Byline: K. Devaraja, Kapil. Sikka, Pankaj. Singh
Yolk sac tumor (YST) is the most common malignant germ cell tumor of head and neck region, constituting around 1% of all malignant germ cells. The rarity of the tumor has led to a relatively sparse collective description of clinical and prognostic factors of such a tumor affecting head and neck region. In this case report with review of the literature, we have highlighted certain peculiar features of YSTs affecting the head and neck region. They seem to commonly affect female children. These tumors seem to fare poorly in children of <1 year of age. Multimodality of treatment consisting of combination chemotherapy with the cisplatin-based regimen and surgery and/or radiotherapy helps in achieving long-term disease-free survival in those affected.
Yolk sac tumor (YST) also known as 'endodermal sinus tumor' is the most common malignant germ cell tumor involving gonads. Although rare, these tumors are also known to arise from an extragonadal site including the head and neck region. While sharing a report of a large YST of temporal region in a 3-year-old boy, we are highlighting certain peculiarities of YST in the head and neck region.
A 3-year-old boy presented with a painless, progressive swelling in the right temporal region for the past 1 year. The swelling of 15 cm x 10 cm was involving temporal region, infratemporal fossa, and parotid region, giving the appearance of a conjoint second head as shown radiologically in [Figure 1]. Open biopsy from the lesion showed mixed germ cell tumor consisting of teratoma and YST. Whole-body positron emission tomography ruled out metastasis. He was given five cycles of cisplatin, bleomycin, and etoposide combination chemotherapy, for which tumor responded significantly with 90% reduction in volume as shown radiologically in [Figure 2]. Although the residual tumor was excised, the pathological examination of surgical specimen did not show any residual tumor. The patient was kept under close follow-up, and he is disease free at 2 years postsurgery with serum alpha-fetoprotein levels of 0.88 ng/ml (normal range: 0.89-8.78 ng/ml) and beta-hCG levels of...