Orbital tumors in USA: Difference in survival patterns

Introduction: There is a wide range of tumors affecting the orbital adnexa. Key such tumors include lymphomas, carcinomas, melanomas and rhabdomyosarcomas. Several studies have proposed that these histological subtypes differ in their survival outcomes. In this study we aim to describe the difference in survival outcomes between such subtypes. Methods: The SEER database was used to gather patient information. All 18 SEER registries were used. Patients diagnosed from 1996 to 2005 were included in the analysis. Observed five-year survival rate was calculated using the SEER*Stat software version 8.1.2. Data were extracted into IBM SPSS version 20 to generate Kaplan Meier curve for each group. Results: There were 2180 patients in the SEER databases who met the selection criteria. Lymphomas were the most common histology in adults. The overall five-year observed survival for all lymphoma patients was 75.9% (95% CI: 73.7–78.1). There was statistically significant difference between observed survival rates of lymphoma subtypes. Carcinomas were the second most common tumors. Their five-year observed survival rate in our study was 60.4%. There was no statistically significant difference between carcinoma subtypes’ observed survival rates in the 20–49 age group, while, in the older age group, the difference was found to be statistically significant. Rhabdomyosarcomas were the most common tumors in children. The overall five-year observed survival rate for rhabdomyosarcomas patients was 89.8%. There was no statistically significant difference between observed survival rates of rhabdomyosarcomas subtypes. There was no statistically significant difference between relative survival rates according to gender and treatment received except within melanomas. Conclusion: In adults, lymphomas have better survival rates than carcinomas. Whereas the lymphoma subtype can be used as a determinant prognostic factor in any age, the carcinoma subtype can be used as such a determinant in older age groups only. In children, rhabdomyosarcomas are the predominant tumors affecting the orbital adnexa. Further studies are needed to determine if the difference between embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma observed survival rates are statistically significant.     

Primary mediastinal malignancies: findings in 219 patients

The purpose of this study was to determine the demographics, histology, methods of treatment, and survival in primary mediastinal malignancies. We did a retrospective review of the statewide New Mexico Tumor Registry for all malignant tumors treated between January 1, 1973 and December 31, 1995. Benign tumors and cysts of the mediastinum were excluded. Two hundred nineteen patients were identified from a total of 110,284 patients with primary malignancies: 55% of tumors were lymphomas, 16% malignant germ cell tumors, 14% malignant thymomas, 5% sarcomas, 3% malignant neurogenic tumors, and 7% other tumors. There were significant differences in gender between histologies (P < 0.001). Ninety-four percent of germ cell tumors occurred in males, 66% of neurogenic tumors were in females; other tumors occurred in males in 58% of cases. There were also significant differences in ages by histology (P < 0.001). Neurogenic tumors were most common in the first decade, lymphomas and germ cell tumors in the second to fourth decades, and lymphomas and thymomas in patients in their fifth decades and beyond. Stage at presentation (P = 0.001) and treatment (P < 0.001) also differed significantly between histologic groups. Five-year survival was 54% for lymphomas, 51% for malignant germ cell tumors, 49% for malignant thymomas, 33% for sarcomas, 56% for neurogenic tumors, and 51% overall. These survival rates were not statistically different (P > 0.50). Lymphomas, malignant germ cell tumors, and thymomas were the most frequently encountered malignant primary mediastinal neoplasms in this contemporary series of patients. Demographics, stage at presentation, and treatment modality varied significantly by histology. Despite these differences, overall five-year survival was not statistically different.     

The novel ETS factor TEL2 cooperates with Myc in B lymphomagenesis

The human ETS family gene TEL2/ETV7 is highly homologous to TEL1/ETV6, a frequent target of chromosome translocations in human leukemia and specific solid tumors. Here we report that TEL2 augments the proliferation and survival of normal mouse B cells and dramatically accelerates lymphoma development in Emu-Myc transgenic mice. Nonetheless, inactivation of the p53 pathway was a hallmark of all TEL2/Emu-Myc lymphomas, indicating that TEL2 expression alone is insufficient to bypass this apoptotic checkpoint. Although TEL2 is infrequently up-regulated in human sporadic Burkitt's lymphoma, analysis of pediatric B-cell acute lymphocytic leukemia (B-ALL) samples showed increased coexpression of TEL2 and MYC and/or MYCN in over one-third of B-ALL patients. Therefore, TEL2 and MYC also appear to cooperate in provoking a cadre of human B-cell malignancies.     

