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National Cancer Institute
Ultima Vez Modificado: 27 de noviembre del 2012
Fortunately, cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975. 1 Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team incorporates the skills of the primary care physician, pediatric surgical subspecialists, radiation therapists, pediatric oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life. Specific information about supportive care for children and adolescents with cancer can be found in the PDQ® Supportive and Palliative Care summaries.
Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics. 2 At these pediatric cancer centers, clinical trials are available for most of the types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients/families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. The majority of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI Web site.
Dramatic improvements in survival have been achieved for children and adolescents with cancer. 1 Between 1975 and 2002, childhood cancer mortality decreased by more than 50%. For gonadal extracranial germ cell tumor (GCT), the 5-year survival rate has increased over the same time from 89% to 98% for children younger than 15 years and from 70% to 95% for adolescents aged 15 to 19 years. For extragonadal GCT, the 5-year survival rate from 1979 to 2002 increased from 42% to 83% for children younger than 15 years. 1 Childhood and adolescent cancer survivors require close follow-up since cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ® summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Childhood GCTs are rare in children younger than 15 years, accounting for approximately 3% of cancer cases in this age group. 3 4 5 In the fetal/neonatal age group, the majority of extracranial GCTs that occur are benign teratomas occurring at midline locations including sacrococcygeal, retroperitoneal, mediastinal, and cervical regions. Despite the small percentage of malignant teratomas that occur in this age group, perinatal tumors have a high morbidity rate due to hydrops fetalis and premature delivery. 6 7 Extracranial GCTs (particularly testicular GCTs) are much more common among adolescents aged 15 to 19 years, representing approximately 14% of cancer diagnoses in this age group. The distribution of extracranial GCTs by 5-year age group and by gender is shown in Table 1 below.
|04 years||59 years||1014 years||1519 years|
GCTs develop from primordial germ cells, which migrate during embryogenesis from the yolk sac through the mesentery to the gonads. 8 9 Childhood extracranial GCTs can be divided into gonadal and extragonadal types. Most childhood extragonadal GCTs arise in midline sites (i.e., sacrococcygeal, mediastinal, and retroperitoneal); the midline location may represent aberrant embryonic migration of the primordial germ cells.
The histologic and genetic properties of these tumors are heterogeneous and vary by primary tumor site and the gender and age of the patient. 10 11 Histologically identical GCTs that arise in younger children have different biological characteristics from those that arise in adolescents and young adults. 12
Childhood extracranial germ cell tumors (GCTs) comprise a variety of histologic diagnoses and can be broadly classified into mature or immature teratomas and malignant GCTs.
Mature teratomas usually occur in the ovary or at extragonadal locations and are the most common histological subtype of childhood GCT. 1 2 3 These teratomas usually contain well-differentiated tissues from the ectodermal, mesodermal, and endodermal germ cell layers, and any tissue type may be found within the tumor. Mature teratomas are benign, though some mature and immature teratomas may secrete enzymes or hormones, including insulin, growth hormone, androgens, prolactin, and vasopressin. 4 5 6
Immature teratomas contain tissues from all three germ cell layers, but immature tissues, primarily neuroepithelial, are also present. Immature teratomas are graded from 0 to 3 based on the amount of immature neural tissue found in the tumor specimen. 7 Tumors of higher grade are more likely to have foci of yolk sac tumor. 8 Immature teratomas occur primarily in young children at extragonadal sites and in the ovaries of girls near the age of puberty, but there is no correlation between tumor grade and patient age. 8 9
GCTs contain frankly malignant tissues of germ cell origin, and rarely, tissues of somatic origin. Isolated malignant elements may constitute a small fraction of a predominantly mature or immature teratoma. 9 10 Malignant germ cell elements of children, adolescents, and young adults can broadly be classified by location (see Tables 2 and 3).
