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| Year : 2014 | Volume
: 60
| Issue : 3 | Page : 351-353 |
Pure yolk sac tumor of testis in an adult: A rare occurrence
S Khan1, S Jetley1, M Pujani1, S Neogi2
1 Department of Pathology, Hamdard Institute of Medical Sciences and Research (HIMSR), New Delhi, India
2 Department of Surgery, Hamdard Institute of Medical Sciences and Research (HIMSR), New Delhi, India
| Date of Web Publication | 14-Aug-2014 |
Correspondence Address:
Dr. M Pujani
Associate Professor, Department of Pathology, Hamdard Institute of Medical Sciences and Research, New Delhi 110062
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 25121392 
How to cite this article:
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 |
Sir,
Yolk sac tumor (YST) of the testis is primarily a tumor of infants and young children, almost always occurs in the pure form and carries an excellent prognosis. However, in adults, it is invariably found admixed with other germ cell tumor components.[1] Pure YST of the testis in an adult is an extremely rare occurrence.[2],[3],[4],[5],[6]
A 33-year-old male patient presented with a rapidly increasing swelling in the scrotum associated with pain following trauma. On examination, the left scrotal sac showed an enlarged, hard, non tender mass free from the overlying skin. Transillumination was negative. Sonography of the scrotum revealed a heterogenous echotexture mass with whorled internal pattern and nodular echogenic areas with cystic changes within. Abdominal ultrasound did not reveal any significant lymphadenopathy. Serum alpha fetoprotein (AFP) was 544 ng/ml (normal reference range < 10 ng/ml), human chorionic gonadotrophin (HCG) and serum lactate dehydrogenase (LDH) were within normal limits.
A high inguinal orchidectomy was performed. Grossly, the left testis was enlarged. On cutting, the entire testis was replaced by a large well circumscribed tumor with variegated appearance and extensive areas of hemorrhage and necrosis measuring 7.0 × 6.5 × 5.0 cm. Normal testis or epididymis could not be identified grossly [Figure 1]. Microscopically, the tumor was composed of cells arranged in a reticular, microcystic pattern as well as in small groups and sheets focally [Figure 2]. The cells were round to cuboidal with scant to moderate amount of vacuolated cytoplasm with round to oval nuclei exhibiting mild to moderate nuclear pleomorphism. Characteristic Schiller Duval bodies were also seen [Figure 2], black arrow] along with few periodic acid schiff (PAS) positive hyaline globules. Tunica albuginea and the spermatic cord were free of tumor. | Figure 1: Grossly, tumor involved entire testis and had a variegated appearance with areas of hemorrhage and necrosis
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 | Figure 2: Microscopically, tumor cells were arranged in a reticular, microcystic pattern; Schiller Duval body (black arrow). (Hematoxylin and Eosin, 100X); inset- strong cytoplasmic positivity for Alpha fetoprotein (Immunohistochemical stain for AFP, 400X)
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Considering the age of the patient and yolk sac tumor as the only component found, we did rigorous sampling from the tumor to identify any other germ cell tumor. But despite extensive sectioning, we did not come across any other component. Moreover, immunohistochemistry revealed a strong positivity for AFP [[Figure 2] inset] as well as cytokeratin while Ki-1(CD 30) and HCG were negative. So, a final diagnosis of pure yolk sac tumor of testis was given in the adult patient.
Yolk sac tumor is characterized by a variety of patterns that recapitulate the embryonal yolk sac, allantois and extraembryonic mesenchyme. YST is found in 2.4% of adult patients where it is usually seen as mixed germ cell tumor, pure YST being extremely rare.[2],[3],[4],[5],[6],[7] The biological behavior of YST is quite variable in the two age groups: Excellent prognosis in infants and children while in adults it carries a much worse prognosis.[1] Serum AFP is elevated in around 90% of the cases and the value is usually above 100 ng/ml but may reach up to 100 fold higher. Ten to twenty percent of the children have metastases at the time of presentation. Lymphatic spread usually occurs to the retroperitoneal nodes.[1]
Histopathological features of YST do not differ with the patient's age. The most commonly encountered pattern is the microcystic or reticular pattern, where there is a meshwork of vacuolated cells. Other patterns of YST are macrocystic, solid, glandular-alveolar, endodermal sinus, papillary, polyvesicular vitelline or rarely hepatoid and enteric patterns. Hallmark of a yolk sac tumor is the Schiller Duval body which is characterized by a central core of thin walled blood vessels lined by a layer of cuboidal cells with clear cytoplasm and prominent nucleoli.[1] Immunohistochemically, AFP is positive in most cases, although, the staining may be variable or weak. Moreover, negative AFP staining does not exclude a diagnosis of YST. Yolk sac tumor is also positive for low molecular weight cytokeratin and alpha-1 antitrypsin. On IHC, negative Ki-1 (CD 30) helped in excluding embryonal carcinoma while negative HCG ruled out choriocarcinoma in the present case.
There is no unanimous consensus regarding the treatment protocol following the inguinal orchidectomy as the pure form is very rare in adults. Medica et al.[4] asserted that nerve sparing retroperitoneal lymph node dissection is potentially curative in adult patients with pure YST. Foster et al.[7] evaluated the clinical behavior of clinical stage I pure YST of testis in adults and compared it with clinical stage I non seminomatous tumors and observed no difference. However, compared to juvenile YST, pure YST in an adult has a strong tendency towards hematogenous metastasis.
We wish to highlight that in view of the rarity of pure yolk sac tumor of testis in adults, one should be extra cautious while reporting and a diagnosis of pure YST should be given only after extensive tissue sectioning to rule out any other germ cell component.
| :: References | |  |
| 1. | Woodward PJ, Mostofi FK, Talerman A, Heidenreich A, Hailemariam S, Kaplan GW, et al. Germ cell tumors. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. WHO classification of tumors. Pathology and Genetics. Tumors of the Urinary system and Male Genital Organs. Lyon: IARC Press; 2004. p. 237-40. 
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| 2. | Izumi H, Shiokawa H, Shibata Y, Kurokawa J, Ohbu M. Pure yolk sac tumor of the testis with brain metastasis: Report of an adult case. [Article in Japanese]. Hinyokika Kiyo 1992;38:1071-4.
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| 3. | Hashimoto Y, Iwase Y, Mogami T, Hayashi Y, Sasaki S, Kato M, et al. A case of adult pure yolk sac tumor of the testis achieving pathological complete response by chemotherapy. [Article in Japanese]. Hinyokika Kiyo 1995;41:813-6.
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| 4. | 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.
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| 5. | Mümtaz D, Melih S, Mehmet B, Levent E, Elif O, Demokan E. Pure yolk sac tumor of testis in an adult patient: Case report. Turkish Journal of Urology 2010;36:322.
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| 6. | Jagtap SV, Nikumbh DB, Kshirsagar AY. Adult Pure Yolk Sac Tumor of the Testis. Online J Health Allied Scs 2012;11:7.
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| 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.
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[Figure 1], [Figure 2]
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