Clinical activity in general practice before sarcoma diagnosis: an Australian cohort study.

BACKGROUND: Increased time to diagnosis in sarcoma is associated with poor prognosis and patient outcomes. Research is needed to identify whether opportunities to expedite the diagnosis of sarcoma in general practice exist. AIM: To examine pre-diagnostic GP clinical activity before sarcoma diagnosis. DESIGN AND SETTING: An Australian retrospective cohort study using hospital registry data (Australian Comprehensive Cancer Outcomes and Research Database [ACCORD]) linked to two primary care datasets (Patron and MedicineInsight). METHOD: The frequency of general practice healthcare utilisation events (general practice attendances, prescriptions, blood test, and imaging requests) were compared in 377 patients with soft tissue sarcoma (STS) and 64 patients with bone sarcoma (BS) in the year pre-diagnosis. Poisson regression models were used to calculate monthly incidence rate ratios (IRR) for the 24 months pre-diagnosis and estimate inflection points for when healthcare use started to increase from baseline. RESULTS: In the 6 months pre-diagnosis, patients with sarcoma had a median of 3-4 general practice attendances, around one-third had a GP imaging request (33% [n = 21] BS and 36% [n = 134] STS), and approximately one in five had multiple imaging requests (19% [n = 12] BS and 21% [n = 80] STS). GP imaging requests progressively increased up to eight-fold from 6 months before sarcoma diagnosis (IRR 8.43, 95% confidence interval [CI] = 3.92 to 18.15, P<0.001) and general practice attendances increased from 3 months pre-diagnosis. CONCLUSION: Patients with sarcoma have increased GP clinical activity from 6 months pre-diagnosis, indicating a diagnostic window where potential opportunities exist for earlier diagnosis. Interventions to help identify patients and promote appropriate use of imaging and direct specialist centre referrals could improve earlier diagnosis and patient outcomes.

Clinicopathological profile of myxoid soft tissue tumors- A retrospective study in a tertiary care hospital in South India.

Background: Myxoid soft tissue tumors are rare and diagnostically challenging group of tumors with varied biological behavior ranging from benign, locally aggressive to distantly metastasizing malignant tumors. Aims: The objectives of the study are to identify the relative frequency and distribution of myxoid soft tissue tumors among patients in a tertiary care hospital and to study the clinicopathological features of these tumors. This was a retrospective cross-sectional study conducted in the department of pathology of a tertiary care hospital from January 2008 to December 2013. Materials and Methods: Clinical and pathological details of all the 80 myxoid soft tissue tumors reported during the study period were retrieved from the records of department of pathology. Corresponding Hematoxylin & Eosin (H & E) slides were reviewed, and Immunohistochemistry (IHC) was carried out for confirmation. The relationship among various prognostic variables was analyzed in case of myxoid sarcomas. Results: Myxoid soft tissue tumors accounted for 3.7% among the soft tissue tumors with a predominance of malignant myxoid sarcomas (71.25%) in contrast to the overall picture of sarcomas. Myxoid neurofibroma (34.78%) was the most common benign tumor, while myxofibrosarcoma (33.33%) was the frequent myxoid sarcoma. A statistically significant correlation was seen between tumor size and depth (P-value: 0.038) and also between presence of vascular invasion and histological grade (P-value: 0.012) of sarcomas. Conclusion: Light microscopic morphology, supplemented by ancillary techniques like IHC, remains the cornerstone for diagnosis of myxoid soft tissue tumors.

What Proportion of Patients With Musculoskeletal Sarcomas Demostrate Symptoms of Depression or Anxiety?

