Teratoma
Teratoma: Excerpt from The 5-Minute Pediatric Consult
Jane E. Minturn, MD
Teratoma - BASICS
Teratoma - description
Embryonal neoplasm containing tissue derived from all 3 germ layers (endoderm, mesoderm, and ectoderm):
- Mature or immature and may occur with or without associated malignant elements
- A subset of the broader class of germ cell tumors
Teratoma - general prevention
There is no known prevention for the development of teratomas and other germ cell tumors.
Teratoma - epidemiology
- Gonadal and extragonadal germ cell tumors account for ~3% of childhood malignancies (<15 years) and 15% of malignancies of ages 15–19 years.
- Incidence of germ cell tumors as a whole is ~2.5 cases per million in white children and 3 cases per million in black children <15 years of age.
- Boys and girls are equally affected.
- One suggestive epidemiologic association is with high maternal hormone levels during pregnancy.
- More controversial associations include younger gestational age; viral infections including herpes simplex virus, varicella-zoster virus, cytomegalovirus, mumps; other congenital anomalies; maternal urinary tract infection or tuberculosis; and paternal occupation in chemical industries.
- Sacrococcygeal teratoma: Accounts for 40% of all childhood germ cell tumors and up to 78% of extragonadal germ cell tumors. Most prevalent in infants; girls more frequently affected
- Testicular and ovarian tumors: Most prevalent in infants and adolescents
- Vaginal tumors: Most prevalent in girls <3 years old
- Mediastinal tumors: Average age of the pediatric patient is 3 years, but also found in adolescents; most common extragonadal germ cell tumor in adults
Teratoma - risk factors
Teratoma - genetics
- Most consistent (80% of germ cell tumors) structural chromosomal abnormality is an isochromosome 12p [i(12p)].
- No pattern of inheritance is known.
Teratoma - pathophysiology
- Absence of normal mitotic/meiotic arrest of primordial germ cells in gonads leading to gonadal tumor formation. Aberrant migration of primordial germ cells during embryonal development, causing germ cells to come to rest outside the gonads leading to extragonadal tumors.
- Mature teratoma: Contains well-differentiated, nonmitotic tissues from all 3 germ layers, such as squamous epithelium, neuronal tissue, muscle, teeth, cartilage, bone, GI, and respiratory epithelium.
- Immature teratoma: Contains various immature tissues representative of all 3 germ layers; divided histologically into 4 grades, 0–3, dependent on degree of immaturity and mitotic activity
- Teratoma with malignant germ cell elements: Foci of malignant tissue that resemble other germ cell tumors such as embryonal carcinoma, yolk sac tumor (endodermal sinus tumor), and choriocarcinoma, in addition to mature or immature tissues
Teratoma - DIAGNOSIS
Teratoma - signs & symptoms
Teratoma - history
- External mass, constipation, urinary abnormalities, lower extremity weakness:
- Sacrococcygeal mass may impinge on nerve structures.
- Anterior sacrococcygeal mass may have no external component.
- Fetal sacrococcygeal teratoma often initially picked up on prenatal sonography.
- Cough, wheeze, dyspnea, hemoptysis, superior vena cava syndrome: Suggest anterior mediastinal mass
- Blood-tinged vaginal discharge: Vaginal teratoma
- Abdominal pain, nausea, vomiting, constipation, urinary tract symptoms: Ovarian tumors present late with a large mass. Symptoms mimicking acute abdomen may indicate ovarian torsion.
- Painless scrotal swelling or painful testicular torsion: Testicular mass may be teratoma.
- Cryptorchidism: Associated with germ cell tumors in boys
Teratoma - physical exam
- Palpable mass either externally or internally, signs of spinal cord compression: Sacrococcygeal tumor
- Vaginoscopy reveals a polyploid lesion arising from the vaginal wall: Examination under anesthesia usually necessary
- Palpable abdominal mass, peritoneal symptoms: Ovarian mass may be large
- Palpable mass in scrotum: Testicular origin
- Decreased breath sounds, consolidation, wheezing, superior vena cava syndrome: Mediastinal mass may be an emergency.
Teratoma - tests
Teratoma - lab
- Serum α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-HCG): Pure teratomas are not associated with elevated tumor markers. Elevation of either of these markers indicates the presence of more malignant germ cell elements and requires review of the histologic material.
