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CORRESPONDENCE
Year : 2018  |  Volume : 14  |  Issue : 12  |  Page : 1241-1243

Paratesticular rhabdomyosarcoma mimicking complicated epididymal cyst


1 Department of Urology, Faculty of Medicine, Bahcesehir University, Medical Park Bursa Hospital, Bursa, Turkey
2 Department of Urology, Istanbul Training and Research Hospital, Istanbul, Turkey
3 Department of Urology, Faculty of Medicine, Kemerburgaz University, Medical Park Bursa Hospital, Bursa, Turkey

Date of Web Publication11-Dec-2018

Correspondence Address:
Mustafa Kadihasanoglu
Department of Urology, Istanbul Training and Research Hospital, Fatih, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.183553

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 > Abstract 


Paratesticular rhabdomyosarcoma (RMS) is a rare nongerm cell intrascrotal malignant tumor in children and young adult/teens resulting from the mesenchymal tissues of the tunica vaginalis, epididymis, spermatic cord, and testis. RMS accounts for approximately 7% of all genitourinary tract RMSs and 12% of all pediatric scrotal neoplasms. Patients usually present with a painless unilateral scrotal mass. We report a 16-year-old boy with a paratesticular RMS mimicking epididymal cyst. To our knowledge, this is the first case reported in the literature presenting as an epididymal cyst.

Keywords: Cyst, epididymis, paratesticular, radical orchiectomy, rhabdomyosarcoma


How to cite this article:
Kilciler M, Kadihasanoglu M, Atahan O. Paratesticular rhabdomyosarcoma mimicking complicated epididymal cyst. J Can Res Ther 2018;14, Suppl S5:1241-3

How to cite this URL:
Kilciler M, Kadihasanoglu M, Atahan O. Paratesticular rhabdomyosarcoma mimicking complicated epididymal cyst. J Can Res Ther [serial online] 2018 [cited 2019 Sep 20];14:1241-3. Available from: http://www.cancerjournal.net/text.asp?2018/14/12/1241/183553




 > Introduction Top


Paratesticular rhabdomyosarcoma (RMS) is a rare highly malignant mesenchymal neoplasm arising from spermatic cord and epididymis. RSM results from the abnormal proliferation of rhabdomyoblasts, which can grow in any part of the body that contains embryonic mesenchymal tissue. It usually seems unilateral, painless, scrotal swelling mass.[1] In general, 5–10% of all childhood tumors are RMS and 7–10% of all RMSs are of paratesticular localization. The incidence of RMS is 4–7 cases/million children/adolescents per year.[2] When it is detected, it generally spreads to para-aortic and renal hilar nodes by lymphatic drainage from the testis or spermatic cord. This malignant fatal disease can be cured by making aggressive surgery, radiotherapy, and chemotherapy in most of the patients.[3]

Primary tumors of paratesticular region are generally known as benign masses. It is expected that this tumor has benign tissue especially if it is a semi-solid cystic mass. If a hydrocele or epididymal cyst is present, it can transilluminate. In this manner, this tumor may mimic complicated epididymal cyst, spermatocele, or hydrocele. We report an unusual case of paratesticular RMS with unusual presentation as epididymal cyst.


 > Case Report Top


A 16-year-old Caucasian boy presented with a painless palpable mass in his right scrotum for 2 months. His family and personal histories were unremarkable. Physical examination revealed a solid mass of the hemiscrotum on the right side. It was nontender, firm, nodular, and measuring 3 cm × 3 cm. The testis was separate from the mass. After a scrotal ultrasonography (USG), it was reported that the size of the right testis was normal and there was no intratesticular pathology. The palpable mass was reported as a 3-cm epididymal cyst with probable hemorrhage, originating from epididymis.

Based on physical examination and USG findings, the patient was transferred to the operating room with the diagnosis of epididymal cyst. In the surgical exploration of the right hemiscrotum, the testis was macroscopically normal and the semi-solid mass was easily excised intact from the epididymis. The mass was white, nodular, firm, well-circumscribed and encapsulated [Figure 1]. Because the mass had a limited shape and was excised easily from the epididymis, the impression was that it was a benign mass. Its pathology report showed that the mass was as a paratesticular embryonal RMS [Figure 2]. Microscopic examination showed a highly cellular tumor composed of rhabdomyoblasts, and a cellular sarcoma with small round- to spindle-shaped cells having scanty cytoplasm and high mitotic activity. The presence of desmin- and myogenin-positive cells in the specimen confirmed the diagnosis. After this pathology report, a radical orchiectomy through an inguinal incision with high dissection and ligation of the spermatic cord was performed. In the pathology of orchiectomy material, it was reported that the testicular parenchyma had not been invaded by tumor and surgical margins were negative.
Figure 1: Macroscopic view of scrotal mass showing a tumor in the paratesticular region

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Figure 2: Histological aspects of paratesticular rhabdomyosarcoma (H and E, ×200)

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Thoraco-abdominopelvic computerized tomography (CT) revealed no evidence of retroperitoneal or mediastinal lymphadenopathy or distant metastases. The bone marrow biopsy was not performed because of the type of RMS and low clinical stage of the patient. After a consultation with medical oncology, the patient, who was classified into Group IA of the intergroup RMS study, was started chemotherapy after a completing tumor staging procedures because there was a localized disease, completely excised, and no evidence of metastasis. After cryopreservation of semen, four cycles of vincristine, cyclophosphamide, and dactinomycin chemotherapy were started. The patient is in the postoperative 48th week and there is no relapse in follow-up.