BACKGROUND: It is estimated that the 12-month prevalence of depression in the United States is 8.6%, and for anxiety it is 2.9%. Although prior studies have evaluated depression and anxiety in patients with carcinoma, few have specifically evaluated patients with sarcoma, who often have unique treatment considerations such as mobility changes after surgery. QUESTIONS/PURPOSES: We evaluated patients with sarcoma seen in our orthopaedic oncology clinic to determine (1) the proportion of patients with depression symptoms, symptom severity, how many patients triggered a referral to mental health professionals based upon our prespecified cutoff scores on the nine-item Patient Health Questionnaire (PHQ-9), and if their symptoms varied by disease state; (2) the proportion of patients with anxiety symptoms, symptom severity, how many patients triggered a referral to mental health professionals based upon our prespecified cutoff scores on the seven-item Generalized Anxiety Disorder Scale (GAD-7), and if they symptoms varied by disease state; (3) whether other factors were associated with the proportion and severity of symptoms of anxiety or depression, such as tumor location in the body (axial skeleton, upper extremity, or lower extremity), general type of tumor (bone or soft tissue), specific diagnosis, use of chemotherapy, length of follow-up (less than 1 year or greater than 1 year), and gender; and (4) what proportion of patients accepted referrals to mental health professionals, when offered. METHODS: This study was a cross-sectional survey study performed at a single urban National Cancer Institute-designated Comprehensive Cancer Center from April 2021 until July 2021. All patients seen in the orthopaedic clinic 18 years of age and older with a diagnosis/presumed diagnosis of sarcoma were provided the PHQ-9 as well as the GAD-7 in our clinic. We did not track those who elected not to complete the surveys. Surveys were scored per survey protocol (each question was scored from 0 to 3 and summed). Specifically, PHQ-9 scores the symptoms of depression as 5 to 9 (mild), 10 to 14 (moderate), 15 to 19 (moderately severe), and 20 to 27 (severe). The GAD-7 scores symptoms of anxiety as 5 to 9 (mild), 10 to 14 (moderate), and 15 to 21 (severe). Patients with PHQ-9 or GAD-7 scores of 10 to 14 were referred to social work and those with scores 15 or higher were referred to psychiatry. Patients with thoughts of self-harm were referred regardless of score. Patients were divided based on disease state: patients during their initial management; patients with active, locally recurrent disease; patients with active metastatic disease; patients with prior recurrence or metastatic lesions who were subsequently treated and now have no evidence of disease (considered to be patients with discontinuous no evidence of disease); patients with no evidence of disease; and patients with an active, noncancerous complication but otherwise no evidence of disease. We additionally looked at the association of gender, chemotherapy administration, and tumor location on survey responses. Data are summarized using descriptive statistics. Differences across categories of disease state were tested for statistical significance using Kruskal-Wallis tests for continuous variables and Fisher exact tests for categorical variables as well as pairwise Wilcoxon rank sum tests. RESULTS: Overall, symptoms of depression were seen in 35% (67 of 190) of patients, at varying levels of severity: 19% (37 of 190) had mild symptoms, 9% (17 of 190) had moderate symptoms, 6% (12 of 190) had moderately severe symptoms, and 1% (1 of 190) had severe symptoms. Depresssion symptoms severe enough to trigger a referral were seen in 17% (32 of 190) of patients overall. Patients scored higher on the PHQ-9 during their initial treatment or when they had recurrent or metastatic disease, and they were more likely to trigger a referral during those timepoints as well. The mean PHQ-9 was 5.7 ± 5.8 during initial treatment, 6.1 ± 4.9 with metastatic disease, and 7.4 ± 5.2 with recurrent disease as compared with 3.2 ± 4.2 if there was no evidence of disease (p = 0.001). Anxiety symptoms were seen in 33% (61 of 185) of patients: 17% (32 of 185) had mild symptoms, 8% (14 of 185) had moderate symptoms, and 8% (15 of 185) had severe symptoms. Anxiety symptoms severe enough to trigger a referral were seen in 16% (29 of 185) of patients overall. Patients scored higher on the GAD-7 during initial treatment and when they had recurrent disease or an active noncancerous complication. The mean GAD-7 was 6.3 ± 3.2 in patients with active noncancerous complications, 6.8 ± 5.8 in patients during initial treatment, and 8.4 ± 8.3 in patients with recurrent disease as compared with 3.1 ± 4.2 in patients with no evidence of disease (p = 0.002). Patients were more likely to trigger a referral during initial treatment (32% [9 of 28]) and with recurrent disease (43% [6 of 14]) compared with those with no evidence of disease (9% [9 of 97]) and those with discontinuous no evidence of disease (6% [1 of 16]; p = 0.004). There was an increase in both PHQ-9 and GAD-7 scores among patients who had chemotherapy. Other factors that were associated with higher PHQ-9 scores were location of tumor (upper extremity versus lower extremity or axial skeleton) and gender. Another factor that was associated with higher GAD-7 scores included general category of diagnosis (bone versus soft tissue sarcoma). Specific diagnosis and length of follow-up had no association with symptoms of depression or anxiety. Overall, 22% (41 of 190) of patients were offered referrals to mental health professionals; 73% (30 of 41) accepted the referral. CONCLUSION: When treating patients with sarcoma, consideration should be given to potential concomitant psychiatric symptoms. Screening, especially at the highest-risk timepoints such as at the initial diagnosis and the time of recurrence, should be considered. Further work should be done to determine the effect of early psychiatric referral on patient-related outcomes and healthcare costs. LEVEL OF EVIDENCE: Level III, therapeutic study.

