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CASE REPORT  
Year : 2020  |  Volume : 63  |  Issue : 3  |  Page : 472-474
Seminoma with focal gonadoblastoma in anatomically normal male: A rare case report


Department of Pathology, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India

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Date of Submission29-Sep-2018
Date of Decision30-Mar-2019
Date of Acceptance01-Apr-2019
Date of Web Publication7-Aug-2020
 

   Abstract 


Gonadoblastomas are unusual gonadal neoplasias that frequently appear in dysgenetic gonads. Approximately 80% of patients are phenotypic females and 20% are males. A very high frequency is associated with malignant germ cell tumor. We present a case of 37-year-old normal fertile man with descended testis who presented with swelling and pain in left testis since 6 months. On examination, left testis was swollen, hard, and tender. Ultrasound examination of left testis showed hypoechoic lesion neoplastic with multiple enlarged lymph nodes in pre- and para-aortic region. After high left inguinal orchidectomy, histopathology of specimen showed tumor tissue composed of cells arranged in large nests separated by fibrous stroma infiltrated by lymphocytes with focal area showing nests of cells with vesicular nucleus and moderate amount of eosinophilic cytoplasm with eosinophilic material which was calcified, suggestive of seminoma testis with focal area of gonadoblastoma.

Keywords: Gonadoblastoma, histopathology, seminoma, testis

How to cite this article:
Giriyan SS, Bharati M B, Shigli NA. Seminoma with focal gonadoblastoma in anatomically normal male: A rare case report. Indian J Pathol Microbiol 2020;63:472-4

How to cite this URL:
Giriyan SS, Bharati M B, Shigli NA. Seminoma with focal gonadoblastoma in anatomically normal male: A rare case report. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Sep 24];63:472-4. Available from: http://www.ijpmonline.org/text.asp?2020/63/3/472/291681





   Introduction Top


Gonadoblastoma, a gonadal tumor comprising of an admixture of germ cells and sex cord stromal cells representing 0.5% of all testicular neoplasm was first described by Scully in 1953.[1] They most commonly occur in phenotypic females who have varying degrees of virilisation and karyotypes of 46XY or 45, X/46, XY; the male patients predominantly have abnormal gonads with cryptorchidism, sliding testicle, hypospadias, and/or female internal sex organs.[1] It is rare to find a gonadoblastoma in an anatomically normal male without gonadal abnormalities.

We report a case of gonadoblastoma accompanied by a germ cell tumor in an anatomically normal male with scrotal testis.


   Case Report Top


A young male presented with swelling in left testis associated with pain since 6 months. Physical examination demonstrated a swelling in left hemiscrotum which was hard in consistency with smooth surface and slight tenderness. External genitalia was normal. He was father of two children.

Ultrasonography revealed two focal hypoechoic lesions measuring about 1.8 × 1.1 cm and 1.5 × 1.2 cm, respectively, in left testis. Right testis and epididymis were normal.

Left high inguinal orchidectomy was performed.

Gross examination revealed left testis with attached spermatic cord. Testis measured 6 × 3.5 × 3 cm. Spermatic cord measured 9.5 cm in length. Capsule was congested and oedematous. Cut surface showed normal testicular tissue with well circumscribed two gray white areas largest measuring 1 cm and other measuring 0.6 cm in diameter. Spermatic cord was oedematous and congested.

Microscopically, the sections studied showed predominantly normal testicular tissue with focal areas of seminoma showing tumor tissue consisting of cells arranged in large nests separated by fibrous stroma. The tumor cells were round to oval with vesicular nucleus, prominent nucleoli, and clear cytoplasm. The stroma was infiltrated by lymphocytes [Figure 1]. Also in the adjacent area was seen a tiny area of gonadoblastoma showing nests of cells having vesicular nucleus, moderate amount of eosinophillic cytoplasm with eosinophillic basement membrane-like material. Area of calcification was seen at places [Figure 2].
Figure 1: Seminoma with tumor cells arranged in large nests separated by fibrous stroma. (H and E, 100X)

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Figure 2: Focal area of gonadoblastoma consisting of nests of cells with vesicular nucleus, eosinophilic cytoplasm with eosinophilic material and area of calcification. (H and E, 400X)

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The diagnosis of Seminoma Testis with focal area of Gonadoblastoma was made.


