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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 109-111

Primary leiomyosarcoma of epididymis


1 Department of Surgical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, New Delhi - 110 029, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi - 110 029, India

Date of Web Publication19-Apr-2012

Correspondence Address:
Dillip Kumar Muduly
Department of Surgical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.95185

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 > Abstract 

Primary leiomyosarcomas rarely arise from epididymis. But they are the most common histopathological types of sarcoma arising from the epididymis. Primary epididymal leiomyosarcoma occurs usually in older patients. We report a young patient of 35 years presenting with leiomyosarcoma of left epididymis. He did not have any metastasis and underwent left high inguinal orchiectomy. He is on regular follow-up and disease free for last two years.

Keywords: Epididymis, leiomyosarcoma, radical orchidectomy


How to cite this article:
Muduly DK, Kallianpur AA, Suryanarayana Deo S V, Shukla NK, Kapali AS, Yadav R. Primary leiomyosarcoma of epididymis. J Can Res Ther 2012;8:109-11

How to cite this URL:
Muduly DK, Kallianpur AA, Suryanarayana Deo S V, Shukla NK, Kapali AS, Yadav R. Primary leiomyosarcoma of epididymis. J Can Res Ther [serial online] 2012 [cited 2019 Sep 21];8:109-11. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/109/95185


 > Introduction Top


Soft tissue sarcomas constitute 1% of all malignancies. Leiomyosarcomas constitute 10 to 20% of soft tissue sarcomas. They arise most often from genitourinary, gastrointestinal tract and retroperitoneal region. [1],[2],[3] Among genitourinary sarcomas in adults, leiomyosarcomas are the most common type and mostly arises in the bladder, kidney, or prostate. [2],[3],[4],[5] We report a case of leiomyosarcoma of epididymis and review the literature.


 > Case Report Top


We describe a case of primary epididymal leiomyosarcoma in a young adult of 35 years, who presented with mild pain in the left groin and scrotum for two months followed by a lump at the posterior aspect of the left testis. Before presentation, he had undergone fine needle aspiration cytology (FNAC) of the lesion in a hospital outside suggestive of malignant cells. On clinical examination, there was a hard mass fixed to the postero-superior aspect of right testis. Left spermatic cord, right testis, scrotal skin and inguinal nodes were free. It was not transilluminant. USG of the scrotum showed a heterogenous mass of 3 cm at the postero-superior aspect with invasion of left testis. CT scan of chest and abdomen revealed no distant metastasis. He underwent right high inguinal orchiectomy. Gross pathological examination revealed a 3 cm solid tumor mass arising from the left epididymis, invading the left testis. On histopathology, the tumor was composed of pleomorphic spindle cells arranged in fascicles [Figure 1]. Immunohistochemistry showed tumor cells to be positive for smooth muscle actin (SMA) and desmin (Des) and negative for cytokeratin (CK), HMB 45, CD117 (C-kit), and CD34. With all the above findings, a diagnosis of leiomyosarcoma of epididymis was confirmed. He was not planned for any adjuvant therapy. He is on regular follow-up and is disease free at two years of surgery.
Figure 1: H&E stained histology (40?) of tumor tissue showing pleomorphic spindle cells

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 > Discussion Top


Primary soft tissue tumors (STTs) arising from the scrotal sac can be classified into four groups; 1) scrotal subcutis/dartos/skin, 2) testicular, 3) paratesticular and 4) those arising from spermatic cord. Scrotal STTs are tumors arising from within the tissues of scrotum or skin of scrotum. Testicular STTs arise from the mesenchymal tissues of the testis. Paratesticular STTs arise from the tissues of epididymis, or coverings of testis and are more common than testicular STTs. In a retrospective review by Chen et al, [1] only one out of 24 had sarcoma and 23 had benign tumors. In another study from Chandigarh, India, out of a total of 228 cases of epididymal nodules who have undergone FNAC only one had leiomyosarcoma (0.4%) and rest had benign lesions. [2] Thus, sarcomas arising from the epididymis are rare and the most common primary epididymal soft tissue sarcoma is leiomyosarcoma. In a retrospective review of 24 cases of leiomyosarcoma of the paratesticular region by Fisher et al. [3] from three institutions, the origin of tumors were from the testicular tunica (10), spermatic cord (10), scrotal subcutis and dartos muscles (1 each), and the epididymis (1). The mean age was 62 years and tumors ranged in size from 2 to 9 cm (mean 5 cm; median 4 cm).

