Sacrococcygeal Germ Cell Tumors Research Articles

Successful Treatment Outcome of a Pediatric Malignant Germ Cell Tumor With an Extensive Cavo-atrial Tumor Thrombus Presenting as an Oncological Emergency

Background: Pediatric germ cell tumor (GCT) with extensive cavo-atrial tumor thrombus presenting as an oncologic emergency is extremely rare. Observation: A 13-month-old female child with sacrococcygeal GCT having inferior vena cava (IVC) and right atrial thrombus presenting with anasarca and respiratory distress is reported. Because of worsening symptoms post initiation of chemotherapy, tumor thrombus debulking from the right atrium and distal end of IVC was performed on day 9 on an emergency basis. Her symptoms improved, and after planned chemotherapy, tumor excision with coccygectomy could be done. The IVC thrombus remained inoperable due to sclerosis. She remains recurrent-free now 12 months post therapy.

Critical elements of pediatrics sacrococcygeal germ cell tumor surgery

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.

Clinicopathological Evaluation of Childhood Sacrococcygeal Germ Cell Tumors: A Single-Center Experience

ObjectiveWe aimed to evaluate the cases of sacrococcygeal germ cell tumors diagnosed in our hospital between 2006 and June 2021.Materials and MethodsWe evaluated 38 sacrococcygeal germ cell tumors cases in our series in terms of age, sex, clinical complaints, localization, macroscopy, tumor size, histopathological diagnosis, surgical, postoperative complications, treatment, recurrence, and prognosis.ResultsThe cases ranged from 1 day to 16 years of age; 14 cases were diagnosed with routine ultrasonographic examination during prenatal period while the rest of the cases most frequently presented with complaints of constipation. In terms of localization, 6 cases were type 1, 11 cases were type 2, 6 cases were type 3, and 15 cases were type 4. In the pathological evaluation, 25 cases were mature teratoma, 8 cases were immature teratoma, and 5 cases were pure yolk-sac tumor. In terms of complications, temporary colostomy was performed as a result of rupture during birth in 2 cases, disseminated intravascular coagulation at birth in 1 case, and colon injury in 2 cases. There was a recurrence in 2 of our cases. Thirty-seven of our cases were alive and 1 died. Alpha-fetoprotein level was high in 28 of our cases.ConclusionIn our series, type 4 cases were observed more frequently, contrary to the literature. We recommend to use a routine ultrasonography to patients who come to the clinic with complaints of constipation and inability to urinate and if a mass is detected, asking for alpha-fetoprotein for further follow-up. Sacrococcygeal germ cell tumors are ultimately a disease that can be successfully treated with multidisciplinary approach, accurate diagnosis in the antenatal and postnatal period, appropriate surgical intervention, and regular follow-up.

Pediatric Malignant Sacrococcygeal Germ Cell Tumor with Intraspinal Extension.

Aims:The study aimed to evaluate the presentation, management, and outcome of patients of sacrococcygeal malignant germ cell tumors (SC-MGCTs) with intraspinal extension.Materials and Methods:Case records of all cases of SC-MGCT were reviewed to identify cases with intraspinal extension. They were evaluated in terms of their presentation, response to therapy, extent of surgical resection, recovery of neurological symptoms if any, and outcome.Results:Of the three cases of SC-MGT, two had intraspinal extension. One had Altman Type 3 disease and another had Altman Type 4 disease; both had Stage 4 disease. The intraspinal extension in both patients was detected on contrast computed tomography scan and magnetic resonance imaging. One patient had bladder and bowel incontinence. All the tumors responded to preoperative chemotherapy. Gross complete local resection could be achieved in all patients. Neurological recovery was complete. All were alive with no recurrence on follow-up.Conclusions:Malignant sacrococcygeal GCTs with intraspinal extension are rare and can be managed with neoadjuvant chemotherapy which obviates the need for extensive exploration for excision and has got good neurological and oncological prognosis.

An Unusual Presentation of Sacrococcygeal Germ Cell Tumor With Autoimmune Hemolytic Anemia as a Paraneoplastic Syndrome

The tumors that induce autoimmune hemolytic anemia (AIHA) are usually hematological neoplasms such as malignant lymphoma and chronic lymphocytic leukemia. AIHA associated with germ cell tumors is very rare with literature limited to case reports with most of them reported with ovarian dermoid, which abated with the removal of the tumor. We report an unusual presentation of AIHA with malignant sacrococcygeal germ cell tumor in a 3-year-old child, which rapidly disappeared after the excision of a sacrococcygeal teratoma.

