Panteli C, Curry J, Kiely E, Pierro A, de Coppi P, Anderson J, Sebire N, Drake D
Department of Paediatric Surgery, Norfolk and Norwich University Hospital, Norwich, United Kingdom.
Eur J Pediatr Surg. 2009 Apr;19(2):96-100. doi: 10.1055/s-0029-1202372. Epub 2009 Apr 9.
BACKGROUND/PURPOSE: The aim of this study was to evaluate the clinical presentation, management and outcome in girls treated for ovarian germ cell tumours at a single unit.
The records of 40 girls (median age 10.5 years) with histologically proven ovarian germ cell tumours operated upon between 1990 and 2007 were reviewed.
Twenty-nine patients had mature teratomas (MT), 5 patients had immature teratomas (IT) and 6 had malignant tumours: 4 malignant mixed germ cell tumours (MMGCT) and 2 germinomas (G). The median age at presentation was 11 years for the MT and IT groups and 8.5 years for the malignant group. The commonest symptoms and signs at presentation were pain (n=28), a palpable mass (n=15) and abdominal distension (n=9). Precocious puberty was noted at presentation in three patients with malignant tumours. Tumour markers were elevated at presentation in all patients with MMGCT. At operation, 14 patients with MT had ovarian torsion. Open unilateral oophorectomy was the procedure most frequently performed (n=30). Surgery alone was the treatment for all patients with MT and IT. Of the patients with malignant tumours, 1 had stage I, 2 had stage II, 1 had stage III and 1 had stage IV disease. All 40 patients are now between 8 months and 17 years post treatment with no contralateral tumours identified. There was one recurrence in a patient with IT, with raised tumour markers.
Ovarian germ cell tumours are uncommon in childhood. Tumour markers are valuable both pre and post-operatively. Malignancy is a risk regardless of age. Surgery is curative in the majority of cases. The overall outlook is very good. However, a close collaboration between surgeons, histologists, radiologists and oncologists is essential to achieve good outcomes.
背景/目的：本研究旨在评估在单一机构接受治疗的卵巢生殖细胞肿瘤女孩的临床表现、治疗及预后情况。
回顾了1990年至2007年间40名经组织学证实患有卵巢生殖细胞肿瘤并接受手术治疗的女孩（中位年龄10.5岁）的病历。
29例患者为成熟畸胎瘤（MT），5例为未成熟畸胎瘤（IT），6例为恶性肿瘤：4例恶性混合性生殖细胞肿瘤（MMGCT）和2例生殖细胞瘤（G）。MT和IT组的中位发病年龄为11岁，恶性组为8.5岁。发病时最常见的症状和体征为疼痛（n = 28）、可触及肿块（n = 15）和腹胀（n = 9）。3例恶性肿瘤患者在发病时出现性早熟。所有MMGCT患者在发病时肿瘤标志物均升高。手术时，14例MT患者发生卵巢扭转。最常施行的手术是开放性单侧卵巢切除术（n = 30）。所有MT和IT患者仅接受手术治疗。恶性肿瘤患者中，1例为I期，2例为II期，1例为III期，1例为IV期疾病。所有40例患者目前均处于治疗后8个月至17年之间，未发现对侧肿瘤。1例IT患者出现复发，肿瘤标志物升高。
卵巢生殖细胞肿瘤在儿童期并不常见。肿瘤标志物在术前和术后均有价值。无论年龄大小，都存在恶性风险。大多数情况下手术可治愈。总体预后非常好。然而，外科医生、组织病理学家、放射科医生和肿瘤学家之间密切合作对于取得良好预后至关重要。