We searched Pubmed with MeSH terms ("Leiomyosarcoma" [MeSH] AND "Epididymis"[MeSH] ) on 24 th September 2011 and could retrieve 18 cases of primary epididymal leiomyosarcoma that have been published. Kwae et al., [4] in 1949 claimed the first case report of primary leiomyosarcoma of epididymis. The demography, treatment and follow-up of the 18 cases are depicted in [Table 1]. They usually occur in older patients with an average age of 60 years. [5] Out of 18 cases reported, only 2 patients are below the age of 50 years (one was a pediatric patient of 6 years and the other was 28 years). But the patient in the current report was a young adult of 35 years presented with the disease. Most common presentation is scrotal mass. Out of the reported 18 cases of primary epididymal leiomyosarcoma, only one had metastasis (into humerus). But the follow-up reported was not adequate (with only nine cases had follow-up of more than or equal to two years). In contrast to this, lungs are reported to be the most common site of metastasis in paratesticular tumors. In a study of 15 cases of paratesticular leiomyosarcoma, five patients died of metastasis within two years of diagnosis. [13] Thus, the biology of epididymal leiomyosarcoma may be less aggressive than paratesticular leiomyosarcomas. They do not spread commonly to the lymph nodes. Metastatic work-up includes CT scan of chest and abdomen. [6]
Table 1: Demography, treatment and follow-up of reported cases of primary epididymal leiomyosarcoma

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Treatment of choice is high inguinal orchiectomy. Lymph node dissection is not required. On immunohistochemistry, they are positive for actin, desmin and CD34. [3] Because of rarity of epididymal leiomyosarcoma, the true biological behavior and role of adjuvant chemotherapy and radiotherapy are not well defined and should be treated according to leiomyosarcoma arising out of other common sites.[17]

 
 > References Top

1.Chen C, Cen H, Huang L. [Primary epididymal tumors: Report of 24 cases]. Zhonghua Wai Ke Za Zhi 1996;34:655-6.   Back to cited text no. 1
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2.Gupta N, Rajwanshi A, Srinivasan R, Nijhawan R. Fine needle aspiration of epididymal nodules in Chandigarh, north India: An audit of 228 cases. Cytopathology 2006;17:195-8.   Back to cited text no. 2
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3.Fisher C, Goldblum JR, Epstein JI, Montgomery E. Leiomyosarcoma of the paratesticular region: A clinicopathologic study. Am J Surg Pathol 2001;25:1143-9.   Back to cited text no. 3
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4.Kwae SZ, Liu Y, Chen SC. Leiomyosarcoma of the epididymis: Case report. J Urol 1949;62:349-53.   Back to cited text no. 4
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5.Kosmehl H, Katenkamp D. [Primary soft tissue tumors of the epididymis, paratesticular tissue and spermatic cord]. Zentralbl Allg Pathol 1985;130:5-12.   Back to cited text no. 5
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6.Sherwin B, Bergman H. Primary malignant neoplasms of the spermatic cord and epididymis; two case reports. J Urol 1952;67:208-15.   Back to cited text no. 6
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7.Abell MR, Holtz F. Testicular and paratesticular neoplasms in patients 60 years of age and older. Cancer 1968;21:852-70.   Back to cited text no. 7
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8.Yadav SB, Patil PN, Karkhanis RB. Primary tumors of the spermatic cord, epididymis and rete testis. J Postgrad Med 1969;15:49-52.   Back to cited text no. 8
    
9.Rushworth GF. Leiomyosarcoma of the epididymis. Proc R Soc Med 1971;64:999.   Back to cited text no. 9
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10.Davides KC, King LM, Paat F. Primary leiomyosarcoma of the epididymas. J Urol 1975;114:642-4.   Back to cited text no. 10
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11.deLuise VP, Draper JW, Gray GF Jr. Smooth muscle tumors of the testicular adnexa. J Urol 1976;115:685-8.   Back to cited text no. 11
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12.Farrell MA, Donnelly BJ. Malignant smooth muscle tumors of the epididymis. J Urol 1980;124:151-3.   Back to cited text no. 12
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13.Helm RH, Al-Tikriti S. Primary leiomyosarcoma of the epididymis. Br J Urol 1986;58:99.   Back to cited text no. 13
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14.Demetriou D, Carpentier F, Hattab B, Goullet E, Mauroy B. [Epididymal leiomyosarcoma. A case report with review of the literature]. Prog Urol 1994;4:1031-5.   Back to cited text no. 14
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15.Planz B, Brunner K, Kalem T, Schlick RW, Kind M. Primary leiomyosarcoma of the epididymis and late recurrence on the penis. J Urol 1998;159:508.   Back to cited text no. 15
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16.Bressenot A, Marcon N, Feuillu B, Gauchotte G, Montagne K. [Primary leiomyosarcoma of the epididymis: A case report with review of the literature]. Prog Urol 2009;19:643-7.   Back to cited text no. 16
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17.Mechri M, Ghozzi S, Khiari R, Bougrine F, Bouziani A, Benrais N. A rare cause of a scrotal mass: primary leiomyosarcoma of epididymis. BMJ Case Rep [Internet]. 2009. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21686870 [Last cited 2011 Sept 28].  Back to cited text no. 17
    


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