Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2013  |  Volume : 56  |  Issue : 4  |  Page : 425--427

Primary intratesticular pleomorphic leiomyosarcoma: A rare case report


Rajshri Pisaram Damle, Kishor H Suryawanshi, Nandkumar V Dravid, Dhananjay V Newadkar 
 Department of Pathology, JMF's ACPM Medical College, Dhule, Maharashtra, India

Correspondence Address:
Rajshri Pisaram Damle
Department of Pathology, JMFSQs ACPM Medical College, Dhule, Maharashtra
India

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.



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;56:425-427


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 2020 Aug 10 ];56:425-427
Available from: http://www.ijpmonline.org/text.asp?2013/56/4/425/125360


Full Text

 Introduction



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}

 Case Report



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}

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}{Figure 3}

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}

 Discussion



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.

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