Evaluation of the prognostic value of morphometric features and cellular DNA content in FIGO I ovarian cancer patients

Approximately 20% to 40% of the patients with a FIGO I ovarian tumor die within five years after the diagnosis. Morphologic studies (typing and grading) of the primary tumor are prognostically important, but poorly reproducible. Therefore, the prognostic value of more objective techniques, such as morphometry and flow cytometric (FCM) DNA determinations, were evaluated in 33 adequately staged FIGO I patients with at least a five-year follow-up. The overall five-year survival in the group was 64%. Three patient categories were defined on the basis of two easily measured morphometric features, the mitotic activity index (MAI) and the volume percentage epithelium (VPE), which an earlier study had proved to be significantly associated with prognosis. The five-year survival rates were 91% for 11 patients in category A (MAI less than 30 and VPE less than 65), 67% for 9 patients in category B (MAI less than 30 and VPE greater than or equal to 65) and 38% for 13 patients in category C (MAI greater than or equal to 30). FCM showed 25 of the tumors to be diploid and 8 to be aneuploid. The cellular DNA content was also of prognostic value: the five-year survival figures for patients with diploid and aneuploid tumors were 68% and 37%, respectively. Combination of the morphometric and FCM features showed that, in diploid tumors, the morphometric features have additional prognostic value: the diploid-tumor-patient survival rates in categories A, B and C were 91%, 63% and 50%, respectively. None of the eight patients with aneuploid tumors fell in the morphometrically favorable category A while seven were in category C.(ABSTRACT TRUNCATED AT 250 WORDS)     

Survival of Patients with Oral Cavity Cancer in Germany

The purpose of the present study was to describe the survival of patients diagnosed with oral cavity cancer in Germany. The analyses relied on data from eleven population-based cancer registries in Germany covering a population of 33 million inhabitants. Patients with a diagnosis of oral cavity cancer (ICD-10: C00-06) between 1997 and 2006 are included. Period analysis for 2002–2006 was applied to estimate five-year age-standardized relative survival, taking into account patients'' sex as well as grade and tumor stage. Overall five-year relative survival for oral cavity cancer patients was 54.6%. According to tumor localization, five-year survival was 86.5% for lip cancer, 48.1% for tongue cancer and 51.7% for other regions of the oral cavity. Differences in survival were identified with respect to age, sex, tumor grade and stage. The present study is the first to provide a comprehensive overview on survival of oral cavity cancer patients in Germany.     

Fractionated total body irradiation and high dose cyclophosphamide: a preparative regimen for bone marrow transplantation for patients with hematologic malignancies in first complete remission

We treated 73 patients with hematologic malignancies in first complete remission (acute lymphoblastic leukemia = 23 patients; acute non-lymphoblastic leukemia = 25 patients; chronic myelogenous leukemia in first chronic phase = 20 patients, and high grade lymphoma = five patients) with a uniform preparative regimen consisting of fractionated total body irradiation (1,320 cGy) and high dose cyclophosphamide (100 mg/kg), followed by allogeneic bone marrow transplantation. By radiation dosimetry we demonstrated that the calculated doses were delivered accurately and reproducibly. Actuarial survival rates (+/- SEM) in complete remission were as follows: Acute lymphoblastic leukemia = 74 +/- 9%; acute nonlymphoblastic leukemia = 50 +/- 11%; and chronic myelogenous leukemia = 55 +/- 11%. Actuarial relapse rates for these three diagnoses were 19 +/- 9%, 17 +/- 11%, and 0% respectively. Three of the five lymphoma patients are alive in complete remission at 22+, 28+, and 54+ months. Overall probability of survival for the 73 patients was 59 +/- 7%. Interstitial pneumonia, usually associated with cytomegalovirus infection and graft-versus-host disease, and relapse of the underlying malignancy were the major causes of death.     