   Discussion Top


Gonadoblastoma occurs rarely in normal testis, less rarely in normal ovaries, and typically in dysgenetic gonads.[2] Gonadoblastoma in an anatomically normal man was first reported in 1970 in a 23-year-old man. Histopathological examination revealed a seminoma with a small focus of gonadoblastoma.[2]

Gonadoblastoma has been variously described as a hamartoma, nodular hyperplasia, and blastomatoid dysgenetic malformation. It was named gonadoblastoma by Scully in 1953 due to similarity between the tumor histological findings and the embryological development of the gonads.[3]

Gonadoblastoma is a tumor composed of an admixture of seminoma-like germ cells and sex cord cells, the latter expressing the FOXL2 protein that is considered a marker of female sex cord cells,[4],[5] but ultrastructural studies providing evidence of sertoli cell features in the form of Charcot-Bottcher filaments.[6],[7] It is felt that a gonadoblastoma locus on Y chromosome, corresponds to testis specific protein on Y chromosome gene (TSPY) is essential for development of gonadoblastoma.[8]

Bilaterality occurs in 30–50% of cases with highest incidence being in dysgenetic gonads.[1]

Gonadoblastoma should be considered an in-situ form of malignant germ cell tumor. In approximately 50% of cases, an invasive germ cell tumor develops, usually a Seminoma. In 8% of cases, however, a more highly malignant germ cell tumor may occur such as embryonal carcinoma, yolk sac tumor, and so on.[1]

Histopathological identification of well defined, rounded nests of seminoma like cells intermingled with round to angulated sex cord cells and basement membrane deposits is essential for diagnosis of gonadoblastoma. The sex cord cells may form a peripheral palisade, surround individual germ cells or be arranged around cylinders of eosinophilic, hyalinized basement membrane, which frequently have superimposed calcification which is seen in 80% of cases and is characteristic of gonadoblastoma.[1],[9]

Our patient had classical histopathological findings of gonadoblastoma with associated seminoma and was fertile anatomically normal male. However, there is a limitation, as, the fact that cytogenetics is not done, mosiacism cannot be ruled out in our case.

The prognosis is variable and depends on other associated germ cell components and extent of germ cell invasion.[10]

Gonadectomy is the treatment of choice for gonadoblastoma. Further treatment with radiation, chemotherapy or a surgery is dependent on other germ cell component of the tumor.[1],[10]


   Conclusion Top


Seminoma with a foci of gonadoblastoma is a rare neoplasm in anatomically normal male without gonadal abnormalities.

It is important to be identified, since 10–50% cases are accompanied by foci of germ cell neoplasms like seminoma, embryonal carcinoma, and yolk sac tumor.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Scully RE. Gonadoblastoma. A review of 74 cases. Cancer 1970;25:1340-56.  Back to cited text no. 1
    
2.
Hughesdon PE, Kumarasamy T. Mixed germ cell tumours (gonadoblastomas) in normal and dysgenetic gonads. Case reports and review. Virchows Arch A Pathol Pathol Anat 1970;349:258-80.  Back to cited text no. 2
    
3.
Scully RE. Gonadoblastoma: A Gonadal tumor related to dysgerminoma (seminoma) and capable of sex hormone production. Cancer 1953;6:455-63.  Back to cited text no. 3
    
4.
Buell-Gutbrod R, Ivanovic M, Montag A, Lengyel E, Fadare O, Gwin K. FOXL2 and SOX9 distinguish the lineage of the sex cord-stromal cells in gonadoblastomas. Pediatr Dev Pathol 2011;14:391-5.  Back to cited text no. 4
    
5.
Hersmus R, Kalfa N, de Leeuw B, Stoop H, Oosterhuis JW, de Krijger R, et al. FOXL2 and SOX9 as parameters of female and male gonadal differentiation in patients with various forms of disorders of sex development (DSD). J Pathol 2008;215:31-8.  Back to cited text no. 5
    
6.
Ishida T, Tagatz GE, Okagaki T. Gonadoblastoma: Ultrasructural evidence for testicular origin. Cancer 1976;37:1770-81.  Back to cited text no. 6
    
7.
Roth LM, Eglen DE. Gonadoblastoma: Immunohistochemical and ultrastructural observations. Int J Gynecol Pathol 1989;8:72-81.  Back to cited text no. 7
    
8.
Hersmus R, de Leeuw BH, Wolffenbuttel KP, Drop SL, Oosterhuis JW, Cools M, et al. New insights into type II germ cell tumor pathogenesis based on studies of patients with various forms of disorders of sex development (DSD). Mol Cell Endocrinol 2008;291:1-10.  Back to cited text no. 8
    
9.
Thomas MU, Daniel MB, Testicular and Parateaticular Tumours. In: Stacey EM, Joel KG, Jason LH, Teri AL, Victor ER editors, Sternberg's Diagnostic Surgical Pathology 6th edn vol 2. Philadelphia. Lippincott Williams and Wilkins. 2015. p. 2217-8.  Back to cited text no. 9
    
10.
Morse MJ, Whitmore WF. Neoplasms of the testis. In: Walsh PC, Gittes RF, Perlmutter AD, Stamey TA, editors. Campbell's Urology. 5th ed, vol 2. Philadelphia: W.B. Saunders Co.; 1986. Chap. 33, p. 1551-82.  Back to cited text no. 10
    

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Correspondence Address:
M B Bharati
Plot No. 86, Nrupatunga Park, Near Shakti Colony, Hubballi - 580 032, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_626_18

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