|Year : 2016 | Volume
| Issue : 4 | Page : 499-502
Pure yolk sac tumor of testis in a 50 years old : A rare case report
Pradhan M Pagaro, Bedarshi Banerjee
Department of Pathology, Dr. DY Patil Medical College, Hospital and Research Centre, Dr. DY Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Web Publication||12-Jul-2016|
Pradhan M Pagaro
Department of Pathology, Dr. D.Y. Patil Medical College and Hospital, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Yolk sac tumor (YST) is most common in infants and children in age range of 3-8 years in pure form; however in adults, YST rarely occurs in pure form, but commonly occurs in association with other germ cell tumors as mixed forms. Less than 10 adult testicular pure YSTs have been reported in literature. We report a 50-year-old male with testicular neoplasm involving the left testis, with pain following trauma. Alpha-fetoprotein was markedly raised 5726 IU/ml, and on histopathology, characteristic features of YST with abundant Schiller–Duval bodies associated with reticular and microcystic pattern, and focal solid areas of nests and trabeculae of cuboidal cells, with prominent nucleoli and mitotic figures, were noted, and extensive histopathological search did not reveal presence of any other type of tumor.
Keywords: Adult rare, Schiller–Duval, yolk sac tumor
|How to cite this article:|
Pagaro PM, Banerjee B. Pure yolk sac tumor of testis in a 50 years old : A rare case report. Med J DY Patil Univ 2016;9:499-502
| Introduction|| |
In adults, yolk sac tumors (YSTs) are rare and histologically are commonly characterized by Schiller–Duval bodies (glomerular-like structures) and reticular and microcystic patterns. Schiller–Duval bodies consist of central blood vessel surrounded by epithelial-like cells, space, and more epithelial-like cells. Other patterns of YST are myxomatous YST, spindle cell pattern which may show rhabdomyoblastic or sarcomatoid differentiation, polyvesicular vitelline pattern occurring in 8% of cases, in one series consisting of vesicles with central constriction lined by flattened to cuboidal cells, hepatic differentiated pattern with polygonal cells, eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli arranged in nested trabecular and sheet-like pattern, or intestinal pattern.
Almost all the YSTs are alpha-fetoprotein positive, they are also positive for ALAP, Glypican-3, cytokeratin (CK)-19, and CK. YSTs are negative for OCT3 (positive in mixed germ cell tumors) and CD30 (positive in embryonal carcinoma).
Prognosis of adult YST is worse as compared to infantile YSTs and metastasis in adults occur through lymphatics, but in infants metastasis is by hematogenous and lymphatic routes. Isochrome 12p is commonly observed in adults but not in infantile YSTs. Intratubular neoplasia is observed in adults but not in infantile YSTs. Genetically adult YST shows aneuploidy and p53 gene mutations whereas infantile YST show 1p, 6q deletions, and 3p duplication with no mutations in p53 gene.
| Case Report|| |
A 50-year-old male came with complaints of swelling and pain in the left testis following trauma. Routine investigations were normal but with a significant increase in alpha-fetoprotein, 5726 IU/ml (normal range is <10 IU/ml), hCG levels were normal. Ultrasonography showed a mass with solid and cystic areas.
The histopathology of the mass showed abundant Schiller–Duval bodies [Figure 1] and [Figure 2] and vesiculo vitelline structures [Figure 3]; there were areas of reticular [Figure 4] and [Figure 5] and microcystic patterns [Figure 6], [Figure 7], [Figure 8]. An extensive search revealed the absence of any other type of germ cell tumor. Hence, histologically it was a pure case of adult YST.