The occurrence and pattern of head and neck sarcomas: a comprehensive cancer center experience.

PURPOSE: This study aimed to analyze the demographics, clinicopathological, treatment, and survival characteristics of head and neck sarcomas diagnosed in a reference center in the Brazilian Northeast. MATERIALS AND METHODS: This retrospective cohort study reviewed the clinical records of patients with head and neck sarcomas. Epidemiologic data consisted in clinical location, age, gender, histopathological diagnosis, clinical TNM staging and treatment. Outcome variables were local recurrence and survival. The statistical analyses were performed by a binary logistic regression analysis. The survival analysis was assessed through the Kaplan-Meier curve. RESULTS: Sixty-nine patients with head and neck sarcomas (male 39; female 30) were analyzed. The most common histologic subtypes were rhabdomyosarcoma, dermatofibrosarcoma, and pleomorphic sarcoma. The mean age of the patients at the time of diagnosis was 38.1 years old. A total of 31 patient died (sarcoma-related death) up to the end of the follow-up, with a mean follow-up rate of 1.63 years. A multivariate analysis revealed that anatomical site, treatment modality, histopathological diagnosis, and clinical stage of the disease were associated with specific survival, reaching statistical significance. CONCLUSION: This study demonstrates the impact of important clinical-pathological parameters on the overall prognosis of head and neck sarcomas.

Sarcoma as a Model for Adolescent and Young Adult Care.

Sarcomas occur across all ages and are relatively abundant in the adolescent and young adult populations compared with older adults. Because of an overall rarity combined with a broad diversity of diagnoses, expertise is often concentrated in comprehensive cancer centers. The sarcoma model of care is an excellent model for overall adolescent and young adult care. We summarize some of the natural advantages of the field for developing adolescent and young adult programs, review management and referral touchpoints, and summarize recent biologic and clinical trial insights that have affected sarcoma management recently.

Quality of life and patients' expectations in soft tissue sarcoma.

Assessment of health-related quality of life (HRQoL) is essential for holistic care. Greater efforts are required to incorporate HRQoL measures into clinical trials and daily practice. Considerable HRQoL data are available for localized soft tissue sarcomas (STS), particularly in the orthopedic setting. In future, HRQoL is expected to become increasingly important in the evaluation of palliative therapy in advanced STS. A patient-centric approach is advocated for STS management. Greater awareness of STS by nonspecialist clinicians, and timely referral to specialized sarcoma reference centers, is crucial for patient welfare. The patient is central to shared decision-making during consultations and during case review in tumor boards. The management approach to STS should be collaborative, involving a multidisciplinary team, multiple centers and patient advocacy groups.

Clinical outcomes of radio-hyperthermo-chemotherapy for soft tissue sarcoma compared to a soft tissue sarcoma registry in Japan: a retrospective matched-pair cohort study.