Malignant sacrococcygeal germ cell tumors in children in Taiwan: A retrospective single-center case series.

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.

Malignant sacrococcygeal germ cell tumors in childhood: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience.

To evaluate clinical features at diagnosis, prognostic factors, and outcomes of malignant sacrococcygeal germ cell tumors (SC-GCTs) in patients enrolled in the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) TCG 2004 protocol. A prospective analysis was conducted on all consecutive patients diagnosed with malignant SC-GCTs between January 2004 and May 2017. Patients with stage I underwent surgery and subsequent surveillance, the others received pediatric cisplatinum-etoposide-bleomycin (pPEB) regimen and eventual deferred surgery. Of 45 patients, 35 were females. Age at diagnosis ranged from 1day to 3.6years (median 1.6years); 26 were stage IV. Of 38 patients who underwent surgery, pathology revealed yolk sac tumor (YST) in 27 and teratoma+YST/embryonal carcinoma in 11, while seven patients were diagnosed based on imaging and elevated levels of alpha-fetoprotein (AFP). Of six patients approached with surgery, only one relapsed and was rescued with first-line chemotherapy. Overall, 38 out of 45 achieved complete remission, three a partial remission, and four were resistant. Ten out of 41 patients who entered remission later relapsed and nine were rescued with a second-line treatment. We observed a global failure percentage of 31% and a 5-year overall survival (OS) and event-free survival (EFS) of 95% and 69%, respectively. Chemotherapyis generally effective in malignant SC-GCTs, even though almost one-third of our patients experienced events salvageable with second-line treatment. Most of the relapses occurred within 1year from diagnosis. A close follow up with serial AFP level monitoring should be done for at least 2years after diagnosis.

Malignant sacrococcygeal germ cell tumors in children: a 30-year experience from a single institution.

Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors. Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded. A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥ 10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis. Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.

Is surgical resection and observation sufficient for stage I and II sacrococcygeal germ cell tumors? A case series and review.

Sacrococcygeal teratoma (SCT) is the most common germ cell tumor (GCT) of infancy. Up to 35% of infants may have malignant elements. The standard of care for SCT with malignant elements (SCT-ME) has been surgery and chemotherapy. However, cases where low-stage SCT-ME have been successfully observed following resection have been reported. To better understand the outcomes of low-stage SCT-ME that do not receive chemotherapy, we reviewed SCT pathology reports from five children's hospitals from 1999 to 2009. Information regarding staging workup, tumor markers, treatment, and outcome was collected for patients with stage I or II SCT-ME. An English language literature review was also performed. Seventy-four SCT were identified: 51 stage I and 23 stage II; 13 (18%) were SCT-ME: 5 stage I and 8 stage II; four stage I and four stage II tumors were not treated with chemotherapy. No stage I tumors recurred; all of the stage II tumors recurred and were successfully salvaged, two had no ME at recurrence. We identified another 10 stage I SCT-ME in the literature managed with active surveillance-two recurred and were successfully treated with surgery and chemotherapy. Overall, of the 14 cases of stage I SCT-ME, 12 survived with no recurrence and the two who did recur were successfully treated with platinum-based chemotherapy (EFS = 86%, overall survival [OS] = 100%); this suggests that patients with stage I SCT-ME could be observed after surgery and treated only upon recurrence. Stage II SCT-ME require further study in a clinical trial setting.

Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood

Purpose. The aim of this study was to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period. Materials and Methods. A retrospective review was conducted of all pediatric patients treated for SC-GCTs at our hospital from 1998 to 2012. Results. Fifty-seven patients were treated for SC-GCTs with the most common in Altman's classification type I. Age at surgery ranged from one day to 5.6 years. Tumor resection and coccygectomy were primarily performed in about 84% of the cases. Pathology revealed mature, immature, malignant sacrococcygeal teratomas (SCTs), and endodermal sinus tumors (ESTs) in 41 (72%), 4 (77%), 6 (10.5%), and 6 (10.5%), respectively. Recurrence of discase occurred in 3 of 41 patients with mature teratomas (7.3%); 2 recurrences with mature teratomas and one recurrence with EST. Five of 6 malignant SCTs and 3 of 6 ESTs responded well to the treatment. Alpha-fetoprotein (AFP) level was elevated in both malignant teratomas and ESTs. No immediate patient death was noted in any of the 57 cases, but 4 patients with malignant tumors and distant metastasis succumbed at home within 2 years of the initial treatment. Conclusion. Benign SCTs have a significant recurrence rate of approximately 7%. Close follow-up with serial AFP level monitoring should be done for 5 years after initial tumor resection and coccygectomy. The survival rate for malignant SC-GCTs with distant metastasis was unfavorable in the present study.

