Germ-cell tumors in childhood and adolescence

Abstract

In mature and immature teratoma the treatment is surgical. The risk of recurrence can be estimated from the parameters primary site (with the coccygeal tumors being most at risk), histological grade of immaturity and completeness of the primary resection including the adjacent organ of origin (coccyx, ovary, testis etc.). In case of a microscopically complete tumor resection there is no role for adjuvant chemo- or radiotherapy irrespective of the histological grade of immaturity. Malignant germ-cell tumors (GCT) account for 2.9% of all malignant tumors of children younger than 15 years of age. More than half of the tumors occur at extragonadal sites such as the ovaries (26%), the coccygeal region (24%), the testes (18%) and the brain (18%) represent then primary sites. In patients with extensive tumor growth, metastatic disease or secreting intracranial tumors a delayed tumor resection after preoperative chemotherapy is preferable. In these patients malignant non-seminomatous GCT may be diagnosed clinically due to the increased serum or cerebrospinal fluid levels of the tumor markers AFP and/or beta-HCG. Current risk adapted treatment protocols containing cisplatinum allow long-term remissions in about 80% including patients with bulky or metastatic tumors. In the cisplatinum era the prognostic factors like histology, primary site of the tumor and initial tumor stage have partly lost their former impressive significance in infants and children. On the other hand the completeness of the primary tumor resection according to oncological standards has been established as the most powerful prognostic parameter superior to tumor marker levels or primary site of the tumor.

Pre‐radiation chemotherapy with response‐based radiation therapy in children with central nervous system germ cell tumors: A report from the Children's Oncology Group

“…Buckner et al noted excellent response and survival in three patients with CSF HCG 79.6-147 mIU/ml [16], whereas Balmaceda et al noted a trend toward increased PD (P 0.06) in 29 patients [23]. It remains unclear whether patients¼with pure germinoma and HCG 50-2,000 mIU/ml (e.g., Patient 24) should be classified as high-risk patients, but our intent was to follow the guidelines of previous European series [5,14,25,33,[35][36][37]. If Patient 24 were to be excluded, the PFS for the high-risk group would be comparable at 76% (10 of 13 patients).…”

“…Studies in new and relapsed patients found responses and/or improved survival after platinum-containing regimens [17][18][19][20][21][22][23][24][25], with one study suggesting synergy with etoposide [26]. Alkylating agents, such as cyclophosphamide (CPM) also were added with apparent efficacy in some trials [12,14,23].…”

Salvage high-dose chemotherapy for children with extragonadal germ-cell tumours

“…Children with relapsing/progressing extragonadal GCT are characterised by a poor prognosis, with a salvage rate in the first relapse in the range of 40 -45% (Marina et al, 1992;Baranzelli et al, 1999;Sawamura et al, 1999;Gobel et al, 2000;Mann et al, 2000;Schmugge et al, 2000). According to extragonadal primary location, histology and the site of recurrence/progression, the salvage strategies are different.…”

Role of regeneration in tissue repairing and therapies

“…Besides, extra gonadial [29] and sacrococcygeal heart are rare forms of teratomas [30]. More exceptionally, malignant cervical teratoma [31], germ cell tumors [32] and malignant retrobulbar and intra cranial teratoma are more common in child and adolescence stages [33]. Similarly, mediastinal teratomas often cause compression of the lungs, chock airways and cause severe chest pain and/ or respiratory tract.…”