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  Table of Contents    
CASE REPORT  
Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 425-427
Primary intratesticular pleomorphic leiomyosarcoma: A rare case report


Department of Pathology, JMF's ACPM Medical College, Dhule, Maharashtra, India

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Date of Web Publication18-Jan-2014
 

   Abstract 

Intratesticular leiomyosarcoma is an extremely rare tumor of the genitourinary system. We report a case of intratesticular pleomorphic leiomyosarcoma in a 68-year-old male who presented with gradual painless enlargement of the right scrotal mass over a period of 1-2 years. Radiological examination revealed a right testicular tumor.Right radical orchiectomy was performed with high ligation of the spermatic cord. Histopathological examination along with immunohistochemistry confirmed the diagnosis of a pleomorphic leiomyosarcoma of the testis. This is, to the best of our knowledge, the 13 th case of primary intratesticular leiomyosarcoma and the third case of high-grade (pleomorphic) intratesticular leiomyosarcoma in an elderly male reported in the literature.

Keywords: Intratesticular, leiomyosarcoma, pleomorphic, testis

How to cite this article:
Damle RP, Suryawanshi KH, Dravid NV, Newadkar DV. Primary intratesticular pleomorphic leiomyosarcoma: A rare case report. Indian J Pathol Microbiol 2013;56:425-7

How to cite this URL:
Damle RP, Suryawanshi KH, Dravid NV, Newadkar DV. Primary intratesticular pleomorphic leiomyosarcoma: A rare case report. Indian J Pathol Microbiol [serial online] 2013 [cited 2019 Dec 13];56:425-7. Available from: http://www.ijpmonline.org/text.asp?2013/56/4/425/125360



   Introduction Top


Primary sarcoma of the testis is rare; both in children and in adults.Various sarcomas reported in the literature include fibrosarcoma, leiomyosarcoma, Kaposi sarcoma, angiosarcoma, osteosarcoma, chondrosarcoma and Ewing's sarcoma. Sarcomatous changes are observed in somatic-type malignancies in germ cell tumor, in spermatocytic seminoma or in sertoli cell- tumor. [1] Other risk factors include post-radiotherapy and use of a high dose of anabolic steroids. Primary intratesticular pleomorphic leiomyosarcoma is extremely rare. Only 12 cases of primary intratesticular leiomyosarcoma have been reported in the literature, with only two cases showing metastasis in other organs. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12] [Table 1].We report a rare case of primary intratesticular pleomorphic leiomyosarcoma without metastasis in an elderly male.
Table 1: Comparison of case reports in the literature

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   Case Report Top


A 68-year-old man presented with discomfort and gradual painless enlargement of the right scrotal mass over a period of 1-2 years. The patient complained of pain since 15 days. He did not present with any other symptoms such as voiding complaints, weight loss, fatigue or fever. Local examination revealed a huge firm-hard scrotal mass of 19 cm × 13 cm × 16 cm without ulceration of the skin.The superficial lymph nodes were not palpated. He had no significant past medical history.

Ultrasonography examination of the right scrotum showed a solid mass of 20 cm × 12 cm × 15 cm with hypoechoic and hyperechoic components suggestive of testicular tumor. The left testicle, both epididymis and spermatic cord, were unremarkable. There was no evidence of hydrocele or paratesticular pathology. A computed tomography scan of the chest, abdomen and pelvis and routine hematologic profiles were all within the normal limits. Liver function and serum level of alfa fetoprotein, lactate dehydrogenase and beta human chorionic gonadotropin were all within normal limits. With a clinical diagnosis of testicular tumor, the patient underwent right inguinal radical orchiectomy without complication.

On gross examination, the specimen measured 19 cm × 13 cm × 16 cm. Externally, the tumor was well-encapsulated, irregular and nodular. The cut section showed a solid, fleshy and greyish white tumor with cystic areas. A whorled character is evident to some degree. Few areas of hemorrhage and necrosis were visible. The tumor mass completely replaced the whole testis [Figure 1].The spermatic cord, epididymis and tunica albuginea were unremarkable.
Figure 1: Cut secti on showing a well-encapsulated, solid, grayish-white tumor mass with few cysti c and yellowish areas, completely replacing the enti re testi s

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Histologically, multiple serial sections showed a highly pleomorphic tumor arranged in intersecting fascicles. The tumor cells were spindle shaped and had characteristically elongated and blunt-ended cigar-shaped nuclei with eosinophilic cytoplasm. Tumor cells showed marked nuclear pleomorphism and hyperchromatism with prominent nucleoli. Multinucleated tumor giant cells and areas of coagulative necrosis were also seen. The mitotic count was 25-30/hpf [Figure 2] and [Figure 3].
Figure 2: Tumor mass with a characteristic whorled appearance. (hematoxylin and eosin ×100)

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Figure 3: (a-c) Pleomorphic tumor cells arranged in a whorled patt ern with nuclear pleomorphism, high N/C rati o and- high mitoti c count with bizarre tumor giant cells. (d) Areas of coagulati ve necrosis visible (hematoxylin and eosin, ×400)

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Immunohistochemistry revealed positive staining for vimentin, SMA, muscle-specific actin and caldesmon [Figure 4].The combined histologic and immunohistochemical findings were diagnostic of primary intratesticular pleomorphic leiomyosarcoma. No metastasis or recurrence was observed in the 6-month follow-up of the patient.
Figure 4: Immunohistochemistry revealed a positive staining for vimenti n, SMA, muscle-specifi c acti n and caldesmon

