Epidemiologic Survey of Myxofibrosarcoma Using Data from the Bone and Soft Tissue Tumor Registry in Japan.

BACKGROUND: Myxofibrosarcoma is a common sarcoma among older patients, with locally infiltrative behavior and a predilection for local postoperative recurrence. Some studies have reported the factors affecting prognosis, although only a few have mentioned the previous staging classification systems. This study investigated the clinical overview and prognosis of myxofibrosarcoma to determine the optimal treatment. METHODS: This retrospective study analyzed the records of 349 patients with myxofibrosarcoma in the nationwide Bone and Soft Tissue Tumor Registry in Japan from 2006 to 2015. Clinical features, treatment options, and patient outcomes were investigated. RESULTS: Ultimately, 349 patients were identified. The overall survival rates were 93.1% at 2 years and 84.3% at 5 years. A multivariate analysis was performed using the Cox proportional hazards model. The study identified four significant prognostic factors for survival: tumor size, depth, compartment status, and location. The prognostic score was calculated by summing the scores of all the factors. The overall survival rate was 69.3% at 5 years for the patients with prognostic scores of 6 or higher. Conversely, the patients with prognostic scores of 2 or lower had a survival rate of 95.6% at 5 years. CONCLUSIONS: Among myxofibrosarcomas, those larger than 5 cm, deep-seated, invaded into the external compartment, or in axial body parts were associated with a significantly worse prognosis. Adjuvant radiotherapy and chemotherapy did not contribute significantly to a better prognosis. Previous staging classification systems are impractical for prognosis prediction. Therefore, new classifications are needed. Further research on new treatment methods for patients with a poor prognosis will be crucial in the future.

Radical Resection of Pediatric Fibrosarcoma Is Not Associated With Survival Benefit: A Nationwide Database Analysis.

INTRODUCTION: Fibrosarcoma is one of the most common nonrhabdomyosarcoma soft tissue sarcomas in the pediatric population. Surgical resection is the cornerstone of treatment; however, optimal factors regarding the extent of fibrosarcoma resection in localized versus regional disease is poorly understood. METHODS: The Surveillance, Epidemiology, and End Results database was queried for patients who are 19 y old or younger with a diagnosis of fibrosarcoma from 1975 to 2016. Kaplan-Meier analyses were performed for disease-specific survival on clinical and pathologic variables. Multivariate analysis was performed based on significant predictors of disease-specific survival. RESULTS: There were 1290 patients (median age 13 [7-17] y) identified with fibrosarcoma. The overall survival rate at 20 y was 93%. Radical resection was performed on 22%, 40%, and 52% of patients with localized, regional, and distant Surveillance, Epidemiology, and End Results disease stage, respectively. Chemotherapy (P < 0.001), radiation (P < 0.001), histology (P < 0.001), and stage (P = 0.004) were significant predictors of increased mortality using univariate Kaplan-Meier analysis. Most patients (86%) with localized or regional stage who underwent radiation had fibrosarcomas of nondermatofibrosarcoma histology (P < 0.001). Dermatofibrosarcoma subtype comprised most radical resections (41%). Excluding distant stage, nondermatofibroma histology (hazard ratio 16.94, 95% confidence interval 3.77-76.01) and regional stage (hazard ratio 8.15, 95% confidence interval 2.93-22.69) were independent prognostic factors of mortality (both P < 0.001). Radical resection was not a significant independent prognostic indicator of survival. CONCLUSIONS: Nondermatofibrosarcoma subtype is independently associated with increased mortality. Although surgery remains the mainstay of treatment, the extent of resection is not a predictor of survival for patients with the localized and regional stage of disease.

Malignant fibrous neoplasms of long bones: analysis of the surveillance, epidemiology, and end results database from 1973 to 2015.

BACKGROUND: Malignant fibrous neoplasms (MFN) of long bones are rare lesions. Moreover, the prognostic determinants of MFN of long bones have not been reported. This study aimed to present epidemiological data and analyse the prognostic factors for survival in patients with MFN. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) programme database was used to screen patients with malignant fibrous neoplasms (MFN) of long bones from 1973 to 2015, with attention to fibrosarcoma, fibromyxosarcoma, periosteal fibrosarcoma and malignant fibrous histiocytoma. The prognostic values of overall survival (OS) and cancer-specific survival (CSS) were assessed using the Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan-Meier method was used to obtain OS and CSS curves. RESULTS: A total of 237 cases were selected from the SEER database. Malignant fibrous histiocytoma was the most common form of lesion in long bones. Multivariate analysis revealed that independent predictors of OS included age, stage, tumour size and surgery. Age, stage, tumour size and surgery were also independent predictors of CSS. Additionally, the most significant prognostic factor was whether metastasis had occurred at the time of initial diagnosis. CONCLUSION: Among patients with MFN of long bones, age (> 60 years), tumour size (> 10 cm), distant stage, and non-surgical treatment are factors for poor survival.

