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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 1  |  Page : 91-93
Primary high-grade testicular leiomyosarcoma


1 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi 221 005, Uttar Pradesh, India
2 Department of Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi 221 005, Uttar Pradesh, India

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   Abstract 

We herein present an extremely rare occurrence of primary intratesticular leiomyosarcoma. A 65-year-old patient presented with painless enlargement of the right testis. A high inguinal orchiectomy was done. Histopathological examination of the excised mass was consistent with high-grade leiomyosarcoma. Pertinent literature is reviewed and the importance of excluding the germ cell tumor and the paratesticular neoplasm is emphasized.

Keywords: Leiomyosarcoma, primary, testis, germ cell tumor

How to cite this article:
Kumar M, Patne SC, Kumar S, Shukla V K. Primary high-grade testicular leiomyosarcoma. Indian J Pathol Microbiol 2009;52:91-3

How to cite this URL:
Kumar M, Patne SC, Kumar S, Shukla V K. Primary high-grade testicular leiomyosarcoma. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Nov 21];52:91-3. Available from: http://www.ijpmonline.org/text.asp?2009/52/1/91/44977



   Introduction Top


Leiomyosarcoma of the testis is known to occur following radiotherapy, following the use of anabolic corticosteroids and in association with testicular germ cell tumors. However, occurrence of testicular leiomyosarcoma without these events (primary intratesticular leiomyosarcoma) is extremely rare. To our knowledge, only nine cases of primary intratesticular leiomyosarcoma have been reported. [1],[2],[3],[4],[5],[6],[7],[8] These cases are reviewed and compared in [Table 1]. We herein present one such rare occurrence of a primary intratesticular leiomyosarcoma in an elderly person.


   Case Report Top


A 65-year-old male presented with discomfort and painless enlargement of the right testis over the past 2 months. No urologic or constitutional symptoms were present. He did not receive any radiation therapy or anabolic corticosteroids in the past. A local examination revealed a hard mass of 13x8x6 cm in the right testis without associated inguinal adenopathy. An ultrasonography of the right scrotum showed an intratesticular complex mass 8.5x6.6 cm in size. Hypoechoic solid and cystic areas were evident in the mass with marginal calcification. The contralateral testis and the spermatic cord were unremarkable. A systemic examination; computed tomography (CT) scans of the chest, abdomen and pelvis; and routine hematologic profiles were all within the normal limits. His serum level of a feto protein (AFP) was 2.3 ng/ml (normal <10 ng/ml), his b human chorionic gonadotropin (b-HCG) level was 3.4 mIU/ml (normal <5 mIU/ml) and his lactate dehydrogenase (LDH) was 352 U/L (normal 240-480U/L). With testicular tumor being the most probable diagnosis, the patient underwent an inguinal orchiectomy with high ligation of the spermatic cord. The excised specimen was fixed in 10% formalin solution for the histopatholgical examination.

Grossly, the specimen measured 13x8x7 cm with the intact tunica albuginea. The testicular mass was hard to cut; cut sections showed a fleshy, gray-white tumor with a whorled disposition. The poorly circumscribed tumor had virtually replaced and extensively invaded the testis [Figure 1A]. Areas of cystic degeneration were also present. The spermatic cord, the epididymis and the tunica albuginea were unremarkable. Hematoxylin- and eosin-stained sections showed a cellular tumor arranged in intersecting fascicles. Individual tumor cells were spindle shaped. The tumor cells had eosinophilic cytoplasm and blunt-ended cigar-shaped nuclei. Evidence of sharply demarcated abrupt areas of the coagulative necrosis was noticed [Figure 1B]. The necrotic area was separated from the viable tumor by the nuclear debris at interface. The tumor cells widely invaded and compressed the seminiferous tubules [Figure 1C]. There was a marked nuclear pleomorphism with the presence of prominent nucleoli [Figure 1D]. The mitotic count was 40-50/10 HPF. Thirty sections examined from the testicular mass did not reveal any evidence of the germ cell component. Masson trichrome stain demonstrated fuschinophilia in the cytoplasm of the tumor cells. Immunohistochemistry against desmin showed moderate to strong cytoplasmic immunoreactivity in the tumor cells. Overall, the findings were consistent with primary intratesticular leiomyosarcoma. The patient did not receive further adjuvant chemotherapy or radiotherapy. Six months after the operation, a CT scan of the abdomen, chest and pelvis was negative for metastatic disease. Serum levels of AFP, β-HCG and LDH were 2.1 ng/ml, 3.7 mIU/ml and 278 U/L, respectively.


