Germ Cell Tumors In Children Research Articles

Malignant germ cell tumours of childhood: new associations of genomic imbalance

Malignant germ cell tumours (MGCTs) of childhood are a rare group of neoplasms that comprise many histological subtypes and arise at numerous different sites. Genomic imbalances have been described in these tumours but, largely because of the paucity of cases reported in the literature, it is unclear how they relate to abnormalities in adult MGCTs and impact on potential systems for classifying GCTs. We have used metaphase-based comparative genomic hybridisation to analyse the largest series of paediatric MGCTs reported to date, representing 34 primary tumours (22 yolk sac tumours (YSTs), 11 germinomatous tumours and one metastatic embryonal carcinoma) occurring in children from birth to age 16, including 17 ovarian MGCTs. The large dataset enabled us to undertake statistical analysis, with the aim of identifying associations worthy of further investigation between patterns of genomic imbalance and clinicopathological parameters. The YSTs showed an increased frequency of 1p- (P=0.003), 3p+ (P=0.02), 4q− (P=0.07) and 6q− (P=0.004) compared to germinomatous tumours. Gain of 12p, which is invariably seen in adult MGCTs, was present in 53% of primary MGCTs of children aged 5–16 and was also observed in four of 14 YSTs affecting children less than 5. Two of these cases (14% of MGCTs in children less than 5) showed gain of the 12p11 locus considered to be particularly relevant in adult MGCTs. Gain of 12p showed a significant association with gain of 12q. Conversely, MGCTs without 12p gain displayed a significantly increased frequency of loss on 16p (P=0.04), suggesting that this imbalance may contribute to tumour development in such cases. This data provides new insight into the biology of this under-investigated tumour group and will direct future studies on the significance of specific genetic abnormalities.

Primary Malignant Teratoma with a Primitive Neuroectodermal Tumor Component in Thyroid Gland: A Case Report

Teratomas comprise the most common extragonadal germ cell tumors in childhood. Most teratomas involving the thyroid are benign and occur in children. However, the adult cases reported are mostly malignant and commonly arise in the thyroid. We report a case of a 31-yr-old female with a huge neck mass. Pathologic examination revealed it to be malignant teratoma composed of primitive neuroepithelial tissue with primitive neural tubes and loose myxoid to fibrous immature mesenchymal stroma. The patient underwent extensive evaluation of the thyroid gland with computed tomography (CT) scan and positron emission tomography (PET) scan, which revealed no evidence of metastatic disease. She underwent total thyroidectomy with bilateral modified radical neck dissection, intensive chemotherapy and radiotherapy. At 22-months of follow-up, the patient has remained euthyroid and showed no evidence of recurrence. This is the first case, to our knowledge, of malignant thyroid teratoma with a exuberant primitive neuroectodermal tumor component in Korea.

Mediastinal Germ Cell Tumors: Clinical Aspects and Outcomes in 7 Children

Mediastinal germ cell tumors presenting during childhood are extremely rare. Publications on this entity are very scarce. This paper reports on the clinical presentations, method(s) of treatment, complications, results and outcomes in a series of children with mediastinal germ cell tumors. A retrospective chart review of 7 children treated between 1971 and 2001 for mediastinal germ cell tumor was carried out. Age at diagnosis and symptoms were recorded. Each patient's surgical treatment, peri- and postoperative complications, histological staging and final outcome were analysed. The median age of the 4 boys and 3 girls was 3 years (range 21 months-15 years). The most frequent symptoms were respiratory distress, persistent coughing, thoracic pain and anorexia/weight loss. Four patients had histologically benign tumors (mature teratoma). Their sole treatment consisted of complete surgical excision of the tumor and (part of) the thymus using either median sternotomy or left-sided thoracotomy. Recovery was uneventful. No recurrences have been observed. All four are alive with no evidence of disease, between 2.5 and 29 years after treatment. Malignant tumors were observed in three patients (1 yolk sac tumor, 1 choriocarcinoma and 1 malignant teratoma). Treatment consisted of either biopsy or debulking followed by chemotherapy (and radiotherapy in 1 case). Two of them died from uncontrollable metastatic disease. The patient with yolk sac tumor survived; he is now in remission, 4 years after diagnosis. Both this study and the literature review testify to the extreme rarity of mediastinal germ cell tumors in childhood. Children with this type of tumor usually are severely symptomatic. Histologically benign tumors carry an excellent prognosis provided surgical excision is complete. Histologically malignant tumors, on the other hand, have a worse prognosis. However, the use of platinum-based combination chemotherapy has considerably increased the survival rates.

