Giulia Sapuppo

Thyroid cancer in thyroglossal duct cysts requires a specific approach due to its unpredictable extension.

CONTEXT Differentiated thyroid cancer (DTC) in thyroglossal duct cysts is uncommon. The requirement of total thyroidectomy and lymph node dissection is still controversial. SETTING The study was performed in a referral thyroid cancer center at an academic hospital. PATIENTS We conducted a single center retrospective study of a consecutive series of 26 patients with DTC in thyroglossal duct cyst, all having undergone cyst resection and total thyroidectomy. MAIN OUTCOME MEASURES Diagnostic modalities, surgical treatment, histopathological features, and clinical outcome were included in the study. RESULTS Thyroglossal duct cyst cancer histotype was papillary in 23 of 26 patients (88.5%) and follicular-Hurthle in 3 of 26 cases (11.5%). A concomitant papillary DTC in the thyroid gland was found in 16 of 26 cases (61.5%), and it was multifocal in 8 of 16 cases (50%). At presentation, the patients with cancer in both the thyroglossal duct cyst and the thyroid were older than the patients who only had cancer in the thyroglossal duct cyst (44.9 ± 7.6 vs 32.0 ± 12.7; P = .006). Lymph node dissection, performed in 17 of 26 patients (65.4%), indicated that the central compartment was involved in 6 patients (35.3%, all having cancer also in the thyroid), the laterocervical compartments in 10 patients (58.8%), and the submental in 4 (23.5%). Six patients (23.1%) had persistent disease at 6-year median follow-up. CONCLUSIONS DTC in thyroglossal duct cysts occurs at a younger age and with more aggressive features at presentation. Concomitant cancer in the thyroid and lymph node metastases is present in most cases. Lymph node compartment involvement is different from that of cancers in the thyroid gland. Therefore, surgical treatment should include both thyroglossal duct cyst resection and total thyroidectomy, with individualized surgical nodal dissection. Subsequent management should follow current DTC guidelines.

Prognostic Factors for Adrenocortical Carcinoma Outcomes

Purpose Adrenocortical carcinoma (ACC) is an aggressive tumor characterized by a high recurrence rate and poor response to treatment. This study analyzes a consecutive series of ACC patients to evaluate the prognostic value of various clinical and pathological characteristics. Methods We retrospectively evaluated 32 ACC patients followed at our Medical Center from 1997 to 2015 and evaluated the prognostic value of age at diagnosis, gender, tumor functional status, stage, and type of treatment with respect to overall survival (OS) and disease-free survival (DFS), as determined by Kaplan–Meier curves. Results ACC was associated with hormonal overproduction in 50% of cases, and patients with isolated hyperandrogenism had a better prognosis. Recurrence was observed in 12/26 (46.2%) patients with no evidence of disease after surgery. Tumor size [hazard ratio (HR) 1.32, 95% confidential intervals (CI) 1.12–1.64; p = 0.007], ki-67 (HR 1.06, 95% CI 1.02–1.11; p = 0.009) and advanced stage at diagnosis (III–IV) (HR 6.51, 95% CI 1.65–24.68; p = 0.006) were associated with recurrence in the 26 R0 patients in the univariate analysis. Advanced stage was an independent risk factor for recurrence in the multivariate analysis (HR 8.10, 95% CI 1.55–41.35; p = 0.01). Five-year survival was 40.0%. Positive resection margins (HR 10.61, 95% CI 3.02–38.31; p = < 0.001), ki-67 (HR 1.04, 95% CI 1.01–1.07; p = 0.01) and advanced stage (HR 11.31, 95% CI 1.45–87.76; p = 0.02) were associated with poor survival in all 32 patients, but only positive resection margins were an independent predictor of mortality in the multivariate analysis (HR 6.22, 95% CI 1.44–26.05; p = 0.01). Conclusion ACC has a poor prognosis with a high recurrence rate. Tumor stage at diagnosis and the completeness of surgical excision are the most relevant prognostic factors.

Familial Non-Medullary Thyroid Cancer Represents an Independent Risk Factor for Increased Cancer Aggressiveness: A Retrospective Analysis of 74 Families.

Objectives To assess whether familial non-medullary thyroid cancer (FNMTC) represents an independent risk factor for increased aggressiveness of the tumor, as concern as the clinical presentation and the long-term follow-up in respect of sporadic differentiated thyroid cancer (SDTC). Design Retrospective study; 1976–2014. Patients and Methods Seventy-four FNMTC families (151 affected individuals): family relationship and number of affected family members were evaluated. Clinical and histopathological features and outcome were compared to that of 643 SDTC patients followed in the same period according to the same institutional protocols. Median follow-up was 57.7 months (range 12–136) in FNMTC and 59.7 (range 15–94.6) in SDTC patients. Results Three cases occurred in 3 families and 2 cases in the other 71. F:M was 3.7:1 in FNMTC and 4.3:1 in SDTC (NS). The family relationship was siblings in 62.2%. Mean age at diagnosis was lower in FNMTC than in SDTC (p < 0.005). Papillary/follicular histotype distribution was similar (86%). Papillary tumors were more frequently multifocal in FNMTC (p = 0.004) and with lymph-node metastases (p = 0.016). Disease-free survival (DFS) was shorter in FNMTC vs. SDTC (p < 0.0001) with 74.8 vs. 90.8% patients free of disease at the last control (p < 0.005). Three patients died in FNMTC group vs. 1 in SDTC (p = 0.02). Conclusion Familial non-medullary thyroid cancer displays distinct characteristics as earlier age of onset and increased aggressiveness at diagnosis and a higher rate of persistent/recurrent disease and mortality with a shorter DFS in respect with SDTC. FNMTC patients, therefore, should be followed accurately. As the specific gene (or genes) responsible for susceptibility for FNMTC has not yet been identified, a low frequency periodic screening of relatives DTC patients may be useful to identify FNMTC patients at early stage of disease.