   Discussion Top


Leiomyosarcomas are malignant soft-tissue tumors, arising from the undifferentiated smooth muscle cells of the mesenchymal origin. [9] Leiomyosarcoma of the scrotum have been classified into the paratesticular and the intratesticular, the latter being an uncommon neoplasm with the rare potential for distant metastasis. [3] Intratesticular leiomyosarcoma is believed to arise from the smooth muscle elements of the testicular parenchyma such as the blood vessels or the contractile cells of the seminiferous tubules. [1],[6] Because of the extreme rarity, diagnosis of intratesticular leiomyosarcoma should only be given after a thorough gross and microscopic examination. Grossly, the more commonly seen paratesticular smooth muscle tumors originating from the spermatic cord and the epididymis should be ruled out. [5],[6] Moreover, co-occurrence of the sarcoma and the germ cell tumor is reported to have an adverse prognosis. [2] Therefore, before establishing the diagnosis of primary or pure intratesticular sarcoma, the presence of the germ cell tumor elements also needs to be excluded.

Patients with intratesticular leiomyosarcoma are usually older than 40 years. The majority of these patients present with painless testicular enlargement and inguinoscrotal discomfort. Owing to only a few reports, histological criteria for the diagnosis of intratesticular leiomyosarcoma are lacking in literature. In the presence of significant nuclear atypia, a mitotic count ≥10/10 high-power field and coagulative necrosis with nuclear debris are criteria for the diagnosis of uterine spindle cell leiomyosarcoma.[10] The same diagnostic criteria may well be used for the diagnosis of intratesticular leiomyosarcoma. As compared with leiomyosarcoma at the other sites, the prognosis of intratesticular leiomyosarcoma remains good. Out of the nine primary cases, only a single case of death due to pulmonary metastatic disease has been reported. [3] Radical orchiectomy (RO)/high inguinal orchiectomy (HIO) followed by surveillance appears to be the treatment of choice. [5],[6] Recently, a postradiotherapy high-grade leiomyosarcoma of the testis was managed by RO and follow-up. [9]

Eight of the previously nine primary intratesticular leiomyosarcoma were of either low grades or unspecified [Table 1]. Our patient has high-grade intratesticular leiomyosarcoma. Despite the high-grade tumor, our patient did not develop metastatic or recurrent disease. This indicates that irrespective of the tumor grade, a high inguinal orchiectomy is adequate treatment for these patients. It also offers further evidence that leiomyosarcoma in this location responds better than at any other site. However, a close follow-up is required, as death due to pulmonary metastasis has been reported 14 months after the orchiectomy in a 70-year-old patient. [3]

 
   References Top

1.Yachia D, Auslaender L. Primary leiomyosarcoma of the testis. J Urol 1989;141:955-6.  Back to cited text no. 1  [PUBMED]  
2.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. 2  [PUBMED]  
3.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. 3  [PUBMED]  
4.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. 4  [PUBMED]  [FULLTEXT]
5.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. 5    
6.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. 6  [PUBMED]  [FULLTEXT]
7.Wakhlu A, Chaudhary A. Massive leiomyosarcoma of the testis in an infant. J Pediatr Surg 2004;39:e16-7.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.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. 8  [PUBMED]  [FULLTEXT]
9.Canales BK, Lukasewycz SJ, Manivel JC, Pryor JL. Postradiotherapy intratesticular leiomyosarcoma. Urology 2005;66:657.  Back to cited text no. 9    
10.Fletcher CD. Diagnostic histopathology of tumours. 3rd ed. New York: Churchill Livingstone Elsevier; 2007.  Back to cited text no. 10    

Top
Correspondence Address:
Mohan Kumar
Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi 221 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.44977

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    Figures

  [Figure 1A], [Figure 1B], [Figure 1C], [Figure 1D]
 
 
    Tables

  [Table 1]

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