Successful treatment of non-midline primary malignant germ cell tumors with yolk sac components in neonates: report of 2 cases

Abstract

Here, the authors present 2 cases of nongerminomatous germ cell tumor (NGGCT): a neonate with a mixed malignant germ cell tumor, 5% yolk sac tumor (YST) and 95% immature teratoma components, originating from the right mesial temporal lobe; and a 2-month-old infant with a pure YST originating from the left middle cranial fossa. These tumors with yolk sac components, which are thought to have a poor prognosis, were successfully treated with complete tumor resection alone and subtotal tumor resection with chemotherapy, respectively. Event-free survival exceeds 5 years for each patient even though neither received radiotherapy. The authors highlight the role of radical surgery and the successful treatment of neonatal YST with aggressive resection (and chemotherapy in 1 case) while avoiding radiation therapy. They also report the very rare non-midline location of these neonatal NGGCTs and emphasize the importance of considering YSTs and mixed NGGCTs with YST components in the differential diagnosis of non-midline hemispheric or skull base tumors in newborns.

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…The most common type of malignant germ cell tumor encountered within the sinonasal cavity is the yolk sac tumor (YST), also commonly referred to as endodermal sinus tumor 1943,1944 . Often found in younger patients and in females, these tumors can be pure or mixed, with most of them being the mixed type 1945–1947 . Mixed YSTs are often found in conjunction with other germ cell tumor components, such as embryonal cell carcinoma or teratoma.…”

“…Many of these are quite sensitive to chemotherapy and a variety of agents have been previously used 1955–1958 . This includes chemotherapeutics such as bleomycin, etoposide, cisplatin, carboplatin, ifosfamide, vincristine, 5‐fluorouracil, and others 1706,1943–1961 . Although tumors may be responsive to multiple forms of therapy, recurrence is commonly local, and early metastases may be present in up to 50% of cases 1961 …”

“…It can occur primarily within the sinonasal cavity or be metastatic from a primary gonadal site 1962,1963 . These may resemble many other tumors radiographically and histologically, but unique to these NSGCTs is the staining pattern and elevated serum levels of beta human chorionic gonadotropin (ß‐hCG) 1946,1964 . Again, this is a particular tumor marker that can help with diagnosis but can also aid in prognostication and cancer surveillance.…”