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Gonadal (Testicular and Ovarian Tumors)

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Gonadal (Testicular and Ovarian Tumors) Gonadal (Testicular and Ovarian Tumors) Gonadal (Testicular and Ovarian Tumors) Authors: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital Erin Gafford, Pediatric Oncology Education Student, St. Jude Children’s Research Hospital; Nursing Student, School of Nursing, Union University Content Reviewed by: Guillermo L. Chantada, MD, Hospital JP Garrahan, Buenos Aires, Argentina Cure4Kids Release Date: 1 September 2006 At 4 weeks gestation, germ cells begin to develop in the yolk sac in an undifferentiated state. These primordial germ cells migrate to the gonadal ridge by the 6th week of gestation, and descend into the pelvis or scrotal sac.This migratory route explains the midline location of most extra-gonadal germ cell tumors (intracranial, mediastinal, retroperitoneal or sacrococcygeal). (A -1 ) Germ cell neoplasms arise either from the primordial germ cells (gonadal) or indirectly through embryonic or extra-embryonic differentiation (extra-gonadal). The stromal tumors arise from the primitive sex cords of either the ovary or the testicles, and the rare epithelial tumors arise from the coelomic epithelium that have undergone neoplastic transformations. Though the morphology of each type of germ cell tumor is similar in all locations (whether gonadal or extragonadal), the morphologic type and biological characteristics vary depending on the site of origin and age of the patient. There are three (A – 2) biologically distinct subsets of germ cell tumors: - tumors of the adolescent testis and ovary - extragonadal germ cell tumors of older children - tumors of infants and young children Testicular Germ Cell Tumors: Approximately 7% of all germ cell tumors are testicular and 75% of all (A – 3) testicular tumors have a germ cell origin. Though 90% are localized, metastatic disease can occur in the lymph nodes of the chest and retroperitoneum. Risk factors include undescended testes; and patients who have orchidopexy should be examined for occurrence of testicular tumors. Most commonly, patients present with a painless, irregular scrotal mass, often associated with hydroceles or inguinal hernias. Most testicular tumors are noted during routine examination or observed by the child’s caretaker. These tumors are irregular, non-tender scrotal masses. In some cases, a hydrocele and inguinal hernia may also be present. Module 13 - Document 17 Page 1 of 12 Gonadal (Testicular and Ovarian Tumors) Diagnostic Workup: Complete history of illness to evaluate for associated symptoms such as constipation, vomiting, genitourinary symptoms. Physical exam to assess for masses, inguinal hernias, hydoceles Ultrasound to determine cystic versus solid characteristics and detect calcifications; localize the scrotal mass, and differentiate between simple and reactive hydrocele. Assessment of serum tumor markers AFP and β-hCG, which are often elevated, since most testicular tumors are of the yolk pathology. These serum markers serve as the basis for staging and monitoring treatment efficacy. Metastatic evaluation including CT scan, and ultrasound of the pelvis, abdomen and pelvis; chest X-ray, and bone scintigraphy. Staging for Testicular Cancers: A (A – 4) staging system developed by the Pediatric Oncology Group and the Children’s Cancer Group that accounts for tumor markers and the trans-scrotal surgery of the tumors. Ovarian Tumors: Ovarian tumors represent approximately 1% of childhood malignancies and are often seen during the first and second decades of life. Two -thirds of all ovarian tumors are of germ cell origin, most commonly benign teratomas. (A – 5) Malignant ovarian tumors include dysgerminoma, yolk sac (endodermal sinus), embryonal carcinoma, mixed germ cell tumor, and immature teratoma. Patients often complain of abdominal distention, varying degrees of acute and chronic abdominal pain, and a palpable abdominal mass. Other symptoms include enuresis, constipation, amenorrhea, precocious puberty, and vaginal bleeding. Diagnostic Workup: Complete history of illness to evaluate for associated symptoms such as vaginal bleeding, amenorrhea, and a review of the menstrual history, if applicable, should be done. Physical exam to assess for (A – 6) abdominal mass and presence of secondary sex characteristics (presence of precocious puberty) Ultrasound to evaluate pelvic masses, localize adnexal masses, differentiate between solid and cystic masses, and to detect calcifications. Abdominal and pelvic CT scans to define the site of origin, tumor extent, presence of calcifications, fat and metastatic disease Assess serum tumor markers AFP, β-hCG, carcinoembryonic antigen, and CA-125. These serum markers serve as basis for staging and monitoring treatment