Sacrococcygeal yolk sac tumor developing after teratoma: A clinicopathological study of pediatric sacrococcygeal germ cell tumors and a proposal of the pathogenesis of sacrococcygeal yolk sac tumors

PurposeWe evaluated the clinicopathological characteristics of pediatric sacrococcygeal germ cell tumors (SGCTs) and yolk sac tumors (YSTs) developing after sacrococcygeal teratoma (SCT) resection, and discussed the pathogenesis of sacrococcygeal YST. MethodsWe retrospectively analyzed pediatric SGCT patients attending 10 Japanese institutions. ResultsA total of 289 patients were eligible, of which 74.6% were girls. The mean age at surgery was 7.1months. There were 194 mature and 47 immature teratomas, and 48 YSTs. YST developed after SCT resection in 13 patients (5.4% of SCTs), and was detected between 5 and 30months after resection. At initial surgery, 9 of these 13 patients were neonates, 12 underwent gross complete resection with coccygectomy, and 9 had histologically mature teratoma without microscopic YST foci. Postoperative serum alpha-fetoprotein (AFP) levels were regularly examined in 11 patients. Intervals of AFP measurement≤4months helped to detect subclinical localized YSTs for resection. ConclusionsThe characteristics of SGCT in Japanese children were similar with those reported in Europe or the United States. YST developed after SCT resection not only in patients with previously reported risk factors. We recommend that patients undergo serum AFP monitoring every 3months for≥3years after SCT resection.

Malignant Sacrococcygeal Germ Cell Tumors in Children: A 30-year Experience from a Single Institution

Our aim was to analyze treatment results and survival characteristics of our patients with malignant sacrococcygeal germ cell tumors. Patient files of children with malignant sacrococcygeal germ cell tumors, treated at our institution between 1979 and 2009, were searched. Patient characteristics, histopathological subtypes, extension of disease, alpha-fetoprotein (AFP) level at the time of diagnosis and relapse, extent of surgical resection, chemotherapy protocols, details of radiotherapy and survival characteristics were recorded. A total of 58 patients (M/F = 20/38) with malignant sacrococcygeal germ cell tumor was included in analysis. With a mean follow-up of 156 months (range, 26 days to 288.8 months) overall and event-free survival rates of the 58 patients were 50.9% and 43.8%, respectively. AFP status of the patients (37% in patients with <10,000 ng/ml, 68.9% in patients with ≥ 10,000 ng/ml), type of resection (total vs others), coccygeal resection, chemotherapy protocol (PEB vs others) and number of chemotherapy courses had an impact on event-free survival in univariate analysis. In multivariate analysis, AFP status had the greatest effect on prognosis. Our treatment results are worse than those reported in the literature. Elevated AFP level at the time of diagnosis had a beneficial effect on prognosis, but year of diagnosis, tumor stage, presence of metastasis, tumor size and histopathological subtype had no impact on survival in patients with malignant sacrococcygeal germ cell tumors.

Protocol for the Examination of Specimens From Pediatric and Adult Patients With Extragonadal Germ Cell Tumors

The College of American Pathologists offers these protocols to assist pathologists in providing clinically useful and relevant information when reporting results of surgical specimen examinations. The College regards the reporting elements in the ‘‘Surgical Pathology Cancer Case Summary (Checklist)’’ portion of the protocols as essential elements of the pathology report. However, the manner in which these elements are reported is at the discretion of each specific pathologist, taking into account clinician preferences, institutional policies, and individual practice. The College developed these protocols as an educational tool to assist pathologists in the useful reporting of relevant information. It did not issue the protocols for use in litigation, reimbursement, or other contexts. Nevertheless, the College recognizes that the protocols might be used by hospitals, attorneys, payers, and others. Indeed, effective January 1, 2004, the Commission on Cancer of the American College of Surgeons mandated the use of the checklist elements of the protocols as part of its Cancer Program Standards for Approved Cancer Programs. Therefore, it becomes even more important for pathologists to familiarize themselves with these documents. At the same time, the College cautions that use of the protocols other than for their intended educational purpose may involve additional considerations that are beyond the scope of these documents.

