Primary yolk sac tumor of the urachus.
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 alpha-fetoprotein, alpha(1)-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.
Huang
Hsuan-Ying
HY
Department of Pathology, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University, Kaohsiung, Taiwan.
Ko
Sheung-Fat
SF
Chuang
Jiin-Haur
JH
Jeng
Yung-Ming
YM
Sung
Ming-Tse
MT
Chen
Wei-Jen
WJ
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001761:Bleomycin / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002945:Cisplatin / Q000627:therapeutic use; D004273:DNA, Neoplasm / Q000032:analysis; D018240:Endodermal Sinus Tumor / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D005047:Etoposide / Q000627:therapeutic use; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007633:Keratins / Q000032:analysis; D008297:Male; D009687:Nuclear Proteins; D011518:Proto-Oncogene Proteins / Q000032:analysis; D051736:Proto-Oncogene Proteins c-mdm2; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014497:Urachus / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D001749:Urinary Bladder Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D000515:alpha 1-Antitrypsin / Q000032:analysis; D000509:alpha-Fetoproteins / Q000032:analysis
D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D009687:Nuclear Proteins; D011518:Proto-Oncogene Proteins; D000515:alpha 1-Antitrypsin; D000509:alpha-Fetoproteins; D001761:Bleomycin; D007633:Keratins; D005047:Etoposide; C497206:MDM2 protein, human; D051736:Proto-Oncogene Proteins c-mdm2; D002945:Cisplatin