GROWING TERATOMA SYNDROME IN CHILDREN AND ADOLESCENTS: PREVALENCE AND
SURGICAL OUTCOME
Abstract
Background and aims Patients affected by metastatic germ cell
tumors may occasionally experience enlargement of metastatic lesions
with concurrent normalization of tumor markers after chemotherapy. This
phenomenon is described as Growing Teratoma Syndrome (GTS). The aim of
the present study is to assess the prevalence of GTS in the pediatric
population and its implications in terms of surgical outcome.
Methods The clinical notes of patients diagnosed with stage III
and IV malignant germ cell tumors from January 2010 until December 2020
at our Institution were retrospectively reviewed. Patients with residual
disease after chemotherapy were studied; the occurrence of GTS,
treatment strategies, survival and outcome were analyzed.
Results Thirty-three patients with high stage malignant germ
cell tumors were diagnosed in our Institution in the analyzed period.
Eleven patients (34%) had radiologic evidence of enlargement of
metastases after chemotherapy; nine of them (28%) had normal markers
after chemotherapy and were classified as GTS patients. All nine
patients underwent resection of metastatic lymph nodes and six had
surgery on visceral metastases. In six patients, radical excision of all
metastatic sites was achieved; five patients are alive and in complete
remission while one died for peri-operative complications. Out of the
three patients who could not achieve radical excision of the metastases,
two died for progressive disease and one is alive with progressive
disease. Conclusions Patients affected by GTS have a risk of
progression of chemotherapy-resistant disease and death. Radical
surgical excision is essential to achieve disease control and long-term
survival.