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OBJECTIVES: Spermatic cord sarcomas are exceedingly rare, often misdiagnosed and subsequently improperly treated at local hospitals. This retrospective study looked at the oncological outcomes of spermatic cord sarcoma cases managed with curative intent resection at a tertiary referral sarcoma centre. We specifically studied how initial inadequate resections impact the oncologic outcomes compared to primary tumour resections at the reference centre. METHODS: One hundred eighteen consecutive patients affected by primary, localized spermatic cord sarcoma surgically managed at our reference centre from January 2001 through January 2021 were included. Primary endpoints were local relapse free (LRFS), distant metastasis free (DMFS) and overall survival (OS). These outcomes were evaluated with multi-nomial logistic regression and Cox proportional hazards regression models for a co-relation to known patient, tumour and treatment-related prognostic factors, including a prior inadequate resection and time from diagnosis to a complete oncologic resection as independent variables. Secondarily, we compared the above variables and treatment intervals among the subgroups of primary versus re-resection surgery. RESULTS: Over a median follow-up of 54 months (IQR 25-105), 12 patients (10.2%) developed local recurrence (LR) and 14 (11.6%) had distant metastasis (DM). 5-year local relapse (LRFS) and distant metastasis-free survival (DMFS) were 89.3% and 86.5%, respectively. Higher tumour grade and size were associated with a worse DMFS (p=<0.05). Likewise, marginal (R1) resection correlated with an inferior LRFS (p=< 0.05). Eighty-four patients (71.2%) had their initial diagnosis established on an inadequate surgical excision performed in a local hospital, followed by a re-excision at our centre (Re-resection group). During the same period, 34 (28.8%) were managed primarily with biopsy and treatment at our reference centre (Primary-resection group). The two groups had statistically significant differences in tumour size, histopathology, surgery duration, rate of postoperative complication and R0 resection (p < 0.005). Additionally, the difference in time intervals to achieve the treatment targets was statistically insignificant and did not correlate to the risk of recurrence as an independent variable. Residual disease was present in 51.2 % (n = 43) of the re-excision specimens. However, following a complete R0 resection, this did not correlate with a higher risk of recurrence (p = 0.481). CONCLUSION: Prompt referral to a tertiary centre, where multidisciplinary evaluation and sound oncologic resections are the standard of treatment, can align the OS and DFS of patients receiving incomplete surgery elsewhere to those treated primarily in referral centres. The primary determinant of prognosis remains surgical margin, tumour size and grade.

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PURPOSE: To evaluate the outcomes of adult patients with spermatic cord sarcoma (SCS). METHODS: All consecutive patients with SCS managed by the French Sarcoma Group from 1980 to 2017 were analysed retrospectively. Multivariate analysis (MVA) was used to identify independent correlates of overall survival (OS), metastasis-free survival (MFS), and local relapse-free survival (LRFS). RESULTS: A total of 224 patients were recorded. The median age was 65.1 years. Forty-one (20.1%) SCSs were discovered unexpectedly during inguinal hernia surgery. The most common subtypes were liposarcoma (LPS) (73%) and leiomyosarcoma (LMS) (12.5%). The initial treatment was surgery for 218 (97.3%) patients. Forty-two patients (18.8%) received radiotherapy, 17 patients (7.6%) received chemotherapy. The median follow-up was 5.1 years. The median OS was 13.9 years. In MVA, OS decreased significantly with histology (HR, well-differentiated LPS versus others = 0.096; p = 0.0224), high grade (HR, 3 versus 1-2 = 2.7; p = 0.0111), previous cancer and metastasis at diagnosis (HR = 6.8; p = 0.0006). The five-year MFS was 85.9% [95% CI: 79.3-90.6]. In MVA, significant factors associated with MFS were LMS subtype (HR = 4.517; p < 10-4) and grade 3 (HR = 3.664; p < 10-3). The five-year LRFS survival rate was 67.9% [95% CI: 59.6-74.9]. In MVA, significant factors associated with local relapse were margins and wide reresection (WRR) after incomplete resection. OS was not significantly different between patients with initial R0/R1 resection and R2 patients who underwent WRR. CONCLUSIONS: Unplanned surgery affected 20.1% of SCSs. A nonreducible painless inguinal lump should suggest a sarcoma. WRR with R0 resection achieved similar OS to patients with correct surgery upfront.