|Malignant Germ Cell Elements||Location|
|Yolk sac tumor||E, O, T|
|Dysgerminoma (rare in young children)||O|
|E = extragonadal; O = ovarian; T = testicular.|
|Malignant Germ Cell Elements||Location|
|Yolk sac tumor||E, O, T|
|Choriocarcinoma||E, O, T|
|Mixed germ cell tumors||E, O|
|E = extragonadal; O = ovarian; T = testicular.|
Yolk sac tumors produce alpha-fetoprotein (AFP), while germinomas (seminomas and dysgerminomas), and especially choriocarcinomas, produce beta-human chorionic gonadotropin, resulting in elevated serum levels of these substances. Most children with malignant GCTs will have a component of yolk sac tumor and have elevations of AFP, 12 13 which is serially monitored during treatment to help assess response to therapy. 9 10 12
Adolescents and young adults present with more germinomas (testicular and mediastinal seminomas in males and ovarian dysgerminomas in females). These tumors are usually treated with chemotherapy. They are also sensitive to radiation therapy, but radiation is rarely recommended. Radiation therapy and surgery for the patient with brain metastases may provide palliation and occasionally, long-term survival. 14[Level of evidence: 3iiiA]
The following paragraphs describe the biologically distinct subtypes of GCTs found in children and adolescents. It should be emphasized that very few pediatric GCT specimens have been analyzed to date. Biologic distinctions between GCTs in children versus adults may not be absolute. 1 2
Ovarian GCTs occur primarily in adolescent and young adult females. While the majority of ovarian GCTs are benign mature teratomas, a heterogeneous group of malignant GCTs occur in females, including immature teratomas, dysgerminomas, yolk sac tumors, and mixed GCTs. Patients with pediatric ovarian GCTs have an excellent prognosis. One series of 66 patients followed over 44 years reported recurrence and mortality rates of 4.5% and 3%, respectively. 12 The malignant ovarian GCT commonly shows increased copies of the short arm of chromosome 12. 13 (Refer to the PDQ® summary on Ovarian Germ Cell Tumors Treatment for more information.)
There is very little data about the potential genetic or environmental factors associated with childhood extracranial GCTs. Patients with Klinefelter syndrome 21 22 23 are at increased risk for mediastinal GCTs, while patients with Swyer syndrome 24 25 are at increased risk for gonadoblastomas and germinomas.
As with other childhood solid tumors, stage directly impacts the outcome of patients with malignant germ cell tumors (GCTs). 1 2 3 The most commonly used staging system in the United States is described below. 4 Retroperitoneal lymph node dissection has not been required in pediatric germ cell trials to stage males younger than 15 years. Data on adolescent males with testicular GCTs are limited. Retroperitoneal lymph node dissection is used for both staging and treatment in adult testicular GCT trials. 5 (Refer to the PDQ® summary on Testicular Cancer Treatment for more information about the staging of adult testicular GCTs.)
Another staging system used frequently by gynecologic oncologists is the International Federation of Gynecologic Oncologists (FIGO) staging system, which is based on an adequate staging operation at the time of diagnosis. 6 (Refer to the PDQ® summary on Ovarian Germ Cell Tumors Treatment for more information.) This system has also been used by some pediatric centers, 2 and is as follows:
Stage IV: Distant organ involvement, including liver parenchyma or pleural space
Prior to effective chemotherapy, children with extracranial malignant germ cell tumors (GCTs) had 3-year survival rates of 15% to 20% with surgery and radiation therapy, 1 2 3 though young boys with localized testicular tumors did well with surgical resection. 4 5 The outcome for most children and adolescents with extracranial GCT is now favorable when appropriate treatment is provided. Prognosis and appropriate treatment depend on factors such as histology (e.g., seminomatous vs. nonseminomatous), age (young children vs. adolescents), stage of disease, and primary site. 6 7 8 9 To maximize the likelihood of long-term survival while minimizing the likelihood of treatment-related long-term sequelae (e.g., secondary leukemias, infertility, hearing loss, and renal dysfunction), it is important that children with extracranial malignant GCTs be cared for at pediatric cancer centers with experience treating these rare tumors. Based on clinical factors, appropriate treatment may involve: surgical resection followed by careful monitoring for disease recurrence; diagnostic tumor biopsy and preoperative platinum-based chemotherapy followed by definitive tumor resection; or initial surgical resection followed by platinum-based chemotherapy. 10 For patients with completely resected immature teratomas at any location (even those with malignant elements) and patients with localized, completely resected (stage I) gonadal tumors, additional therapy may not be necessary; however, close follow-up is important. 11 12 The watch-and-wait approach requires scheduled serial physical examination, tumor marker determination, and primary tumor imaging to ensure that a recurrent tumor is detected without delay.