Effectiveness of mass screening for endometrial cancer

OBJECTIVE: To investigate the effectiveness of mass screening for endometrial cancer using Endocyte (Laboratoire CCD, Paris, France) endometrial smears. STUDY DESIGN: The study subjects were consecutive patients with documented endometrial cancer diagnosed between January 1, 1989, and December 31, 1997, at 22 hospitals in Japan. One hundred twenty-six cases were detected by mass screening and 1,069 diagnosed in outpatient clinics. We compared the stage of cancer at diagnosis and survival rate of patients in the two groups. RESULTS: Early stage was significantly more frequent in the screening group (P < .001); stage I comprised 88.1% of the screening group as compared with 65.3% of the outpatient group. Well-differentiated adenocarcinoma was significantly more frequent in the screening group (P < .01); grade 1 constituted 74.7% of the screening group as compared with 61.0% of the outpatient group. The five-year survival rate was significantly higher in the screening group than in the outpatient group (94.0% vs. 84.3%, P = .041). The crude hazard ratio (HR) of dying of endometrial cancer for the screening group as compared to the outpatient group was .47 (95% CI .22-.99, P = .048). HR became .96 (95% CI .45-2.08, P = .925) after adjustment for age, study area and cancer stage. CONCLUSION: The results suggest that an endometrial cancer screening program would lead to early detection and improved survival among women with endometrial cancer.     

The prognostic value of the tumor marker CA 15-3 at initial diagnosis of patients with breast cancer

CA 15-3 has been most widely used as a serum tumor marker in follow-up and detection of breast cancer recurrence. In this study we have specifically focused upon the prognostic implications and utility of preoperative CA 15-3 levels. We have identified on our database 414 patients with breast cancer in whom serial levels of the serum tumor marker CA 15-3 had been determined at diagnosis and follow-up. We have analyzed the follow-up and clinical outcomes in these patients and from this data we have assessed the potential of CA 15-3 as a predictor of five-year overall and disease-free survival. Our results show that an initially elevated CA 15-3 level is associated with a very poor prognosis in both early and late stage disease. Elevated pre-biopsy CA 15-3 levels are associated with 14% five-year disease-free survival rates and 17% overall survival rates at five years. In contrast, normal CA 15-3 levels are associated with 47% five-year disease-free survival rates and 54% overall survival rates at five years (p<0.01). Comparison of five-year survival rates between patients with elevated and normal CA 15-3 levels in early breast cancer (stage I and II) also showed significant differences, with survival being 41% and 75%, respectively (p<0.01).     

Carcinoma of the Larynx: Results of Therapy

Prognosis of laryngeal carcinoma varies considerably, depending on its site and stage of development. In the past, laryngectomy was considered the treatment of choice for all but very early lesions. Results of therapy and five-year survival rates were relatively good, but the patient deprived of his larynx frequently presented difficulties in rehabilitation.Recent advances in radiotherapy techniques have permitted treatment of a greater proportion of patients with laryngeal carcinoma by this means, with encouraging results. Results of a survey in the Toronto area suggest that radiotherapy should be used as primary treatment in early and intermediate stages of the disease; radical excision combined with radiotherapy is indicated for treatment failures among early cases and for those with far-advanced disease or carcinoma outside the larynx proper. With this program five-year survival rates are comparable to those achieved when laryngectomy is the primary treatment used, and two-thirds of those who survive maintain laryngeal function.     

DNA quantification in colorectal carcinoma using flow and image analysis cytometry

