Anaplastic Thyroid Cancer: a Retrospective analysis of 120 cases.

INTRODUCTION: Anaplastic Thyroid Cancer (ATC) is one of the most lethal malignancies with very short survival and extremely poor treatment outcome. ATC accounts for 2-5% of all thyroid cancers worldwide with an annual incidence of about 2 million. OBJECTIVE: To review the natural history and treatment outcome of ATC patients treated at King Faisal Specialist Hospital and Research Centre (KFSH and RC) located at Riyadh, Saudi Arabia. MATERIAL AND METHODS: Retrospective review of 120 Saudi cancer patients collected from registry data at KFSH & RC from 1976-2008. Search key words included: thyroid cancer, anaplastic, undifferentiated and not otherwise specified. Search was not restricted to particular age, gender, treatment or tumor size. Demographic information, baseline patient characteristics including date of diagnosis, type of treatment and date of death were obtained from KFSH & RC registry data and Saudi death registry. RESULTS: A total of 120 cases were identified at our cancer centre from 1976 to 2008. Of these total, 73 were females (60.8%) and 47 were males (39.2%). The average age at diagnosis was 63.34 +/- 12.8 years. Thirty-four patients underwent surgery (28.3%), 52 had a palliative radiation treatment (43.3%) and only 5 had chemotherapy (4.2%). The median survival was 53 days (0-457). CONCLUSION: Our review proves that ATC is rapidly fatal cancer and is unresponsive to currently available therapeutic options. More research is needed to understand the tumor biology and novel treatment options. KEYWORDS: thyroid cancer, anaplastic, undifferentiated, radiation treatment, chemotherapy, median survival, Riyadh and Saudi Arabia.

Association between normal tissue complications after radiotherapy and polymorphic variations in TGFB1 and XRCC1 genes.

Genetic predictive biomarkers of radiosensitivity are being sought to individualize radiation treatment of cancer patients. In this pilot case-control study, we tested the association between TGFB1 T869C codon 10 Leu/Pro (rs1982073), XRCC1 G28152A codon 399 Arg/Gln (rs25487), and XRCC3 C18067T codon 241 Thr/Met (rs861539) single-nucleotide polymorphisms (SNPs) and late reaction to radiotherapy in 60 nasopharyngeal cancer patients. Subcutaneous and deep tissue fibrosis was scored using the RTOG/EORTC grading system. Patients with moderate to severe fibrosis (radiosensitive cases, G2-3, n = 30) were matched and compared to those with little or no reaction (controls, G0-1, n = 30). The three nonsynonymous SNPs were genotyped by direct DNA sequencing. Significant association was observed for TGFB1 T869C and XRCC1 G28152A genotypes (P < or = 0.05). Both variant alleles, TGFB1 869C and XRCC1 28152A, were associated with a lower grade of fibrosis (odds ratios were 0.41, 95% CI: 0.20-0.86, P = 0.02 and 0.30, 95% CI: 0.10-0.89, P = 0.02, respectively), and therefore the wild-types were the risk alleles. Interestingly, there was a significant difference in the median number of risk alleles between the radiosensitive and the control groups (P = 0.006). We conclude that radiotherapy complications are associated with genetic variations in our nasopharynx cancer patients. Our findings support the assumption of the combined effects of multiple SNPs. Large-scale studies are required to confirm these findings before polymorphisms can be used as predictive markers to individualize radiation therapy on genetic bases.

Nasopharyngeal carcinoma in Saudi Arabia: clinical presentation and diagnostic delay.

Nasopharyngeal carcinoma is commonly advanced at diagnosis. In this study we evaluated the clinical presentation, diagnostic delay and factors affecting delay in nasopharyngeal carcinoma. Data were collected prospectively for 307 newly diagnosed patients, including detailed demographic data, disease history, health care consultations and referral process. Diagnostic delay was classified as patient, professional and overall. Neck lump and nasal obstruction were the commonest presenting symptoms. There was a significant association between delay time of > or = 3 months and advanced stage. Patient's age and otological symptoms were associated with increased overall delay time. Advanced clinical stage at diagnosis was associated with paitents' sociodemographic characteristics.

