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Presented by George Netto, M.D. and prepared by Amy Duffield, M.D., Ph.D.
Case 1: A 76 years old man presented with a 9 cm left groin mass
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1. Question
Week 319: Case 1
A 76 years old man presented with a 9 cm left groin mass. On immunostains, the tumor was diffusely positive for Muscle specific actin, Smooth Muscle Actin and Desmin, Focally positive for CD34 and Cytokeratin Cam5.2.images/1A_7_30_07.jpg
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Answer: Paratesticular leiomyosarcoma
Histology: The lesion is composed of anastomosing bundles of spindle cells with ovoid cigar shaped nuclei that are focally indented by paranuclear vacuoles. No significant increase in mitotic activity is present. There is focal moderate cytologic atypia and focal evidence of necrosis. Cytoplasmic cross-striations were absent. No “lipomatous” component was present. On additional immunostains, the tumor was negative for S100, Myogenin and
Myo-D1.Discussion: In a recent study from Memorial Sloan-Kettering Cancer Center reviewing the author’s experience with adult genitourinary sarcoma during a 25-year period, paratesticular region was the most common primary site, accounting for 57/131 cases. The most common histological subtypes were leiomyosarcoma and liposarcoma, accounting for 29% and 26% of cases, respectively. The latter two histologic types had a more favorable prognosis. Predictors of disease specific survival included localized disease at presentation, complete tumor resection, tumor grade, size, location and histological subtype.
In the largest series on paratesticular leiomyosarcoma (24 cases), Fisher et al. found the testicular tunica and spermatic cord to be the most common sites of origin. Like in our case, the immunohistochemical profile was that of muscle-specific actin, smooth muscle actin and desmin positivity in over 90% of cases. CD34 was positive in 3 tumors, all of which were also strongly desmin positive. Focal reactivity for cytokeratin and S-100 protein was occasionally present. 29% recurrence and 29 % metastatic rates were encountered. All four patients with high-grade (grade 3/3) leiomyosarcomas were dead of disease.
Reference(s):
– Fisher C, Goldblum JR, Epstein JI, Montgomery E. Leiomyosarcoma of the paratesticular region: a clinicopathologic study. Am J Surg Pathol. 2001; 25(9):1143-9.
– Dotan ZA, Tal R, Golijanin D, Snyder ME, Antonescu C, Brennan MF, Russo P. Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience. J Urol. 2006 Nov;176(5):2033-8; discussion 2038-9.Incorrect
Answer: Paratesticular leiomyosarcoma
Histology: The lesion is composed of anastomosing bundles of spindle cells with ovoid cigar shaped nuclei that are focally indented by paranuclear vacuoles. No significant increase in mitotic activity is present. There is focal moderate cytologic atypia and focal evidence of necrosis. Cytoplasmic cross-striations were absent. No “lipomatous” component was present. On additional immunostains, the tumor was negative for S100, Myogenin and
Myo-D1.Discussion: In a recent study from Memorial Sloan-Kettering Cancer Center reviewing the author’s experience with adult genitourinary sarcoma during a 25-year period, paratesticular region was the most common primary site, accounting for 57/131 cases. The most common histological subtypes were leiomyosarcoma and liposarcoma, accounting for 29% and 26% of cases, respectively. The latter two histologic types had a more favorable prognosis. Predictors of disease specific survival included localized disease at presentation, complete tumor resection, tumor grade, size, location and histological subtype.
In the largest series on paratesticular leiomyosarcoma (24 cases), Fisher et al. found the testicular tunica and spermatic cord to be the most common sites of origin. Like in our case, the immunohistochemical profile was that of muscle-specific actin, smooth muscle actin and desmin positivity in over 90% of cases. CD34 was positive in 3 tumors, all of which were also strongly desmin positive. Focal reactivity for cytokeratin and S-100 protein was occasionally present. 29% recurrence and 29 % metastatic rates were encountered. All four patients with high-grade (grade 3/3) leiomyosarcomas were dead of disease.
Reference(s):
– Fisher C, Goldblum JR, Epstein JI, Montgomery E. Leiomyosarcoma of the paratesticular region: a clinicopathologic study. Am J Surg Pathol. 2001; 25(9):1143-9.
– Dotan ZA, Tal R, Golijanin D, Snyder ME, Antonescu C, Brennan MF, Russo P. Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience. J Urol. 2006 Nov;176(5):2033-8; discussion 2038-9.
