GERM CELL TUMORS
 
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GERM CELL TUMORS

 

DEFINITION:

A tumor arising from germ cells giving rise to gonadal, embryonic, and extraembryonic tumors.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • ovarian - peaks < 2years and after 6 years
    • testicular - peaks < 2years and after 14 years
  • risk factors:
    • F > M (?)
    • cryptorchidism - increases risk by 50%
      • 20% arise in descended testis

PATHOGENESIS:

1. Tumor Origin

1. Gonadal

  • most common

2. Extragonadal

  • CNS, mediastinum, retroperitoneum, sacrococcygeum
  • thought to represent aberrancies in the migration of germ cells from the yolk sac into the developing fetus

2. Histologic Types

  • germ cells differentiate into:

1. Gonadal Tumors

  • seminomas (testes)
  • dysgerminomas (ovaries)

2. Totipotential Tumors

  • embryonic tumors -> teratoma (ectoderm, mesoderm, and/or endoderm)
  • extra-embryonic tumors-> choriocarcinoma (trophoblasts)
    • yolk sac carcinoma (yolk sac cells)

    (totipotential tumors have multipotential characteristics based upon the differentiated cell type)

    • extra-embryonic tumors have a poorer prognosis
    • sacrococcygeal teratomas are the most common solid tumor in newborns (incidence: 1/40,000 live births)

CLINICAL FEATURES:

1. Primary Tumor

1. Systemic Manifestations

  • weight loss, anorexia, lethargy

2. Gonadal Tumors

  • testes - painless scrotal mass in 1st year of life
  • ovaries - pain, nausea/vomiting, abdominal mass

3. Extragonadal Tumors

  • mediastinal - respiratory difficulies/distress
  • retroperitoneal - mass
  • sacrococcygeal - mass, GI/GU obstruction

2. Metastases

  • 1. Regional Lymph Nodes
  • 2. Lung
  • 3. Bone

    • INVESTIGATIONS:

    1. Imaging Studies

    • MRI, CT, ultrasound, chest x-ray, bone scan

    2. Pathology

    • tumor
    • bone marrow

    3. Biologic

    • serum - alpha-fetoprotein, beta-hCG

    MANAGEMENT:

    1. Surgery

    2. Radiotherapy

    • seminomas and dysgerminomas are highly radiosensitive

    3. Chemotherapy

    • adjuvant
    • cisplatin, bleomycin, vinblastine, etoposide, ifosfamide

     

     

     

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