Numerous studies using flow cytometry (FCM) have shown that DNA quantification and ploidy classification can provide information of prognostic significance for patients with colorectal carcinoma; recent advances in image analysis cytometry (image cytometry, ICM) provide a new, alternative technique for DNA quantification. This study investigated whether (1) patients with colorectal carcinomas that exhibit a diploid pattern of DNA distribution have improved five-year survival statistics as compared to their non-diploid counterparts and (2) ICM provides quantitative data comparable to that obtained by FCM. DNA quantification and ploidy classification of 27 cases of primary colorectal carcinoma was performed on archival paraffin-embedded tissue by both FCM and ICM; 70% (19) of the tumors were classified as nondiploid by ICM while 56% (15) were similarly classified by FCM. Diploid tumors were associated with Dukes' stage A while nondiploid tumors were associated with Dukes' stage D. The overall five-year survival rate was 75% for patients with ICM diploid tumors and 67% for patients with FCM diploid tumors. The five-year survival was only 53% for patients with nondiploid tumors identified by both techniques. This study confirmed that DNA quantification is an important prognostic indicator for patients with colorectal carcinoma. It also showed that ICM provides data comparable to that of FCM and may be more sensitive.     

Incidence patterns and trends of malignant gonadal and extragonadal germ cell tumors in Germany, 1998–2008

BackgroundMalignant gonadal (GGCT) and extragonal germ cell tumors [GCT (EGCT)] are thought to originate from primordial germ cells. In contrast to well reported population-based data of GGCTs in males, analyses of GGCTs in females and EGCTs in both sexes remain limited.MethodsIn a pooling project of nine population-based cancer registries in Germany for the years 1998–2008, 16,883 malignant GCTs and their topographical sites were identified using ICD-O morphology and topography for persons aged 15 years and older. We estimated age-specific and age-standardized incidence rates.ResultsAmong males, the incidence of testicular GCTs increased over time. In contrast, there was no increase in the incidence of EGCTs. Among females, rates of ovarian GCTs were stable, while rates of EGCTs declined over time. The most frequent extragonadal sites were mediastinum among males and placenta among females.ConclusionsOur results underline different incidence trends and distinct age-specific incidence patterns of malignant GGCTs and EGCTs, as reported recently by several population-based registries. The differences suggest that GGCT and EGCT may have different etiologies.     

Etiology and treatment of malignancy-associated anaemia

Patients with cancer frequently develop anaemia. Various factors, including the type of malignancy and the intensity of chemotherapy influence the prevalence of anaemia and need of transfusions. Among the numerous causes of its development, the most frequent type is cancer anaemia, the so-called "anaemia of chronic disorders". Anaemia of chronic disorders is diagnosed when neoplastic disease is accompanied by an otherwise unexplained microcytic anaemia with compromised iron utilisation and decreased erythropoietin secretion. In 50-70% of patients with solid tumors or hematological malignancies, mainly with multiple myeloma and malignant lymphomas, transfusion can be avoided, or significantly decreased by the use of recombinant erythropoietin. This review provides tools to decide the best candidates for this treatment and a guideline to monitor its efficacy.     

Pediatric oncology

Intensive use of cytotoxic agents in multimodality therapeutic regimens has resulted in almost 80% five-year disease-free survival and cure in the majority of childhood cancer patients. However, such success has come at the expense of severe acute or delayed toxicities and an increased occurrence of secondary cancers. With an increasing understanding of the genetic changes that underlie transformation in childhood cancer, rational approaches using agents that target these transforming events are being developed. Current and future strategies in developing tumor-selective therapy using inhibitors of signaling pathways dysregulated in leukemias (FLT3, NOTCH1) and solid/brain tumors (ErbB1-4, IGF-IR, PTCH1), and the challenges in developing less toxic, but equally effective treatments in pediatric oncology are presented.     

Progressive multifocal leukoencephalopathy: two cases with electron microscopic and viral studies.

Part I of this review described the pathogenesis of lung cancer and emphasized that it was largely a preventable disease. In the present paper, attention is drawn to the prevalent but false impression that treatment of established disease is quite in-effective. In eight consecutive series of cases (over 2300 patients) the authors have seen a change in the clinical environment in which lung cancer is treated—from one of discouragement and apathy to one of outspoken encouragement and enthusiasm.Complete preoperative assessment—an evaluation of the biology of the tumour-host relationship as well as technical resectability—avoids unnecessary surgical intervention and stimulates a trend to earlier referral. This has permitted increasing use of resection with a declining mortality and a continuing improvement in overall survival. On the basis of present resectability rates (37.5%) and a 39% five-year survival rate in those who have had curative resection, it is estimated that current over-all five-year salvage should exceed 13%. This is more than a five-fold increase in survival for all patients compared to that achieved by treatment before 1952.     