[An analysis of incidence trends and characteristics of soft tissue sarcoma in Beijing, 1999-2013].

Objective: To analyze the incidence trends and to describe the characteristics of soft tissue sarcoma (STS) among residents in Beijing from 1999 to 2013. Methods: Medical information of the cases diagnosed as STS(ICD10: C47&C49) from 1999 to 2013 in Beijing was extracted from the population-based database of Beijing Cancer Registry.Crude incidence rate, age-standardized incidence rates to Chinese population (ASRC)and the world population(ASRW) were calculated. The characteristics of the STS cases in different gender and age groups from urban and rural areas were respectively analyzed.The incidence trends and annual percentage changes (APC) during last 15 years were analyzed by using JoinPoint 3.4.3 software. Results: A total of 2 048 cases were diagnosed as STS during the study period and the incidence rate of STS was 1.15 per 100 000 person-years. The ASRC was 0.74 per 100 000 person-years and ASRW was 0.86 per 100 000 person-years, respectively. Except for the impact of changes in the age composition, the APC of ASRW from 1999 to 2013 was 3.95%. For males, the incidence rate was increased from 0.65 per 100 000 in 1999 to 1.51 per 100 000 in 2013, and the APC was 4.27% (P<0.05); For females, the incidence rate was increased from 0.61 per 100 000 in 1999 to 0.91 per 100 000 in 2013, and the APC was 3.38% (P<0.05). In urban area, the incidence rate increased from 1.14 per 100 000 in 2000 to 1.35 per 100 000 in 2013, and the APC was 2.70% (P=0.05); In rural area, the incidence rate increased from 0.57 per 100 000 in 2000 to 0.98 per 100 000 in 2013, and the APC was 4.66% (P=0.04). 85.89%cases were pathologically diagnosed.Among them, undifferentiated pleomorphic sarcoma was the most common subtype, which accounting for 19.22%, followed by liposarcoma (19.04%), malignantnerve sheath tumor (10.18%), fibrosarcoma (10.06%)and sarcoma without definite type(7.22%). Conclusions: The incidence rate of STS is still increasing, especially in the rural area of Beijing. The most common subtypes of STS in different age-groups were different. Further study is needed to explore the high risks of STS in the population with specific characteristics.

Diagnosis and sequence analysis of avian leukosis virus subgroup J isolated from Chinese Partridge Shank chickens.

The diagnosis of avian leukosis virus subgroup J (ALV-J) infection in Chinese Partridge Shank chickens was confirmed by necropsy, histopathological examinations, antibody tests, viral isolation, immunofluorescence assays, and sequence analysis. Myelocytoma, myeloma, and fibrosarcoma were simultaneously found in Partridge Shank flock with ALV-J infection. Sequence analysis of the env genes of ALV-J demonstrated that both gp85 and gp37 were highly homologous among the three strains from local chickens of those among ALV-J strains isolated from white meat-type chickens. The phylogenetic trees indicated that the three strains isolated in this study were closely related to reference strains isolated in so-called Chinese yellow chickens and some strains isolated from white meat-type chickens, both from the USA and China. The observed ALV-J infection was the first report on Partridge Shank chickens, and myelocytoma, myeloma, and fibrosarcoma were found at the same time in this batch of local chickens.

Myxofibrosarcoma: prevalence and diagnostic value of the "tail sign" on magnetic resonance imaging.

OBJECTIVE: Myxofibrosarcoma frequently shows curvilinear extensions of high T2 signal that also enhance on magnetic resonance imaging; these "tails" represent fascial extension of tumor at histopathological examination. This study was performed to determine whether the tail sign is helpful in distinguishing myxofibrosarcoma from other myxoid-containing neoplasms. MATERIALS AND METHODS: The study group consisted of 44 patients with pathologically proven myxofibrosarcoma; the control group consisted of 52 patients with a variety of other myxoid-predominant tumors. Three musculoskeletal radiologists independently evaluated T2-weighted (and/or short-tau inversion recovery) and post-contrast MR images for the presence of one or more enhancing, high-signal intensity, curvilinear projections from the primary mass. Sensitivity and specificity for the diagnosis of myxofibrosarcoma were calculated for each reader. Interobserver variability was assessed with kappa statistic and percentage agreement. RESULTS: A tail sign was deemed present in 28, 30, and 34 cases of myxofibrosarcoma and in 11, 9, and 5 of the controls for the three readers respectively, yielding a sensitivity of 64-77 % and a specificity of 79-90 %. The interobserver agreement was moderate-to-substantial (kappa=0.626). CONCLUSION: The tail sign at MRI is a moderately specific and sensitive sign for the diagnosis of myxofibrosarcoma relative to other myxoid-containing tumors.