Imbalances of chromosome arm 1p in pediatric and adult germ cell tumors are caused by true allelic loss: A combined comparative genomic hybridization and microsatellite analysis

Previous studies on childhood germ cell tumors (GCTs) report highly variable frequencies of losses at chromosome arm 1p. Since deletions at 1p portend a poor prognosis in other embryonal tumors, this study aims to clarify the question of the frequency of true allelic loss at 1p and whether it constitutes a prognostic parameter. We analyzed 13 GCTs from different gonadal and extragonadal sites of children (4 teratomas, 9 malignant GCTs) and 18 GCTs of adolescents and adults (3 teratomas; 15 malignant GCTs) using automated microsatellite analysis with 23 polymorphic markers and chromosomal "high resolution" comparative genomic hybridization (HR-CGH). With this combined approach, we detected loss of heterozygosity (LOH) at 1p in 8/9 childhood malignant GCTs with concordant data from HR-CGH and microsatellite analyses. In contrast, LOH at 1p was not detected in childhood teratomas (0/4) and constituted a rare event in GCTs of adolescence and adulthood (3/18). The commonly deleted region was located at distal 1p36-pter, with a proximal boundary between the markers D1S450 and D1S2870. These data unequivocally demonstrate that deletion at 1p is common in childhood GCTs and results in allelic loss. This observation argues for the presence of a classical tumor suppressor at distal 1p. Considering the high frequency of LOH at 1p and the overall favorable prognosis of childhood GCTs, a prognostic impact of LOH at 1p in childhood GCTs appears unlikely. However, since two postpubertal tumors with LOH at 1p progressed, a prognostic relevance in this age group seems possible, warranting a prospective evaluation.

No association between in utero exposure to female hormones and germ cell tumors in children

No association between in utero exposure to female hormones and germ cell tumors in children

Malignant germ cell tumors in childhood

Malignant germ cell tumors are a very heterogeneous group of neoplasms that have historically carried a poor prognosis. A review of the cooperative studies on malignant extracranial germ cell tumors in children was performed to update the progress in treatment and outcome for this rare group of tumors. The advent of platinum-based chemotherapy in the 1980s dramatically improved the prognosis for these tumors and allowed redefinition of risk groups. Chemotherapy regimens have been sequentially modified to reduce toxicity while maintaining survival. Surgical management has evolved from an aggressive en bloc resection at diagnosis to a more tailored approach, with resection and meticulous staging for low stage tumors at diagnosis and biopsy with neoadjuvant chemotherapy for advanced stage tumors. Improved surgical margins and prognosis are seen in post chemotherapy resections for high stage tumors. Large persisting masses after chemotherapy are often due to coexisting elements of benign teratoma, and aggressive attempts at resection are warranted in these patients with expectation of successful outcome.

In utero exposure to female hormones and germ cell tumors in children

Maternal exposure to exogenous female hormones during pregnancy has been implicated as a risk factor for malignant germ cell tumors (GCTs) in the offspring in some epidemiologic studies of testicular and ovarian carcinoma in adults. From 1996 to 2002, 278 children younger than 15 years of age with malignant GCTs and 423 healthy controls, frequency-matched for geographic location, age, and sex were enrolled in a case-control study to investigate whether in utero exposure to female hormones is associated with the risk of malignant GCT in children. Cases were recruited from 84 institutions in the U.S. and controls were enrolled through random digit dialing. Information was obtained through telephone interview with the biological mothers of the subjects and through blinded review of the mothers' medical records. Neither self-reported (odds ratio [OR] = 1.15; 95% confidence interval [CI], 0.63, 2.12) nor medical chart based (OR = 1.14; 95% CI, 0.75, 1.73) maternal exposure to exogenous female hormones was related to malignant GCT risk. Pregnancy-related conditions that may have altered serum levels of circulating female hormones were also unrelated to the risk of GCT in the offspring. This study failed to provide strong evidence to support the hypothesis that maternal exposure to exogenous female hormones during pregnancy increases the risk of GCT in the offspring.