Influence of Tumor Site and Histology on Long-Term Survival in 193 Children with Extracranial Germ Cell Tumors

Although germ cell tumors (GCT) supposedly share the same cell type of origin, their clinical course differs considerably depending on tumor site and histology. The aim of this work was to study long-term survival stratified for tumor site and tumor histology. The medical records of 193 consecutive infants and children with extracranial GCT were studied. The GCT arose in the following anatomical sites: sacrococcygeal (n = 70), ovary (n = 66), testis (n = 20), retroperitoneum (n = 12), neck (n = 8), mediastinum (n = 7), and miscellaneous (n = 10). Histological analysis revealed 152 teratomas (mature: 115, immature: 37), 27 yolk sac tumors, 8 mixed tumors, 2 dysgerminomas, 2 gonadoblastomas, 1 choriocarcinoma and 1 embryonal carcinoma. Overall survival (OS) for the whole patient group was 0.91 +/- 0.02, and event-free survival (EFS) was 0.88 +/- 0.02 at ten years. Patients with gonadal GCT had a higher probability of OS than those with extragonadal GCT (p = 0.029). Patients with cervical and mediastinal tumors had a lower probability of EFS than those with gonadal, retroperitoneal or sacrococcygeal GCT (p = 0.018). Patients with choriocarcinoma, embryonal carcinoma, immature teratoma, yolk sac tumor and mixed GCT had a lower probability of EFS than patients with mature teratoma or gonadoblastoma (p < 0.001). Mortality in children with extracranial germ cell tumors is not only dictated by malignant histology, but also, as in the case of mature teratomas, by occurrence at certain sites.

The distribution and behavior of extragonadal primordial germ cells in Bax mutant mice suggest a novel origin for sacrococcygeal germ cell tumors

In the mouse, germ cells that do not reach the genital ridges rapidly die by a wave of apoptosis that requires the pro-apoptotic protein Bax. In Bax-null embryos, large numbers of ectopic (extragonadal) germ cells fail to die. We have studied the fates of these, in an effort to understand the etiology of human extragonadal germ cell tumors, which are thought to arise from ectopic germ cells. We find that ectopic germ cells in which apoptosis is blocked form a heterogeneous population, which partially differentiates along the gonocyte pathway to different extents in different regions of the embryo, and in the two genders. In particular, a previously undescribed population of ectopic germ cells was identified in the tail. These germ cells retained primitive markers for longer than ectopic germ cells in other regions, and represent a possible origin for sacrococcygeal type I extragonadal germ cell tumors found in neonates and infants. This hypothesis is supported, but not proved, by the finding of cells expressing the germ cell marker Oct4 associated with a coccygeal germ cell tumor in a human infant.

SACROCOCCYGEAL TUMORS IN INFANCY AND CHILDHOOD; A RETROSPECTIVE HISTOPATHOLOGICAL REVIEW OF 85 CASES

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth—3 years versus median 1 week, range birth—10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z = 1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).

Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89.

To evaluate the prognostic value of metastases, extension into bone, and alpha-fetoprotein (AFP) elevation in children with malignant sacrococcygeal germ cell tumors (GCTs) prospectively collected in two cooperative Maligne Keimzelltumoren (MAKEI) protocols (83/86 and 89). Between October 1983 and October 1995, 76 of 210 registered patients with sacrococcygeal primaries presented either with pure yolk sac tumor, embryonal carcinoma (EC), or yolk sac tumor and EC mixed with immature and mature teratoma elements. Stages T1 and T2 disease were diagnosed in 15 and 61 children, respectively, 41 patients had metastases, and 35 children presented with extension into bone. At diagnosis, 22 children had an AFP elevation of less than 10,000 ng/mL. Thirty-six children showed an AFP level between 10,000 and 100,000 ng/mL, and 12 patients had values of greater than 100,000 ng/mL. Five patients died of complication during treatment and were excluded from further evaluation. Seventy-one patients could be analyzed. The 5-year relapse-free survival rate (RFS, Kaplan-Meier) was 0.76 +/- 0.03 (54 of 71 patients; median observation time, 54 months after diagnosis). The RFS of patients with and without metastases was different, but not significantly so (0.71 v 0.82). The outcome of patients with extension into bone (n = 31) and without this extension (n = 40) was 0.71 versus 0.80 (RFS, 5 years). Above-normal AFP level had no prognostic significance (P =.52). In children with malignant sacrococcygeal GCTs treated with an intensive, short-interval, platinum-based regimen, the stage, extent of metastases, extension into bone, and AFP level had no prognostic significance.