- CBC and chemistry profile, with electrolytes, BUN, creatinine, liver function tests, uric acid, and lactate dehydrogenase: Workup to rule out other malignancies or associated organ dysfunction
Teratoma - imaging
- Plain radiograph: May reveal mature calcified tissues, such as bone or teeth, within tumor
- Chest radiograph: Shows mediastinal mass
- CT scan: Necessary to evaluate the primary site and regional disease
- Prenatal MRI for fetal sacrococcygeal teratoma diagnosed by ultrasound: Allows more accurate prenatal counseling and improved preoperative planning
- Chest CT and bone scan: If malignancy is suspected or proven, these are indicated for evaluation of metastasis.
- Ultrasound, if CT is not readily available:
- May be helpful, but will rarely suffice as the sole imaging study of the primary site
- May be 1st evidence of anterior sacrococcygeal mass or to differentiate testicular mass from hydrocele
Teratoma - differencial diagnosis
- Sacrococcygeal: Pilonidal cyst, meningocele, lipomeningocele, hemangioma, abscess, bone tumor, epidermal cyst, chondroma, lymphoma, ependymoma, neuroblastoma, glioma
- Abdominal: Wilms tumor, neuroblastoma, lymphoma, rhabdomyosarcoma, hepatoblastoma, retained twin fetus
- Vaginal: Rhabdomyosarcoma (sarcoma botryoides), clear cell carcinoma
- Ovarian: Cyst, appendicitis, pregnancy, pelvic infection, hematocolpos, sarcoma, lymphoma, other ovarian tumors
- Testicular: Epididymitis, testicular torsion, infarct, orchitis, hernia, hydrocele, hematocele, rhabdomyosarcoma, lymphoma, leukemia, other testicular tumors
- Mediastinal: Hodgkin and non-Hodgkin lymphoma, leukemia, thymoma
Teratoma - TREATMENT
Teratoma - surgery
- Every effort should be made to preserve fertility in gonadal teratomas. An experienced pediatric–gynecology oncologic surgeon is critical.
- Sacrococcygeal teratomas should undergo complete resection to include the coccyx, and patients followed closely postoperatively with tumor markers.
- Mature teratoma: Full surgical excision, irrespective of site, is curative in prepubescent patients. One exception is in postpubescent testicular teratoma, where aggressive surgical resection, lymph node dissection, and postsurgical chemotherapy are recommended.
- Immature teratoma:
- Complete surgical resection is therapy of choice.
- In cases of elevated AFP and incomplete surgical resection; chemotherapy should be offered given risk of microscopic foci of endodermal sinus tumor.
- Teratoma with malignant components:
- Surgery plus chemotherapy with etoposide, cisplatin or carboplatin, and bleomycin
- Patients with residual disease should have additional surgery and additional chemotherapy if total resection is not possible.
- High-dose chemotherapy with autologous stem cell support and radiation are reserved for salvage therapy in recurrent disease.
Teratoma - FOLLOW UP
- Serial physical exams and imaging studies of primary site
- Tumor markers (AFP or β-HCG) if elevated at diagnosis
- If chemotherapy or radiation therapy used, need to monitor for secondary malignancies, long term. Short term, need to monitor blood counts, chemistries, renal function, and audiology
Teratoma - bibliography
- Allen MS. Presentation and management of benign mediastinal teratomas. Chest Surg Clin North Am. 2002;12:659–664.
Cushing B, Perlman EJ, Marina NM, et al. Germ cell tumors. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2002:1091–1113.- Danzer E, Hubbard AM, Hedrick HL, et al. Diagnosis and characterization of fetal sacrococcygeal teratoma with prenatal MRI. Am J Roentgenol. 2006;187:W350–W356.
- De Backer A, Madern GC, Oosterhuis JW, et al. Ovarian germ cell tumors in children: A clinical study of 66 patients. Pediatr Blood Cancer. 2006;46:459–464.
- Hedrick HL, Flake AW, Crombleholme TW, et al. Sacrococcygeal teratoma: Prenatal assessment, fetal intervention, and outcome. J Pediar Surg. 2004;39:430–438.
Teratoma - CODES
Teratoma - icd9
Teratoma - FAQ
- Q: What is the chance of cure for malignant teratomas?
- A: With current chemotherapy as outlined above, diseasefree survival is 85%.
- Q: Can a benign tumor recur? If so, can it then be malignant?
- A: Yes. If there is residual tissue left behind, the tumor can recur. If there were unrecognized areas of malignancy, the recurrence can be a malignant teratoma. The greatest risk for the latter is with the immature teratomas.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Medical Books Excerpts
- Scrotal swelling
- "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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