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Leiomyosarcoma of the Epididymis
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Leiomyosarcoma of the Epididymis A 57-year-old man presented with an epididymal mass and radical orchiectomy was performed. Gross examination revealed a 3.6 cm solid mass arising from the epididymis. Histological sections showed malignant spindle cells with eosinophilic cytoplasm arranged in fascicles, marked nuclear atypia and numerous mitotic figures, including atypical forms (figs. 1 and 2). This histomorphology is diagnostic of a high grade leiomyosarcoma with origin in the epididymis. Paratesticular sarcomas constitute a significant percentage of sarcomas of the adult genitourinary tract but they are rare.1 Paratesticular leiomyosarcomas most often involve the testicular tunica and spermatic cord.2 Leiomyosarcomas arising in the epididymis are exceedingly rare, comprising approximately 4% of paratesticular leiomyosarcomas,2 with about 23 documented cases of primary epididymal leiomyosarcoma reported.3 The majority of patients are older than 50 years and present with an intrascrotal mass grossly measuring 3 to 13 cm.3 The histological differential diagnosis includes other mesenchymal neoplasms that can arise in the epididymis, such as leiomyoma and rhabdomyosarcoma. Leiomyoma is the most common benign mesenchymal neoplasm of the epididymis but lacks the nuclear
Figure 2. Leiomyosarcoma displaying nuclear atypia
atypia and mitotic activity of leiomyosarcoma, as seen in this case. Rhabdomyosarcoma of the paratesticular region is usually seen in children and is typically embryonal, which has the appearance of a small, blue cell tumor, although there is a spindle cell variant. Immunophenotyping is important in the diagnosis of rhabdomyosarcoma and can be useful in select cases of paratesticular leiomyosarcoma.2 Most patients with epididymal leiomyosarcoma are treated with radical orchiectomy.3 Recurrence and metastasis occur in a minority of patients with paratesticular and epididymal leiomyosarcoma,2,3 and spread to lymph nodes, lung and liver may occur. For paratesticular leiomyosarcomas histological grade is a significant prognostic indicator, with high grade leiomyosarcomas behaving aggressively. Peter A. Humphrey Department of Pathology and Immunology Washington University School of Medicine St. Louis, Missouri 1. Dotan ZA, Tai R, Golijanin D et al: Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience. J Urol 2006; 176: 2033. 2. Fisher CM, Goldblum JR, Epstein JI et al: Leiomyosarcoma of the paratesticular region: a clinicopathologic study. Am J Surg Pathol 2001; 25: 1143.
Figure 1. Leiomyosarcoma with bundles of spindle cells and adjacent benign epididymis.
0022-5347/13/1895-1907/0 THE JOURNAL OF UROLOGY® © 2013 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
AND
RESEARCH, INC.
3. Petersen RO, Sesterhenn IA and Davis CJ: Testicular adnexa. In: Urologic Pathology, 3rd ed. Philadelphia: Lippincott Williams & Wilkins 2009; pp 421– 431.
http://dx.doi.org/10.1016/j.juro.2013.02.082 Vol. 189, 1907, May 2013 Printed in U.S.A.
www.jurology.com
1907
Leiomyosarcoma of the Epididymis A 57-year-old man presented with an epididymal mass and radical orchiectomy was performed. Gross examination revealed a 3.6 cm solid mass arising from the epididymis. Histological sections showed malignant spindle cells with eosinophilic cytoplasm arranged in fascicles, marked nuclear atypia and numerous mitotic figures, including atypical forms (figs. 1 and 2). This histomorphology is diagnostic of a high grade leiomyosarcoma with origin in the epididymis. Paratesticular sarcomas constitute a significant percentage of sarcomas of the adult genitourinary tract but they are rare.1 Paratesticular leiomyosarcomas most often involve the testicular tunica and spermatic cord.2 Leiomyosarcomas arising in the epididymis are exceedingly rare, comprising approximately 4% of paratesticular leiomyosarcomas,2 with about 23 documented cases of primary epididymal leiomyosarcoma reported.3 The majority of patients are older than 50 years and present with an intrascrotal mass grossly measuring 3 to 13 cm.3 The histological differential diagnosis includes other mesenchymal neoplasms that can arise in the epididymis, such as leiomyoma and rhabdomyosarcoma. Leiomyoma is the most common benign mesenchymal neoplasm of the epididymis but lacks the nuclear
Figure 2. Leiomyosarcoma displaying nuclear atypia
atypia and mitotic activity of leiomyosarcoma, as seen in this case. Rhabdomyosarcoma of the paratesticular region is usually seen in children and is typically embryonal, which has the appearance of a small, blue cell tumor, although there is a spindle cell variant. Immunophenotyping is important in the diagnosis of rhabdomyosarcoma and can be useful in select cases of paratesticular leiomyosarcoma.2 Most patients with epididymal leiomyosarcoma are treated with radical orchiectomy.3 Recurrence and metastasis occur in a minority of patients with paratesticular and epididymal leiomyosarcoma,2,3 and spread to lymph nodes, lung and liver may occur. For paratesticular leiomyosarcomas histological grade is a significant prognostic indicator, with high grade leiomyosarcomas behaving aggressively. Peter A. Humphrey Department of Pathology and Immunology Washington University School of Medicine St. Louis, Missouri 1. Dotan ZA, Tai R, Golijanin D et al: Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience. J Urol 2006; 176: 2033. 2. Fisher CM, Goldblum JR, Epstein JI et al: Leiomyosarcoma of the paratesticular region: a clinicopathologic study. Am J Surg Pathol 2001; 25: 1143.
Figure 1. Leiomyosarcoma with bundles of spindle cells and adjacent benign epididymis.
0022-5347/13/1895-1907/0 THE JOURNAL OF UROLOGY® © 2013 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
AND
RESEARCH, INC.
3. Petersen RO, Sesterhenn IA and Davis CJ: Testicular adnexa. In: Urologic Pathology, 3rd ed. Philadelphia: Lippincott Williams & Wilkins 2009; pp 421– 431.
http://dx.doi.org/10.1016/j.juro.2013.02.082 Vol. 189, 1907, May 2013 Printed in U.S.A.
www.jurology.com
1907