The enigma of myxofibrosarcoma of the extremity.

BACKGROUND: The reported high rate of local recurrence (LR) in myxofibrosarcoma raises the question of whether this sarcoma histology should be considered radioresistant. In this study, the authors compared rates and patterns of LR of high-grade (HG) myxofibrosarcoma with rates and patterns of HG leiomyosarcoma, which was chosen because of the similarity in incidence and general treatment approach. METHODS: Two hundred two patients with primary, nonmetastatic extremity myxofibrosarcoma (n = 114) and leiomyosarcoma (n = 88) underwent limb-sparing surgery and were followed prospectively. All 202 patients had HG tumors, and 138 patients (68%) received adjuvant radiation therapy. RESULTS: The groups were comparable in terms of age, sex, and receipt of chemotherapy. Compared with leiomyosarcoma, myxofibrosarcoma presented more frequently with tumors >5 cm (P < .001), deep location (P = .036), and upper extremity site (P = .015). In addition, rates of positive/close margins (P < .001) and the receipt of radiation therapy (P < .001) were significantly higher in the myxofibrosarcoma group. The 5-year overall LR rate was not significantly different according to histology (14.6% for myxofibrosarcoma, 13.2% for leiomyosarcoma; P = .594). The only predictor of LR for the whole cohort of patients was positive/close margins (P = .01). Of 17 myxofibrosarcoma LRs, 8 (47%) occurred out of field, versus 1 of 12 (8%) leiomyosarcoma LRs (P = .04). Leiomyosarcoma more commonly recurred distantly (54.1% vs 24.3% at 5 years; P = .014). CONCLUSIONS: Despite more adverse clinical features, myxofibrosarcoma recurred distantly less often than leiomyosarcoma, whereas the LR rates were comparable between the 2 groups, suggesting that adjuvant radiation therapy is effective in myxofibrosarcoma. Myxofibrosarcoma LRs more commonly occurred out of field. Reduction in radiation field margins may not be advisable in patients with myxofibrosarcoma.

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007.

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

Bone and/or joint attachment is a risk factor for local recurrence of myxofibrosarcoma.

BACKGROUND: Myxofibrosarcoma is characterized by a high local recurrence rate despite optimal surgical treatment. The definition of prognostic factors for recurrence offers high-risk patients a closer follow-up and a multidisciplinary therapeutic approach. METHODS: A cohort of 23 patients treated for primary myxofibrosarcoma was retrospectively analyzed. The patients (sex and age), tumors (size, stage, tumor location, bone and/or joint attachment), radiological findings (abnormal signal extension in MRI), histological findings (FNCLCC grade and microscopic extension along the muscle fascia), and treatment (surgical margin) characteristics were included in univariate prognostic factor analysis. RESULTS: After a median follow-up of 63.3 months (range 15-191), the overall recurrence rate was 34.7%. Median time between initial surgery and recurrence was 24.8 months (range 8-52). Inadequate surgical margins (p = 0.026) and bone and/or joint attachment (p = 0.001) were associated with an increased recurrence rate. CONCLUSION: For the further improvement of local recurrence-free survival of patients with myxofibrosarcoma, accurate diagnosis of the tumor extension and adequate planning for the surgical margin should be focused on in cases with bone and/or joint attachment.

The impact of local control in the treatment of children with advanced infantile and adult-type fibrosarcoma: Experience of the cooperative weichteilsarkom studiengruppe (CWS).