Nasopharyngeal carcinoma in Saudi Arabia: clinical presentation and diagnostic delay

Nasopharyngeal carcinoma is commonly advanced at diagnosis. In this study we evaluated the clinical presentation, diagnostic delay and factors affecting delay in nasopharyngeal carcinoma. Data were collected prospectively for 307 newly diagnosed patients, including detailed demographic data, disease history, health care consultations and referral process. Diagnostic delay was classified as patient, professional and overall. Neck lump and nasal obstruction were the commonest presenting symptoms. There was a significant association between delay time of >/= 3 months and advanced stage. Patient's age and otological symptoms were associated with increased overall delay time. Advanced clinical stage at diagnosis was associated with paitents' sociodemographic characteristics

Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience.

BACKGROUND: Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct clinico-pathological subtype of diffuse large B-cell lymphoma (DLBCL). The optimal treatment is unknown, with some studies suggesting a superior outcome with dose-intensive chemotherapy regimens, and the role of radiotherapy remains ill-defined. PATIENTS AND METHODS: The British Columbia Cancer Agency lymphoma database was searched and records reviewed to identify those patients presenting with a prominent mediastinal mass and considered to be PMBCL based on the current REAL/WHO classifications. Patients were treated based on era-specific BCCA guidelines (1980-1992 MACOPB/VACOPB; 1992-2001 CHOP-type; 2001-present CHOP-R). Beginning in January 1998 involved-field radiotherapy was recommended to be routinely administered following chemotherapy. Prior to this, use of radiotherapy was individualized in advanced disease. RESULTS: In total, 153 patients with newly diagnosed PMBCL were identified between 28 July 1980 and 30 June 2003. The median age was 37 years (range 13-82) and the majority had stage I/II (74%), bulky mediastinal disease (75%). Overall (OS) and progression-free (PFS) survival at 5 years for the entire cohort were 75% and 69%, respectively. In direct comparison with a cohort of patients with DLBCL (n = 1273), OS (P = 10(-4)) and PFS (P = 0.0001) favored PMBCL. The age-adjusted International Prognostic Index (aaIPI) was not predictive of survival (P = 0.18). Five-year OS in patients < 65 years old treated with MACOPB/VACOPB, CHOP-R and CHOP-type was 87%, 81% and 71% respectively (P = 0.048). In pair-wise survival comparisons, only MACOPB/VACOPB and CHOP-type treated patients were significantly different (P = 0.016). In Cox multiple regression analysis, poor performance status remained the only predictor of survival, with treatment received demonstrating a trend to worse outcome for patients treated with CHOP-type regimens (P = 0.09). In an intention-to-treat analysis comparing the era before radiotherapy was routinely administered with after, there was no significant difference in 5-year PFS (74% versus 62%; P = 0.09) or OS (78% versus 69%; P = 0.14). CONCLUSIONS: In this single institution, population-based retrospective study, we found that PMBCL patients have excellent survival rates and a distinct plateau is observed in PFS, in striking comparison to DLBCL. The aaIPI was not predictive of survival in this population, suggesting that other prognostic models may be better suited for risk stratification. Dose-intensified chemotherapy with MACOPB or VACOPB demonstrated a trend to superior outcome over CHOP-type chemotherapy. However, further randomized studies are needed and the impact of rituximab on these comparisons must be considered. Finally, the routine addition of radiotherapy does not improve survival.

Primary thyroid lymphoma: a retrospective analysis of prognostic factors and treatment outcome for localized intermediate and high grade lymphoma.

