Pediatric Oncology Unit, Fondazione IRCCS National Cancer Institute, Milano, Italy.
Pediatr Blood Cancer. 2010 Apr;54(4):532-7. doi: 10.1002/pbc.22397.
Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse.
The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC. Clinical files and pathology specimens were reviewed.
The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1). Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1). Three patients were in stage I, four in stage II, three in stage III, and four in stage IV. All but one patient underwent surgery and only females showed carcinoma components. Nine patients relapsed or progressed and eight died. Six patients are alive and disease-free. Two patients underwent complete resection and four were treated based on transformed histologies. Relapse-free and overall survival rates were 35.7% and 42.8%, respectively (median follow-up, 31 months).
Prognosis for germ cell tumors (GCTs) containing MSC is worse than that for GCTs. The pediatric disease appears to be more heterogeneous in tumor site distribution and MSC histology than in adults. Our series suggests no effects of age, histology, or gender on outcome. Surgery has an essential role in localized disease, with complete resection highly desirable. Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive. Malignant GCT warrants GCT-directed chemotherapy.
含有恶性体细胞成分的畸胎瘤（TMSC）较为罕见，但已有成人病例的相关报道，而儿科病例的相关信息则较为匮乏。
意大利儿科血液学和肿瘤学会（AIEOP）共鉴定出 14 例 TMSC 病例。对这些患者的临床病历和病理标本进行了回顾性分析。
本研究系列（9 例女性，5 例男性）的发病部位如下：睾丸（2 例）、骶尾部（3 例）、卵巢（3 例）、腹膜后（3 例）、纵隔（2 例）和足部软组织（1 例）。体细胞成分的分布为：癌（4 例）、胰腺神经内分泌肿瘤（1 例）、神经母细胞瘤（3 例）、横纹肌肉瘤（3 例）、横纹肌肉瘤合并脂肪肉瘤、软骨肉瘤、神经源性肉瘤（1 例）、软骨肉瘤合并神经外胚层肉瘤（1 例）、恶性外周神经鞘肿瘤（1 例）。3 例患者处于 I 期，4 例处于 II 期，3 例处于 III 期，4 例处于 IV 期。除 1 例患者外，其余所有患者均接受了手术治疗，仅女性患者出现了癌性成分。9 例患者复发或进展，8 例患者死亡。6 例患者存活且无疾病进展，2 例患者接受了完全切除术，4 例患者接受了基于转化组织学的治疗。无复发生存率和总生存率分别为 35.7%和 42.8%（中位随访时间 31 个月）。
含有 MSC 的生殖细胞瘤（GCT）的预后较单纯 GCT 更差。与成人相比，儿科 TMSC 疾病在肿瘤部位分布和 MSC 组织学上似乎更具异质性。本研究系列表明，年龄、组织学和性别对预后均无影响。手术在局限性疾病中具有重要作用，强烈建议行完全切除术。针对组织学优化的化疗应包括针对体细胞恶性肿瘤的药物，如果其对化疗敏感的话。恶性 GCT 需行 GCT 导向的化疗。