BACKGROUND AND OBJECTIVES: This study aims at examining the potential survival benefits of primary versus secondary surgery of children diagnosed with advanced infantile (iFS) and adult-type fibrosarcoma (aFS). METHODS: Treatment and outcome of 89 children with FS treated within prospective Cooperative Studiengruppe (CWS) trials (1981-2016) were analyzed retrospectively. RESULTS: Localized disease (LD) was diagnosed in 87 patients: 64/66 patients with iFS (≤2 years) and 23 with aFS (>2 ≤ 18 years). Two patients (iFS) had metastatic disease. Resection was the mainstay of therapy of patients with LD resulting in microscopically complete (R0, IRS group I) (n = 29/87, 33%), microscopically incomplete (R1, IRS group II) (n = 17/87, 20%) and macroscopically incomplete (R2, IRS group III) (n = 41/87, 47%). Advanced LD (IRS group III) was present in 32/64 (50%) patients with iFS and in 9/23 (39%) with aFS. Chemotherapy was added predominantly in patients with advanced disease and an assessable objective response to CHT was seen in 71% iFS and 75% aFS. The 5-year event-free survival (EFS) of patients with iFS and aFS was 81% (±10, 95% CI) and 70% (±19, 95% CI) (p = 0.24); the 5-year overall survival (OS) was 98% (±3, 95% CI) and 82% (±16, 95% CI) (p = 0.02). Primary resection was no prognostic factor. Secondary R0/ R1 resection in patients with advanced disease improved 5-year EFS and OS in aFS (p = 0.002 and p = 0.000) but not in infants. CONCLUSIONS: Secondary resection improves outcome in advanced aFS but not in infants. Mutilating surgery in infants should be avoided. TYPE OF STUDY AND LEVEL OF EVIDENCE: Treatment study: patients were enrolled in five prospective studies and one registry, prognosis study: retrospective study. LEVEL OF EVIDENCE: II/ III. MINI-ABSTRACT: Fibrosarcoma is a very rare malignant tumor. Little is known about differences of local treatment of advanced infantile and adult-type. Data of 89 patients registered in five prospective trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2016) were analyzed.

Radiotherapy-induced malignancies in breast cancer patients with TP53 pathogenic germline variants (Li-Fraumeni syndrome).

The risk of radiotherapy-induced malignancies (RIMs) is a concern when treating Li-Fraumeni syndrome (LFS) or Li-Fraumeni Like (LFL) patients. However, the type of TP53 pathogenic germline variant may possibly influence this risk. TP53 p.R337H mutation is particularly prevalent in Brazil. We aimed to evaluate the outcomes of patients with pathogenic TP53 variants treated for localized breast cancer in a Brazilian cohort. We evaluated retrospectively a cohort of patients with germline TP53 pathogenic variants treated for localized breast cancer between December 1999 and October 2017. All patients were followed by the Hereditary Cancer Group of an academic cancer center. Our primary objective was to evaluate the occurrence of RIMs after adjuvant radiotherapy. Sixteen patients were evaluated; 10 (62.5%) had a germline TP53 p.R337H pathogenic variant. Median age was 39.8 years. Thirteen patients had invasive ductal carcinoma: 8 (61.5%) were hormone receptor-positive; 6 (46.1%), human epithelial growth factor receptor 2 (HER2)-amplified. Three patients had ductal carcinoma in situ. Most patients (N = 12/16, 75%) received adjuvant radiotherapy. After a median follow-up of 52.5 months, 2 patients (2/12, 16.6%) had RIMs. One had a fibrosarcoma and the other, a low-grade leiomyosarcoma. In the group treated with radiotherapy, one distant recurrence was diagnosed (1/12), and no loco-regional recurrence occurred. Among 4 patients who did not receive radiotherapy, 2 presented with loco-regional recurrence. In this cohort of patients with LFS enriched in TP53 p.R337H pathogenic variant, the incidence of RIMs after treatment of localized breast cancer was lower than previous literature. Nevertheless, rates of RIMs were still alarming. Early molecular diagnosis and careful evaluation of treatment risks and benefits are essential for these patients.

Cardiovascular safety of rapidly accelerated fibrosarcoma B-type and/or mitogen-activated extracellular signal-regulated kinase inhibitors: A mixed approach combining a meta-analysis and a pharmacovigilance disproportionality analysis.

