Sacrococcygeal site

Sacrococcygeal GCTs are common extragonadal tumors that occur in very young children, predominantly young females.[10] The tumors are usually diagnosed at birth, when large external lesions predominate (usually mature or immature teratomas), or later in the first years of life, when presacral lesions with higher malignancy rates predominate.[10]

Malignant sacrococcygeal tumors are usually very advanced at diagnosis. Two-thirds of patients have locoregional disease, and metastases are present in 50% of patients.[8,11,12] Because of their advanced stage at presentation, the management of sacrococcygeal tumors requires a multimodal approach with platinum-based chemotherapy followed by delayed tumor resection.

Platinum-based therapies, with either cisplatin or carboplatin, are the cornerstone of treatment. The PEb regimen or the JEb regimen produces EFS rates of 85%.[8,9] Surgery may be facilitated by preoperative chemotherapy. In any patient with a sacrococcygeal GCT, resection of the coccyx is mandatory.[8,9]

Completeness of surgical resection is an important prognostic factor, as shown in the following circumstances:[8,9,13]