Regional hyperthermia is considered to enhance the antitumor effects of chemotherapy and radiotherapy. In this study, we confirmed the efficacy of concomitant radiotherapy, hyperthermia, and chemotherapy (RHC) for neoadjuvant treatment of malignant soft tissue sarcoma (STS). From 1994 to 2013, we performed RHC in 150 patients. This study was limited to 60 patients using the following exclusion criteria: salvage for recurrence or unplanned excision, trunk location, metastasis at initiation, non-STS, and no definitive surgery. As a control group, we collected data from 11,031 patients in the Bone and Soft Tissue Tumor Registry in Japan (BSTT). We performed multivariate logistic regression analysis, and propensity scores were created for comparisons between groups. The primary outcome of this study was to compare oncologic outcomes (5-year local control rate [LC] and overall survival rate [OS]). In the RHC group, two local recurrences (3.3%) occurred, and no patients underwent amputation. Margins of definitive surgery were not identical between groups [wide margins (60.0% vs. 85.3%), marginal margins (28.3% vs. 10.5%), and intralesional margins (7.4% vs. 4.2%), RHC and BSTT groups, respectively, P < 0.001]. After adjustment, the difference in OS was not significant between groups (HR = 1.26, P = 0.532); however, a statistically significant difference in LC was observed (HR = 4.82, P = 0.037). RHC resulted in a high LC at 5 years compared to the BSTT group, and amputation was averted in the RHC group, despite the wider margins in the BSTT group. This indicates that less invasive surgery might be achieved with effective neoadjuvant therapy.

Algorithm for the surgical management of mesenchymal tumors of the perineum in adults.

BACKGROUND: Perineal soft tissue tumors are rare, so that little is known about their management and the outcome of treatment. OBJECTIVE: The aim of this study is to describe the presentation, management, and outcome of the surgical treatment of soft tissue tumors and to provide a final decision algorithm. DESIGN: This is a retrospective study. SETTINGS: The study was conducted in a single tertiary care hospital with a dedicated unit on sarcoma. PATIENTS: Fifty-one consecutive patients from 1998 to 2013 were included. MAIN OUTCOME MEASURES: The primary outcomes measured are patient demographics, treatment decisions, and outcome of surgical treatment. RESULTS: Forty-nine patients presented with a primary soft tissue tumor, and 2 underwent simple excisions for isolated metastases. The median tumor size was 75 mm (50-110). Symptoms were nonspecific, and MRI had insufficient specificity for malignancy so that a preoperative biopsy was systematically performed according to European Society for Medical Oncology and National Comprehensive Cancer Network soft tissue tumor guidelines. Six benign soft tissue tumors (3 lipomas, 3 leiomyomas), 16 intermediate soft tissue tumors (12 aggressive angiomyxoma, 4 desmoid tumors), and 27 sarcomas were identified. Treatments and surgery were tailored from the beginning according to histology. All but 1 benign soft tissue tumor were treated by 'shelling out.' Aggressive angiomyxoma were treated with en bloc resection sparing uninvolved organs. Nonsurgical treatments were our first choice for desmoid tumors. Wide en bloc surgery was planned for all sarcomas (n = 27) after the induction treatment for 16 patients (chemotherapy, n = 12; radiotherapy, n = 4). In the sarcoma group, the 5-year estimated metastasis-free, local recurrence-free, and overall survival rates were 68.1% (95% CI, 50.7-91.5), 84.7% (95% CI, 66.7-100), and 85.7% (95% CI, 71.8-100). In the benign and intermediate tumor groups, there were no deaths, local recurrences, or progression. LIMITATIONS: This study was limited by the small number of patients, given the rarity of this disease in the perineum. CONCLUSION: We provide useful indications for the best strategy necessary to treat these rare tumors located in a complex site.

[Histopathological diagnostic concordance in bone and soft tissue sarcomas between two comprehensive cancer centers from eastern and western Europe: a collaborative experience]. / La concordance du diagnostic histopathologique des sarcomes osseux et des tissus mous entre deux centres anticancéreux de l'Europe de l'Est et de l'Ouest : une expérience de collaboration.

