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Background: Primary cardiac tumors are extremely rare. Most primary cardiac tumors are benign and around one quarter is malign. Sarcomas are accounting for 95% of these malign tumors and they show different histologies. The prognosis is poor with a mean survival of 3 months to 1 year, even with complete radical resection. We report the cases of two patients with primary cardiac sarcoma treated with surgery and radiation and/or chemotherapy. In addition we retrospectively collected data of patients with primary cardiac sarcoma treated between 2005 and 2019 with minimum follow-up of 12 months. Clinical characteristics, treatment modalities and outcomes were collected and analyzed. Finally a literature review was done.Case Presentation: The first patient presented with cerebellar infarction. When she developed a recurrence analysis showed a suspicious myocardial lesion for which irradical surgery (R2) was performed. Histopathology showed an intimal sarcoma of the left atrium. Postoperative radiotherapy was applied without complications. Three months after treatment multiple metastases were diagnosed and she died 13 months after initial diagnosis. The second patient presented with pericardial effusion. A tumor was found located in the right atrium and radical surgery was performed. Histopathology showed an angiosarcoma, without signs of metastases. Adjuvant radiotherapy was added because of close margins and based on high risk of recurrence and metastases it was decided to add chemotherapy. One year after finishing treatment, evaluation showed local recurrence together with pulmonary metastases.Conclusions: Surgery combined with postoperative radiotherapy is feasible in patients with resectable cardiac sarcoma. Distant metastases occur frequently. In patients with an irresectable sarcoma of the heart primary radiotherapy should be considered. [ABSTRACT FROM AUTHOR]

Introduction. Nonsurgical management of patients with desmoid-type fibromatosis (DF) is increasing. This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. Patients and Methods. From the Dutch Pathology Registry (PALGA), patients with extra-abdominal or trunk/abdominal wall DF, diagnosed between 1993 and 2013, were identified. First-line treatment was analyzed. Best response (BR) using RECIST criteria from start of treatment/surveillance until change of treatment or last follow-up was analyzed. Results. Ninety-one of the 1141 identified patients had first-line nonsurgical management. The percentage of patients treated nonsurgically increased from 0.6% in 1993–1998 to 12.8% in 2009–2013. Thirty-seven patients had surveillance (41%), 35 radiotherapy (38%), and 19 systemic treatment (21%). BR for surveillance was complete response (CR) in 2/37, partial response (PR) in 4/37, stable disease (SD) in 21/37, progressive disease (PD) in 5/37, and unknown in 5/37 patients. BR for radiotherapy was CR in 4/35, PR in 11/35, SD in 16/35, and unknown in 4/35. BR for systemic treatment was CR in 1/19, PR in 1/19, SD in 10/19, PD in 2/19, and unknown in 5/19. Totally, 91% of patients did not progress. Discussion. Given the low percentage (9%) of PD of nonsurgical management, these data can be used in shared decision making with the patient regarding optimal treatment. [ABSTRACT FROM AUTHOR]

Treatment of advanced-stage cervical cancers with (chemo)radiation causes cytotoxicity through induction of high levels of DNA damage. Tumour cells respond to DNA damage by activation of the 'DNA damage response' (DDR), which induces DNA repair and may counteract chemoradiation efficacy. Here, we investigated DDR components as potential therapeutic targets and verified the predictive and prognostic value of DDR activation in patients with cervical cancer treated with (chemo)radiation. In a panel of cervical cancer cell lines, inactivation of ataxia telangiectasia mutated (ATM) or its substrate p53-binding protein-1 (53BP1) clearly gave rise to cell cycle defects in response to irradiation. Concordantly, clonogenic survival analysis revealed that ATM inhibition, but not 53BP1 depletion, strongly radiosensitised cervical cancer cells. In contrast, ATM inhibition did not radiosensitise non-transformed epithelial cells or non-transformed BJ fibroblasts. Interestingly, high levels of active ATM prior to irradiation were related with increased radioresistance. To test whether active ATM in tumours prior to treatment also resulted in resistance to therapy, immunohistochemistry was performed on tumour material of patients with advanced-stage cervical cancer ( n = 375) treated with (chemo)radiation. High levels of phosphorylated (p-)ATM [ p = 0.006, hazard ratio (HR) = 1.817] were related to poor locoregional disease-free survival. Furthermore, high levels of p-ATM predicted shorter disease-specific survival ( p = 0.038, HR = 1.418). The presence of phosphorylated 53BP1 was associated with p-ATM ( p = 0.001, odds ratio = 2.206) but was not related to any clinicopathological features or survival. In conclusion, both our in vitro and patient-related findings indicate a protective role for ATM in response to (chemo)radiation in cervical cancer and point at ATM inhibition as a possible means to improve the efficacy of (chemo)radiation. [ABSTRACT FROM AUTHOR]

