9071/3 Embryonal carcinoma, infantile



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C71
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Yolk sac tumour is a non-seminomatous malignant germ cell tumour composed of primitive germ cells. It is the most common malignant germ cell tumour in the pediatric population. The tumour mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumour is very responsive to chemotherapy regimens that include cisplatinum.

Cervical yolk sac tumours are polypoid, friable masses that protrude into the vagina. Patients typically present with abnormal vaginal bleeding1
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Thymus
ICD-O-3 topography code: C37
ICD10: C37
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Ovary
ICD-O-3 topography code: C56
ICD10: C56
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.

Histological variants:
> Polyvesicular vitelline tumour
> Glandular variant
> Hepatoid variant



Testis/Male genital organs
ICD-O-3 topography code: C62-C63
ICD10: C62-C63
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumor is very responsive to chemotherapy regimens that include cisplatinum.



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Yolk sac tumour is a non-seminomatous malignant germ cell tumour composed of primitive germ cells. It is the most common malignant germ cell tumour in the pediatric population. The tumour mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). This tumour is very responsive to chemotherapy regimens that include cisplatinum.

In the vulva, yolk sac tumour is very rare. It occurs predominantly in children and young women
2
Click to access Pubmed
Flanagan CW, Parker JR, Mannel RS, Min KW, Kida M (1997)
Primary endodermal sinus tumor of the vulva: a case report and review of the literature.
Gynecol Oncol 66: 515-8



3
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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