Search

Background: Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery., Methods: Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (N0) were prospectively enrolled in the low-risk (n = 70, subgroup A; age < 10 years and tumour size ≤ 5 cm) or standard-risk group (n = 108, subgroup B; age ≥ 10 years or tumour size > 5 cm. Subgroup A received 8 courses of vincristine and dactinomycin (VA) for 22 weeks; subgroup B received 4 courses of VA with ifosfamide (IVA) and 5 courses of VA for 25 weeks., Results: The 5-year event-free survival (EFS) and overall survival (OS) were 90.8% (95% confidence interval [CI]: 85.0-94.4) and 95.7% (95% CI: 90.5-98.1), respectively (n = 178). The EFS and OS were 95.5% (95% CI: 86.8-98.5) and 100% (subgroupA), and 87.8% (95% CI: 79.3-93.0) and 93.0% (95% CI: 84.8-96.8)(subgroup B), respectively. Bearman stage 2 veno-occlusive disease (VOD) occurred in 4 very young patients., Conclusion: VA treatment for 8 courses was effective and well tolerated by the subgroup of patients with low-risk ERMS (group A). Four courses of IVA and 5 courses of VA instead of 9 courses of IVA also has very good results. Careful monitoring for liver toxicity is important in very young patients. European union drug regulating authorities clinical trials EUDRACT No. 2005-000217-35., Competing Interests: Conflict of interest statement The authors do not have competing interests., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Open biopsy is recommended for a soft-tissue sarcoma (s-t-S) diagnosis. Core needle biopsy (CNB) was recently associated with minimal morbidity, cost and time-consumption, but also potential inaccuracy. Its diagnostic utility was investigated retrospectively in 110 patients with soft-tissue masses (s-t-M) undergoing CNB between September 1994 and September 2000. Sensitivity (Se), specificity (Sp), positive (PPV) and negative (NPV) predictive values were determined for malignancy (benign/malign), soft-tissue tumour (yes/no), and sarcoma diagnosis (yes/no), comparing CNB and the best standard test available; concordance was evaluated. 103/110 CNB were suitable for analysis. Final diagnosis was 23 benign tumours (19%), 65 s-t-S (59%), 9 lymphomas (8%), 6 fibromatoses (desmoid) (5%) and 7 carcinomas (6%). CNB Sp and PPV were 100%, Se was 95, 99 and 92%, and NPV 85, 95 and 88% for diagnosing malignancy, soft-tissue tumour and sarcoma. CNB Se and NPV were 100% for malignancy, connective tumour and sarcoma in lymphomas, high-grade sarcomas and desmoid tumours. In low grade sarcomas, Se was 94 and 85%, and NPV 84 and 77% for malignancy and sarcoma. Histological grade on CNB seems uneasy, except for grade-III tumours. CNB is accurate, not misleading for s-t-M diagnosis, avoids open biopsy complications, and allows one-surgery or neo-adjuvant chemotherapy planning when combined with appropriate imaging.

The International Neuroblastoma Staging System (INSS) criteria for diagnosis requires an unequivocal pathological diagnosis and favours the identification of prognostic markers in the samples. Surgical biopsies of the primary tumour and bone marrow (BM) sampling in metastatic disease constitute the major sources of tumour material for the laboratory. We analysed the possibility of percutaneous fine needle aspiration cytology (FNAC) constituting an alternative procedure to the conventional technique of sampling of the primary tumour in children with advanced neuroblastoma. From July 1987 through July 1998, 64 consecutive children suspected of having advanced neuroblastoma and referred to our institution underwent percutaneous FNAC of deeply located tumours. FNAC was performed using 22-gauge needles under ultrasound guidance, before any chemotherapy and within the first days following admission. No complication occurred after FNAC. The median number of the extracted tumour cells was 2.3x10(6) (range: 0-40.6x10(6)). Cytology analysis was possible in 59/64 cases (92%) and immunocytochemistry in 56/64 (88%) allowing confirmation of the diagnosis. N-Myc analysis was available in 46/64 (72%). In addition, the presence of a partial deletion of chromosome 1p (del 1p) was assessed, since 1992, in 24/47 cases (51%), where enough cells were available. FNAC of deeply located advanced neuroblastoma is safe and information is available in a few hours after admission. The provided material is reliable for confirmation of diagnosis and analysis of biological prognostic markers in the majority of cases. More invasive tumour sampling procedures are required only in selected cases.