Testicular germ cell tumors in children: Management and outcome in a series of 20 patients

Testicular germ cell tumors occurring during childhood are extremely rare. This study reports the clinical presentation, pathological diagnosis, treatment methods and outcome in a series of 20 boys, aged between 3.5 months and 16 years (median: 1.5 years; 19 were prepubertal), who were treated between 1963 and 2003. Histologically, mature teratoma was present in seven, immature teratoma in four and yolk sac tumor in nine. Nineteen patients were stage I; only one patient was stage IV. Of the 11 teratomas, 10 were treated by orchiectomy and one by testis-sparing tumor excision only. All 11 patients have survived and show no evidence of disease between 10 and 28 years after surgery. The nine patients with yolk sac tumor were managed by orchiectomy, in two plus retroperitoneal lymphadenectomy, and in eight plus chemotherapy. One patient is in remission for 10 months, seven are alive with no evidence of disease for 5.5–23 years, and one patient died from a T-cell acute lymphoblastic leukemia, 2 years after the end of treatment of the testicular tumor. A gradual switch towards less invasive treatment has been observed over the years. This study confirms the excellent cure rates obtained in children with testicular germ cell tumor, provided diagnosis is prompt and treatment accurate.

Ovarian germ cell tumors in children: A clinical study of 66 patients

Ovarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two-center series of girls with ovarian germ cell tumor. The records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44-year-span, were reviewed. Pain and an abdominal mass were the most frequent symptoms. The tumors were right-sided in 35, left-sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo-oophorectomy was the most frequently performed procedure (n = 46), and ovarian-sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra-operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment. With a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT).

Environmental exposure to residential pesticides, chemicals, dusts, fumes, and metals, and risk of childhood germ cell tumors

We examined relationships between exposure to residential pesticides, chemicals, dusts, fumes, and metals, and childhood germ cell tumors (GCTs) in the largest case-control study to date on the topic. We recruited 272 children under 15 years old who had GCT diagnosed between January 1, 1993 and December 31, 2001. Controls were selected by random-digit dialing and were frequency matched to cases by sex, age, and geographic area. Telephone interviews and self-administered questionnaires of parents were used to collect exposure information. We used unconditional logistic regression to estimate odds ratios (OR) and 95% confidence intervals (CI). Maternal and paternal exposure from 6 months before pregnancy to during breastfeeding and children's postnatal exposure to residential pesticides were generally unrelated to risk of childhood GCT. Elevated OR were observed for mothers’ exposure to hair dyes 1 month before pregnancy for boys (OR=1.7, 95% CI=1.0–2.8) and during breastfeeding for boys and girls combined, and for girls (OR=1.5, 95% CI=1.0–2.2 and OR=1.7, 95% CI=1.1–2.6, respectively). Among boys, OR for paternal exposure to insecticides more often than four times or exposure to indoor insecticides more often than three times were 0.5 (95% CI=0.2–0.9, trend test p = 0.05 ) and 0.3 (95% CI=0.2–0.8, trend test p = 0.02 ) during the index pregnancy. Overall this study produced no strong evidence linking parental and child residential exposure to pesticides, certain chemicals, dusts, fumes, and metals to increased risk of childhood GCT. Statistically significant associations need to be confirmed in future studies.

Does delay in the diagnosis of childhood testicular germ cell tumors (GCT) lead to more advanced disease stage and inferior survival?

8554 Background Stage I testicular GCT in childhood is readily curable with orchiectomy alone. Stage II-IV disease requires the addition of chemotherapy that may cause long-term toxicity, and survival is slightly less favorable. It is unknown if a prolonged time interval from initial symptoms to diagnosis (TSD) is associated with more advanced disease stage and a lower likelihood of cure. Methods Single institution retrospective chart review of 49 pediatric patients with testicular germ cell tumors treated between 12/68 and 12/03. The relationships between TSD (as a continuous variable), tumor size, disease stage, age group (< 10 years or ≥ 10 years), and survival were examined using the Spearman correlation coefficient, the Wilcoxon rank sum test, Fisher’s exact test, and Cox regression, as appropriate. Results Median age at diagnosis was 2.1 years (range, 0.3–19.9 years), and 22 patients were ≥10 years. TSD range was 1 to 447 days (median 52 days). Twenty-nine patients had stage I disease and 20 patients had stage II-IV. Longer TSD was correlated with larger tumor volume (p<0.001). TSD was longer in patients with stage II-IV disease than in patients with stage I disease (median 122 vs. 41 days, p=0.001). Patients ≥ 10 years of age had a longer TSD than younger patients (median 106 vs. 33 days, p=0.001) and were more likely to have high stage disease (p<0.001). TSD was significantly predictive of survival (p=0.03). Conclusions In pediatric testicular GCT patients, longer TSD is associated with larger tumor volume, higher disease stage, and inferior survival. Patients ≥ 10 years of age are at the greatest risk for long TSD. Interventions aimed at early diagnosis, particularly in adolescents, may improve the likelihood of survival. Early diagnosis may also reduce the proportion of patients requiring chemotherapy, thereby diminishing long-term morbidity in survivors. No significant financial relationships to disclose.