|Figure 1: Yolk sac tumor scanner view show many glomeruloid Schiller–Duval bodies ×4|
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|Figure 2: Glomeruloid Schiller–Duval bodies (high power). Note the anaplastic nature of cells ×40|
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|Figure 3: Low power view of yolk sac tumor showing many Schiller–Duvan and vesiculo vitelline structures with reticular pattern ×10|
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|Figure 4: Yolk sac tumor showing Schiller–Duval structures and reticular pattern ×10|
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|Figure 6: Yolk sac tumor showing glomeruloid structures with microcystic pattern ×10|
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| Discussion|| |
YSTs resemble yolk sac, allantois, and extraembryonic mesenchyme. Prognosis of adult YST is worse as compared to infantile YSTs and metastasis in adults occur through lymphatics; however in infants, metastasis is by hematogenous and lymphatic routes. Hypermethylation of RUNX3 promoter gene and overexpression of GATA-4 genes is seen in infantile YST and not in adult YST. 12pi is noted in adult and not in juvenile YST, aneuploidy, and p53 mutations are noted in adult and 1p, 6q deletions, and 3p duplication noted in infantile YST. Lymphatic metastasis occurs in adult, and vascular and lymphatic spread occurs in infantile YST.
Alpha-fetoprotein is seen in cells and blood of almost all patients of YSTs with <10,000 units - good prognosis >10,000 units - poor prognosis.
These tumors are also positive for ALAP, CK, and Glypican-3.
Negative for OCT 3/4 and CD30.
There is noted, differences genetically as mentioned above between infantile and adult YSTs. Furthermore, there is different clinical behavior between the two in the form of different ways of the spread of tumors and also prognosis, which is better in infantile YSTs.
Grossly the tumor shows solid and cystic areas with foci of hemorrhage and necrosis.
Histologically, the classic feature is the presence of glomeruloid structures called Shiller–Duval bodies [Figure 1] and [Figure 2], and also show lace-like reticular [Figure 7] and microcystic [Figure 6] patterns; in one series 8% of YSTs showed vesiculo vitelline pattern [Figure 3].
Uncommon patterns include myxomatous, and spindle cell pattern with rhabdomyoblastic and sarcomatoid differentiation, hepatoid, intestinal, glandular, and papillary patterns., Shah et al. have reported in the population-based analysis of 788 cases that mixed germ cell tumors with yolk sac element or pure YSTs in adults have poor prognosis.
Almost all the authors of cases of adult YST agree that adult YSTs are very rare. Khan et al. reported a pure YST in a 33-year-old man with alpha-fetoprotein >544 ng/ml  (normal <10 ng/ml). Stubinski et al. while reporting a 44-year-old with pure YST said in literature <10 pure adult testicular YSTs have been reported. Mumtaz et al. reported a pure YST of testis in a 22-year-old. Talerman reported that YST elements were found in 22/68 germ cell tumors with unfavorable prognosis. Chong et al. reported that pure YSTs can be successfully treated with aggressive chemotherapy and surgery with high cure rate. Grady et al. reported different clinical behavior, with adult YSTs show metastasis through lymphatics, and infantile YSTs show hematogenous metastasis. Kato et al. noted hypermethylation of RUNX3 gene and loss of heterozygosity at 1p36 gene in 8 of 10 cases of infantile YSTs not observed in adult YSTs indicating pathogenetic differences between the two. Cohen MB and others describe a pure YST in adult testis with intestinal differentiation with YST confirmed by immunohistochemistry and electron microscopy. Czaja and Ulbright TM observed in 6 of 10 cases in which foci of an abrupt change from seminoma to various patterns of YST occurred, indicating seminoma may serve as a precursor to YST. Manivel et al. noted adult YST with intratubular germ cell neoplasm but such change was absent in infantile YST indicating divergent pathogenetic mechanisms between the two. Grady et al. have described that metastasis in adult YST occurs via lymphatic route whereas in infantile YST occurs through mainly hematogenous route. Kao et al. have described solid patterns of YST which may be confused with granulosa cell tumor which is AFP negative  Debbag et al. reports a case of 23-year-old with pure YST. Springerlink encyclopedia page 3973-3974 describes adult YST with classic patterns of perivascular Schiller–Duval bodies with eosinophilic cytoplasmic globules which are alpha-fetoprotein positive and these tumors are usually radioresistant but respond to combination therapy. However, Hashimoto et al. described a pure YST of adult testis achieving pathological complete response by chemotherapy.