 > Discussion Top


Paratesticular RMS usually presents as asymptomatic scrotal mass followed by trauma, hydrocele, or hernia.[4] It can be misdiagnosed as testicular torsion, hydrocele, epididymo-orchitis, inguinal hernia, and mumps orchitis.[1] These extratesticular lesions can be distinguished by clinical features and high-resolution USG, which reveals a solid heterogeneous mass with increased vascularity on color Doppler.[1] However, sometimes, it is not easy to make definitive diagnosis with clinical findings and USG before the histopathological examination. After the excision of the epididymal cyst in our patient, the pathological report demonstrated that this cyst was a paratesticular RMS. To our knowledge, this is the first case of paratesticular RMS presenting as an epididymal cyst. We performed radical inguinal orchiectomy to our case at the same day the pathology report was taken.

Paratesticular RMS has a better prognosis than RMS and 5-year overall survival for paratesticular RMS is between 30% and 95.5%.[5],[6] Surgery alone produced approximately a 50% 2-year relapse-free survival before using systemic chemotherapy.[7] Reported risk factors are stage, tumor size (>5 cm), nodal involvement, and age.[8] Although surgical guidelines recommend a high inguinal orchiectomy, a scrotal approach is used in up to 25% of cases because, as in our case, the definitive diagnosis can sometimes be made after the pathological examination in the presence of testicular torsion, hydrocele, epididymitis, inguinal hernia, mumps orchitis, and epididymal cyst.[1],[6] In contrast with other primary sites, up to 82% of paratesticular RMS are completely resectable.[1] The current multimodal treatment of RMS including surgery, radiation, and systemic chemotherapy yields 5-year survival rates of 80%.[2],[8] Because of this, we performed additional chemotherapy to our case. Retroperitoneal lymph node (RPLN) is found in approximately 25% of patients at presentation.[1] The most appropriate supplementary therapy seems chemotherapy after orchiectomy without positive RPLN. If the RPLN is negative, additional therapy must be limited. Patient race, histology, and tumor laterality are not significant predictors of nodal metastasis.[9] For further staging and treatment, highly selective RPLN dissection (RPLND) is needed after chemotherapy if the positive nodes are present. Radiotherapy is not recommended for patients with completely resected localized disease and is only recommended to the patients with locally advanced disease or nodal involvement on RPLND.[10] According to these acknowledges, we did not perform RPLND and radiotherapy because our patient had local disease and there is no RPLN on the CT scan. Our case is in the 48th week and we encountered no relapse condition.

Paratesticular RMS is rare and malign neoplasm in young adults with multimodality approach in diagnosis as well as treatment. Cystic paratesticular RMS can be confused with complicated epididymal cyst, spermatocele, or hydrocele. However, it should be remembered that these paratesticular masses can be paratesticular RMS as in our case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Dangle PP, Correa A, Tennyson L, Gayed B, Reyes-Múgica M, Ost M. Current management of paratesticular rhabdomyosarcoma. Urol Oncol 2016;34:84-92.  Back to cited text no. 1
    
2.
Ferrari A, Casanova M, Massimino M, Luksch R, Piva L, Fossati-Bellani F. The management of paratesticular rhabdomyosarcoma: A single institutional experience with 44 consecutive children. J Urol 1998;159:1031-4.  Back to cited text no. 2
    
3.
Raney RB Jr., Hays DM, Lawrence W Jr., Soule EH, Tefft M, Donaldson MH. Paratesticular rhabdomyosarcoma in childhood. Cancer 1978;42:729-36.  Back to cited text no. 3
    
4.
Metcalfe PD, Farivar-Mohseni H, Farhat W, McLorie G, Khoury A, Bägli DJ. Pediatric testicular tumors: Contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170(6 Pt 1):2412-5.  Back to cited text no. 4
    
5.
Olney LE, Narayana A, Loening S, Culp DA. Intrascrotal rhabdomyosarcoma. Urology 1979;14:113-25.  Back to cited text no. 5
    
6.
Seitz G, Fuchs J, Martus P, Klingebiel T, Leuschner I, Schuck A, et al. Outcome, treatment, and treatment failures in patients suffering localized embryonal paratesticular rhabdomyosarcoma: Results from the “Cooperative Weichteilsarkom Studiengruppe” trials CWS-86, -91, -96, and -2002P. Ann Surg 2015 [Epub ahead of print].  Back to cited text no. 6
    
7.
Sutow WW, Sullivan MP, Ried HL, Taylor HG, Griffith KM. Prognosis in childhood rhabdomyosarcoma. Cancer 1970;25:1384-90.  Back to cited text no. 7
    
8.
Ferrari A, Bisogno G, Casanova M, Meazza C, Piva L, Cecchetto G, et al. Paratesticular rhabdomyosarcoma: Report from the Italian and German Cooperative Group. J Clin Oncol 2002;20:449-55.  Back to cited text no. 8
    
9.
Alanee S, Holland B, Dynda D, Kamel O, Ganai S. Primary tumor size predicts pathologic findings in the retroperitoneal lymph nodes in patients with paratesticular rhabdomyosarcoma. Virchows Arch 2014;465:697-701.  Back to cited text no. 9
    
10.
Wu HY, Snyder HM 3rd, Womer RB. Genitourinary rhabdomyosarcoma: Which treatment, how much, and when? J Pediatr Urol 2009;5:501-6.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2]



 

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