Fo r c i t at i o n: D e g t ya rev V. A . , G rac h ev N . S . , M o ise e n ko R . A . , S t rad o ms kaya T.V. , Te l es hova M .V. , B a bas k i n a N .V. , M i t rofa nova A . M . , Vo roz htsov I . N . , Ut a l i eva D.T. , S ha m a ns kaya T.V. , Kacha nov D.Yu . M a l i g na nt g e rm cell tumors of the head and neck region in children. Head and neck. Russian Journal. 2022;10(1):35–45 (In Russian).

Doi: 10.25792/HN.2022.10.1.35–45

Germ cell tumors (GCT) are a heterogeneous group of neoplasms occurring predominantly in childhood and including several histological types characterized by different lesion localization and different clinical symptoms.  Malignant GCT, such as yolk sac tumor (YST), in the head and neck region are extremely rare, therefore, only few clinical observations have been described in the literature.

The aim of this study was to analyze the clinical data and treatment results of patients with yolk sac tumors of the head and neck region.

Material and methods. During the period from 02.2012 to 02.2021 (108 months), 4 patients with YST of the head and neck area were treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. Median age at diagnosis was 22.2 months (range 6.9–36.2 months). Primary tumor was located in the orbit in 2 (50%) patients, in maxilla in 1 (25%) patient, and in nasal cavity in 1 (25%) case. Median alfa-fetoprotein (AFP) level before therapy was 4395 ng/ml (range 541–19453 ng/ml). The diagnosis was morphologically confirmed in all cases. Distant metastases in lungs were detected in 1 (25%) patient. The stage distribution was the following: II – 1 (25%), III – 2 (50%), IV – 1 (25%).

Results. All the patients have received combined treatment according to the protocols of the German MAKEI group. Orbito-facial resections were required in 2 (50%) cases. By the time of the analysis, all patients are alive with no signs of recurrence and the median follow-up of 30.9 months (range 12.7–89.0).

Conclusions. The main conclusion of this study is that the combined treatment, which is the standard of care, allows to achieve high overall survival rates, however, in some cases, complex anatomical localization of the tumor may require mutilation operations.

Key words: germ cell tumor, yolk sac tumor, head and neck, chemotherapy, surgery

Conflicts of interest. The authors have no conflicts of interest to declare.

Funding. There was no funding for this study.

For citation: Degtyarev V.A., Grachev N.S., Moiseenko R.A., Stradomskaya T.V., Teleshova M.V., Babaskina N.V., Mitrofanova A.M., Vorozhtsov I.N., Utalieva D.T., Shamanskaya T.V., Kachanov D.Yu.

Malignantgerm cell tumors of the head and neck region in children. Head and neck. Russian Journal. 2022;10(1):35–45  (In Russian).

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