Primary yolk sac tumor of the urachus: Case report with immunohistochemical and flow cytometric studies

Hsuan Ying Huang, Sheung Fat Ko, Jiin Haur Chuang, Yung Ming Jeng, Ming Tse Sung, Wei Jen Chen*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

26 Scopus citations


Pure yolk sac tumor is the most common malignant gonadal tumor of infants and toddlers. However, the majority of extragonadal germ cell tumors in the midline are either seminomas (germinomas) or teratomas, and pure yolk sac tumors account for only a small fraction of these lesions. To date, only 1 primary urachal pure yolk sac tumor has been reported in the literature. We describe another case, occurring in a 7-month-old male infant who presented with a rapidly enlarging intra-abdominal tumor with marked engorgement of the superficial venous plexus around the umbilicus. With periodic follow-up for 3 years following surgical extirpation of the tumor and adjuvant chemotherapy, this patient is still alive without evidence of disease. Notably, the glandular elements predominating in the frozen sections resulted in the initial misdiagnosis of the tumor as a urachal adenocarcinoma, although the entirely resected specimen revealed typical histologic patterns and Schiller-Duval bodies. Immunohistochemistry showed that the tumor cells were diffusely reactive to α-fetoprotein, α-antitrypsin, and cytokeratin. Tumor cells were negative for p53 protein, but revealed overexpression for MDM2 protein. Flow cytometry demonstrated a diploid DNA content with S-phase being as high as 55.36%. This case emphasizes that pure yolk sac tumor can occur primarily in the remnant of the urachus in young children.

Original languageEnglish
Pages (from-to)1106-1109
Number of pages4
JournalArchives of Pathology and Laboratory Medicine
Issue number9
StatePublished - 09 2002
Externally publishedYes


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