Difference between revisions of "Germ cell tumor classification"

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Revision as of 16:54, 13 September 2019

Germ cell tumors can be classified based on their histologic features into:


 
 
 
 
 
 
 
 
Germ cell tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Germinomatous/Undifferentiated/Immature
 
 
 
 
 
 
 
 
Nongerminomatous/Differentiated/Embryonal
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Dysgerminoma(Ovary)
 
 
Seminoma(Testis)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Embryonal carcinoma
 
 
 
 
 
embryonic tissue
 
 
 
 
Extraembryonic tissue
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Teratoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yolk sac tumor
 
Choriocarcinoma
 


 
 
 
 
 
 
 
 
Germ cell tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Germinomatous/Undifferentiated/Immature
 
 
 
 
 
 
 
 
Nongerminomatous/Differentiated/Embryonal
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Dysgerminoma(Ovary)
 
 
Seminoma(Testis)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Embryonal carcinoma
 
 
 
 
 
embryonic tissue
 
 
 
 
Extraembryonic tissue
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Teratoma
 
 
Yolk sac tumor
 
Choriocarcinoma
 
 










Germ cell tumors classification is based on the histologic features and whether they are differentiated or not into:

  • Germinomatous tumors: (non-differentiated): Gonadal and Extra-gonadal
    • Germinoma ( Gonadal: dysgerminoma and seminoma), (Extra-gonadal: mediastinum, or pineal region)
    • Dysgerminoma (Ovary)
    • Seminoma (testes)
  • Nongerminomatous tumors: all other germ-cell tumors, pure and mixed
    • Embryonal carcinoma
    • Endodermal sinus tumor, also known as yolk sac tumor (EST, YST)
    • Choriocarcinoma
    • Teratoma including mature teratoma, dermoid cyst, immature teratoma, teratoma with malignant transformation
    • Polyembryoma
    • Gonadoblastoma
  • Mixed germ cell tumors:

Germ cell tumors can also be classified based on their location into:

  • Gonadal (ovary and testes)
  • Extra-gonadal (MC:mediastinum, retroperitoneal. Less common: Pineal gland, sacrococcigeal)


  • Ovarian germ cell tumors (OGCTs ): The histologic types that arise from the ovary are similar to those arising from the testes of men
    • Embryo-like neoplasms
      • Teratomas and their subtypes
      • Dysgerminomas: The female version of the male seminoma (comprised of immature germ cells)
    • Extraembryonic fetal-derived (placenta-like) cell populations
      • Yolk sac/primitive placenta forms (epithelial neoplasms differentiate into yolk sac tumors)
    • Rare OGCTs
      • Pure embryonal carcinomas
      • Nongestational choriocarcinomas
      • Pure polyembryoma.
    • Mixed germ cell tumors (teratoma with yolk sac, dysgerminoma, and/or embryonal carcinoma)
  • Extragonadal germ cell tumors (GCTs): no evidence of a primary tumor in the testes or ovaries
    • Typically arise in midline locations,
    • Specific sites vary with age
    • The most common sites in order of frequency
    • In adults:
      • Anterior mediastinum
        • Mature teratomas
        • Immature teratoma
        • Mediastinal seminoma
        • Mediastinal non-seminomatous GCT:
          • Yolk sac tumor (most common, pure or mixed)
          • Choriocarcinoma (less common)
          • Embryonal carcinoma (infrequent)
          • Mixed GCTs (a mixture of teratoma, seminoma, and other cell types)
      • Retroperitoneum
        • Retroperitoneal seminoma
        • Retroperitoneal non-seminomatous GCTs (Embryonal carcinoma is common)
        • Retroperitoneal teratomas (rare)
      • Pineal and suprasellar regions
    • In infants and young children:
      • Sacrococcygeal
      • Intracranial GCTs


Types Subtypes Signs and Symptoms Histopathology Lab finding Treatment Prognosis
Gonadal

Seminoma (Testis)

Gross: pale gray to yellow nodules that are uniform or slightly lobulated and often bulge from the cut surface
  • Complete blood count and blood chemistry tests.
  • Abnormal serum tumor marker levels (LDH, HCG).[1]
  • CT: Metastases to the para-aortic, inguinal, or iliac lymph nodes. Visceral metastasis may also be seen.
  • Pelvic MRI: may be diagnostic. multinodular tumors of uniform signal intensity
  • Hypo- to isointense on T2-weighted images and inhomogenous enhancement on contrast enhanced T1-weighted images.
  • Other diagnostic studies for seminoma include biopsy, FDG-PET scan, and bone scan.

Dysgerminoma

(Ovarian germ cell tumor)

  • Chemotherapy: except those with stage 1a, stage 1a, 1b dysgerminoma
  • Radiotherapy:


** Dysgerminoma is radiosensitive.

** Radiotherapy is not anymore the first option of treatment for dysgerminoma considering its association with ovarian failuredevelopment.

  • Surgery: for diagnostic grading and therapy depending on if the patient prefers to preserve the ovary or not.


Germinoma (Brain)

Extragonadal Embryonic

Teratoma

  • AFP
  • MSAFP
  • CT scans are often used to diagnose teratoma.


  • For malignant teratomas, usually, surgery is followed by chemotherapy.
  • Teratomas that are in surgically inaccessible locations, or are very complex, or are likely to be malignant (due to late discovery and/or treatment) sometimes are treated first with chemotherapy.


The prognosis of teratoma depends on the following:

  • Whether or not the tumor can be removed by surgery.
  • The size and location of the tumor
  • The patient’s general health
  • Teratomas are not dangerous for the fetus unless there is either a mass effect or a large amount of blood flow through the tumor (known as vascular steal). The mass effect frequently consists of obstruction of normal passage of fluids from surrounding organs. The vascular steal can place a strain on the growing heart of the fetus, even resulting in heart failure, and thus must be monitored by fetal echocardiography. After surgery, there is a risk of regrowth in place, or in nearby organs [1]

Extraembryonic

Coriocarcinoma

Yolk sac tumor

References


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