Cisplatin-based chemotherapy has dramatically improved the outcome for children with extracranial GCTs, with 5-year survival rates of more than 90%. 6 7 8 9 The standard chemotherapy regimen for both adults and children with malignant nonseminomatous GCTs includes cisplatin, etoposide, and bleomycin (PEB), though children receive fewer doses of bleomycin than adults. 6 7 13 14 The combination of carboplatin, etoposide, and bleomycin (JEB) has undergone clinical investigation in the United Kingdom in children younger than 16 years and is reported to have a similar event-free survival (EFS) by site and stage as PEB. 8 15 The use of JEB appears to be associated with less ototoxicity and nephrotoxicity than PEB. 8 Adult studies have substituted standard-dose carboplatin for cisplatin in combination with etoposide alone and in combination with etoposide and low-dose bleomycin, 16 but the carboplatin regimens demonstrated inferior EFS and overall survival (OS) compared with cisplatin-containing therapy among patients with malignant GCTs. No randomized comparison of PEB versus JEB has been conducted in children. [Note: See Table 4 for pediatric PEB and JEB chemotherapy dosing schedules.]
The approach to the management of extracranial GCTs has been informed by the results of two intergroup studies conducted by the Children's Cancer Group (CCG) and the Pediatric Oncology Group (POG). 6 7 11 These studies explored the use of PEB for the treatment of localized gonadal GCT 6 and the benefit of increasing the dose of cisplatin (high-dose [HD]-PEB: 200 mg/m2 vs. PEB: 100 mg/m2 of cisplatin) in a randomized manner in patients with extragonadal and advanced gonadal GCTs. 7
The intensification of cisplatin in the HD-PEB regimen provided some improvement in EFS but no difference in OS; however, the use of HD-PEB was associated with a significantly higher incidence and severity of ototoxicity and nephrotoxicity. In a subsequent study, amifostine was not effective in preventing hearing loss in patients who received HD-PEB. 17
|Pediatric PEB (every 21 days)||15 units/m, day 1||100 mg/m, days 15||20 mg/m, days 15|
|Pediatric JEB (every 2128 days)||15 units/m, day 3||120 mg/m, days 13||600 mg/m or GFR-based dosing, day 2|
|GFR = glomerular filtration rate; JEB = carboplatin, etoposide, and bleomycin; PEB = cisplatin, etoposide, and bleomycin.|
Table 5 provides an overview of standard treatment options for children with extracranial GCTs. Treatment requires a multidisciplinary approach with various surgical subspecialties and pediatric oncologists. Specific details of treatment by primary site and clinical condition are described in subsequent sections.
|Mature teratoma||All sites||Localized||Surgery + Observation|
|Immature teratoma||All sites||Localized||Surgery + Observation|
|Malignant germ cell tumors||Testicular||Stage I||Surgery + Observation|
|Stages IIIVa||Surgery + PEB|
|Ovarian||Stage Ib||Surgery + PEB|
|Stages IIIV||Surgery + PEB|
|Extragonadal||Stages III||Surgeryc + PEB|
|Stages IIIIVa||Surgeryc + PEB|
|PEB = cisplatin, etoposide, and bleomycin.|
The treatment of GCTs with other non-GCT elements is complex and scant data exist to inform treatment. Specific treatment for both the malignant GCT and non-GCT elements may be required. 18 However, the optimal treatment strategy for other malignant elements found in GCT has not been determined.
Mature and immature teratomas arise primarily in the sacrococcygeal region of neonates and young children and in the ovaries of pubescent girls. These tumors are also less commonly found in the testicular region of boys younger than 4 years, the mediastinum of adolescents, and other sites. 1 2 3
The sacrococcygeal region is the primary tumor site for the majority of benign and malignant germ cell tumors (GCTs) diagnosed in neonates, infants, and children younger than 4 years. These tumors occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported. 4 Sacrococcygeal tumors present in two clinical patterns related to the child's age, tumor location, and likelihood of tumor malignancy. Neonatal tumors present at birth protruding
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