Risk of childhood germ cell tumors in association with parental smoking and drinking

The etiology of childhood germ cell tumors (GCT) is not well understood. The Children's Oncology Group conducted the largest case-control study of childhood GCT to investigate whether parental exposures to smoking and alcohol contributed to the disease. Cases included 274 children with GCT diagnosed between January 1, 1993 and December 31, 2001 who were age <15 years. Controls (n=421) were selected by random digit dialing and were frequency matched based on gender, age (+/-1 year), and geographic area. Exposure information was collected from subjects' parents using independent telephone interviews and self-administrated questionnaires. No association was found between parental smoking or drinking alcohol and risk of childhood GCT (for smoking: odds ratio [OR]=1.0, 95% confidence interval [95% CI], 0.8-1.3 and OR = 1.2, 95% CI, 0.9-1.5, for mothers and fathers, respectively; for drinking: OR=0.9, 95% CI, 0.7-1.2 and OR=1.0, 95% CI, 0.8-1.3, for mothers and fathers, respectively). No significant trend was observed for length of maternal exposure to passive smoking during the index pregnancy and GCT risk (for total subject: P=0.77; boys: P=0.52; girls: P=0.93). The authors found no evidence that childhood GCT was related to prenatal exposure to parental cigarette smoking, alcohol drinking, and maternal passive smoking.

Cortical laminar necrosis in childhood intracranial germ cell tumor survivors

We report two childhood germ cell tumor survivors treated for multifocal or large suprasellar tumors followed by the presentation of acute neurological deficits and extensive cortical laminar necrosis on magnetic resonance imaging (MRI), 7 and 34 months after cranial irradiation. This complication, though uncommon, represents another manifestation of delayed treatment-related brain injury that can be catastrophic and leads to significant morbidity.

Brain metastases of malignant germ cell tumors in children and adolescents.

Brain metastases of pediatric germ cell tumors are uncommon, and there is limited information regarding their incidence, clinical presentation, response to treatment, and influence on survival. The authors reviewed the experience with brain metastases from pediatric germ cell tumors at St. Jude Children's Research Hospital (Memphis, TN) over a 40-year period. Between March 1962 and February 2002, 16 of 206 patients with germ cell tumors (7.8%) had brain metastases at the time of initial presentation (n = 2), later in the course of the illness (n = 12), or at autopsy (n = 2). Twelve of 16 patients (75%) had symptoms referable to the brain (nausea/emesis, headaches, or seizures), and 14 (88%) had pulmonary metastases at the time brain metastases were identified. Patients with brain metastases were more likely to have an extragonadal primary tumor (P = 0.013), advanced-stage disease at initial presentation (P = 0.016), and choriocarcinoma within the primary tumor (P < 0.001). The incidence of brain metastases was significantly lower in the second 2 decades of the study period (5 of 135 patients [3.7%]) than in the first 2 decades (11 of 71 patients [15.5%]; P = 0.005). Two of the 16 patients in the current study are long-term survivors. Brain metastases are uncommon in childhood germ cell tumors, and their incidence appears to be decreasing. In the current study, most patients with such metastases were symptomatic and had pulmonary metastases at the time brain metastases were identified. Patients with the highest risk of developing brain metastases include those with extragonadal tumors, those with high disease stage at initial presentation, and those with choriocarcinoma as a component of the primary tumor. The probability of survival is poor, although a small proportion of patients may become long-term survivors.

Testicular Germ Cell Tumors in Childhood: Treatment Results of 52 Patients

We analysed the treatment results of 52 children with testicular germ cell tumors. Histopathological diagnoses were endodermal sinus tumor (63.4%), embryonal carcinoma (28.8%), teratocarcinoma (5.7%), and mixed tumors (2.1%). Radical inguinal orchiectomy was performed in 42 patients and retroperitoneal lymph node dissection in 10 (3/10 positive). Overall survival rates were: whole group: 71.2%; stage I: 89.7%; II: 68.5%; III: 31.2%; IV: 30% (p= .001). Five-year overall survival rates were 85.8% and 100% for stage I patients who received chemotherapy or not (p= .27); BEP regimen: 85.7%; classical VAC: 67.9%; vinblastine + bleomycin: 63.6%. Chemotherapy is not required in stage I. BEP regimen is effective in testicular germ cell tumors.

