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Features of a Metastatic Poorly Differentiated Carcinoma from an Unknown Primary Site Potentially Responsive to Chemotherapy (Unrecognized Extragonadal Germ Cell Cancer Syndrome)

Description

Richardson et al described features of metastatic poorly differentiated carcinomas from an unknown primary site which respond to cisplatin-based or other chemotherapeutic regimens. The group was initially called the "extragonadal germ cell cancer syndrome" or "atypical teratoma syndrome" since many cases were thought to represent metastatic germ cell tumors.


 

Features:

(1) metastatic poorly differentiated or undifferentiated carcinoma from an unknown primary site

(2) patient age < 50 years

(3) involvement in one of the following:

(3a) a midline structure (mediastinum or retroperitoneum)

(3b) multiple pulmonary nodules

(3c) lymph nodes

(4) elevated serum levels of alpha-fetoprotein (AFP) or human chorionic gonadotropin (HCG)

(5) evidence of rapid tumor growth

(6) tumor responsive to radiation and/or chemotherapy

 

A patient who meets these conditions may respond to a cisplatin-based or other chemotherapeutic regimen.

 

NOTE: Today many tumors of unknown primary site can be better characterized with newer diagnostic and imaging techniques (immunohistochemistry, cytogenetics, MRI, etc.).

 


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