Cancer Health Center

Mature and immature teratomas arise primarily in the sacrococcygeal region of neonates and young children and in the ovaries of pubescent girls. These tumors are also less commonly found in the testicular region of boys younger than 4 years, the mediastinum of adolescents, and other sites.[1,2,3]

Sacrococcygeal Tumors in Children

Standard treatment options

The sacrococcygeal region is the primary tumor site for the majority of benign and malignant germ cell tumors (GCTs) diagnosed in neonates, infants, and children younger than 4 years. These tumors occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported.[4] Sacrococcygeal tumors present in two clinical patterns related to the child's age, tumor location, and likelihood of tumor malignancy. Neonatal tumors present at birth protruding from the sacral site are usually mature or immature teratomas. Among infants and young children, the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum.[1] These pelvic tumors have a greater likelihood of being malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a malignant tumor incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis.[5] The pelvic site of the primary tumor has been reported to be an adverse prognostic factor, which may be due to either delayed diagnosis because it was unappreciated at birth or incomplete resection at the time of original surgery.[5,6,7,8]

After successful resection, neonates diagnosed with benign mature and immature teratomas are observed with close follow-up exams and serial serum alpha-fetoprotein (AFP) determinations for several years to ensure that the expected physiological normalization of AFP levels occurs and to facilitate early detection of tumor relapse.[9] A significant rate of recurrence among these benign tumors has been reported by several groups, ranging from 10% to 21%, with most relapses occurring within 3 years of resection.[4,9,10,11] While there is no standard follow-up schedule, follow-up should include scans and tumor markers for 3 years. Importantly, 43% to 50% of these recurrent tumors will be malignant and require adjuvant chemotherapy. Complete resection of the coccyx is vital to minimize the likelihood of recurrent tumor;[2] however, one study reported that 11 out of 12 patients with microscopic residual benign immature teratoma had no recurrence.[12] Long-term survivors should be followed for complications of extensive surgery, which include constipation, fecal and urinary incontinence, and psychologically unacceptable cosmetic scars.[13]

Nonsacrococcygeal Teratomas in Children

Standard treatment options

Mature teratoma and epidermoid cyst in the prepubertal testis are relatively common benign lesions and may be amenable to testis-sparing surgery.[14] Children with mature teratomas, including mature teratomas of the mediastinum, can be treated with surgery and observation with an excellent prognosis.[1,15] In a review of 153 children with nontesticular mature teratoma, the 6-year relapse-free survival for completely resected disease was 96% versus 55% for incomplete resection.[2] Head and neck GCTs in neonates should be cared for by a multidisciplinary team. While most are benign, they do present significant challenges to surgeons. Some tumors develop malignant elements, which may change the treatment strategy.[16]