Reported cases of yolk sac tumors in head and neck region over the last 20 years

YSTs of the head and neck appear to have a female predilection [Table 1], in contrast to the gonadal germ cell tumors which frequently involve males. With respect to the laterality, YSTs in the head and neck region frequently seems to arise on the right side similar to the gonadal germ cell tumors. From [Table 1], it is clear that the YST in head and neck region is the disease of childhood. According to the MAKEI group, among all those children with head and neck germ cell tumor, there is a statistically higher chance of having malignant component (of YST) in those over the age of 1 year, as compared to those diagnosed in infancy.[1] This peculiar observation seems to hold good even for the index case and for the majority listed in [Table 1].

In general, the YSTs in head and neck are commonly found to involve sino-nasal and orbital regions [Table 1]. However, it was seen in the soft tissue of the temporal and infratemporal fossae in our case. Similar involvement of soft tissue areas like parapharyngeal space and skull base has also been described rarely in the literature.[9],[10]

In the MAKEI study group, all patients with YST except one had a favorable outcome with multimodal therapy. It is interesting to find that all children with YST in the group were over the age of 1 year, suggesting an improved survival with age. Similarly, cases in [Table 1] also seem to follow the same trend. Among the three cases described by Kusumakumari, two patients who had treatment failure were actually of age <1 href="https://www.thieme-connect.com/products/ejournals/html/10.4103/ijmpo.ijmpo_135_17#JR_2" xss=removed>2] It seems that the age of diagnosis of YST influences the overall prognosis in those affected. However, this fact needs to be verified by a systematic review or meta-analysis.

Another important factor which seems to have a direct bearing on survival in YSTs of head and neck is the therapeutic regimen. Unlike for benign germ cell tumors, mere excision of the YSTs may not be adequate in achieving the cure. In fact, as it is evident from [Table 1], any treatment modality for YST, which is less than multimodality regimen, has resulted in the unfavorable outcome. The combination chemotherapy based on high-dose cisplatin along with surgical excision and/or radiotherapy seems to be the optimal treatment regimen for treating YSTs, and this has contributed to the betterment of disease-free survival rates in recent times [Table 1]. However, radiotherapy is known to be deleterious in children and might hamper their overall growth. Thus, surgery is the preferred modality in children along with combination chemotherapy consisting of cisplatin, etoposide/ifosfamide, and bleomycin.[1] In our case, although the excised specimen did not show any residual tumor tissue, we believe that the surgical excision has helped in achieving a complete cure, by eradicating the microscopic and molecularly altered tissue. Similar results of favorable outcome have been reported in the past by excising the pathologically tumor-free primary site, after the significant remission with chemotherapy.[9] Furthermore, there are reports of recurrence at the unattended primary sites even after dramatic response to the chemotherapy.[3]

In summary, the YST of head and neck commonly involve children over the age of 1 year. However, it seems to be more aggressive if diagnosed in children of <1>

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflict of Interest

There are no conflicts of interest.

References