Primary yolk sac tumor of the urachus: Case report with immunohistochemical and flow cytometric studies
Journal
Archives of Pathology and Laboratory Medicine
Journal Volume
126
Journal Issue
9
Pages
1106-1109
Date Issued
2002
Author(s)
Abstract
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.
SDGs
Other Subjects
alpha fetoprotein; bleomycin; cisplatin; cytokeratin; DNA; etoposide; protein MDM2; protein p53; trypsin inhibitor; abdominal distension; abdominal mass; abdominal tumor; abdominal wall; adenocarcinoma; adjuvant chemotherapy; article; cancer surgery; cell cycle S phase; chemotherapy; computer assisted tomography; diagnostic error; diploidy; embryonal rhabdomyosarcoma; flow cytometry; follow up; hemoperitoneum; histology; hormone blood level; human; human tissue; immunohistochemistry; infant; male; metastasis; necrosis; peripheral vein; physical examination; protein expression; testis; tumor cell; tumor volume; umbilical vein; umbilicus; urachus; vomiting; yolk sac tumor; alpha 1-Antitrypsin; alpha-Fetoproteins; Antineoplastic Combined Chemotherapy Protocols; Bleomycin; Chemotherapy, Adjuvant; Cisplatin; DNA, Neoplasm; Endodermal Sinus Tumor; Etoposide; Flow Cytometry; Humans; Immunohistochemistry; Infant; Keratins; Male; Nuclear Proteins; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-mdm2; Tomography, X-Ray Computed; Treatment Outcome; Tumor Markers, Biological; Urachus; Urinary Bladder Neoplasms
Type
journal article