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Primary intratesticular rhabdomyosarcoma, a rare entity: A case report with review of literature


1 Department of Radiation Oncology, MNJIO and RCC, Hyderabad, Telangana, India
2 Department of Pathology, MNJIO and RCC, Hyderabad, Telangana, India

Correspondence Address:
Pavan Kumar Lachi,
Department of Radiation Oncology, MNJIO and RCC, Hyderabad - 500 082, Telangana
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_585_15

 > Abstract 


Paratesticular rhabdomyosarcoma is a very rare mesenchymal tumor. It is an intrascrotal tumor that is localized in paratesticular structures such as the epididymis or spermatic cord. Rhabdomyosarcoma is most often observed in children and adolescents, presenting as a painless scrotal mass. An 18-year-old man presented with a painless left scrotal mass and lump abdomen that had evolved over four months. A histological examination of the lesion revealed rhabdomyosarcoma. Chemotherapy with alternative cycles of Vincristine, Adriamycin, Cyclophosphamide followed by Ifosphamide, Etoposide was given. Paratesticular rhabdomyosarcoma is a rare aggressive tumor manifesting in children and very young adults. Localized forms have a good prognosis whereas metastatic tumors show very poor results. A well-defined treatment based on surgery and chemotherapy yields good results.

Keywords: Chemotherapy, rhabdomyosarcoma, surgery



How to cite this URL:
Lachi PK, Armugham N, Triveni B, Sheela B, Krishna M R. Primary intratesticular rhabdomyosarcoma, a rare entity: A case report with review of literature. J Can Res Ther [Epub ahead of print] [cited 2019 Dec 7]. Available from: http://www.cancerjournal.net/preprintarticle.asp?id=264224




 > Introduction Top


A paratesticular rhabdomyosarcoma is a very rare mesenchymal tumor. It is an intrascrotal tumor that is localized in paratesticular structures such as the epididymis or spermatic cord. Rhabdomyosarcoma is most often observed in children and adolescents where it is usually presents as a painless scrotal mass.[1]

We report the case of an 18-year-old man who presented with a large testicular mass with abdominal lymphadenopathy developed over 4 months. An investigation into blood tumoral markers was negative. A histological examination and immunohistochemistry confirmed paratesticular rhabdomyosarcoma. Adjuvant chemotherapy was initiated.


 > Case Report Top


Our patient was an 18-year-old man who presented with a painless left scrotal mass and lump abdomen evolved over 4 months. A clinical examination revealed a hard testicular mass in his left side with a diameter of 15 cm; the mass was renitent, static, and suspicious. Abdominal examination revealed a diffuse mass in the epigastric region measuring 7 cm × 7 cm moving with respiration and firm to hard in consistency [Figure 1]. An ultrasound revealed mass lesion in the right scrotum of 15 cm × 10 cm in size with solid mixed echoic areas. A computed tomography (CT) scan of his thorax, abdomen, and pelvis showed a large heterogeneous soft-tissue mass in the right testis measuring 16 cm × 11 cm, and a large retroperitoneal nodal mass measuring 10 cm × 8 cm was seen. Our patient's levels of tumoral markers such as alpha-fetoprotein and beta-human chorionic gonadotropin were normal. An inguinal orchiectomy was performed. A histological examination of the lesion showed round-to-oval cells with mild nuclear atypia, scant amount of cytoplasm, and with areas of necrosis. Immunohistochemistry with desmin, vimentin, and CD99 was positive and CD117, pancytokeratin, and placental alkaline phosphatase was negative, and these confirm rhabdomyosarcoma [Figure 2] and [Figure 3]. Three chemotherapy sessions of alternative cycles of vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide were performed for every 3 weeks and planned for total 42 weeks. The patient was assessed 2 months after his last chemotherapy session and demonstrated good clinical improvement.
Figure 1: Clinical photograph showing enlarged right scrotum and epigastric mass

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Figure 2: Histopathological image showing round-to-oval cells with mild nuclear atypia, scant amount of cytoplasm, and with areas of necrosis

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Figure 3: Immunohistochemistry with desmin, vimentin, and CD99 was positive and CD117, pancytokeratin, and placental alkaline phosphatase was negative; these confirm rhabdomyosarcoma