Outcomes and Impact of HIV Prevention,ART and TB Programs in Swaziland – Early Evidence from Public Health Triangulation

Introduction

Swaziland’s severe HIV epidemic inspired an early national response since the late 1980s, and regular reporting of program outcomes since the onset of a national antiretroviral treatment (ART) program in 2004. We assessed effectiveness outcomes and mortality trends in relation to ART, HIV testing and counseling (HTC), tuberculosis (TB) and prevention of mother to child transmission (PMTCT).

Methods

Data triangulated include intervention coverage and outcomes according to program registries (2001-2010), hospital admissions and deaths disaggregated by age and sex (2001-2010) and population mortality estimates from the 1997 and 2007 censuses and the 2007 demographic and health survey.

Results

By 2010, ART reached 70% of the estimated number of people living with HIV/AIDS with CD4<350/mm³, with progressively improving patient retention and survival. As of 2010, 88% of health facilities providing antenatal care offered comprehensive PMTCT services. The HTC program recorded a halving in the proportion of adults tested who were HIV-infected; similarly HIV infection rates among HIV-exposed babies halved from 2007 to 2010. Case fatality rates among hospital patients diagnosed with HIV/AIDS started to decrease from 2005–6 in adults and especially in children, contrasting with stable case fatality for other causes including TB. All-cause child in-patient case fatality rates started to decrease from 2005–6. TB case notifications as well as rates of HIV/TB co-infection among notified TB patients continued a steady increase through 2010, while coverage of HIV testing and CPT for co-infected patients increased to above 80%.

Conclusion

Against a background of high, but stable HIV prevalence and decreasing HIV incidence, we documented early evidence of a mortality decline associated with the expanded national HIV response since 2004. Attribution of impact to specific interventions (versus natural epidemic dynamics) will require additional data from future household surveys, and improved routine (program, surveillance, and hospital) data at district level.     

Chromosome-mediated alterations of the MYC gene in human cancer

The step-wise accumulation of genetic and epigenetic alterations in cancer development includes chromosome rearrangements and viral integration-mediated genetic alterations that frequently involve proto-oncogenes. Protooncogenes deregulation lead to unlimited, self-sufficient cell growth and ultimately generates invasive and destructive tumors. C-MYC gene, the cellular homologue of the avian myelocitic leukemia virus, is implicated in a large number of human solid tumors, leukemias and lymphomas as well as in a variety of animal neoplasias. Deregulated MYC expression is a common denominator in cancer. Chromosomal rearrangements and integration of oncogenic viruses frequently target MYC locus, causing structural or functional alterations of the gene. In this article, we illustrate how genomic rearrangements and viruses integration affect MYC locus in certain human lymphomas and solid tumors.     

Response to treatment with imatinib mesylate in previously treated chronic-phase chronic myeloid leukemia patients in a hospital in Brazil

We analyzed the results of treatment with imatinib mesylate in 70 patients with chronic-phase chronic myeloid leukemia who had previously been treated (with second-line or higher imatinib), many of them in a late chronic phase. The median follow-up period was 60.5 months (range 3-100 months). Our objective was to assess the efficacy and safety of treatment. The mean dose was 400 mg per day. The hematologic response rate was 92.1% at six months, while the cumulative rates of major and complete cytogenetic responses were 73.6 and 66.3%, respectively. Molecular response rate improved slowly and steadily over time, reaching 65.8% at 60 months, remaining stable for up to 96 months. The five-year progression-free survival and overall survival were 84 and 89%, respectively. Cytogenetic response by 12 months significantly correlated with overall survival (P = 0.0007) and progression-free survival (P = 0.0280). Sokal risk score did not differ significantly between subgroups. The medication was well tolerated, with only 16% of patients showing hematologic toxicity grades 3 and 4. At the end of the assessment, 57% of the patients were still on imatinib mesylate; most of those who discontinued treatment (17/30) did so because of unsatisfactory response. Treatment with imatinib mesylate in previously treated chronic-phase chronic myeloid leukemia induced durable responses in a high proportion of patients and was related to satisfactory long-term and event-free survival.     

