Malignant sacrococcygeal germ cell tumors in children in Taiwan

Abstract

Although the incidence of malignant sacrococcygeal germ cell tumors (MSGCTs) is high in the East Asian countries, information about MSGCTs from this region is limited. This report aimed to analyze the data of children with MSGCTs in a single medical center in Taiwan. Patients aged 18 years or younger with primary MSGCTs or malignant recurrence of a sacrococcygeal teratoma who underwent surgery during the neonatal period between January 1999 and December 2016 were identified from the Linkou Chang Gung Cancer Center registry. The clinical features, laboratory data, and treatment outcomes were reviewed. Fifteen children (1 man and 15 women) with MSGCTs were identified. Sacrococcygeal tumors were present at birth in 7 patients. All patients presented with a bulging mass at the buttock region and they had normal alpha-fetoprotein levels at the time of diagnosis. They underwent primary excision of the tumor. Immature teratoma was histologically diagnosed in 5 neonates, and mature teratoma in 2. Only 1 patient with grade 3 immature teratoma received adjuvant chemotherapy. Two patients with mature teratoma developed malignant recurrence 1.6 and 2.1 years later, respectively. Eight patients were diagnosed with MSGCTs after the neonatal period. The common presenting symptoms included buttock asymmetry (37.5%), abdominal distension (25%), and constipation (12.5%). Seven patients had elevated alpha-fetoprotein levels for their age. They were administered neoadjuvant chemotherapy followed by tumor excision if a residual tumor was present. The histology of the excised tumor included mature teratoma (66.7%) and necrosis (33.3%). One patient with a normal alpha-fetoprotein level underwent primary tumor excision followed by adjuvant chemotherapy. Grade 2 immature teratoma with embryonal carcinoma was diagnosed histologically. Among the 15 patients with MSGCTs, 3 had a recurrence (at age of 2.1, 0.5, and 2.4 years, respectively) and 1 died (at age of 6.1 years) of disease progression. The 5-year overall and event-free survival rates were 90% and 80%, respectively. Children with MSGCTs had good overall prognoses in this case series. For those with sacrococcygeal mature teratoma or low-grade immature teratoma in the neonatal period, we recommend close follow-up for at least 3 years after surgery to detect malignant recurrence.

Mixed germ cell tumor of sacrococcygeal region; A case report with literature review

“…Sacrococcygeal germ-cell tumors are often identified antenatally on routine imaging and, if large enough, can cause hydrops fetalis or labor obstruction [ 12 ]. In neonates, it generally manifests as an external palpable lump; however, it can also manifest as discomfort while sitting, buttock asymmetry, or bladder, bowel, and lower limb dysfunction [ 13 ]. Any midline tumor should be evaluated for a germ-cell tumor diagnosis [ 12 ].…”

Giant malignant sacrococcygeal germ cell tumor in a newborn: A rare case report

“…Malignant germ cell tumors account for approximately 3%-4% of all neoplasms in children under 15 years [1] , [2] . They are a histologically heterogeneous group of tumors with a common origin in primordial germ cells, the embronic precursors of adult gametes.…”

“…Malignant germ cell tumors occur predominantly in the gonads (ovaries or testes), but can also develop in extragonadal sites (sacrococcyx, mediastinum, intracranial region, retroperitoneum) due to the abnormal or arrested migration of germ cells precursors during embryogenesis [2] , [3] , [4] . In infants and young children, more than half of these neoplasms are extragonadal and sacrococcygeal teratoma (SCT) is the most common germ cell tumor found in newborns, occurring in approximately 1 in 35,000-40,000 live births with a female prevalence in a 3:1 to 4:1 ratio [1] , [2] , [4] , [5] . Immature and mature SCTs are usually benign and, in rare cases, they may be cancerous for the presence of malignant components, such as yolk sac tumor and less frequently embryonal carcinoma or choriocarcinoma [1] , [2] , [6] , [7] .…”

“…In infants and young children, more than half of these neoplasms are extragonadal and sacrococcygeal teratoma (SCT) is the most common germ cell tumor found in newborns, occurring in approximately 1 in 35,000-40,000 live births with a female prevalence in a 3:1 to 4:1 ratio [1] , [2] , [4] , [5] . Immature and mature SCTs are usually benign and, in rare cases, they may be cancerous for the presence of malignant components, such as yolk sac tumor and less frequently embryonal carcinoma or choriocarcinoma [1] , [2] , [6] , [7] . Malignant SCTs represent about 12%-14% of sacrococcygeal germ cell tumors; they are not common in infants under 2 months and approximately 70% of SCTs are cancerous at 9 months of age [1] , [2] , [4] , [5] , [6] , [7] .…”

“…Immature and mature SCTs are usually benign and, in rare cases, they may be cancerous for the presence of malignant components, such as yolk sac tumor and less frequently embryonal carcinoma or choriocarcinoma [1] , [2] , [6] , [7] . Malignant SCTs represent about 12%-14% of sacrococcygeal germ cell tumors; they are not common in infants under 2 months and approximately 70% of SCTs are cancerous at 9 months of age [1] , [2] , [4] , [5] , [6] , [7] . In this paper, we report a rare case of a male newborn with a mixed malignant sacrococcygeal germ cell tumor containing immature teratoma and small foci of the yolk sac.…”