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 > Discussion Top


Rhabdomyosarcoma usually occur in the extremities. A paratesticular localization is rare; the tumor develops from mesenchymal tissues of the spermatic cord and epididymis. There are two frequency peaks found for the development of rhabdomyosarcoma, the first at the age of 4 years and the second at the age of 18 years, as in our case. There is no predilection for race.[1] The consensus is that this tumor derives from mesenchymal elements of the testis envelope, epididymis, and spermatic cord. The tumor manifests as a hard painless inguinoscrotal swelling, the size and duration of development are varied, and it rarely invades the scrotal skin.[2] Differential diagnoses include testicular torsion, orchiepididymitis, scrotal abscess, and rarely, testicular tuberculosis. A testicular ultrasound was routinely performed for a scrotal mass. This imaging modality shows a mass with heterogeneous echogenicity and inguinoscrotal extension in 80% of cases.[3] This allows the nature of the intrascrotal tissue mass to be determined and specifies the exact topography. A thoraco-abdomino-pelvic CT scan allows for any deep invasion of the lymph nodes to be investigated, especially lombo-aortic and pelvic metastases as well as possible metastases to the liver or lung. In rhabdomyosarcoma, tumoral markers including alpha-fetoprotein, beta-human chorionic gonadotropin, and carcinoembryonic antigen are usually normal. This was the case with our patient. A malignant tumor might be suspected in masses sitting in the distal cord with a hard and irregular form adhering to surrounding structures; a rapid increase in tumor volume might be noted. However, the diagnosis is mainly made using histology.[4] There are four histological types of rhabdomyosarcoma: pleomorphic, alveolar, botryoidal, and embryonal. The characteristic cell is rhabdomyoblast, which is not necessary for the diagnosis. Whenever rhabdomyoblasts are not present, immunohistochemical investigations are conducted using a panel of antibodies including myosin and desmin.[5] Our patient showed small round cell morphology which was an atypical rhabdomyosarcoma. Rhabdomyosarcoma may be included in the differential diagnosis for other paratesticular sarcomas such as leiomyosarcoma, liposarcoma, and fibrosarcoma.

Multidisciplinary treatment approaches have greatly improved the prognosis of intratesticular rhabdomyosarcoma.[6] The Intergroup Rhabdomyosarcoma Study Group management guidelines for the pediatric population have resulted in reducing morbidity and increasing survival from 25% to 70% over 20 years since 1970.[6] For localized intratesticular tumors, orchidectomy followed by chemotherapy is the best treatment.[7],[8] Postorchidectomy retroperitoneal lymph node dissection (RPLND) used to be carried out as a routine staging procedure, more commonly for a therapeutic purpose.[7] RPLND combined with chemotherapy revealed excellent long-term results. The chemotherapy agents used are vincristine, actinomycin D, and cyclophosphamide. Patients with unresectable tumors who undergo treatment with chemotherapy should be considered for surgery after downgrading. Radiotherapy is recommended more commonly to control local recurrence, metastasis, or for unfavorable histology, such as alveolar rhabdomyosarcoma.[8]


 > Conclusion Top


Paratesticular rhabdomyosarcoma is a rare aggressive tumor manifesting in children and very young adults. Localized forms have a good prognosis whereas metastatic tumors show very poor results. A well-defined treatment based on surgery and chemotherapy yields good results. Radiotherapy is indicated in cases of residual foci and retroperitoneal lymph nodes. Strict follow-up has to be instituted for all patients.

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.
Radouane B, El Fenni J, Chaouir S, Amil T, Hanine A, Ben Ameur M. Paratesticular rhabdomyosarcoma. A case report. J Radiol 2004;85:779-81.  Back to cited text no. 1
    
2.
Mason BJ, Kier R. Sonographic and MR imaging appearances of paratesticular rhabdomyosarcoma. AJR Am J Roentgenol 1998;171:523-4.  Back to cited text no. 2
    
3.
Wiener ES, Lawrence W, Hays D, Lobe TE, Andrassy R, Donaldson S, et al. Retroperitoneal node biopsy in paratesticular rhabdomyosarcoma. J Pediatr Surg 1994;29:171-7.  Back to cited text no. 3
    
4.
Kourda N, El Atat R, Derouiche A, Bettaib I, Baltagi S, Zermani R. Paratesticular pleomorphic rhabdomyosarcoma in an adult: Dignosis and managment; Cancer Radiother 2007;11:280-3.  Back to cited text no. 4
    
5.
Goldfarb B, Khoury AE, Greenberg ML, Churchill BM, Smith CR, McLorie GA, et al. The role of retroperitoneal lymphadenectomy in localized paratesticular rhabdomyosarcoma. J Urol 1994;152:785-7.  Back to cited text no. 5
    
6.
Stewart LH, Lioe TF, Johnston SR. Thirty-year review of intrascrotal rhabdomyosarcoma. Br J Urol 1991;68:418-20.  Back to cited text no. 6
    
7.
Liu ZW, Zhang XQ, Hou GL, Zhang ZL, Qin ZK, Han H, et al. Primary adult intratesticular rhabdomyosarcoma: Results of the treatment of six cases. Int J Urol 2011;18:171-4.  Back to cited text no. 7
    
8.
Kelly B, Lundon D, Rowaiye B, Daly P, Walsh K. Embryonal rhabdomyosarcoma of the testis. Can Urol Assoc J 2011;5:E7-10.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
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