Histology, Fusion Status, and Outcome in Alveolar Rhabdomyosarcoma With Low-Risk Clinical Features: A Report From the Children's Oncology Group
Histology, Fusion Status, and Outcome in Alveolar Rhabdomyosarcoma With Low-Risk Clinical Features: A Report From the Children's Oncology Group
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DOI：
10.1002/pbc.25862
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发表时间：
2016-04-01
影响因子：
3.2
通讯作者：
Walterhouse, David O.
中科院分区：
文献类型：
--
作者：
Arnold, Michael A.;Anderson, James R.;Walterhouse, David O.
背景区分肺泡型横纹肌肉瘤（ARMS）与胚胎性横纹肌肉瘤（ERMS）对于预后和治疗具有重要意义。从 1995 年到 2013 年，这些实体的分类标准发生了显着变化。ARMS 与较差的结果相关；因此，具有肺泡组织学特征的患者通常被排除在低风险治疗之外。然而，患有ARMS和低风险分期和组（1期，I/II组/眼眶III；或2/3期，I/II组）的患者有资格参加1997年至1999年儿童肿瘤组（COG）低风险横纹肌肉瘤（RMS）研究D9602。这些患者的特征和结果以前没有报道过，并且这些病例的组织学也没有使用当前的研究进行审查。程序我们使用当前的组织学标准重新审查了 D9602 上被分类为 ARMS 的病例，确定了 PAX3/PAX7-FOXO1 融合状态，并将这些数据与这一独特患者组的结果进行比较。结果 38 名 ARMS 患者被纳入 D9602。根据当前的组织学标准，只有三分之一的载玻片可供重新审查的病例 (11/33) 仍被归类为 ARMS。大多数病例被重新分类为 ERMS（17/33，51.5%）。仍被分类为 ARMS 的病例通常为融合阳性（8/11，73%），因此当前的分类导致所有临床风险组的融合阳性 ARMS 比例相似。结合 COG 中危治疗方案 D9803 的数据，我们的数据证明了具有低风险临床特征的融合阴性 ARMS 的良好结局。结论具有低风险临床特征的融合阳性 RMS 患者应分类为中危并接受治疗，而融合阴性 ARMS 患者可适当接受降低强度治疗。
BackgroundDistinguishing alveolar rhabdomyosarcoma (ARMS) from embryonal rhabdomyosarcoma (ERMS) is of prognostic and therapeutic importance. Criteria for classifying these entities evolved significantly from 1995 to 2013. ARMS is associated with inferior outcome; therefore, patients with alveolar histology have generally been excluded from low-risk therapy. However, patients with ARMS and low-risk stage and group (Stage 1, Group I/II/orbit III; or Stage 2/3, Group I/II) were eligible for the Children's Oncology Group (COG) low-risk rhabdomyosarcoma (RMS) study D9602 from 1997 to 1999. The characteristics and outcomes of these patients have not been previously reported, and the histology of these cases has not been reviewed using current criteria.ProcedureWe re-reviewed cases that were classified as ARMS on D9602 using current histologic criteria, determined PAX3/PAX7-FOXO1 fusion status, and compared these data with outcome for this unique group of patients.ResultsThirty-eight patients with ARMS were enrolled onto D9602. Only one-third of cases with slides available for re-review (11/33) remained classified as ARMS by current histologic criteria. Most cases were reclassified as ERMS (17/33, 51.5%). Cases that remained classified as ARMS were typically fusion-positive (8/11, 73%), therefore current classification results in a similar rate of fusion-positive ARMS for all clinical risk groups. In conjunction with data from COG intermediate-risk treatment protocol D9803, our data demonstrate excellent outcomes for fusion-negative ARMS with otherwise low-risk clinical features.ConclusionsPatients with fusion-positive RMS with low-risk clinical features should be classified and treated as intermediate risk, while patients with fusion-negative ARMS could be appropriately treated with reduced intensity therapy.