INTRODUCTION: This study aims to assess the degree of concordance of histological diagnosis of bone and soft tissue sarcomas between a Comprehensive Cancer Center (CCC) of Eastern Europe - not specialized in this area of pathology - and an important CCC of Western Europe, which is one of the coordinators of a clinical reference network in sarcoma pathology. The goal is to have an overview of the sarcomatous pathology in a region of Eastern Europe and to discover diagnostic discrepancies between the two centers, while determining their cause. MATERIALS AND METHODS: The initial diagnosis was compared with the revised diagnosis on 110 specimens from 88 patients with bone or soft tissue sarcomas from East-European CCC, in a one-year period of time. RESULTS: Complete diagnostic agreement was observed in 55 cases (62.5%), a partial agreement in 23 cases (26.1%) and a major disagreement in 10 cases (11.4%). Major discrepancies of the histological type was observed in only 3 cases (3.4%): one case of discordance benign/malignant and 2 cases of discordance mesenchymal/non mesenchymal. Minor histological discrepancies - not affecting the management of the patient - were observed in 18 cases (20.4%). A major discordance in grading - potentially changing the management of the patient - was noted in 7 cases (7.9%), and a minor discrepancy in 5 cases (5.7%). DISCUSSIONS: Some histological types were clearly overdiagnosed, like "adult fibrosarcomas" and "malignant peripheral nerve sheet tumors" (MPNST), mostly converted after the audit into "undifferentiated spindle cell sarcomas" or other types of sarcomas. Some "unclassified" sarcomas and "undifferentiated pleomorphic sarcomas" could be re-classified with the aid of an extensive panel of antibodies. Overall, immunohistochemistry was responsible, but not in exclusivity, for half of the minor discrepancies, and for 2 out of 3 cases of major histological discrepancies. Otherwise, the main cause of discrepancies was the difficulties in the interpretation of the morphology. Molecular biology was decisive in one case. Most grading discrepancies resulted from the appreciation of the mitotic index. CONCLUSIONS: The profile of the sarcomatous pathology in the northwest region of Romania does not appear to differ significantly from other parts of Europe or the world, but a prospective epidemiological study would be necessary to confirm this assessment. The expansion of immunohistochemical antibody panel, the over-specialization of pathologists and, in the future, the establishment of a national network of referral centers in sarcoma pathology, are required for a high level of histological diagnosis in Eastern Europe. A periodic external audit, continuing this trans-European collaboration between the two centers, would be beneficial for monitoring progress.

Survey among German gynecologists on the clinical management of patients with sarcomas of the uterus.

AIM: To gain more information about the knowledge of the clinical management of uterine sarcoma. MATERIALS AND METHODS: This survey was performed among members of the North-Eastern German Society of Gynecological Oncology (NOGGO) and the German Society of Psychosomatic Medicine in Gynecology and Obstetrics (DGPFG) on the treatment of uterine sarcomas. RESULTS: Altogether, 374 gynecologists took part. When asked about the surgical therapy of leiomyosarcoma, 64% indicated hysterectomy with bilateral adenectomy and lymph node dissection. Answers on the extent of lymphadenectomy in leiomyosarcoma differed widely. When asked about the preferred chemotherapy regimen for metastatic uterine sarcoma, more than 60% of all gynecologists would not apply any chemotherapy. Almost 40% recommended any kind of radiotherapy in this situation. CONCLUSION: There is a great uncertainty about the standard treatment of uterine sarcoma, even among specialists of gynecological oncology. It is time for organized efforts to improve the treatment of uterine sarcoma.

Multidisciplinarity and medical decision, impact for patients with cancer: sociological assessment of two tumour committees' organization.

PURPOSE: Medical practices in oncology are expected to be multidisciplinary, yet few articles studied how this may be concretely applied. In the present study, we evaluated the organization of two multidisciplinary committees, one for breast cancer and one for sarcoma, in a French Comprehensive Cancer Centre. METHODS: Both tumours were specifically chosen so as to emphasise substantial differences in relation with incidence, histological subtypes, management strategy, and scientific evidence. Between 2003 and 2004, 404 decision processes were observed, 210 for sarcoma (26 meetings) and 194 for breast cancer (10 meetings). The number of physicians who took part in the discussions and their medical specialties were systematically noted as well as the number of contradictory discussions, medical specialties represented in these contradictory discussions and the topics of contradiction. The last measured data was whether the final committee's decision was in conformity with the referent preferences or not. All these measures were related to the referent's medical speciality and working place, to the stage of the disease and to the disease management stage. RESULTS: Committees' specificities concerned their organization, referent's medical specialties, the number of participants in discussions and their medical specialties. Discussions in the sarcoma committee tended to be more multidisciplinary, involving more specialties. Initial strategy proposal for one patient was modified during the discussions for 86 patients out of 210 (41%) and for 62 out of 194 (32%) respectively for sarcoma and breast cancer. However, there was no significant difference in the rate of contradictory discussions between breast cancer and sarcoma committees (32% versus 41% respectively; P = 0.08). The rates of contradictory discussions were similar for localized cancers, local relapse and metastasis disease (37%, 41% and 34% respectively; P = 0.86). CONCLUSIONS: The present study reports more than 30% of changes concerning strategy for patient with cancer due to multidisciplinary discussions. This indicates that, providing tumour committees are adapted to the pathologies' characteristics, they can promote a collective and multidisciplinary approach to oncology.

