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 Table of Contents  
CASE REPORTS
Year : 2021  |  Volume : 9  |  Issue : 4  |  Page : 159-161

A rare case report of testicular seminomatous mixed germ cell tumor with components of teratoma, embryonal carcinoma, and choriocarcinoma


1 Department of Pathology, C. U. Shah Medical College, Surendranagar, Gujarat, India
2 Department of Pathology Forensic Medicine and Toxicology, All India Institute of Medical Sciences (AIIMS), Bibinagar, Hyderabad Metropolitan Region, Telangana, India

Date of Submission21-Nov-2022
Date of Acceptance21-Jan-2022
Date of Web Publication15-Jul-2022

Correspondence Address:
Gunvanti B Rathod
Department of Pathology, All India Institute of Medical Sciences (AIIMS), Bibinagar, Hyderabad Metropolitan Region, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/dypj.DYPJ_62_21

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  Abstract 

The tumors arising in testis are of germ cell origin in most of the cases. Approximately 95% of all testicular neoplasms reported in literature are germ cell tumors (GCTs). Nowadays, there is an increase in the incidence of testicular tumors all over the world because of unknown reasons. Mixed GCTs consisting of various combinations are generally rare. In the literature, very few cases have been reported till today with the combination of seminomatous GCT with choriocarcinoma, teratoma, and embryonal carcinoma. We want to report such a rare case to make clinicians and pathologists aware of this rare entity.

Keywords: Mixed germ cell tumor, rare entity, testicular neoplasms


How to cite this article:
Choksi TS, Rathod GB, Parmar PB. A rare case report of testicular seminomatous mixed germ cell tumor with components of teratoma, embryonal carcinoma, and choriocarcinoma. D Y Patil J Health Sci 2021;9:159-61

How to cite this URL:
Choksi TS, Rathod GB, Parmar PB. A rare case report of testicular seminomatous mixed germ cell tumor with components of teratoma, embryonal carcinoma, and choriocarcinoma. D Y Patil J Health Sci [serial online] 2021 [cited 2022 Aug 8];9:159-61. Available from: http://www.dypatiljhs.com/text.asp?2021/9/4/159/351083




  Introduction Top


Generally 90–95% of testicular tumors are of germ cell origin.[1] Mixed germ cell tumors (GCTs) consisting of various combinations are generally rare.[2] Testicular mixed GCT combinations usually consist of yolk sac tumor with embryonal carcinoma, choriocarcinoma, and mature teratoma, but combinations of seminoma with immature teratoma are rare. In the literature, very few cases have been reported till today with this combination of seminomatous GCT with choriocarcinoma, teratoma, and embryonal carcinoma. We want to report such a rare case to make clinicians and pathologists aware of this rare entity.


  Case Report Top


A 50-year-old male patient came to surgery OPD with complaint of swelling in the genital region since the last 1 year. On examination, the patient had huge, painless mass in the right testis. Contralateral testis was normal. The patient underwent surgery and right testis with spermatic cord being removed. Surgical pathology department received a 9 × 7×5 cm sized testicular specimen along with a 2 × 1.5 cm sized attached cord. Gross examination showed grayish white to grayish brown outer surface and it was capsulated. On cut section, it showed grayish white to grayish brown variegated appearance with chalky white hard areas [Figure 1]. Multiple sections were taken from the testicular mass and examined microscopically. H&E-stained sections showed population of cells which were highly atypical with high N:C ratio. They showed moderate-to-severe pleomorphism along with nucleated cells and prominent nucleoli along with eosinophilic cytoplasm. These cells were forming solid sheets along with tubular pattern. Some medium-sized atypical cuboidal as well as columnar cells along with large atypical cells were also seen. They were forming lobular patterns which were separated by fibrous septa. There were decidual cell-like sheets containing trophoblastic cells within. There were many areas of hemorrhage and necrosis seen. In stroma, many cartilaginous structures were also seen along with proliferation of stroma comprising the epithelial tubules. Stromal cells show atypia along with desmoplastic reaction. From the above findings, the final diagnosis was given as mixed GCT with predominant components of seminoma, along with components of post-pubertal teratoma, embryonal carcinoma, and choriocarcinoma [Figure 2][Figure 3][Figure 4].
Figure 1: Grayish white nodular areas with variegated appearance on gross examination

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Figure 2: Seminomatous component with nest of tumor cells (H & E stain, 10×)

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Figure 3: Decidual cell-like sheets with trophoblastic cells of choriocarcinoma (H & E stain, 10×)

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Figure 4: Multinucleated syncytiotrophoblast suggesting choriocarcinoma (H & E Stain, 20×)

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  Discussion Top


The tumors arising in the testis are of germ cell origin in most of the cases. Approximately 95% of all testicular neoplasms reported in the literature are GCTs. Nowadays, there is an increase in the incidence of testicular tumors all over the world because of unknown reasons.[3] We have to be cautious as it is the most malignant tumor and third leading cause of death among men between 20 and 40 years of age group.[4] GCTs are common. But some mixed GCTs with rare combination are uncommon and there are very few cases reported in the literature till today. Lovrić et al.[5] described an unusual mixed GCT consisting of yolk sac tumor and teratoma with rhabdomyosarcomatous element. Terrier-Lacombe et al.[6] have also described embryonal rhabdomyosarcoma arising in a mature teratoma of testis. Koshy et al.[7] found mixed GCTs to be rare with a common combination of dysgerminoma with yolk sac tumor.