efficacy. Metastatic evaluation by CT scan, ultrasound of the pelvis, abdomen and pelvis, chest X- ray, CT and bone scintigraphy. CNS imaging may be necessary in patients with disseminated disease. Module 13 - Document 17 Page 2 of 12 Gonadal (Testicular and Ovarian Tumors) Staging for Ovarian Cancers: A staging system currently used for ovarian cancers in the adult population developed by the (A-7) International Federation of Gynecology and Obstetrics (FIGO) serves as a basis for a simpler system being used by the (A-8) Pediatric Oncology Group/Children’s Cancer Group (POG\CCG) in their cooperative studies. Treatment: Treatment for germ cell tumors should be multimodal and individualized. Surgical resection is the treatment of choice in treating benign germ cell tumors and is also important in treating malignant tumors if removal does not sacrifice vital structures. In cases of tumors that are unresectable, chemotherapy can sometimes be used to debulk some tumors so that they can be resected. Young patients with completely excised testicular yolk sac tumors whose AFP values return to normal after surgery, may be managed without adjuvant therapy and monitored closely with serial AFP levels for early detection of relapse. Standard agents currently used in the Germ Cell Tumor Protocols include cisplatin (platinol), etoposide (VP – 16), and bleomycin (PEB). Newer agents include ifosfamide (Ifex), carboplatin, and topotecan. Even though dysgerminomas are exquisitely radiosensitive, this modality has been recently replaced by chemotherapy in most cases. The introduction of effective chemotherapy has greatly improved the prognosis of germ cell tumors. Relapsing patients with chemosensitive disease can be rescued with higher dose chemotherapy. Future Directions: The challenges for germ cell tumors include further understanding these diverse tumors through risk groupings, identification of molecular and genetic alterations associated with these tumors, and the development of novel therapies with lower toxicity and fewer late effects than current drugs. Module 13 - Document 17 Page 3 of 12 Gonadal (Testicular and Ovarian Tumors) Helpful Web links: Lucile Packard Children’s Hospital at Stanford, Palo Alto, CA http://www.lpch.org/diseaseHealthInfo/healthLibrary/oncology/gct.html St. Jude Children's Research Hospital http://www.stjude.org/disease-summaries/0,2557,449_2167_7407,00.html eMedicine.com – Teratomas and Other Germ Cell Tumors http://www.emedicine.com/ped/topic3023.htm eMedicine.com - Germ Cell Tumors http://www.emedicine.com/med/topic863.htm The National Cancer Institute This website contains comprehensive information on extracranial germ cell tumors. http://jncicancerspectrum.oxfordjournals.org/cgi/pdq/jncipdq;CDR0000062854 Related www.Cure4kids.org Seminars Seminar #658 Ovarian Masses in Pediatric Age Groups Himesh Gupta, MD, Stephen Shochat, MD, Joseph D. Khoury, MD and Fredric Hoffer, MD https://www.cure4kids.org/seminar/658 Module 13 - Document 17 Page 4 of 12 Gonadal (Testicular and Ovarian Tumors) APPENDIX: A – 1 Histogenesis of the Gonadal Tumors Embryonic Gonads Mesenchymal Tissue Primordial germ cell Celomic epithelium : Dysgerminoma Sex-cord stromal Epithelial tumors tumors Embryonal differentiation Embryonal carcinoma Extra-embryonic tumors Endodermal sinus tumor Embryonic tumors Choriocarcinoma Teratoma Polyembryoma Distribution and Characteristics of Germ Cell Tumors: Germ Cell Median Age Pathology Clinical Tumor Presentation Gonadal Pre-adolescents & Dysgerminomas Palpable abdominal Ovarian adolescents Yolk sac tumors mass (10 – 14 years) Immature teratoma Abdominal distention Malignant mixed germ cell tumors Varying degree of acute Embryonal carcinomas or chronic abdominal pain. - depending on patient age, histology and the presence of ovarian torsion Infants Endodermal sinus tumor (yolk sac) Testicular Adolescents teratomas Irregular, non tender scrotal mass Module 13 - Document 17 Page 5 of 12 Gonadal (Testicular and Ovarian Tumors) Germ Cell Median Age Pathology Clinical Tumor Presentation Extragonadal Infancy Endodermal sinus tumor (Yolk sac) Variable – depending upon Sacrococcygeal Embryonal location and histology Benign Teratoma Immature Malignant Children Teratoma Coughing Mediastinal Adolescents Embryonal carcinoma Wheezing anterior Endodermal sinus tumor (yolk sac) Dyspnea superior Choriocarcinoma Chest pain especially in large posterior tumors Under 2 years Benign or malignant Abdominal pain Abdominal Constipation Retroperitoneum Urinary difficulties Stomach Omentum Liver Children Germinomas Headaches Intracranial Non-germinomas Visual disturbances Pineal Mixed with yolk sac Incoordination Suprasellar Choriocarcinoma Diabetes Insipidus Infrasellar Teratocarcinoma Hypopituitaris, Anorexia Precocious puberty Infants Usually benign Variable, depending
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