| Conclusion|| |
YSTs usually occur in infants and young children but are rarely observed in postpubertal adults. Very few YST in adults have been reported, however in this age group the YST commonly occur in association as mixed germ cell tumors. Authors have reported different chromosomal mutations in the two age groups with YST resulting in differences as of pathology and clinical behavior. Metastasis in infantile YST occurs via hematogenous and lymphatic route whereas in adult YST occur through lymphatics only. Furthermore, the worst prognosis noted in adult YSTs may be due to different chromosomal abnormalities noted in YSTs in the two age groups. We report this tumor, as it is rare, to understand the pathogenetic differences which would help to explain the differences with respect to clinical behavior and prognosis, and to achieve understanding for devising proper treatment modalities.
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| References|| |
Mills S. Sternberg's Textbook of Surgical pathology. 5th
ed. Philadelphia: Wolters Kluwer; 2015. p. 1980-5.
Young RH. The yolk sac tumor: Reflections on a remarkable neoplasm and two of the many intrigued by it-Gunnar Teilum and Aleksander Talerman-and the bond it formed between them. Int J Surg Pathol 2014;22:677-87.
Talerman A. The incidence of yolk sac tumor (endodermal sinus tumor) elements in germ cell tumors of the testis in adults. Cancer 1975;36:211-5.
Shah JP, Kumar S, Bryant CS, Ali-Fehmi R, Malone JM Jr. Deppe G, et al.
A population-based analysis of 788 cases of yolk sac tumors: A comparison of males and females. Int J Cancer 2008;123:2671-5.
Khan S, Jetley S, Pujani M, Neogi S. Pure yolk sac tumor of testis in an adult: A rare occurrence. J Postgrad Med 2014;60:351-3.
Medica M, Germinale F, Giglio M, Stubinski R, Campodonico F, Raggio M, et al.
Adult testicular pure yolk sac tumor. Urol Int 2001;67:94-6.
Mumtaz D, Melin S, Serbeta B, Emir ML, Elif OS, Demokan E. Pure yolk sac tumor in adult testis a case report. Turk Urol Derg 2010;36:322.
Chong CD, Logothetis CJ, von Eschenbach AC, Ayala AG, Samuels ML. Successful treatment of pure endodermal sinus tumors in adult men. J Clin Oncol 1988;6:303-7.
Foster RS, Hermans B, Bihrle R, Donohue JP. Clinical stage I pure yolk sac tumor of the testis in adults has different clinical behavior than juvenile yolk sac tumor. J Urol 2000;164:1943-4.
Kato N, Tamura G, Fukase M, Shibuya H, Motoyama T. Hypermethylation of RUNX3 gene promoter in testicular YST in infants not seen in adults YST. Am J Pathol 2003;163:387-91.
Cohen MB, Friend DS, Molana JJ, Talerman A. Pure YST in adult testis with exclusive intestinal differentiation. Pathol Res Pract 1987;182:609-16.
Czaja JT, Ulbright TM. Evidence for the transformation of seminoma to yolk sac tumor, with histogenetic considerations. Am J Clin Pathol 1992;97:468-77.
Manivel JC, Simton S, Wold LE, Dehner LP. Absence of intratubular germ cell neoplasia in juvenile YST. Arch Pathol Lab Med 1988;112:641-45.
Grady RW, Ross JH, Kay R. Patterns of metastatic spread in prepubertal and adult YSTs in testis. J Urol 1995;153:1259-61.
Kao CS, Idrees MT, Young RH, Ulbright TM. Solid pattern yolk sac tumor: A morphologic and immunohistochemical study of 52 cases. Am J Surg Pathol 2012;36:360-7.
Debbag A, Benani S, Jouhadi S, Jouval A, El Mrini M, Kahlan A, et al
. Pure yolk sac tumor in adult testis a case report. Ann Urol 2001;35:356-8.
Schwab M. Encyclopedia of cancer. Berlin: Springer; 2009. p
Hashimoto Y, Iwase Y, Mogani T, Hayashi Y, Sasaki S, Kato M, et al
. Adult YST achieving complete remission with chemotherapy. Hinyokiko Kiya 1995;41:813-16.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]