We are all very well aware about cryptorchidism as the most important risk factor for the development of GCTs. There are also other risk factors, e.g., testicular dysgenesis, a history of contralateral tumor, and genetic factors. Recent studies are also considering HIV infection as a risk factor for the development of testicular tumor.[8] Family history also plays a major role, and it is documented in the literature that the relative risk for first-degree relatives is 3–10-folds for the development of GCT.[9] If one-sided testis has malignant neoplasm, then the contralateral testis has 500–1000-folds greater chance of developing GCT. The pathogenesis of mixed GCTs includes chromosomal aberrations in the form of isochromosome formation and deletion of chromosome 12. The pluripotency of homeobox genes NANOG is expressed in human GCTs.[10] OCT3/4 and SOX2 genes are transcription factors expressed in embryonic stem cells. Through a cooperative interaction of these two, there occurs pluripotent expression of genes like NANOG that drives the cells into a feedback autoregulatory loop. Increased levels of these transcription factors SOX2 and OCT3/4 maintain the pluripotent state of the cells of these mixed GCTs.[11]

Mixed GCTs arise usually around 30 years of age. Because of lack of awareness or social stigma, many of the patients present late with morbid complications. Unfortunately, such patients are difficult to treat and carry bad prognosis. Though if early, treatment with the chemotherapy can increase survival period reasonably, depending on the associated complications. The route of metastasis for the GCTs is lymphogenous, except spermatocytic seminoma.[12] GCT mostly spreads lymphogenously to retroperitoneal and mediastinal nodes and to the left supraclavicular node, which is also known as Virchow’s node. The most common organs for hematogenous metastases of GCT are lungs, liver, brain, or bones. Mostly 20% for seminomas and 30–60% for nonseminomatous tumors are presenting with metastatic disease itself.[8] The microscopic examination of metastasis reveals the histology of the basic primary tumor. However, many times it happens that different histologic cell types are found more in metastases than in primary tumors. The possible reason for this may be due to maturation of one germ cell type into another cell type. The cure rate for the patients with localized tumor is 90–100% and for patients with metastatic disease is 70%. The treatment modalities and prognosis of testicular tumors are mostly dependent on clinical stage and histological type of the tumor. One more very important factor responsible for the definite cure is completion of the treatment and regular follow-up of the patient. In most of the cases, a 2-year disease-free interval indicates cure in about 90% of the patients.


  Conclusion Top


We report the first known example of a GCT with an unusual combination of seminoma, choriocarcinoma, teratoma, and embryonal carcinoma. This case not only reflects the heterogeneity in the biology of GCT of the testis, but also highlights the aggressiveness of these tumors and challenges in treatment. One should keep in mind such rare entity while diagnosing GCT so that we can improve patient’s prognosis.



 
  References Top

1.
Rosai J Female reproductive system. In: Rosai J, editor. Rosai and Ackermans Surgical Pathology, 9th ed. Vol. 2. New Delhi, India: Elsevier, a Division of Reed Elsevier; 2004. p. 1636-761.  Back to cited text no. 1
    
2.
Oosterhuis JW, Looijenga LH, van Echten J, de Jong B Chromosomal constitution and developmental potential of human germ cell tumors and teratomas. Cancer Genet Cytogenet 1997;95:96-102.  Back to cited text no. 2
    
3.
Epstein JI The lower urinary tract and male genital system. In: Vinay K, editor. Robbins and Cotran Pathologic Basis of Disease. 8th ed. Philadelphia: Saunders Elsevier; 2010. p. 987.  Back to cited text no. 3
    
4.
Coleman MP, Esteve J, Damiecki P, Arslan A, Renard H Trends in cancer incidence and mortality. IARC Sci Publ 1993;121:1-806.  Back to cited text no. 4
    
5.
Lovrić E, Hizak DB, Balja MP, Lenicek T, Kruslin B An unusual mixed germ cell tumor of the testis consisting of rhabdomyosarcoma, mature teratoma and yolk sac tumor. Asian J Androl 2010;12:451-2.  Back to cited text no. 5
    
6.
Terrier-Lacombe MJ, Martinez-Madrigal F, Porta W, Rahal J, Droz JP Embryonal rhabdomyosarcoma arising in a mature teratoma of the testis: A case report. J Urol 1990;143:1232-4.  Back to cited text no. 6
    
7.
Koshy M, Vijayananthan A, Vadiveloo V Malignant ovarian mixed germ cell tumour: A rare combination. Biomed Imaging Interv J 2005;1:e10.  Back to cited text no. 7
    
8.
Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL Urologic surgery. In: Coburn M, editor. Sabiston Text Book of Surgery. 19th ed. Philadelphia: Saunders; 2012, p. 2076.  Back to cited text no. 8
    
9.
Brodsky GL Pathology of testicular germ cell tumors. Hemtol/Oncol Clin N Am 1991;5:1095-125.  Back to cited text no. 9
    
10.
Tai MH, Chang CC, Kiupel M, Webster JD, Olson LK, Trosko JE Oct4 expression in adult human stem cells: Evidence in support of the stem cell theory of carcinogenesis. Carcinogenesis 2005;26:495-502.  Back to cited text no. 10
    
11.
Hart AH, Hartley L, Parker K, Ibrahim M, Looijenga LH, Pauchnik M, et al. The pluripotency homeobox gene Nanog is expressed in human germ cell tumors. Cancer 2005;104:2092-8.  Back to cited text no. 11
    
12.
Sesterhenn IA, Davis CJ Jr. Pathology of germ cell tumors of the testis. Cancer Control 2004;11:374-87.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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