Malignant germ cell tumors in childhood: results of the first Italian cooperative study "TCG 91".

About 20% of patients with germ cell tumor (GCT) are still resistant to therapy. To investigate which features are present in resistant patients, a multicenter study on GCT in children was undertaken to correlate clinical and laboratory parameters with the outcome. Patients aged less than 16 years, with histologically proven extracranial GCT were included. Ninety-five patients (median age 33 months, 45 males) were eligible. The site of the primary tumor was gonadal in 59, extragonadal in 36. The stage was I in 39; II in 5; IIIa (microscopic residue) in 7; IIIb (macroscopic residue) in 16; IIIc (unresectable) in 13; IV in 15. The treatment was surgery alone in 31; surgery plus radiotherapy in 1; chemotherapy +/- surgery in 63. Post-chemotherapy resection in 19 (10 complete, 9 partial). The chemotherapy regimen was carboplatin 400 mg/m2/day on days 1, 2; etoposide 150 mg/m2/day on days 1, 2; ifosfamide 1,500 mg/m2/day on days 21, 22; dactinomycin 1.5 mg/m2/day on day 21; vincristine 1.5 mg/m2/day on day 21. Three patients died because of toxicity and two non-responders (to primary chemotherapy), died of progression; among the remaining 90 patients 20 relapsed, 9 are in second remission, 2 are alive with disease, and 9 died of disease progression (one from progression and intracranial hemorrhage). Overall survival was 82.7% and event-free survival: 71.5%. Survival according to: (a) site: testis: 100%; ovary: 88%; sacrococcyx: 69.6%; other sites: 33.3% (P < 0.001); (b) stage: I and II: 100%; IIIa: 83.3%; IIIb: 84.6%; IIIc: 60.6%; IV: 53.2% (P < 0.001); (c) AFP levels: normal: 85.5%; 42-9,470 ng/ml: 84.6%; >/=10,000 ng/ml: 58.7% (P = 0.02). All the pts who had complete resection of the primary tumor at diagnosis or at delayed surgery, remained in remission. Multivariate analysis showed that the primary site of tumor was the only independent prognostic factor for survival and EFS.

Follow-up in sacrococcygeal teratomas

Sacrococcygeal teratomas (SCT) are the most common type of childhood germ cell tumor in extragonadal locations, occurring at rate of approx. 1 in 38,000 live births. The objective of this study was to evaluate the results of surgical treatment in children with SCT with regard to urinary tract function. Between 1992 and 2000, 23 children underwent SCT resection, 20 with external and 3 with presacral/pelvic locations. Mortality rate was 13% (3 deaths). Histopathologic examination revealed mature SCT in 22 specimens and malignant foci in one. Children were seen 6 months to 6 years after operation. Urinalysis and culture, ultrasound examination, voiding cystouretherography, and IVP were performed. There were no pathologic findings in the results of these examinations. Bladder capacity, pressure, and urethral function were evaluated during urodynamic examination in 8 children showing normal values of measured parameters. The maximum intrabladder pressure was 11.28 cm H2O (±6.1), and detrussor pressure was 10.45 cm H2O (±4.24) during collecting and 44.29 cm H2O (±0.66) and 43,75 cm H2O (±11.29), respectively during voiding phase. The authors recommend urinary tract evaluation (including urodynamics) as a routine in postoperative assessment of children with SCT to exclude associated anomalies.—Jerzy K. Niedzielski Sacrococcygeal teratomas (SCT) are the most common type of childhood germ cell tumor in extragonadal locations, occurring at rate of approx. 1 in 38,000 live births. The objective of this study was to evaluate the results of surgical treatment in children with SCT with regard to urinary tract function. Between 1992 and 2000, 23 children underwent SCT resection, 20 with external and 3 with presacral/pelvic locations. Mortality rate was 13% (3 deaths). Histopathologic examination revealed mature SCT in 22 specimens and malignant foci in one. Children were seen 6 months to 6 years after operation. Urinalysis and culture, ultrasound examination, voiding cystouretherography, and IVP were performed. There were no pathologic findings in the results of these examinations. Bladder capacity, pressure, and urethral function were evaluated during urodynamic examination in 8 children showing normal values of measured parameters. The maximum intrabladder pressure was 11.28 cm H2O (±6.1), and detrussor pressure was 10.45 cm H2O (±4.24) during collecting and 44.29 cm H2O (±0.66) and 43,75 cm H2O (±11.29), respectively during voiding phase. The authors recommend urinary tract evaluation (including urodynamics) as a routine in postoperative assessment of children with SCT to exclude associated anomalies.—Jerzy K. Niedzielski

