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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 3  |  Page : 437-439
Spermatocytic seminoma with rhabdomyoblastic differentiation: Case report and review of literature


1 Department of Pathology, HBT Medical College and Dr. R. N. Cooper Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, HBT Medical College and Dr. R. N. Cooper Municipal General Hospital, Mumbai, Maharashtra, India
3 Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India

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Date of Web Publication13-Jul-2018
 

   Abstract 


Spermatocytic seminoma (SCS) is an indolent germ cell tumor of the testis. It has an excellent prognosis and orchidectomy is generally curative. Very rarely, it can be complicated by a sarcomatous transformation which is associated with a very aggressive behavior and requires adjuvant therapy. SCS with sarcomatous component is a very rare occurrence with <20 cases described in the world literature of which eight showed rhabdomyoblastic differentiation. We report a case of SCS with rhabdomyosarcomatous differentiation in a 60-year-old male along with a short review of literature.

Keywords: Rhabdomyosarcoma, spermatocytic seminoma, testis

How to cite this article:
Pandey V, Khatib Y, Khade AL, Pandey R, Khare MS. Spermatocytic seminoma with rhabdomyoblastic differentiation: Case report and review of literature. Indian J Pathol Microbiol 2018;61:437-9

How to cite this URL:
Pandey V, Khatib Y, Khade AL, Pandey R, Khare MS. Spermatocytic seminoma with rhabdomyoblastic differentiation: Case report and review of literature. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 6];61:437-9. Available from: http://www.ijpmonline.org/text.asp?2018/61/3/437/236603





   Introduction Top


Spermatocytic seminoma (SCS) is an uncommon germ cell tumor which accounts for 1%–2% of all testicular tumors.[1] It is a slow-growing tumor presenting after the 5th decade of life with a favorable outcome.[1] The presence of sarcomatous component converts this otherwise slow growing, localized, indolent neoplasm into a rapidly growing, infiltrative, aggressive tumor with metastatic potential, and poor prognosis. Identification of this component is very crucial for proper management. The development of rhabdomyosarcoma in SCS is extremely rare with eight cases having been reported till date, with only two earlier case reports from India.[2],[3]


   Case Report Top


A 60-year-old male presented with a painless, slowly enlarging right testicular mass of 4 years' duration with a rapid increase in the size for 2 months. There was no history of smoking or any drug intake. Clinical examination revealed a nontender enlarged right testis. There were no palpable inguinal lymph nodes. Ultrasonography revealed a large lobulated mass in the right testis measuring 18 cm × 8 cm × 7 cm which showed heterogeneous echogenicity with necrotic areas [Figure 1]a. Computed tomography of abdomen and pelvis and X-ray chest did not reveal any metastatic or lymphatic involvement. Serum tumor markers beta human chorionic gonadotropin, alpha-fetoprotein (AFP), and lactate dehydrogenase were normal. The patient underwent right inguinal orchidectomy. On gross examination, a large lobulated tumor measuring 18 cm × 7.5 cm × 6.3 cm in size was seen replacing the entire right testis [Figure 1]b. On cut section, the tumor was gray-white with a heterogeneous appearance. There were fleshy, glistening, solid areas interrupted by friable necrotic areas. The testicular capsule appeared breached, and epididymis and normal testicular tissue could not be identified.
Figure 1: Ultrasonography showing right testicular mass replacing the testes with heterogenous echogenicity. (a) Gross appearance of tumor: Testis is replaced by large tumor having variegated appearance with grey white fleshy areas. (b) Spermatocytic seminoma showing three population of cells small, intermediate, and large (c, H and E, ×100) High power of spermatocytic seminoma showing filamentous chromatin (d, H and E, ×400)

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Microscopic examination showed a tumor comprising of two distinct components. Majority of the sections showed features of a classical SCS. The tumor cells displayed a sheet-like pattern with three distinct populations of small, intermediate, and large cells. Intermediate cells were predominant with filamentous chromatin [Figure 1]c and [Figure 1]d. These tumor cells were immunonegative for CD 30, C-kit, placental alkaline phosphatase and AFP. The second component showed spindle cell proliferation in haphazard fascicles with brisk mitosis. Amidst this spindle cell proliferation cells resembling rhabdomyoblasts with eccentric nuclei, eosinophilic cytoplasm, and prominent nucleoli were seen [Figure 2]a. The sarcomatous component infiltrated the epididymis and spermatic cord, and focally the tunica albuginea [Figure 2]b and [Figure 2]c. These cells stained strongly for Desmin antibody [Figure 2]d. No areas of ITCGN or other germ cell component were identified. A diagnosis of SCS with rhabdomyoblastic differentiation was given based on the morphology and IHC studies. Further treatment with adjuvant chemotherapy was advised, but the patient refused the same. Two months' postsurgery, the patient is symptomatically doing well.
Figure 2: Rhabdomyosarcomatous component showing spindle cells with rhabdomyoblast (inset) having abundant eosinophilic cytoplasm and nucleus showing prominent nucleoli. (a, H and E, ×100). Rhabdomyosarcomatous component, infiltrating tunica (b, H and E, ×100) Rhabdomyosarcomatous component encasing spermatic cord. (c, H and E, ×100) Rhabdomyosarcomatous component showing immunoreactivity for Desmin (d)

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   Discussion Top


SCS is an uncommon germ cell neoplasm first described by Masson.[3] The usual age of presentation is in the 5th or 6th decade of life as compared with classic seminoma which occurs usually in the age group of 30–40 years.[1],[4] It presents as a slowly growing painless swelling with symptoms ranging from few months to 4 years. Bilateral involvement is seen in 10% of the cases.[1],[2] The diagnosis of SCS is based on the presence of a tripartite population of small lymphocyte-like, intermediate and large cells with filamentous chromatin. Unlike classical seminoma, SCS is not associated with ITGCN or other germ cell components.[1] It is an indolent neoplasm which presents at an early stage with the absence of metastasis and excellent prognosis.

