Optimal surgical treatment for paratesticular leiomyosarcoma: retrospective analysis of 217 reported cases

Abstract

Background Paratesticular leiomyosarcoma (LMS) is a rare tumor. Conventionally, tumor resection by high inguinal orchiectomy is performed as the preferred treatment approach for paratesticular sarcoma. On the other hand, testis-sparing surgery has recently attracted attention as a less-invasive treatment option for paratesticular sarcoma. However, the prognostic predictors and optimal treatment strategy for paratesticular LMS remain unclear because of its rarity. In this study, we systematically reviewed previously reported cases of paratesticular LMS to evaluate the prognostic factors and establish the optimal treatment strategy. Methods A systematic search of Medline, Web of Science, Embase, and Google was performed to find articles describing localized paratesticular LMS published between 1971 and 2020 in English. The final cohort included 217 patients in 167 articles. The starting point of this study was the time of definitive surgical treatment, and the end point was the time of local recurrence (LR), distant metastasis (DM), and disease-specific mortality. Results Patients with cutaneous LMS had a slightly better LR-free survival, DM-free survival, and disease-specific survival than those with subcutaneous LMS (p = 0.745, p = 0.033, and p = 0.126, respectively). Patients with higher grade tumors had a significantly higher risk of DM and disease-specific mortality (Grade 3 vs Grade 1 p < 0.001, and Grade 3 vs Grade 1 p < 0.001, respectively). In addition, those with a microscopic positive margin had a significantly higher risk of LR and DM than those with a negative margin (p < 0.001, and p = 0.018, respectively). Patients who underwent simple tumorectomy had a slightly higher risk of LR than those who underwent high inguinal orchiectomy (p = 0.067). Subgroup analysis of cutaneous LMS demonstrated that the difference in LR between simple tumorectomy and high inguinal orchiectomy was limited (p = 0.212). On the other hand, subgroup analysis of subcutaneous LMS revealed a significant difference in LR (p = 0.039). Conclusions Our study demonstrated that subcutaneous LMS and high-grade tumors are prognostic factors for paratesticular LMS. For subcutaneous LMS, tumorectomy with high inguinal orchiectomy should be the optimal treatment strategy to achieve a negative surgical margin.

Epididymal leiomyosarcoma: Report of a rare case

“…Patients treated by simple tumorectomy were reported to have a significantly higher risk of a positive surgical margin (9 of 17 vs. 5 of 27, p = .024), which they described to be an independent risk factor for local recurrence. However, there was no significant difference in terms of DM and DSS between simple tumorectomy and high inguinal orchiectomy 9 . Given that the presence of a positive margin leads to reoperation, 9 it is important to adopt an appropriate surgical approach from the beginning if this diagnosis is suspected.…”

“…However, there was no significant difference in terms of DM and DSS between simple tumorectomy and high inguinal orchiectomy. 9 Given that the presence of a positive margin leads to reoperation, 9 it is important to adopt an appropriate surgical approach from the beginning if this diagnosis is suspected. In the present case, close epididymal margin and the final diagnosis of LMS, led to the decision for reoperation.…”

Management of epididymal leiomyosarcoma: literature narrative, case report and discussion on the use of simple epididymectomy or high radical orchiectomy

“…In general, LMS are managed with a high-volume radical orchiectomy, which provides negative margins, a low recurrence rate and a high survival rate [ 6 ]. A negative effect of a radical orchiectomy is a reduced postoperative testosterone level [ 6 ], which may, in part, explain why 22.5% of patients post-orchiectomy experience one type of sexual disorder [ 7 ], including but not limited to erectile or ejaculatory dysfunction [ 8 ]. In our case, we used a radical orchiectomy.…”

“…When comparing simple tumorectomy with high-radical orchiectomy in the setting of subcutaneous vs. cutaneous paratesticular tumor management, we found both techniques offered no significant difference in terms of local recurrence, disease metastasis and disease-specific survival [ 6 ]. Though both surgical approaches achieve similar local recurrence rates, in a subgroup of subcutaneous LMS, simple tumorectomy had a significantly increased risk of local recurrence compared to high-radical orchiectomy [ 6 ]. Therefore, ultrasound, computed tomography chest or magnetic resonance abdominopelvic surgery is recommended post-resection.…”

“…Management of high-grade (2–3) tumors (high mitotic index) seldom requires adjuvant chemotherapy, radiotherapy or retroperitoneal lymph node dissection [ 6 , 9 – 12 ]. Radiotherapy has decreased the local recurrence rate in patients who receive orchiectomy [ 11 ].…”