this study was to assess the behavior of FVPTC, especially its encapsulated form, and shed more light on its true position in the classification scheme of well differentiated thyroid carcinoma., Methods: All patients with FVPTC, follicular thyroid adenoma (FTA), and follicular thyroid carcinoma (FTC) who were diagnosed between 1980 and 1995 were reviewed and reclassified according to the currently accepted definition of FVPTC. The tumors were separated into encapsulated and nonencapsulated (infiltrative/diffuse) types. Encapsulated tumors were subdivided further into tumors with or without capsular/vascular invasion. These different subtypes of FVPTC were correlated with outcome and with other clinicopathologic parameters., Results: After review by 4 pathologists, 78 patients were included in the study. Sixty-one of 78 patients (78%) had encapsulated tumors (18 invasive, 43 noninvasive), and 17 patients had nonencapsulated tumors (infiltrative/diffuse). The gender distribution, age at presentation, and tumor size did not differ between patients with encapsulated and nonencapsulated FVPTC. Patients who had encapsulated FVPTC had a significantly lower rate of marked intratumor fibrosis (18%), extrathyroid extension (5%), and positive margins (2%) compared with patients who had nonencapsulated tumors (88%, 65%, and 50% respectively; P < .0001). Regional lymph node metastases were present in 14 of 78 patients (18%), and no patients had distant metastases. The lymph node metastatic rate was significantly higher in patients who had nonencapsulated tumors (11 of 17 patients; 65%) compared with patients who had encapsulated neoplasms (3 of 61 patients; 5%; P < .0001). In addition, lymph node metastases were not detected in any noninvasive, encapsulated FVPTCs. With a median follow-up of 10.8 years, only 1 patient developed a recurrence, which occurred in an encapsulated FVPTC that had numerous invasive foci. None of the patients with noninvasive, encapsulated FVPTCs developed recurrences, including 31 patients who underwent lobectomy alone, with a median follow-up of 11.1 years., Conclusions: FVPTC appeared to be a heterogeneous disease composed of 2 distinct groups: an infiltrative/diffuse (nonencapsulated) subvariant, which resembles classic papillary carcinoma in its metastatic lymph node pattern and invasive growth, and an encapsulated form, which behaves more like FTA/FTC. Patients who had noninvasive, encapsulated FVPTCs did not develop lymph node metastases or recurrences and could be treated by lobectomy alone. If the current findings are confirmed, then strong consideration should be given to reclassifying encapsulated FVPTC as an entity that is close to the FTA/FTC class of tumors., ((c) 2006 American Cancer Society.)

Background: The objective of the current study was to evaluate the ability of serum thyroglobulin mRNA assay in detecting local and distant recurrences in patients who underwent surgery for thyroid carcinoma., Methods: Sixty-six consecutive patients were studied. One year after surgery, all patients underwent clinical examination and radioiodine scan, and a blood sample was taken for serum thyroglobulin (Tg) immunoassay and for Tg mRNA assay by reverse transcription-polymerase chain reaction (RT-PCR). RNA was extracted from cells pellet and analyzed by RT-PCR using specific primers for Tg., Results: Thyroglobulin mRNA was detected in 14 (21.2%) patients. Seven of 16 patients with elevated serum thyroglobulin had detectable Tg mRNA. Six of 30 (20%) patients with absent or minimal thyroid bed radioiodine uptake and 7 of 36 (19.4%) patients with significant thyroid bed uptake had detectable Tg mRNA. Among 5 patients with metastases, only 1 (20%) showed circulating Tg mRNA. Overall, the sensitivity, specificity, and accuracy of Tg mRNA assay in predicting the results of the (131)I whole-body scans was 25%, 80%, 25%, respectively. Fourteen of 53 (26.4%) patients with papillary thyroid carcinoma had detectable thyroglobulin mRNA whereas none of the patients with other histologic types did. The sensitivity, specificity, and accuracy of Tg mRNA assay in predicting the results of the (131)I whole-body scans in patients with papillary thyroid carcinoma was 100%, 75%, and 100%, respectively. Of note, the percentage of cases with detectable Tg mRNA was similar among patients who did not receive postoperative (131)I and those who had postoperative radioiodine treatment., Conclusions: The current study suggests that the validity of the Tg mRNA assay varies according to the histologic type of thyroid carcinoma and that this assay may play a role in the identification of metastatic disease in the subgroup of patients affected by papillary thyroid carcinoma but does not appear to be sensitive or active enough to direct clinical management., (Copyright 2001 American Cancer Society.)

An unusual case of a metastasizing dermatofibrosarcoma protuberans with fibrosarcomatous change (DFSP-FS) is reported. The tumor metastasized to unusual sites, including soft tissues of the cheek, forearm, and retroperitoneum 5 years after surgical resection without local recurrence. In addition, the patient had a second, histologically distinct fibrohistiocytic tumor: a malignant giant cell tumor of soft parts. The occurrence of these two tumor types in the same patient highlights the relationship of DFSP to the family of fibrohistiocytic tumors. The late onset of metastases in the absence of local relapse, although a rare event, reflects the potential difficulty in predicting its biologic behavior. DFSP-FS may be a more aggressive tumor than ordinary DFSP.