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Spermatic cord Leiomyosarcoma is an extremely rare intrascrotal tumour. Owing to its rarity, no definitive management guidelines have been formulated as yet. The majority of published literature comprises of case reports or case series and show varying outcomes depending upon multiple patient- and disease-related factors. Almost all cases are older adults with majority in the sixth or seventh decades of life. It is commonly labelled as an indolent curable tumour if treated Spermatic cord Leiomyosarcoma is an extremely rare intrascrotal tumour. Owing to its rarity, no definitive management guidelines have been formulated as yet. The majority of published literature comprises of case reports or case series and show varying outcomes depending upon multiple patient- and disease-related factors. Almost all cases are older adults with majority in the sixth or seventh decades of life. It is commonly labelled as an indolent curable tumour if treated early by radical orchiectomy. The role of lymphadenectomy, adjuvant radiotherapy or chemotherapy is unclear. This case report concerns a young 38-year-old man who suffered from a painless firm left hemiscrotal mass for the past two years. Ultrasonography showed an intrascrotal paratesticular mass. Metastatic workup was negative. Left radical orchiectomy was performed and histopathology of the surgical specimen revealed leiomyosarcoma of the spermatic cord. The patient is on post-surgery follow-up and disease-free for six months. A literature review is also presented.

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Sarcoma in the spermatic cord is rare, and accurate diagnosis of the histopathological subtypes are often difficult. A 62-year-old male smoker with a five-month history of left inguinoscrotal swelling was admitted to my hospital. Ultrasonography and computed tomography examinations showed a 4.0 cm×5.5 cm×4.5 cm solid mass engulfing the spermatic cord. The boundaries between the mass and the left testicle was distinct. Ultrasound-guided fine needle aspiration biopsy showed tumor originated from mesenchymal tissue, which is likely to be malignant, hence, enabling its removal by a left spermatic cord tumor resection via a left inguinal approach, under general anesthesia. The final pathological report showed an undifferentiated spermatic cord sarcoma. No recurrence and metastasis were observed in the patient during the subsequent 26 months follow-up period.

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Introduction: Spermatic cord sarcomas represent a rare genitourinary malignancy with a challenging diagnostic and therapeutic pathway. Different histotypes have been described and prognostic factors remain poorly defined due to the paucity of data presented in literature. Methods: Retrospective chart review of 22 adult patients treated for spermatic cord sarcoma in a single institution in the last 20 years was performed. Clinicopathological characteristics of the tumors were collected with primary and subsequent treatment. Survival analysis was performed in order to identify prognostic factors of disease-specific survival. Results: The median age at diagnosis was 68 years (58-78), the most common histotype was liposarcoma (14/22), and most patients (63.6%) were found to have positive surgical margins after surgery. The 5-year cancer specific survival was 91.3%. Grading (p = 0.480), histotype (p = 0.327), and type of intervention (p = 0.732) were not associated with survival. All patients dead of disease had positive surgical margins (p = 0.172). Conclusion: We report a good prognosis at 5 years. Wide radical resection remains the first and probably the most important step; thus, according also to literature, negative surgical margins should be aimed.

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Spermatic cord sarcomas are rare tumors for which the most important is the initial diagnostic procedure. They are frequently misdiagnosed after surgery for inguinal hernia, inguinal lymphadenectomy or testicular malignancy. Any clinical suspicion has to lead to perform imaging with MRI and a core needle biopsy in order to obtain an accurate preoperative diagnosis. Liposarcoma and leiomyosarcoma are the most common histological subtypes in elderly adults, rhabdomyosarcoma in children or in young adults. A CT scan will precede the treatment in order to look for distant metastasis and abdominal involvement. The therapeutic strategy as well as the surgical planning are then adapted to the histological, morphological and prognostic factors. Surgery is the cornerstone for the treatment of spermatic cord sarcoma. The minimum requirements for the surgical procedure are a wide excision of the tumor en bloc with radical orchidectomy, excision of the ipsilateral scrotum and high spermatic cord ligation. It could be enlarged to the anterior abdominal wall and adjacent organs some required a soft tissue flap. Spermatic cord sarcoma and trunk wall sarcoma have the same prognosis for which local recurrence could significantly decrease survival. Consequently, surgeon in charge with these tumors has to be familiar with soft tissue sarcoma and the management of these patients must be carried out under the supervision of a multidisciplinary team within the Netsarc network.

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A 70-year-old man presented with a left scrotal swelling. A computed tomography scan showed an 8-cm left scrotal mass and no metastasis. Radical orchiectomy with high ligation of the spermatic cord was performed. The tumor was classified as a high-grade leiomyosarcoma of the left testis. An intensive follow-up including repeated computed tomography scans was performed. A computed tomography scan 34 months after the surgery showed a retroperitoneal mass in front of the left kidney. Resection of the mass was performed. Microscopically, the mass was metastatic leiomyosarcoma. Intratesticular leiomyosarcoma is rare; only 18 cases have been reported. This is the first case in which leiomyosarcoma metastasized to the retroperitoneal space postoperatively. We herein review the literature and discuss how intratesticular leiomyosarcoma metastasized to the retroperitoneal space in this patient.