With the combined treatment procedure of isolated limb perfusion (ILP), delayed surgical resection and external beam radiotherapy (EBRT) for locally advanced soft tissue sarcomas (STS) of the extremities, limb salvage rates of more than 80% can be achieved. However, long-term damage to the healthy surrounding tissue cannot be prevented. We studied the late effects on the normal tissue using the LENT-SOMA scoring system. A total of 32 patients—median age 47 (range 14–71) years—were treated for a locally advanced STS with ILP, surgical resection and often adjuvant 60–70 Gy EBRT. After a median follow-up of 88 (range 17–159) months, the patients were scored, using the LENT-SOMA scales, for the following late tissue damage: muscle/soft tissue, peripheral nerves, skin/subcutaneous tissue and vessels. According to the individual SOM parameters of the LENT-SOMA scales, 20 patients (63%) scored grade-3 toxicity on one or more separate items, reflecting severe symptoms with a negative impact on daily activities. Of these patients, 3 (9%) even scored grade-4 toxicity on some of the parameters, denoting irreversible functional damage necessitating major therapeutic intervention. In evaluating long-term morbidity after a combined treatment procedure for STS of the extremity, using modified LENT-SOMA scores, two-thirds of patients were found to have experienced serious late toxic effects. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the long-term limb salvage rate and overall survival after isolated limb perfusion (ILP) with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma (STS). From 1991 to 2003, 73 patients (36 men, 37 women, median age 54 [range 14–80] years) with biopsy-proven STS underwent 77 perfusions followed by delayed surgical resection, with or without adjuvant radiation. Limb salvage and overall survival curves were calculated by the Kaplan-Meier method. A total of 21 amputations (28%) were performed. Overall 1, 5, and 10 years’ limb salvage was 80.1% ± 4.8%, 68.2% ± 6.5%, and 60.6% ± 9.2%, respectively. We found that the risk of amputation was linked to three time periods. The first was within a year after perfusion, mainly as a result of massive necrosis of the tumor and overlying skin, resulting in soft tissue deficit or recurrent disease (n = 17). The second was within 5 years, with two amputations performed for late local recurrence. The third occurred 10 years after perfusion, with two amputations performed for critical leg ischemia. Another two patients developed a pathological fracture of the femur due to radiation osteonecrosis. These four patients received adjuvant radiotherapy. Overall, 1, 5, and 10 years’ survival was 82.9% ± 9.2%, 58.7% ± 13.1%, and 42.5% ± 18.2%, respectively. ILP treatment with tumor necrosis factor alpha and melphalan followed by delayed surgical resection and adjuvant radiation treatment is an effective limb salvage treatment regimen for locally advanced STS. However, we observed late morbidity, with two amputations performed for critical leg ischemia and two pathological fractures of the femur in patients receiving adjuvant radiotherapy. [ABSTRACT FROM AUTHOR]

We report a patient with a nonresectable histologically benign solitary fibrous tumor who suffered from paraneoplastic non-islet cell tumor hypoglycemia (NICTH). Diagnostic workup revealed malignant characteristics in which the tumor showed up as, presumably, false-negative on fluorodeoxyglucose-positron emission tomography (FDG-PET), while being positive on tyrosine-PET. Neoadjuvant treatment, which consisted of combined chemo-radiation and consecutive selective embolization of the tumor feeding vessels, caused such a therapeutic effect, on both NICTH and reduction in tumor volume, that a secondary resection, with the patient in a normoglycemic status, was possible. Our report highlights several important issues in the management of the patient with a nonresectable solitary fibrous tumor with severe episodes of hypoglycemia due to NICTH. [ABSTRACT FROM AUTHOR]