BACKGROUND: The risk of cardiovascular adverse events from rapidly accelerated fibrosarcoma B-type (BRAF) and mitogen-activated extracellular signal-regulated kinase (MEK) inhibitors is not fully characterized. AIM: To evaluate the cardiovascular adverse events risks related to BRAF and/or MEK inhibitors in randomized placebo-controlled clinical trials and in the real-life setting. METHODS: We used two approaches. First, we conducted a systematic review and meta-analysis of randomized placebo-controlled clinical trials reporting the incidence of cardiovascular adverse events for BRAF and/or MEK inhibitors in cancer patients. Second, we performed a disproportionality analysis, using age- and sex-adjusted reporting odds ratios (arORs) and their 95% confidence intervals (CIs) from the World Health Organization's pharmacovigilance database (VigiBase®) of anticancer drug-associated reports, to investigate real-life data. RESULTS: MEK inhibitors increased the risk of ejection fraction decrease (odds ratio [OR] 3.35, 95% CI 1.58-7.07), peripheral oedema (OR 2.87 95% CI 1.93-4.27) and syncope (OR 6.71, 95% CI 3.00-14.99) compared with placebo in randomized placebo-controlled clinical trials. BRAF and MEK inhibitor combination therapy further increased the risk of ejection fraction decrease. In the disproportionality analysis, we found over-reporting of ejection fraction decrease (arOR 8.42, 95% CI 7.03-10.09), peripheral oedema (arOR 1.39, 95% CI 1.17-1.66), syncope (arOR 1.56, 95% CI 1.22-1.99), torsade de pointes/QT prolongation (arOR 6.13, 95% CI 5.04-7.47) and supraventricular arrhythmias (arOR 1.50, 95% CI 1.21-1.85) for BRAF and MEK inhibitors. BRAF and MEK inhibitors were not associated with hypertension in either approach. CONCLUSIONS: In conclusion, MEK inhibitors increase the risk of ejection fraction decrease, peripheral oedema and syncope in randomized placebo-controlled clinical trials. Real-life data confirm these findings, and suggested additional risks of torsade de pointes/QT prolongation and supraventricular arrhythmias with BRAF/MEK inhibitors.

Pediatric head and neck fibrosarcomas: A demographical, treatment, and survival analysis and review of a rare case.

OBJECTIVE: To examine pediatric head and neck fibrosarcoma cases and review the demographics, management, and survival for these patients. METHODS: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of a head and neck fibrosarcoma using ICD-O-3 head and neck primary sites and histology codes. Patients were included from birth-18 years of age. Additionally, a pediatric case of a head and neck infantile fibrosarcoma treated at the Nemours Children's hospital in Orlando, Florida is presented. RESULTS: One hundred-thirteen pediatric head and neck fibrosarcomas were identified within the SEER database over the study period. The mean age at diagnosis was 9.8 years (SD: 6.2, range: 0.0-18.0). The mean age at diagnosis for infantile fibrosarcomas was 1.7 years (SD: 3.2, range: 0.0-12.0). Fifty-one (45.1%) patients were female. A majority (N = 67, 59.3%) of patients had dermatofibrosarcoma followed by 18 (15.9%) who had infantile fibrosarcomas. Nearly all patients (N = 107, 94.7%) received surgical intervention. 27.8% of patients with an infantile fibrosarcoma received chemotherapy as a part of their care compared to 1.5% of patients with a dermatofibrosaroma (p = .004). The 5-year disease-specific survival was 97%. CONCLUSIONS: Pediatric patients with head and neck fibrosarcomas are most likely to present in Caucasian males or females during late childhood or early adolescence. Infantile fibrosarcomas present in pediatric patients at a much earlier age. Surgical management is common for pediatric head and neck fibrosarcomas. Additionally, chemotherapy may be used for infantile fibrosarcomas of the head and neck. Survival rates for pediatric patients with a head and neck fibrosarcoma are excellent.

Oral benign neoplasms: A retrospective study of 790 patients over a 14-year period.

INTRODUCTION AND OBJECTIVE: Oral benign neoplasms (OBNs) exhibit some features that can guide the professionals to the correct diagnosis and best treatment. Through retrospective studies, medical records can be reviewed to better describe a given population and, furthermore, help clinicians in routine practice. In this context, the objective of this paper was to analyze the cases of OBNs of an oral pathology referral department, from 2003 to 2017, in order to better understand their epidemiological and clinicopathological characteristics. METHODS: A total of 8355 histopathological reports were analyzed. Lesions diagnosed as OBNs were selected and the following variables were recorded: gender, age, histological type of the lesion, anatomical location, rate and pattern of growth, type of base, color, symptomatology and diagnostic hypotheses on clinical examination. RESULTS: OBNs represented 9.4% of all lesions diagnosed. The most frequent histopathological types were fibroma (39.9%), papilloma (22%), fibroblastoma (13.1%), lipoma (10.2%) and hemangioma (6.1%). Overall, most cases affected females (n=518; 65.6%) and in the fifth decade of life (n=148; 18.7%). The oral mucosa was the most common site (n=265; 33.5%). The most common features of each OBN were also highlighted. CONCLUSION: The most common OBNs were fibroma, papilloma, fibroblastoma, lipoma and hemangioma. Overall, the OBN presented common clinical features; however, in particular cases, there are some characteristics that can lead the professionals to the correct diagnosis. Nevertheless, in general, histopathological analysis must be performed to confirm diagnosis. Intraosseous tumors and large lesions may require imaging tests to help diagnosis.

