Choriocarcinoma Syndrome as an Initial Presentation of Testicular Cancer

Abstract

Choriocarcinoma syndrome (CS) is a rare clinical entity within the spectrum of nonseminomatous germ-cell tumors (NSGCT). It is characterized by the abrupt establishment of rapidly progressive and hemorrhagic tumors associated with very high levels of the beta fraction of human chorionic gonadotropin (β-hCG) and with a very poor prognosis, particularly in patients with β-hCG values above 50,000 IU/L. We present the case of a 17-year-old man with a sudden onset nonmassive hemoptysis. Physical examination revealed a right testicular mass. Imaging studies showed metastatic lung, bone, and retroperitoneal disease. β-hCG serum levels were 222,493.21 IU/L, AFP 1.56 ng/mL, and DHL 457 IU/L. Histopathological study after right radical orchiectomy showed a mixed germ-cell tumor. Based on poor-risk characteristics, chemotherapy was started with an adequate clinical response. Physicians should be aware of the potential complications of CS in the treatment of testicular cancer with high β-hCG levels since they could be associated with a rapidly progressive and high-volume disease. Patients in this category should be referred to the centers experienced in the treatment of advanced germ-cell tumors. Due to the severity of the presentation, hemodynamic monitoring, ideally in an intensive care unit, is essential as well as timely administration of cytotoxic treatment.

A rare manifestation of choriocarcinoma syndrome in a child with primary intracranial germ cell tumor and extracranial metastases: A case report and review of the literature

“…The clinical course of our patient aligns with the description of choriocarcinoma syndrome with pulmonary hemorrhage after initiation of chemotherapy in the setting of extremely elevated β‐hCG. The pathogenesis of choriocarcinoma syndrome is not well described but is hypothesized to involve tumor invasion into adjacent blood vessels or development of aneurysms secondary to necrotic tumor emboli, which perhaps occurred in our patient 5,13 . This highlights the importance of considering rare but life‐threatening complications like choriocarcinoma syndrome in pediatric patients with GCTs, while providing aggressive management.…”

“…Although intracranial hemorrhage has been reported with choriocarcinoma syndrome in the setting of extragonadal mixed GCTs, those cases have primarily been in adult patients with either CNS metastases or infarcts presumably secondary to tumor emboli 13 . Most reports of choriocarcinoma syndrome with pulmonary hemorrhage are of adult patients with primary gonadal disease and hemorrhagic manifestations at presentation or soon after starting systemic therapy, 5,14,15 or with gestational choriocarcinoma, 16 barring one report by Peng and colleagues describing a pediatric patient with nongestational ovarian carcinoma 9 . In that report, a 12‐year‐old female had a presentation similar to our patient's, with choriocarcinoma syndrome manifesting soon after the initiation of chemotherapy.…”

“…In extra‐CNS GCTs with complete or partial components of choriocarcinoma, the hemorrhagic propensity of metastatic foci is described as choriocarcinoma syndrome. This complication has been reported in about 10% of gonadal and extragonadal advanced GCTs, mostly in adults, at various sites of metastasis 5 . Choriocarcinoma syndrome typically develops soon after initiation of chemotherapy; however, this phenomenon can also occur at presentation 6 .…”

“…The hallmark of this syndrome is markedly elevated serum β‐human chorionic gonadotropin (β‐hCG) and hemorrhage at sites of metastatic disease. β‐hCG levels >50,000 IU/L are associated with very poor prognosis 5,6 . Most reports of choriocarcinoma syndrome are in adults, aged 20–30 years, when GCTs are most common.…”

An Exceptionally Rare Cause of Refractory Gastrointestinal Bleed: Choriocarcinoma Syndrome

“…Choriocarcinoma syndrome (CS) is an exceedingly rare and often fatal manifestation of choriocarcinoma and is likely a reflection of massive tumor burden [ 2 ]. The majority of cases present with pulmonary hemorrhage; however, there are rare cases that demonstrate spontaneous hemorrhage from the gastrointestinal tract (GIT), liver, and brain [ 3 , 4 ]. CS usually develops after initiation of chemotherapy and is rarely the initial feature of choriocarcinoma [ 5 ].…”