Differentiated thyroid cancer in children: Heterogeneity of predictive risk factors

To correlate clinical and pathological characteristics at diagnosis with patient long‐term outcomes and to evaluate ongoing risk stratifications in a large series of paediatric differentiated thyroid cancers (DTC).

L’ipertiroidismo è un fattore di rischio di trombosi venosa e arteriosa: descrizione di un caso clinico

L’incidenza dell’associazione tra ipertiroidismo e trombosi non è nota. Le alterazioni coagulative che potrebbero essere coinvolte sono: l’aumentato turnover dei fattori della coagulazione con una loro aumentata sintesi endoteliale, emofilia A acquisita, sindrome da antifosfolipidi, ridotta attività fibrinolitica, un’eccessiva attività adrenergica. Il distretto più frequentemente interessato è quello cerebrale [1]. Il paziente, maschio di 57 anni con febbre, algie addominali, tachicardia e decremento ponderale, presentava TSH 0,0 microUI/ml (0,34–4,2), FT3 19,1 pmol/L (3,8–6), FT4 68,1 pmol/L (6,8–16), leucocitosi neutrofila (GB 14.600, Ne 80%), VES 99 mm (< 14), PCR 11,4 mg/dl (< 0,5), PT 65% (80–120), PTT 41 sec (23–35), fibrinogeno 668 mg/dl (1,5–4,5). L’esame obiettivo evidenziava una tiroide aumentata di volume, dolente alla deglutizione; l’ecografia tiroidea rilevava un volume aumentato (lobo destro 35 × 32 × 53 mm, lobo sinistro 32 × 34 × 49 mm); la scintigrafia tiroidea indicava scarsa fissazione del radioiodio. Veniva trattato con paracetamolo, corticosteroidi e beta bloccante con miglioramento clinico. Dopo circa 6 giorni il paziente lamentava dolore addominale a intensità crescente, con aumento degli indici di flogosi e D-Dimero. La TAC torace e addome evidenziava malattia trombo-embolica arteriosa e venosa (Fig. 1) e il paziente veniva sottoposto a terapia trombolitica e trasferito in UTIC.

Ruolo della 18FDG PET nel follow-up del carcinoma differenziato della tiroide (CDT) in presenza di valori di Tireoglobulina (Tg) indosabili, AAT elevati e TBS post-131-I negativo: esperienza clinica in una paziente

Circa il 20% dei pazienti tiroidectomizzati per carcinoma tiroideo differenziato (CDT) presentano alla diagnosi valori di anticorpi-anti-tireoglobulina (AAT) al di sopra del range di normalità che, nella maggior parte dei casi, si riducono progressivamente fino a negativizzarsi con una mediana di circa 3–5 anni [1]. Valori elevati di AAT possono interferire con il dosaggio della tireoglobulina (Tg) rendendola falsamente negativa pur in presenza di malattia residua. I pazienti in cui gli AAT non presentano alcun decremento temporale o si positivizzano durante il follow-up, o in cui si osserva un trend in ascesa richiedono un’attenta valutazione per escludere persistenza/recidiva di malattia.

Anaplastic Thyroid Cancer in Sicily: The Role of Environmental Characteristics

Background Anaplastic thyroid cancer (ATC) is a rare but extremely aggressive cancer of the thyroid, contributing up to 30–40% of thyroid cancer-specific mortality. We analyzed ATC characteristics and survival rates in Sicily to evaluate the possible influence of environmental factors. With this aim, data regarding ATC incidences in urban/rural and industrial, iodine-deficient, and volcanic vs control areas were compared in Sicily as well as ATC data from Sicily and USA. Methods Using the Sicilian Register of Thyroid Cancer (SRTC) database incidence, age, gender, tumor size and histotype, extrathyroidal extension, stage, and coexistence with pre-existing differentiated thyroid cancer (DTC) were evaluated in different areas of Sicily and also compared with Surveillance Epidemiology and End Results data in USA. Results Forty-three ATCs were identified in Sicily in the period 2002–2009. In our series only age <70 years at diagnosis (p = 0.01), coexistence with DTC (p = 0.027) and tumor size ≤6 cm (p = 0.012) were significant factors for increased survival at univariate analysis (only age at multivariate analysis). No difference in ATC incidence was found in urban vs rural areas and in iodine-deficient and industrial vs control areas. By contrast, in the volcanic area of Sicily, where DTC incidence is doubled relative to the rest of the island, also ATC incidence was increased. ATC data in Sicily were similar to those reported in the same period in the USA where overall survival rate at 6 and 12 months, however, was smaller. Conclusion The similar ATC data observed in Sicily and USA (having different genetic background and lifestyle) and the increased ATC incidence in the volcanic area of Sicily paralleling the increased incidence of papillary thyroid cancer are compatible with the possibility that casual additional mutations, more frequent in a background of increased cell replication like DCT, are the major causes of ATC rather than genetic background and/or direct environmental influences.