Sacrococcygeal germ cell tumor and spinal deformity in association with Sotos syndrome

Sacrococcygeal germ cell tumor and spinal deformity in association with Sotos syndrome

Treatment of recurrent malignant sacrococcygeal germ cell tumors: analysis of 22 patients registered in the German protocols MAKEI 83/86, 89, and 96.

To evaluate therapeutic options for recurrent malignant sacrococcygeal germ cell tumors (GCT) following three-agent, cisplatinum-based, first-line chemotherapy and tumor resection. Twenty-two patients were evaluated in 22 first-, 14 second-, five third-, and two fourth-relapse situations. One patient, who relapsed with pure teratoma, was excluded from the analysis of adjuvant treatment. Seventeen patients presented with an isolated local recurrence, two patients showed a distant relapse, and three patients suffered from a combined local and distant recurrence. Twelve patients achieved complete remission (CR) after surgery (n = 12) and adjuvant platinum chemotherapy (n = 10). Seven of these patients remain in continuous CR, and five patients relapsed. All patients who achieved only a partial remission developed a second relapse. Three of 14 patients could be cured after a second (or further) relapse. Altogether, 10 patients survived disease free, and 12 patients died as a result of tumor progression (n = 11) or therapy-related complications (n = 1). The completeness of salvage surgery and clinical remission status after first salvage treatment were the most important prognostic parameters. In addition, patients in first or second relapse with locally advanced or poorly responding tumors benefited from preoperative chemotherapy in combination with regional hyperthermia (RHT). In some patients after microscopically incomplete resection, irradiation at doses > 45 Gy contributed to a favorable outcome. The complete resection of the local recurrence represents the cornerstone of salvage treatment. Preoperative platinum-based chemotherapy, combined with RHT in some patients, facilitates complete tumor resection. Radiotherapy should be reserved for those patients with microscopically incomplete tumor resection. As the chance of cure decreases with further relapses, it is important to establish a stringent therapeutic strategy to avoid significant treatment delays and, most importantly, insufficient local therapy.

Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89.

To evaluate a multimodal approach including surgery and cisplatinum chemotherapy for treatment of children with malignant sacrococcygeal germ cell tumors (GCT) and to compare adjuvant and neoadjuvant strategies in advanced tumors. Between 1983 and 1995, 71 patients with malignant sacrococcygeal GCT were prospectively enrolled onto the German protocols for nontesticular GCT Maligne Keimzelltumoren 83/86 and 89. Five patients who received no chemotherapy (n = 2) or nonplatinum chemotherapy (n = 2) or who did not undergo tumor resection (n = 1) were excluded from this analysis. Among the 66 patients analyzed were 14 boys and 52 girls. The median age was 17.4 months (range, 7 months to 119 months). Median follow-up was 79 months (range, 4 months to 145 months). Fifty-two patients presented with locally advanced stage T2 tumors, and 30 patients had distant metastases at diagnosis. Patients received a median of eight cycles (range, four to nine cycles) of cisplatinum-based chemotherapy. Thirty-five patients underwent tumor resection at diagnosis and received adjuvant cisplatinum-based chemotherapy (group A). Thirty-one patients received up-front chemotherapy followed by delayed tumor resection (group B). Group B included more metastatic tumors than group A (group B, 19 of 31 patients; group A, 11 of 35 patients, P =.01). Preoperative chemotherapy facilitated complete tumor resections (group B, 20 of 31 patients; group A, five of 35 patients, P <.001) and avoided second-look surgery. Metastases at diagnosis and completeness of the first attempt of tumor resection were significant prognostic predictors; however, metastases were not predictive for patients treated with up-front chemotherapy. At 5 years follow-up, event-free survival was 0.76 +/- 0.05 (50 of 66 patients), and overall survival was 0.81 +/- 0.05 (54 of 66 patients). Four patients died as a result of therapy-related complications, and eight patients died of their tumors. Patients with locally advanced and metastatic tumors (T2b M1) fared better with neoadjuvant treatment [overall survival: 0.83 +/- 0.09 (16 of 19 patients) versus 0.45 +/- 0.15 (five of 11 patients), P =.01]. Even locally advanced and metastatic sacrococcygeal GCT can be successfully treated with up-front cisplatinum-based chemotherapy followed by delayed but complete tumor resection.