Description

The "Choriocarcinoma Syndrome" (also known as very high risk NSGCT) is a rare syndrome seen in a patient with metastatic nonseminomatous germ cell tumor (NSGCT).


 

Clinical and laboratory features:

(1) rapid development of multiple large metastases, resulting in a high tumor burden

(2) very high serum HCG concentration (some use >= 25,000 IU/L, others >= 50,000 IU/L)

(3) dyspnea or low PaO2 (< 80 mm Hg) is often seen at presentation

(4) some patients have gynecomastia or breast tenderness

 

Fulminant complications following standard chemotherapy:

(1) massive intra-alveolar hemorrhage

(2) acute respiratory distress syndrome

(3) acute pneumonia

(4) hemorrhage at other metastastic sites (liver, brain, other) associated with sudden tumor necrosis

 

The mortality rate for this subgroup of patients is high, and prompt and careful management can be life-saving. Initial chemotherapy may be at reduced dosages to reduce hemorrhagic complications. Control of hemorrhage is especially important, and massive hemorrhage can be controlled by infusion of recombinant factor VIIa.

 


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