Non-Hodgkin's lymphoma presenting in the thyroid gland is uncommon. A review of the King Faisal Specialist Hospital and Research Centre (KFSH & RC) experience was performed to assess treatment outcome and prognostic factors in this rare extranodal presentation of localized lymphoma. Sixty patients treated at KFSH & RC between 1975 and 1995 were identified, and their records were reviewed retrospectively. Eight patients who had stage III or IV disease, low grade, or did not complete their prescribed treatment were excluded from the study. There were 38 female and 14 male patients with a median age of 59.5 years at the time of diagnosis (range: 10-87 years). Thirty-five of the 52 patients underwent diagnostic partial or total thyroidectomy at other institutions based on a preoperative assumption of thyroid carcinoma. All 52 patients had non-Hodgkin's lymphoma of intermediate (94%) or high (6%) grade. Detailed staging was carried out in all patients; 16 patients (31%) had disease confined to the thyroid gland (stage IE), whereas 36 (69%) had associated disease in cervical lymph nodes and/or the mediastinum (stage IIE) disease. All patients were treated with curative intent. A total of 18 patients (35%) were treated with a single-modality treatment--radiotherapy alone in 2, chemotherapy alone in 13, and surgery alone in the remaining 3 patients. The majority of patients (34/52; 65%) were treated with a combined-modality approach. The overall relapse-free survival (RFS) and overall survival (OS) at 5 years were 72% and 88%, respectively. There were no significant differences in outcome between those treated with single-modality and those with combined-modality therapy. A univariate analysis showed that the presence of mediastinal lymph node involvement was the most important prognostic factor affecting both RFS and OS. Patients with Hashimoto thyroiditis and without "B" symptoms were found to have a significantly higher RFS without influence on the OS. However, patients who had a good performance status (PS) of 0, 1, and 2 were found to have a significantly higher overall survival in comparison to those with poor performance status. Age, sex, stage, histology, lactic acid dehydrogenase level, tumor bulk, and the treatment modality were not found to correlate with RFS or OS. Mediastinal involvement and PS were found to be the most important independent prognostic factors influencing RFS and OS.

A simplified shielding approach for limiting fetal dose during radiation therapy of pregnant patients.

PURPOSE: To investigate the effectiveness of a simple and practical shielding device to reduce fetal dose for a patient undergoing radiation therapy of nasopharyngeal carcinoma. METHODS AND MATERIALS: Using 5-cm-thick lead bricks and a heavy-duty steel cart, a 50 x 50-cm portable shield was designed and fabricated to reduce fetal dose due to collimator scatter and head leakage radiation. With the gantry at 90 degrees /270 degrees the shield can be easily positioned between the machine head and the fetus to reduce peripheral dose. Dose measurements for 6-MV X-rays and 9-MeV electrons have been made, utilizing a Rando phantom, to quantify the effect of the shield. RESULTS: Measurements show that the peripheral dose to the fetus can be reduced by 60% when the simple shielding device is used.

Early stage carcinoma of oral tongue: prognostic factors for local control and survival.

The purpose of this study was to assess prognostic factors, treatment outcomes and patterns of relapse in patients with early stage (T1-2 N0) squamous cell carcinoma of oral tongue treated primarily by surgery. The medical records of all patients with early stage (T1-2 N0) oral tongue cancer, radically treated at King Faisal Specialist Hospital and Research Center between January 1980 and December 1997, were reviewed. Eighty-five patients were identified for analysis, 38 male and 47 female. With a median follow-up for surviving patients of 64 months, 5-year actuarial overall, disease-specific (DSS), and relapse-free survival (RFS) were 71, 75, and 63%, respectively. Univariate analysis for DSS showed survival advantage for patients with tumor thickness (TT) of < or =10 mm (P=0.0002) and distance from resection margin (DFRM) of > 5 mm (P=0.005). The effect of TT of < or =10 mm was maintained (P=0.001) on multivariate analysis. Higher RFS was observed with TT of < or =10 mm (P=0.0002), DFRM of > 5 mm (P=0.0002) and DFRM of >10 mm (P=0.007). On multivariate analysis higher RFS was also found for TT < or =10 mm (P=0.01) and DFRM >5 mm (P=0.01). Salvage of local tongue recurrence was higher than neck node failure, with 5-year DSS of 71 and 19%, respectively (P=0.007). Time interval for recurrence showed no significant impact on outcome. In T1-2 N0 oral tongue cancer, TT, and DFRM are significant prognostic factors for both local control and survival. Neck node recurrence is associated with poor prognosis and low salvage rate.

Squamous cell carcinoma of the oral tongue: an analysis of prognostic factors.