The aim was to investigate the value of adjuvant radiotherapy for locally advanced soft tissue sarcoma after hyperthermic isolated limb perfusion (ILP) with tumor necrosis factor α and melphalan followed by limb-saving surgery. From 1991 to 2003, 73 patients (median age, 54 years; range, 14–80 years) underwent 77 ILPs, followed by resection in 68 patients (93%). Radiotherapy was administered in case of marginally or microscopically positive resection margins. Local recurrences were scored and calculated according to the Kaplan-Meier method and log-rank test. After residual tumor mass resection, 58% received radiotherapy (external beam radiotherapy [EBRT]+ group), and 42% did not (EBRT− group). The median follow-up was 28 months (range, 2–159 months). A significantly better local control rate was observed in the EBRT+ compared with the EBRT− group ( P < .0001). When only R0 resections in patients without metastasis were considered, the significance remained between groups ( P = .0003). In the EBRT− group, an R1 or R2 resection resulted in earlier relapse of local disease compared with R0 resections ( P = .0475). Adjuvant EBRT reduces the risk for local recurrence after delayed resection in soft tissue sarcoma patients treated with ILP and tumor necrosis factor and is indicated when resection margins are close or microscopically positive. It also seems beneficial after an R0 resection. [ABSTRACT FROM AUTHOR]

This study investigates emotional and behavioural problems in children of parents diagnosed with cancer and examines the relationship with demographic and illness-related variables. Furthermore, agreement and differences between informants regarding child's functioning were examined. Members of 186 families in which a parent had been diagnosed with cancer participated. More emotional problems were reported for latency-aged sons (ill parents) and adolescent daughters (ill parents; self-reports), whereas also better functioning was reported in adolescent children (spouses), compared to the norm group. Age and gender-effects were found: latency-aged sons were perceived as having more emotional problems than adolescent sons (ill parents); adolescent daughters as having more emotional and behavioural problems than adolescent sons (ill parents; self-reports). Results indicated a higher prevalence of problems when the father was ill than when the mother was (spouses and self-reports). The treatment intensity affected adolescent daughter's functioning (spouses), whereas adolescent son's functioning was affected by relapsed disease (self-reports). Adolescents and mothers perceived comparable levels of problems, but fathers perceived problems in children to be less prevalent. Findings suggest that adolescent daughters and latency-aged sons are at risk for emotional problems following the diagnosis of cancer in a parent. The perception of child's functioning and potential influencing variables varied according to informant. Copyright © 2005 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Treatment of radiation-induced sarcoma (RIS) remains an unsolved problem. To provide more insight into the disease process, its characteristics, outcome, and potential outcome determinants were defined. From 1978 to 2003, 27 patients—20 females (74%) and 7 males (26%) with a median age 44 years (range, 1–73 years) at the time of diagnosis of the primary tumor—developed an RIS after a median interval of 8 years (range, 3–41 years). The histology of the RIS was 10 (37%) undifferentiated high-grade pleomorphic sarcomas, 7 (26%) angiosarcomas, 6 (22%) fibrosarcomas, 2 (7%) osteosarcomas, 1 (4%) pleomorphic rhabdomyosarcoma, and 1 (4%) pleomorphic leiomyosarcoma. Surgical resection was performed in 21 patients: 13 (62%) R0 (microscopically radical), 4 (19%) R1 (microscopically irradical), 2 (9.5%) R2 (macroscopically irradical), and 2 (9.5%) RX (unknown radicality). Six (22%) patients underwent no resection. The 5-year disease-free and overall survival rates were 27% and 30%, respectively. The local failure rate after R0 resection was 54%. The distant failure rate for the entire group was 41%. Patients with an R0 resection had a significantly better survival rate ( P < .05) than patients with an R1, R2, or no resection. RISs are aggressive malignancies with a high tendency for local recurrence and distant metastases. Previously applied treatment often hampers adequate resection. Therefore, radical surgical resection is the only chance to improve disease-free and overall survival, but it may also have a palliative role. Still, the overall prognosis remains poor. [ABSTRACT FROM AUTHOR]

Soft tissue sarcomas (STSs) are rare tumors, notorious for early hematogenous metastasizing. Metastatic disease is seldom amenable to curative treatment; therefore new treatment modalities are required. Treatment-related and tumor-related prognostic factors can be assessed to estimate the risk for subsequent metastases, as will be discussed. By this means, high-risk patients can be defined; they are the candidates for clinical trials mandatory for treatment development. The metastatic process as well as the reaction to chemotherapy depends on the biological make-up of the tumor. Current chemotherapy regimens do not improve the survival rates of patients with metastatic disease, due to resistance mechanisms of tumor cells. New drugs with direct access to the cell death machinery in tumor cells might contribute to more effective treatment of STS patients. [ABSTRACT FROM AUTHOR]