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["成熟型骶尾部畸胎瘤的恶性复发。4例临床病理研究"]

[Malignant recurrence of "mature" sacrococcygeal teratoma. A clinicopathological study of 4 cases].

作者信息

Njim L, Moussa A, Ziadi J, Dhouibi A, Maazoon K, Zakhama A

机构信息

Service d'Anatomie et de Cytologie Pathologiques, Hôpital Fattouma Bourguiba 5000 Monastir, Tunisie.

出版信息

Rev Med Liege. 2011 Jan;66(1):25-9.

PMID:21374957
Abstract

Sacrococcygeal teratomas (SCT) are uncommon neonatal tumours which are usually benign. Our interest was aroused by encountering patients with reportedly benign SCT which later, after surgery, recurred as malignant tumours. We conducted a retrospective study of 17 patients treated for benign SCT during a period of 9 years. Of these patients, 4 developed malignant recurrence (3 females and 1 male) with a mean age of 19 months. The average time to recurrence was 17 months. Recurrence presented as a gluteal mass in one case, urinary and digestive compression signs in another case and as elevated alpha-fetoprotein levels in the two remaining cases. Histologically, one of the original tumors included tiny immature foci but none contained a malignant component after reading slides. Recurrences were as endodermal sinus tumour in all cases. After surgery and adjuvant chemotherapy, only one child died from complications related to chemotherapy. The other three were alive and well at mean follow-up of 5 years. These results emphasise the need for close clinical and biological follow-up for at least 2 years in all patients who had undergone excision of a neonatal SCT.

摘要

骶尾部畸胎瘤(SCT)是一种罕见的新生儿肿瘤,通常为良性。我们遇到了一些据报道为良性SCT的患者,这些患者在手术后后来复发为恶性肿瘤,这引起了我们的兴趣。我们对9年间接受良性SCT治疗的17例患者进行了回顾性研究。在这些患者中,4例出现恶性复发(3例女性和1例男性),平均年龄为19个月。复发的平均时间为17个月。1例复发表现为臀部肿块,另1例表现为泌尿和消化压迫症状,其余2例表现为甲胎蛋白水平升高。组织学上,最初的肿瘤中有1例包含微小的未成熟灶,但读片后均未发现恶性成分。所有病例的复发均为内胚窦瘤。手术和辅助化疗后,只有1名儿童死于与化疗相关的并发症。另外3例在平均5年的随访中存活且状况良好。这些结果强调,对于所有接受新生儿SCT切除的患者,至少需要进行2年的密切临床和生物学随访。

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