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   Discussion Top


Testicular tumors are classified depending upon the tissue of origin into germ cell tumors and non-germ cell tumors. Malignant mesenchymal tumors of the testis are extremely rare. Leiomyosarcomas are malignant soft tissue tumors arising from the smooth muscle cells of mesenchymal origin. Leiomyosarcomas of the scrotum have been classified into intratesticular and paratesticular, the latter being a common site in the scrotum. [4]

Intratesticular leiomyosarcoma is a rare disease entity with a rare potential for distant metastasis. The origin of intratesticular leiomyosarcoma has been attributed to the contractile cells of seminiferous tubules, smooth muscle of blood vessels and tunica albuginea. [2],[7] The associated risk factors that have been reported for the development of leiomyosarcoma are following radiotherapy, high dose of anabolic steroids and chronic inflammation. [11]

Intratesticular leiomyosarcoma is known to occur with germ cells tumors. Therefore, before making a diagnosis of primary intratesticular leiomyosarcoma, the presence of germ cell tumor as well as the origin of the tumor from the paratesticular smooth muscle of spermatic cord and epididymis should be ruled out grossly and microscopically by thorough sectioning of the tumor mass.

Very few cases of primary intratesticular leiomyosarcoma have been reported in the literature, with age of patients ranging from 30 to 76 years. Only one case was reported in an infant. Majority of the patients presented clinically with a gradual, painless, increasing scrotal swelling. All these cases reported in the literature were clinical stage I tumors as all cases diagnosed as slow-growing local disease. [11]

Histological criteria for the diagnosis of uterine leiomyosarcoma are significant nuclear atypia, mitotic count >10/10 hpf and coagulative necrosis with nuclear debris. The same diagnostic criteria may be used for the diagnosis of intratesticular leiomyosarcoma. [10] The prognosis of intratesticular leiomyosarcoma appears good as of 12 published cases, only two cases showed metastasis. The various tumor markers used as adjuants for the diagnosis and prognosis of testicular tumors have a limited role in case of intratesticular leiomyosarcoma, as in our case.

Based on a review of the literature, treatment for an intratesticular leiomyosarcoma is radical orchiectomy followed by surveillance. However, close follow-up is mandatory to look for metastasis as death due to metastasis has been reported 1.5 years after orchiectomy in the literature. Additional chemotherapy is required in cases with advanced disease.

Only two cases of high-grade intratesticular leiomyosarcoma have been reported in the literature. [8],[10] In our case, histomorphology shows high grade (grade 3) with bizarre tumor giant cells, marked nuclear pleomorphism, prominent nucleoli and high mitotic count. No metastasis or recurrence is observed in the 6-month follow-up of the patient despite the pleomorphic nature and the high grade of the tumor. A review of the literature showed that irrespective of the high grade of the tumor, high inguinal orchiectomy is adequate treatment for these patients and prognosis remains good in clinical stage I disease.

To conclude, primary intratesticular pleomorphic leiomyosarcoma is an extremely rare, indolent tumor with a potential for distant metastasis.

 
   References Top

1.Rosai J. Male reproductive system - Testis. In: Rosai J, editor. Rosai and Ackerman's Surgical Pathology. 9 th ed. New Delhi: Elsevier, A division of Reed Elsevier, India Private Limited; 2004: p.1443.  Back to cited text no. 1
    
2.Yachia D, Auslaender L. Primary leiomyosarcoma of the testis. J Urol 1989;141:955-6.  Back to cited text no. 2
    
3.Washecka RM, Mariani AJ, Zuna RE, Honda SA, Chong CD. Primary testicular sarcoma: Immunohistochemical ultrastructural and DNA flow cytometric study of three cases with a review of the literature. Cancer 1996;77:1524-8.   Back to cited text no. 3
    
4.Hachi H, Bougtab A, Amhajji R, Otmany F, al Bouzidi A, Laalou L, et al. A case report of testicular leiomyosarcoma. Med Trop (Mars) 2002;62:531-3.  Back to cited text no. 4
    
5.Ali Y, Kehinde EO, Makar R, Al-Awadi KA, Anim JT. Leiomyosarcoma complicating chronic inflammation of the testis. Med Princ Pract 2002;11:157-60.  Back to cited text no. 5
    
6.Sattary M, Hazraty B, Saraii MB. Primary pure testicular low-grade leiomyosarcoma. Iran J Med Sci 2003;28:48-50.   Back to cited text no. 6
    
7.Singh R, Chandra A, O'Brien TS. Primary intratesticular leiomyosarcoma in a mixed race man: A case report. J Clin Pathol 2004;57:1319-20.   Back to cited text no. 7
    
8.Wakhlu A, Chaudhary A. Massive leiomyosarcoma of the testis in an infant. J Pediatr Surg 2004;39:e16-7.  Back to cited text no. 8
    
9.Takizawa A, Miura T, Fujinami K, Kawakami S, Osada Y, Kameda Y. Primary testicular leiomyosarcoma. Int J Urol 2005;12:596-8.   Back to cited text no. 9
    
10.Kumar M, Patne SC, Kumar S, Shukla VK. Primary high grade testicular leiomyosarcoma. Indian J Pathol Microbiol 2009;52:91-3.  Back to cited text no. 10
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11.Yoshimine S, Kono H, Nakagawa K, Kikuchi E, Miyajima A, Kameyama K, et al. Primary intratesticular leiomyosarcoma. Can Urol Assoc J 2009;3:E174-6.  Back to cited text no. 11
    
12.Labanaris AP, Zugor V, Smiszek R, Nutzel R, Kuhn R. Primary leiomyosarcoma of the testis. A case report. Anticancer Res 2010;30:1725-6.  Back to cited text no. 12
    

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Correspondence Address:
Rajshri Pisaram Damle
Department of Pathology, JMF's ACPM Medical College, Dhule, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.125360

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    Figures

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