Scheer Monika, Dantonello Tobias, Brossart Peter, Dilloo Dagmar, Schweigerer Lothar, Feuchtgruber Simone, Sparber-Sauer Monika, Vokuhl Christian, Bielack Stefan S, Klingebiel Thomas, Koscielniak Ewa, von Kalle Thekla
Stuttgart Cancer Center, Peadiatric 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Kriegsbergstrasse 62, D-70174, Stuttgart, Germany.
Department of Oncology, University Hospital Bonn, Bonn, Germany.
Pediatr Radiol. 2018 May;48(5):648-657. doi: 10.1007/s00247-017-4066-8. Epub 2018 Jan 24.
Alveolar rhabdomyosarcoma commonly arises in the extremities and is characterized by aggressive biology and high frequency of metastases. Whole-body imaging is increasingly employed in pediatric oncology but not recommended as standard in the staging of soft-tissue sarcomas.
After observing patients with a large symptomatic alveolar rhabdomyosarcoma lesion and a smaller silent lesion in the more distal part of an extremity we sought to estimate the frequency of this constellation.
We retrospectively evaluated the data of prospectively registered paediatric patients (age <21 years) with alveolar rhabdomyosarcoma in the SoTiSaR (Soft Tissue Sarcoma Registry) of the Cooperative Weichteilsarkom Studiengruppe (CWS) 09/2011-04/2015 with regard to whole-body imaging.
Seventy-five patients were eligible. Images of 57 patients had been submitted for reference consultation, including 80 whole-body examinations in 36 patients. Among them were 5 patients (14%, 95% confidence interval 3-25%) who had been diagnosed because of a symptomatic lesion while an additional silent lesion in the distal part of an extremity had remained unnoticed and had only been detected by later whole-body imaging. It is noteworthy that in 42 (53%) of all 80 whole-body examinations, the hands and feet had been only partially covered or completely excluded.
In alveolar rhabdomyosarcoma silent lesions can be overlooked when the distal parts of the limbs are not thoroughly examined and not completely covered by imaging. Missing them influences treatment decisions and prognosis. Our results should be considered when evaluating the potential role of whole-body imaging in rhabdomyosarcoma.
肺泡状横纹肌肉瘤常见于四肢，具有侵袭性生物学行为和高转移率的特点。全身成像在儿科肿瘤学中的应用日益广泛，但在软组织肉瘤分期中不被推荐作为标准方法。
在观察到患有较大症状性肺泡状横纹肌肉瘤病灶且在肢体更远端存在较小无症状病灶的患者后，我们试图评估这种情况的发生频率。
我们回顾性评估了合作软组织肉瘤研究组（CWS）09/2011 - 04/2015的软组织肉瘤登记处（SoTiSaR）中前瞻性登记的年龄小于21岁的肺泡状横纹肌肉瘤儿科患者关于全身成像的数据。
七十五名患者符合条件。57名患者的图像已提交以供参考咨询，其中36名患者进行了80次全身检查。其中有5名患者（14%，95%置信区间3 - 25%）因症状性病灶被诊断，而肢体远端的另一个无症状病灶未被注意到，仅在后来的全身成像中被发现。值得注意的是，在所有80次全身检查中的42次（53%）中，手和脚仅被部分覆盖或完全未被检查。
在肺泡状横纹肌肉瘤中，如果肢体远端未被彻底检查且成像未完全覆盖，无症状病灶可能会被忽略。遗漏这些病灶会影响治疗决策和预后。在评估全身成像在横纹肌肉瘤中的潜在作用时应考虑我们的结果。