Soft tissue sarcoma - a population-based, nationwide study with special emphasis on local control.

BACKGROUND: A prospective diagnostics and treatment protocol for extremity and trunk wall soft tissue sarcoma (STS) was introduced by the Scandinavian Sarcoma Group in 1986 and it was also widely adopted in Finland. We have updated the protocol and made it more detailed at the Helsinki University Central Hospital. We retrospectively compared diagnostics and treatment of STS in a nationwide population-based material to this protocol with special emphasis on local control. METHODS: Data for 219 patients with an STS of extremity or trunk wall diagnosed during 1998-2001 was retrieved from the nationwide Finnish Cancer Registry. Histologic review was performed. Treatment centres were divided into high-, intermediate- and low-volume centres based on the number of patients with final surgery during the study period. RESULTS: Significantly more patients were operated with a preoperative histological or cytological diagnosis at high-volume centres. No preoperative diagnosis was a strong predictor for the patient to undergo more than one operation (p < 0.0001). Wide surgical margin was achieved more often at high-volume centres, but in all centre categories a considerable percentage of patients with inadequate surgical margin did not receive adjuvant radiation therapy. Local control at five years was 82% at high-volume centres, 61% at intermediate-volume centres treating highest percentage of deep tumours and 69% at low-volume centres (p = 0.046). Local control improved as the number of patients operated (surgical volume of the centre) increased. CONCLUSION: The present quality-control study is the first nationwide population-based study to assess diagnostics and treatment of STS. When referred to a specialised sarcoma centre even patients with inadequate surgery can achieve good local control. STS is a rare cancer and its treatment should be centralised in Finland, which has 5.4 million inhabitants and approximately 100 new STSs of extremities and trunk wall annually.

Survey of orbital tumors at a comprehensive cancer center in the United States.

BACKGROUND: The purpose of this study was to evaluate the frequencies of various types of orbital lesions seen at a comprehensive cancer center in the United States. METHODS: In this retrospective case series, we reviewed the medical records of 268 consecutive patients referred to our orbital oncology service for evaluation of an orbital mass between November 1998 and February 2009. Each orbital lesion was documented by CT and/or MRI, and in most cases diagnosis was established with a tissue biopsy. Patients who were seen for second opinions and had inadequate follow-up data were excluded, as were patients with thyroid eye disease or orbital hemorrhage. RESULTS: The study included 134 men and 134 women aged 1 to 89 years at diagnosis (median, 55 years). Follow-up ranged from 0.06 to 192 months (median, 15 months). Of the tumors, 171 (64%) were primary orbital, 69 (26%) were secondary orbital, and 28 (10%) were metastatic tumors. Lesion types were as follows: secondary orbital tumors, 69 (26%); lymphoproliferative lesions, 68 (25%); metastases, 28 (10%); epithelial lacrimal gland tumors, 27 (10%); inflammatory conditions, 21 (8%); vascular lesions, 20 (7%); mesenchymal tumors, 18 (7%); optic nerve and nerve sheath tumors, 7 (3%); peripheral nerve tumors, 3 (1%); histiocytic lesions, 3 (1%); cystic lesions, 3 (1%); and other lesions, 1 (<1%). The most common histopathologic diagnoses were lymphoma, 50 cases (19%); orbital extension of sinus tumor, 25 (9%); lacrimal gland adenoid cystic carcinoma, 18 (7%); cavernous hemangioma, 15 (6%); orbital extension of brain tumor, 14 (5%); idiopathic orbital inflammation, 14 (5%); plasmacytoma, 8 (3%); reactive lymphoid hyperplasia, 7 (3%); metastatic breast cancer, 7 (3%); orbital extension of ocular adnexal basal cell carcinoma, 7 (3%); orbital extension of ocular adnexal melanoma, 6 (2%), rhabdomyosarcoma, 6 (2%); metastatic gastrointestinal cancer, 5 (2%); sarcoidosis, 5 (2%); and other less common lesions. Forty-two lesions (16%) were intraconal, and 226 (84%) were extraconal. There were 169 (63%) malignant tumors and 99 (37%) were benign tumors. The rate of malignant lesions was 65% in children and 63% in adults (≥18 years). Malignant conditions encountered at a higher rate than previously reported included lymphoma (19% vs 8% to 13%), secondary orbital tumors (26% vs 13% to 20%), orbital metastases (10% vs 2% to 7%), and malignant epithelial lacrimal gland tumors (9% vs 2% to 3%). CONCLUSION: Our findings highlight the distinctive nature of the orbital oncology experience at a comprehensive cancer center. We found higher than previously reported rates of malignant tumors (63% of all tumors), particularly secondary tumors, malignant epithelial lacrimal gland tumors, and orbital metastases.