However, in a few cases, sarcomatous transformation occurs in SCS which changes its growth pattern renders the tumor a metastatic potential with poor prognosis. On review of literature, <20 cases of sarcomatous transformation have been reported with three case reports from India. Out of these two cases showed rhabdomyosarcomatous differentiation and one showed the presence of undifferentiated sarcoma [2],[3],[5] The sarcomatous component was rhabdomyosarcoma in 8 of these cases [2],[3],[4],[6],[7],[8],[9] [Table 1]. In majority of the reported cases of SCS with rhabdomyosarcomatous component, the patients were in 5th or 6th decade of life with three patients <45 years.[4],[7],[8] The youngest reported case was that of a 38-year-old man Narang et al.[3] The sudden increase in size of a previously slowly growing swelling suggests the emergence of a sarcomatous component. This was also seen in the present case. SCS component was well circumscribed while the sarcomatous component was seen infiltrating into the epididymis and rete testis as has been reported in earlier cases.[2],[3] The sarcomatous component showed the presence of spindle cells intermixed with rhabdomyoblasts which stained positive with desmin stain confirming the diagnosis.
Table 1: Cases of spermatocytic seminoma with rhabdomyosarcoma published in English literature

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In these cases, it is important not to mistake the epithelial structures of rete testis and epidydimus for an epithelial component of a mixed NSGCT.[2],[5]

Metastatic disease at the time of presentation was detected in five cases to distant sites such as lung liver and bone.[3],[4],[5],[6] All of these cases died within a year of diagnosis. Hence, the presence of sarcomatous differentiation in SCS is associated with aggressive behavior presence of metastasis and poor outcome.

The mechanism of this sarcomatous transformation is unknown. One hypothesis is anaplastic conversion of SCS.[3],[6] The rapid growth phase seen clinically probably represents this period of transition and histological differentiation into sarcoma.[3],[6] Another theory is differentiation of totipotent germ cells to somatic tissues and subsequent malignant transformation or by malignant transformation of a preexisting teratomatous element.[6] The gain of chromosome 9 is the consistent chromosomal anomaly found in SCS. However, chromosomal studies have not been done in the limited reported cases of SCS with rhabdomyosarcomatous differentiation.[8] As very few case reports are available, prognostic factors in SCS with rhabdomyosarcomatous differentiation have not been established. Most cases irrespective of age, site and size showed an aggressive behavior; however, those with extratesticular disease with metastasis had a short survival.

The treatment of choice for SCS is orchidectomy. However, in the presence of sarcomatous component, adjuvant chemotherapy or radiotherapy may be beneficial.


   Conclusion Top


SCS with rhabdomyosarcomatous differentiation is an exceptionally rare tumor with only eight cases reported in the literature reported so far. All these cases were reported in patients with a neglected testicular mass over a period of years; therefore, there is a need for early detection and surgical removal. Second, in a few of these cases, majority of the tumor remained well circumscribed with focal infiltration on gross inspection. Hence, a meticulous sampling of the tumor to detect areas of sarcomatous transformation is imperative. Third, these tumors often present with concurrent metastatic disease; and thus, treatment with adjuvant chemotherapy following radical inguinal orchidectomy should be considered in all cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Talerman A. Spermatocytic seminoma: Clinicopathological study of 22 cases. Cancer 1980;45:2169-76.  Back to cited text no. 1
    
2.
Menon S, Karpate A, Desai S. Spermatocytic seminoma with rhabdomyosarcomatous differentiation: A case report with a review of the literature. J Cancer Res Ther 2009;5:213-5.  Back to cited text no. 2
    
3.
Narang V, Gupta K, Gupta A, Kumar S. Rhabdomyosarcomatous differentiation in a spermatocytic seminoma with review of literature. Indian J Urol 2012;28:430-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
True LD, Otis CN, Delprado W, Scully RE, Rosai J. Spermatocytic seminoma of testis with sarcomatous transformation. A report of five cases. Am J Surg Pathol 1988;12:75-82.  Back to cited text no. 4
    
5.
Trivedi P, Pasricha S, Gupta A. Spermatocytic seminoma associated with undifferentiated sarcoma: A rare case report. Indian J Pathol Microbiol 2011;54:138-40.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Floyd C, Ayala AG, Logothetis CJ, Silva EG. Spermatocytic seminoma with associated sarcoma of the testis. Cancer 1988;61:409-14.  Back to cited text no. 6
    
7.
Matoska J, Talerman A. Spermatocytic seminoma associated with rhabdomyosarcoma. Am J Clin Pathol 1990;94:89-95.  Back to cited text no. 7
    
8.
Robinson A, Bainbridge T, Kollmannsberger C. A spermatocytic seminoma with rhabdomyosarcoma transformation and extensive metastases. Am J Clin Oncol 2007;30:440-1.  Back to cited text no. 8
    
9.
Chelly I, Mekni A, Gargouri MM, Bellil K, Zitouna M, Horchani A, et al. Spermatocytic seminoma with rhabdomyosarcomatous contingent. Prog Urol 2006;16:218-20.  Back to cited text no. 9
    

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Correspondence Address:
Archana Laxman Khade
Department of Pathology, C Wing, 1st Floor, Hospital Building, HBT Medical College and Dr. R. N. Cooper Municipal General Hospital, Juhu, Mumbai - 400 056, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_243_17

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