Incidence and time trends of soft tissue sarcomas in German children 1985-2004 - a report from the population-based German Childhood Cancer Registry.

The incidence of soft tissue sarcoma (STS) in Europe is increasing, but it is unclear whether this increase can also be seen in Germany. We analysed the heterogeneous group of STS recorded to the German Childhood Cancer Registry (GCCR) between 1985 and 2004 with respect to incidence data. Age-specific, age-standardised and cumulative incidence rates were calculated. Additionally, the average annual percent change (AAPC), derived from a Poisson regression model, was estimated, using time in years as the explanatory, continuous variable. Two thousand sixty-one children were diagnosed at a median age of 72 months. Most common are rhabdomyosarcomas (RMS) (n=1202) and fibrosarcomas (n=174). The age-standardised incidence rate (ASR) is 0.9 per 100,000/year for all STS, 0.70 for rhabdomyosarcoma-like (n=1588) and 0.18 for non-rhabdomyosarcoma-like (n=411) STS. AAPC is +0.4% (95%-confidence interval [-0.4;+1.2%]) for STS. In Germany, the increase in incidence rate is less evident than in other European countries.

Sarcomas of the oral and maxillofacial region: a review of 88 cases in Zimbabwe.

We reviewed the case notes of 88 patients who were treated for sarcomas of the oral and maxillofacial region over a period of 24 years. There were 51 men (mean age 23 years) and 37 women (mean age 29). Forty-six tumours were in the mandible. Osteosarcoma accounted for 31 cases, and rhabdomyosarcoma 21 (usually under the age of 10 years).

Low-grade myofibroblastic sarcoma: A population-based study.

OBJECTIVES/HYPOTHESIS: Low-grade myofibroblastic sarcoma (LGMS) is a rare entity that is described as having a predilection for occurring in the head and neck region. Here we analyze its demographics, clinic-pathologic, and survival characteristics. STUDY DESIGN: Retrospective database analysis. METHODS: A cohort from the Surveillance, Epidemiology, and End Results Program database of cases with LGMS between 2001 and 2012. RESULTS: There were 49 cases with a 5-year overall survival of 71.6% and disease- specific survival of 76.3%. The majority of cases were in patients <60 years old, female, and white ethnicity. The most common sites were the extremities in 40.8% of cases followed by the head and neck region with 26.5% of cases. Multivariate analysis showed that only older age was significantly associated with worse survival (P < .05). CONCLUSIONS: LGMS is uncommon in the United States and occurs most commonly in the extremities followed by the head and neck region, despite an existing characterization of a predilection for the head and neck region. Treatment most commonly involves surgery, but the optimal surgical extent and/or radiotherapy needs to be further investigated. LEVEL OF EVIDENCE: 2c Laryngoscope, 127:116-121, 2017.

Occurrence of spontaneous tumors in the germfree F344 rat.

The incidence of spontaneously occurring neoplasms was observed over 10 years in a colony of germfree F344 rats (78 males and 102 females) representing 10 generations of inbredding. Leukemia was the most common neoplasm (25.6% of males, 36% of females) followed by mammary tumors (11.5% of males, 19.6% of females). Various other tumors developed in 9% of the males and 5% of the females. The overall incidence of spontaneous tumors was comparable to that reported for conventional rats of different strains. This was particularly true of leukemia for which the most data were available. However, for unknown reasons, significantly fewer solid tumors were observed in germfree than in conventional male rats.

[A case of primary fibrosarcoma of the pulmonary artery].

A 38-year-old-man was admitted to our hospital complaining of exertional dyspnea. Chest radiography and CT showed a huge mass that completely occluded the left main pulmonary artery and spread into the right main pulmonary artery. The tumor was diagnosed as sarcoma by transbronchial biopsy. Radiation therapy obtained temporary partial response. He died of respiratory insufficiency after 14 months. The autopsy revealed primary pulmonary artery sarcoma: fibrosarcoma arising from the left pulmonary artery. To our knowledge, this is the first case report of fibrosarcoma of the pulmonary artery in Japan.