[Multimodality therapy concepts for soft tissue sarcomas]. / Multimodale Therapiekonzepte bei Weichteilsarkomen.

Despite the fact that soft tissue sarcomas are representing a rare tumor entity with a low incidence rate of about 2-4 per 100,000 per year, they highly require a multimodality therapeutic approach. Based on a reference pathology a complete surgical resection is the first treatment goal. After accomplished R0 resection the local relapse rate can be further decreased by an adjuvant radiotherapy. For primarily irresectable or only partially respectable tumors a neoadjuvant chemotherapy combined with regional hyperthermia should be considered. Patients with metastasized soft tissue sarcomas should receive an anthracyclin-based chemotherapy in a palliative intention. Prognostically more favorable are gastrointestinal stroma tumor, also in advanced stages with metastases, since the tyrosine kinase inhibitors imatinib and sunitinib can induce durable remissions.

Pesticides, soft-tissue sarcoma and non-Hodgkin lymphoma--historical aspects on the precautionary principle in cancer prevention.

BACKGROUND: After the 2(nd) World War a long range of chemical agents have been introduced on the market, both in Sweden and most other countries. From the 1950's several pesticides gained increasing use in agriculture and forestry. In the 1970's public concern increased in Sweden especially regarding use of phenoxy herbicides to combat deciduous wood, although statements from different authorities were reassuring of the safety. MATERIALS AND METHODS: At the end of the 1970's the author and his colleagues published the first scientific evidence of an association between exposure to phenoxyacetic acids, chlorophenols and certain malignant tumours, i.e., soft-tissue sarcoma and malignant lymphoma. The study subjects were also exposed to contaminating dioxins such as 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). Later studies showed also an association between certain persistent organic pollutants such as polychlorinated biphenyls and non-Hodgkin lymphoma (NHL) with an interaction with titers of antibodies to Epstein-Barr virus early antigen. These results have been corroborated in other studies. DISCUSSION: Over the years industry and its allied experts have attacked our studies, but in 1997 IARC classified TCDD as a human carcinogen, Group I. The increasing incidence of NHL in Sweden levelled off about 1990. The author postulated that the regulation or ban of the use of chlorophenols, certain phenoxy herbicides and some persistent organic pollutants in Sweden back in the 1970s has contributed to the now decreasing incidence of NHL. Unfounded criticism from industry experts may prohibit the precautionary principle and early warnings of cancer risk can be ignored. Cancer risks by certain chlorinated phenols may serve as a model of how the precautionary principle should be used by taking early warnings seriously.

Epidemiological aspects of soft tissue sarcomas (STS)--consequences for the design of clinical STS trials.