Reliability of recording uterine cancer in death certification in France and age-specific proportions of deaths from cervix and corpus uteri

French uterine cancer recordings in death certificates include 60% of "uterine cancer, Not Otherwise Specified (NOS)"; this hampers the estimation of mortalities from cervix and corpus uteri cancers. The aims of this work were to study the reliability of uterine cancer recordings in death certificates using a case matching with cancer registries and estimate age-specific proportions of deaths from cervix and corpus uteri cancers among all uterine cancer deaths by a statistical approach that uses incidence and survival data. Deaths from uterine cancer between 1989 and 2001 were extracted from the French National database of causes of death and case-to-case matched to women diagnosed with uterine cancer between 1989 and 1997 in 8 cancer registries. Registry data were considered as "gold-standard". Among the 1825 matched deaths, cancer registries recorded 830 cervix and 995 corpus uteri cancers. In death certificates, 5% and 40% of "true" cervix cancers were respectively coded "corpus" and "uterus, NOS" and 5% and 59% of "true" corpus cancers respectively coded "cervix" and "uterus, NOS". Miscoding cervix cancers was more frequent at advanced ages at death and in deaths at home or in small urban areas. Miscoding corpus cancers was more frequent in deaths at home or in small urban areas. From the statistical method, the estimated proportion of deaths from cervix cancer among all uterine cancer deaths was higher than 95% in women aged 30-40 years old but declined to 35% in women older than 70 years. The study clarifies the reason for poor encoding of uterus cancer mortality and refines the estimation of mortalities from cervix and corpus uteri cancers allowing future studies on the efficacy of cervical cancer screening.     

Pleural effusions in non-Hodgkin's lymphoma. A cytomorphologic, cytochemical and immunologic study

Review of the records of 243 cases of cytologically diagnosed non-Hodgkin's lymphomas (NHL) revealed pleural effusions in 21 (8.6%). Cytologic examination of pleural fluid was done in 17 cases, of which 16 were reported as positive. Cytologic examination was supplemented with cytochemical staining (acid phosphatase, alpha naphthyl acetate esterase and periodic-acid-Schiff reactions) and E-rosetting studies in 12 cases. Of the 16 positive cases, 11 were malignant lymphomas consisting of convoluted lymphocytes. Acute lymphatic leukemia of the prothymocytic type (T-ALL) and chronic lymphocytic leukemia of the T-cell type (T-CLL) comprised one case each, and there were three cases of follicular center cell lymphomas, two of the cleaved-cell type and one of the Burkitt-type. Comparison of the cytomorphology of the tumor cells in the pleural effusion with those in fine needle aspiration smears from the solid tumors in 14 cases showed an identical appearance in 13 cases; in one, the Burkitt-type lymphoma, the cells were larger and more pleomorphic in the pleural effusion. This study indicates that the cytologic diagnosis and categorization of NHL of the convoluted-cell type is greatly enhanced by the study of neoplastic lymphocytes in a pleural effusion.     

Secondary malignant neoplasms in patients with non-Hodgkin's lymphoma

In 1979-1987, 570 patients with non-Hodgkin's lymphomas (226 female and 344 male patients) were treated at the Department of Hematology of the Pomeranian Medical Academy and hematological outpatient clinic. The second malignant tumors were diagnosed in 25 (4.4%) patients. Two patients suffered from three malignant tumors. The most frequent combination of 2 tumors were: non-Hodgkin lymphoma and cancer of the lungs in 8 patients, non-Hodgkin lymphoma and cancer of the skin in 6 patients, non-Hodgkin lymphoma and cancer of the larynx in 4 patients, non-Hodgkin lymphoma combined with cancer of the stomach in 2 patients, and non-Hodgkin lymphoma together with cancer of the bladder in 2 patients. Moreover, non-Hodgkin lymphoma coexisted with cancer of the colon, cervix and prostate (one case of each). The authors stress the possibility of other malignant tumors in patients with non-Hodgkin lymphomas and occurrence of the subsequent neoplasms without preceding chemo- or radiotherapy.