Pure yolk sac tumor of sacrococcygeal region
DOI:
https://doi.org/10.4322/acr.2021.287Keywords:
Child, Preschool, Sacrococcygeal Region, TeratomaAbstract
The sacrococcygeal region is the most common site for the extragonadal germ cell tumors comprising seminomatous and non-seminomatous tumors. Seminomatous tumors are seminomas, and non-seminomatous tumors comprise mainly teratoma (mature and immature), yolk sac tumor (YST), embryonal carcinoma (EC), and choriocarcinoma. These tumors occur in newborns, infants, and adolescents. Other common sites for extragonadal germ cell tumors are the brain and mediastinum, although they may occur anywhere in the body. These tumors may occur in mixed as well as pure form. So, sectioning from different areas should be done before labeling them as pure germ cell tumors. YST, in its pure form, is rare and therefore should not be missed as it is chemosensitive. The patient should be thoroughly assessed clinically. Imaging also becomes necessary while evaluating swelling in the sacrococcygeal region and can aid in differentials. When the clinical and imaging suspicion of either Sacrococcygeal teratoma or other germ cell tumor is high, serum biomarkers as alfa-fetoprotein should be requested. The serum levels are necessary and should be done preoperatively, postoperatively, and during the course of chemotherapy as follow-up. However, the final diagnosis rests on the histopathological diagnosis. We report one such case of pure YST in the sacrococcygeal region in a 9-month-old female child. The imaging suggested sacrococcygeal teratoma type 4, and high alfa-fetoprotein levels were determined postoperatively.
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Gharpure V. Sacrococcygeal teratoma. J Neonatal Surg. 2013;2(2):28. http://dx.doi.org/10.47338/jns.v2.40. PMid:26023448.
Davidoff AM, Hebra A, Bunin N, Shochat SJ, Schnaufer L. Endodermal sinus tumor in children. J Pediatr Surg. 1996;31(8):1075-8, discussion 1078-9. http://dx.doi.org/10.1016/S0022-3468(96)90090-1. PMid:8863237.
Teilum G. Classification of endodermal sinus tumour (mesoblatoma vitellinum) and so-called "embryonal carcinoma" of the ovary. Acta Pathol Microbiol Scand. 1965;64(4):407-29. http://dx.doi.org/10.1111/apm.1965.64.4.407. PMID: 5318716.
Yoon HM, Byeon SJ, Hwang JY, et al. Sacrococcygeal teratomas in newborns: a comprehensive review for the radiologists. Acta Radiol. 2018;59(2):236-46. http://dx.doi.org/10.1177/0284185117710680. PMid:28530139.
Talerman A, Haije WG, Baggerman L. Serum alphafetoprotein (AFP) in diagnosis and management of endodermal sinus (yolk sac) tumor and mixed germ cell tumor of the ovary. Cancer. 1978;41(1):272-8. http://dx.doi.org/10.1002/1097-0142(197801)41:1<272::AID-CNCR2820410138>3.0.CO;2-Z. PMid:75054.
Teilum G. Special tumors of ovary and testis and related extragonadal lesions: comparative pathology and histological indentification. Copenhagen: Munksgaard; 1976.
Talerman A, Vang R. Germ cell tumors of the ovary. In: Kurman RJ, Ellenson LH, Ronnett BM, editors. Blaustein's pathology of female genital tract. 6th ed. USA: Springer science+business media LLC; 2011. p. 857-862. http://dx.doi.org/10.1007/978-1-4419-0489-8_16.
Papic JC, Finnell SM, Slaven JE, Billmire DF, Rescorla FJ, Leys CM. Predictors of ovarian malignancy in children: overcoming clinical barriers of ovarian preservation. J Pediatr Surg. 2014;49(1):144-7, discussion 147-8. http://dx.doi.org/10.1016/j.jpedsurg.2013.09.068. PMid:24439599.
Billmire DF. Germ cell tumors. Surgical Clinics. 2006;86(2):489-503, xi. PMid:16580936.
Norgaard‐Pedersen B, Albrechtsen R, Teilum G. Serum alpha‐foetoprotein as a marker for endodermal sinus tumor (yolk sac tumor) or a vitelline component of ‘teratocarcinoma’. Acta Pathol Microbiol Scand [A]. 1975;83(6):573-89. http://dx.doi.org/10.1111/j.1699-0463.1975.tb01385.x. PMid:52995.
Ein SH, Mancer K, Adeyemi SD. Malignant sacrococcygeal teratoma—endodermal sinus, yolk sac tumor—in infants and children: a 32-year review. J Pediatr Surg. 1985;20(5):473-7. http://dx.doi.org/10.1016/S0022-3468(85)80468-1. PMid:3903096.
Hawkins EP, Finegold MJ, Hawkins HK, Krischer JP, Starling KA, Weinberg A. Nongerminomatous malignant germ cell tumors in children: a review of 89 cases from the Pediatric Oncology Group, 1971–1984. Cancer. 1986;58(12):2579-84. http://dx.doi.org/10.1002/1097-0142(19861215)58:12<2579::AID-CNCR2820581204>3.0.CO;2-V. PMid:3022907.
De Backer A, Madern GC, Pieters R, et al. Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors. Eur J Pediatr Surg. 2008;18(1):1-6. http://dx.doi.org/10.1055/s-2007-989399. PMid:18302061.
Wang F, Liu A, Peng Y, et al. Diagnostic utility of SALL4 in extragonadal yolk sac tumors: an immunohistochemical study of 59 cases with comparison to placental-like alkaline phosphatase, alpha-fetoprotein, and glypican-3. Am J Surg Pathol. 2009;33(10):1529-39. http://dx.doi.org/10.1097/PAS.0b013e3181ad25d5. PMid:19574883.
Merchant A, Stewart RW. Sacrococcygeal yolk sac tumor presenting as subcutaneous fluid collection initially treated as abscess. South Med J. 2010;103(10):1068-70. http://dx.doi.org/10.1097/SMJ.0b013e3181efb572. PMid:20818302.
Khanchel-Lakhoua F, Koubâa-Mahjoub W, Jouini R, Bel Haj Salah M, Kaabar N, Chadli-Debbiche A. Sacrococcygeal yolk sac tumor: an uncommon site. APSP J Case Rep. 2012;3(3):17. PMid:23061033.
Yoshida M, Matsuoka K, Nakazawa A, et al. Sacrococcygeal yolk sac tumor developing after teratoma: a clinicopathological study of pediatric sacrococcygeal germ cell tumors and a proposal of the pathogenesis of sacrococcygeal yolk sac tumors. J Pediatr Surg. 2013;48(4):776-81. http://dx.doi.org/10.1016/j.jpedsurg.2012.08.028. PMid:23583133.
Pawar NP, Mahajan SV, Chaudhari RA, Chavan SD. Extragonadal GCT: A rare case report of sacrococcygeal pure yolk sac tumor. Indian J Pathol Microbiol. 2013;56(3):329-31. http://dx.doi.org/10.4103/0377-4929.120421. PMid:24152532.
Ben Nsir A, Darmoul M, Arous SB, Hattab N. Metastatic sacrococcygeal yolk sac tumor: a misleading diagnosis. J Neurosci Rural Pract. 2015;6(3):395-8. http://dx.doi.org/10.4103/0976-3147.158772. PMid:26167024.
Mondal K, Mandal R. Bilateral lung metastases unveils an asymptomatic sacrococcygeal yolk sac tumor. Indian J Pathol Microbiol. 2017;60(4):565-7. http://dx.doi.org/10.4103/IJPM.IJPM_385_16. PMid:29323075.
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