The purpose of the study was to gain insight into epidemiological aspects of soft tissue sarcomas (STS), based on the population-based cancer registry of the Comprehensive Cancer Center North-Netherlands (CCCN), and to provide data for the development of future STS clinical trials. 456 primary STS (Kaposi, urogenital and gastro-intestinal STS excluded), registered from 1989 to 1995 by the cancer registration of the Comprehensive Cancer Center North-Netherlands (CCCN), were analysed. The annual, age-adjusted, STS incidence was 3.6 per 100,000. Incidence increased with age. Half of the patients were over the age of 65 years. Malignant fibrous histiocytomas and liposarcomas were most frequently encountered. At presentation, nodal involvement was rare (3-8%). Distant metastases were more frequently encountered (9-14%), and appeared to be related to tumour size and site. Above 70 years of age, 16% of patients received no treatment at all, especially for metastatic disease.

Sensitivity of hyperthermia trial outcomes to temperature and time: implications for thermal goals of treatment.

PURPOSE: In previous work we have found that the cumulative minutes of treatment for which 90% of measured intratumoral temperatures (T90) exceeded 39.5 degrees C was highly associated with complete response of superficial tumors. Similarly, the cumulative time for which 50% of intratumoral temperatures (T50) exceeded 41.5 degrees C was highly associated with the presence of > 80% necrosis in soft tissue sarcomas resected after radiotherapy and hyperthermia. In the present work we have calculated the time for isoeffective treatments with T90 = 43 degrees C and T50 = 43 degrees C, respectively, using published thermal isoeffective dose formulae. The purpose of these calculations was to determine the sensitivity of treatment outcome to variations in thermal isoeffective dose. METHODS AND MATERIALS: The basis for the calculations were the thermal parameters and treatment outcomes in three patient populations: 44 patients with moderate or high grade soft tissue sarcoma treated preoperatively with hyperthermia and radiation; 105 patients with superficial tumors treated with hyperthermia and radiation, and 59 patients with deep tumors treated with hyperthermia and radiation. RESULTS: The thermal dose values calculated are strongly associated with outcome in multivariate logistic regression analysis. Simple dose-response equations result from the analysis, and we use these equations to assess the sensitivity of outcome upon variations in thermal dose. This information, in turn, allows us to estimate the number of patients required in Phase II and III trials of hyperthermia and radiation therapy. CONCLUSIONS: For regimens of 5 to 10 hyperthermia treatments, improvements in median T90 (superficial tumors) and T50 (deep tumors) parameters by 1.2-1.5 degrees C could result in response rates high enough (compared to radiotherapy alone) to justify Phase III trials. A similar improvement in response rates would require an increase in overall duration of treatment by a factor of 3 to 5. This would be difficult to achieve while also avoiding thermal tolerance induction. Achieving these temperature goals may be possible with improvements in hyperthermia technology. Alternatively, there may be ways to increase the sensitivity of cells to temperatures that can be achieved currently, such as pH reduction or chemosensitization.

Adjuvant brachytherapy for treatment of chest wall sarcomas.

Thirty patients treated with surgical resection and brachytherapy for chest wall sarcoma at Memorial Sloan-Kettering Cancer Center from 1980 through 1987 were reviewed. Patients selected to receive adjuvant irradiation were those for whom there was doubt as to the completeness of surgical resection. Overall 5-year survival and locoregional control after brachytherapy were 65% and 54%, respectively. Locoregional control was similar for tumors treated at initial diagnosis (12 patients), at the time of recurrence (13 patients), or for tumors that were metastatic to the chest wall (five patients). Six patients with tumors larger than 10 cm in maximum dimension had a locoregional recurrence rate of 69% versus a recurrence rate of 39% for 18 patients with smaller tumors (p = 0.27). Fifty-four percent of high-grade tumors recurred locoregionally versus 28% of low-grade tumors (p = 0.37). Bone invasion or the presence of positive resection margins was not clearly associated with a higher locoregional failure rate. Only one patient (1/28; 7%) was known to have had recurrence within the irradiated area. Eight patients (8/28; 37%) had recurrence adjacent to the implanted area, and the precise failure site could not be determined for the remaining two patients. Because of the relatively high risk of regional versus in-field recurrence, patients with chest wall sarcoma who receive adjuvant treatment should be treated primarily with external-beam irradiation to allow more generous coverage of the tumor bed. Brachytherapy could be used as a tumor bed "boost" treatment. In patients undergoing resection of recurrent tumor in a previously irradiated site, adjuvant brachytherapy, without external-beam irradiation, should be considered to reduce the risk of extensive soft tissue necrosis.