Primary Extragonadal Yolk Sac Tumor Arising from the Urachus: A Case Report with Literature Review

  • Kenneth Steven Taylor PA, Department of Pathology and Laboratory Medicine Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • Kristian Theo Schafernak Department of Pathology and Laboratory Medicine Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • Lisa McMahon Department of Surgery Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • David Notrica Department of Surgery Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • Masayo Watanabe Center for Hematology and Oncology Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • Carrie Schaefer Department of Radiology Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
  • Paul S Dickman Department of Pathology and Laboratory Medicine Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
Keywords: Extragonadal germ cell tumor, yolk sac tumor, urachus

Abstract

Germ cell tumors in extragonadal sites are uncommon; those arising in the urachus are exceedingly rare and limited to a few anecdotal reports. Herein we document an occurrence of yolk sac tumor in a 9-month-old male who presented with abdominal distension. Laboratory investigation revealed elevated lactate dehydrogenase and α-fetoprotein levels; imaging studies were inconclusive as to tumor type or site of origin. The tumor was resected from the base of the umbilicus and histologic examination revealed neoplastic cells arranged in solid nests or microcystic patterns in a fibromyxoid stroma. Schiller-Duval bodies were conspicuous. Immunohistochemical analysis showed lesional cells reactive to α-fetoprotein, SALL4, placental alkaline phosphatase, Glypican-3 and CD117. No teratomatous or other germ cell elements were identified. These findings supported a diagnosis of pure yolk sac tumor arising from the urachus; the clinical, radiological and pathological findings are discussed.DOI:10.276/APALM.1832

Author Biography

Kenneth Steven Taylor, PA, Department of Pathology and Laboratory Medicine Phoenix Children's Hospital 1919 E Thomas Rd Phoenix, Arizona 85016 USA
Department of Pathology and Laboratory MedicineMHS, PA-CM

References

1. Romero-Rojas AE, Messa-Botero OA, Melo-Uribe MA, Diaz-Perez JA, Chinchilla-Olaya SI. Primary yolk sac tumor of the urachus. Int J Surg Pathol. 2011; 19(5):658-661.
2. Sheldon CA, Clayman RV, Gonzalez R, Williams RD, Fraley EE. Malignant urachal lesions. J Urol. 1984; 131:1-8.
3. Huang HY, Ko SF, Chuang JH, Jeng YM, Sung MT, Chen WJ. Primary yolk sac tumor of the urachus. Arch Pathol Lab Med. 2002; 126:1106-1109.
4. D’Allessio A, Verdelli G, Bernardi M, et al. Endodermal sinus (yolk sac) tumor of the urachus. Eur J Pediatric Surg. 1994; 4(3):180-181.
5. Harano K, Ando M, Sasajima Y, et al. Primary yolk sac tumor of the omentum: a case report and literature review. Case Rep Oncol. 2012; 5:671-675.
6. Lim SH, Kim YH, Yim GW, et al. Primary omental yolk sac tumor. Obstet Gynecol Sci. 2013; 56(6):412-415.
7. Zeremski V, Mawrin C, Fischer T, Schalk E. Diagnostic and therapeutic challenges in extragonadal yolk sac tumor with hepatoid differentiation: a case report. Mol Clin Oncol. 2017; 6:79-82.
8. Ravishankar S, Malpica A, Ramalingam P, Euscher ED. Yolk sac tumor in extragonadal pelvic sites. Still a diagnostic challenge. Am J Surg Pathol. 2017; 41(1):1-11.
9. Joseph MG, Fellows FG, Hearn SA. Primary endodermal sinus tumor of the endometrium. A clinicopathologic, immunocytochemical, and ultrastructural study. Cancer. 1990; 65:297-302.
10. Lin SC, Li XH, Sun CH, et al. CT findings of intrarenal yolk sac tumor with tumor thrombus extending into the inferior vena cava: a case report. Korean J Radiol. 2014; 15(5):641-645.
11. Euscher ED. Unusual presentations of gynecologic tumors. Extragonadal yolk sac tumor of the vulva. Arch Pathol Lab Med. 2017; 141:293-297.
12. Arumugam D, Thandavarayan P, Chidambaram L, Boj S, Marudasalam S. Primary nasopharyngeal yolk sac tumor: a case report. J Clin Diagn Res. 2016; 10(5):ED06-ED07.
13. El-Bahrawy M. α-Fetoprotein-producing non-germ cell tumors of the urological system. Rev Urol. 2011;13(1):14-19.
14. Mori H, Matsubara N, Fujii M, et al. Alpha-fetoprotein producing rhabdomyosarcoma of the adult liver. Pathol Int. 1979;29(3):485-491.
15. Li YK, Zheng Y, Lin JB, et al. Radiological-pathological correlation of yolk sac tumor in 20 patients. Acta Radiol. 2016;57(1):98-106.
16. Li YK, Zheng, Lin JB, et al. CT imaging of ovarian yolk sac tumor with emphasis on differential diagnosis. Sci Rep. 2015; 5:11000; doi: 10.1038/srep11000.
17. Baba T, Su S, Umeoka S, et al. Advanced extragonadal yolk sac tumor serially followed up with 18F-fluorodeoxyglucose-positron emission tomography and computerized tomography and serum alpha-fetoprotein. J Obstet Gynaecol Res. 2012;38(3):605-609.
18. Takahashi M, Kanamori Y, Takahashi M, Momose T, Iwanaka T. Detection of a metastatic lesion and tiny yolk sac tumors in two teenage patients by FDG-PET: report of two cases. Surg Today. 2014;44:1962-1965.
19. Liu M, Chen G, Fu Z, Li Z, Li Q. Occult mediastinal yolk sac tumor producing α-fetoprotein detected by 18F-FDG-PET. Clin Nuc Med. 2016;41(7):585-586.
20. Gilbert KL, Bergman S, Dodd LG, Volmar KE, Creager AJ. Cytomorphology of yolk sac tumor of the liver in fine-needle aspiration: a pediatric case. Diagn Cytopathol. 2006;34(6):421-423.
21. Gupta R, Mathur SR, Arora VK, Sharma SG. Cytology features of extragonadal germ cell tumors. A study of 88 cases with aspiration cytology. Cancer. 2008;114(6):504-511.
22. Kataria SP, Misra K, Singh G, Kumar S. Cytological findings of an extragonadal yolk sac tumor presenting at an unusual site. J Cytol. 2015;32(1):62-64.
23. McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: a review with emphasis on pathologic features, clinical prognostic variables, and differential diagnostic considerations. Adv Anat Pathol. 2007;14(2):69-92.
24. Frazier AL, Hale JP, Rodriguez-Galindo C, et al. Revised risk classification for pediatric extracranial germ cell tumors based on 25 years of clinical trial data from the United Kingdom and United States. J Clin Oncol. 2015;33(2):195-201.
25. Murray MJ, Huddart RJ, Coleman N. The present and future of serum diagnostic tests for testicular germ cell tumors. Nat Rev Urol. 2016;13(12):715-725.
Published
2018-03-22
Issue
Vol 5 No 3 (2018)
Section
Case Report

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