AIM: To identify the prognostic significance of different factors in patients with squamous cell carcinoma of the tongue. PATIENTS AND METHODS: Seventy-seven patients with carcinoma of the tongue were treated radically at the King Faisal Specialist Hospital and Research Centre between 1980 and 1989. Twenty patients (26%) were treated by resection alone, 11 (14%) with radiotherapy alone, and 46 (60%) with combined resection and radiotherapy. RESULTS: Forty-seven patients (61%) had T(1-2), 28 (36%) T(3-4), and two T(x) tumours. The regional nodes were clear in 53 (69%) and contained metastases in 24 patients (31%). Thirty patients (39%) developed recurrences, which were local in 9, regional in 14, locoregional in 5, and locoregional with metastatic disease in 2. The five and 10-year overall actuarial survival for all patients were 65% and 53%, respectively, and the corresponding relapse-free survival 56% and 50%. Univariate and multivariate analyses were done of seven variables - age (<40 compared with >/=40 years), sex, chewing tobacco use, smoking, TNM stage, surgical margins (clear or invaded), and treatment (resection, radiotherapy, or the combination). On univariate analysis chewing tobacco (P=0.04), smoking (P=0.01), invaded resection margins (P=0.04), involved regional lymph nodes (P=0.009), T4 tumours, and patients treated with radiotherapy alone (P=0.001) were associated with poor overall survival. Factors associated with shorter relapse-free survival were age >40 (P=0.03), chewing tobacco (P=0.04), invaded resection margins (P=0.01), and smoking (P=0.01). On multivariate analysis, invaded resection margins and smoking (P=0.04)(P=0.02) were associated with shorter overall survival and relapse-free survival (P=0.03 and (P=0.01), while chewing tobacco independently influenced relapse-free survival only (P=0.03). CONCLUSION: Invaded resection margins and smoking were the only independent prognostic factors that affected both overall and relapse-free survival. Those who chewed tobacco were at high risk of locoregional failure.

Primary pericardial synovial sarcoma: a case report and literature review.

Primary synovial sarcoma of the heart is a rare tumor, with only six previous cases having been reported in the literature. Treatment has included surgery with or without chemotherapy. We present the first case of a documented synovial sarcoma arising from the pericardium in a 19-year-old man. Molecular analysis for t(X; 18) SYT-SSX gene fusion was positive. Radiation treatment was given postoperatively to the entire heart with a boost to the area where the margins were positive.

Carcinoma of the tonsil: prognostic factors.

OBJECTIVES: This study was conducted to provide a review of the prognostic factors of tonsillar carcinoma. DESIGN: A retrospective analysis. SETTING/PATIENTS: Patients with squamous cell carcinoma of the tonsil, treated in Northern Alberta, at the Cross Cancer Institute from 1975 to 1995 were analyzed using a population-based, head and neck cancer registry. There were 102 patients, 73 male and 29 female, ranging in age from 35 years to 83 years, with a mean of 60 years. The clinical stages were T1: 5 patients; T2: 27 patients, T3: 33 patients; T4: 11 patients; and Tx: 3 patients. The nodal stages were N0: 33 patients, N1: 26 patients; N2: 34 patients, N3: 7 patients; Nx: 2 patients. METHOD: The patients were treated with various modalities: surgery alone: 2 patients; surgery plus radiation: 26 patients; radiation treatment alone: 61 patients; and others: 13 patients. Patients were classified according to the UICC TNM 1992 criteria. The overall 5-year Kaplan-Meier survival in our series was 39%. The cause-specific 4-year survival was 57%. Various prognostic factors and their impact on survival were studied. RESULTS: On univariate analysis, the following factors were found to be significant. Age < 50 vs. > 50 (p = .02); endophytic growth pattern vs. exophytic growth of the primary (p = .01); ulcerated lesions vs. nonulcerated lesions (p = .000); various T stages (p = .003); clinical extension vs. no extension of primary disease (p = .02); combined modality of treatment (surgery and radiation treatment) had the best chance of survival compared to radiation treatment alone (p = .03). Nodal stages N0 vs. N+ disease (p = .2); sex of the patient, female vs. male (p = .83); and dose of radiation treatment < 5000 cGy vs. > 5000 cGy (p = .41) were found not to be significant. When the above significant factors were stratified according to the stage of the disease, only two were significant; ulcerated lesions vs. nonulcerated lesions (p = .04), and the modality of treatment chosen (e.g., radiation alone vs. radiation plus surgery) (p = .02). CONCLUSIONS: In this series of patients, combined-modity approach using surgery and radiation treatment was found to be the best way to treat carcinoma of the tonsil. However, each treatment strategy should be individualized taking into account various prognostic factors.