Management of germ cell tumours in childhood

Germ cell tumours are a group of rare benign and malignant neoplasms of unknown cause, and may be gonadal or extragonadal (intracranial or extracranial). Teratomas are the most common and are usually benign. Malignant germ cell tumours often secrete the tumour markers alpha-fetoprotein and human chorionic gonadotrophin, which are useful in diagnosis and monitoring. The treatment is surgical for teratoma and non-metastatic gonadal tumours. Chemotherapy, reserved in the UK for stage 2–4 extracranial malignant germ cell tumours, achieves a 5-year survival of around 90%. Intracranial tumours are typically pineal or suprasellar. Intracranial germinomas are cured in over 90% of cases with radiotherapy or combined chemoradiotherapy, whereas non-germinomatous tumours fare less well despite combination chemotherapy and radiotherapy.

Germ cell tumours of the central nervous system in children-controversies in radiotherapy.

Primary germ cell tumours (GCT) of the central nervous system (CNS) are rare tumours of children and adults. As few prospective, randomised trials have been published, management decisions are often based on retrospective and historical studies, histology, age, performance status, and extent of disease at diagnosis. This article reviews the current controversies in the radiotherapeutic management of primary GCT of the CNS. A computerised literature search was performed using the Medline database from 1975 to 2000, this being limited to publications written in the English language on CNS GCT in children up to the age of 16 years. Issues in radiation therapy currently undergoing review include the role of cranio-spinal irradiation (CSI), radiation therapy field sizes, the radiation therapy dose, the extent of surgery in combination with radiotherapy, and the role and timing of chemotherapy in combination with radiotherapy. The excellent outcome in children with pure germinoma of the CNS and the long-term morbidity associated with standard therapy justifies attempts to limit both the total radiation dose and field sizes, with or without the addition of chemotherapy. The poorer outcome associated with non-germinoma GCT justifies a more aggressive approach in children, combining chemotherapy, surgery and virgule, or radiation therapy, based on known prognostic factors.

Malignant germ cell tumors: Clinical characteristics, treatment, and outcome. A report from the study group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan

Purpose: This study aims to assess the prognostic factors and optimal treatments for malignant germ cell tumors (MGCT) in childhood. Methods: Among 117 MGCT, the clinical features were analyzed. Regarding the histology, there were 89 embryonal carcinomas, 13 dysgerminomas, 4 choriocarcinomas, and 11 others. The prognostic factors and treatments were assessed based on the 5-year survival rate. Results: (1) Stage: 100% for stage I (n = 54), 75.0% for stage II (n = 4), 67.3% for stage III (n = 14), and 54.8% for stage IV (n = 33); Unknown: n = 12. (2) Primary site: 93.4% for the testis (n = 52), 86.7% for the ovary (n = 31), 56.9% for the sacrococcygeal (n = 21), and 60.6% for others (n = 12); unknown: n = 1. (3) Surgical intervention for primary tumor: 100% for stage I with a complete resection (n = 53), 78.4% for stage III, IV with a complete resection (n = 26), and 33.3% for stage III, IV with an incomplete resection (n = 21). (4) Type of chemotherapy for the stage III and IV: 83.9% for the PVB (cisplatin, vinblastin, bleomycin; n = 13), 66.7% for the VAC (vincristine, actinomycin D, cyclophosphamide; n = 6), and 47.1% for other regimens (n = 25). Conclusions: An early stage, a diagnosis under 1 year of age and a primary site in the gonads were favorable prognosis factors, whereas histologic findings of choriocarcinoma and liver or lung metastasis were unfavorable. Radical complete resection alone is a sufficient treatment for localized MGCT. The PVB regimen is optimal chemotherapy for advanced MGCT; however, high-risk cases still may require more aggressive treatment. J Pediatr Surg 37:1703-1706. Copyright 2002, Elsevier Science (USA). All rights reserved.