The infrequent large pelvi-perineal tumors as a surgical dilemma: en bloc resection and long-term results

To assess long-term results of surgical resections of the sporadic extra-large pelvi-perineal soft tissue tumors, a retrospective series of patients referred to the National Cancer Institute 2001–2022 (tertiary care cancer university hospital in Egypt) was designed. Thirteen patients (3 males) averaged age 57 years had large 29 cm (18–38) pelvi-perineal tumors (10 to the paraanal ischiorectal spaces and 3 to the vulva) plus upper intra-abdominal extensions in 9 patients (69%). Symptoms were nonspecific with delayed presentation that averaged 22-month. The entire underwent combined open abdomino-perineal approach to widely en masse resect tumors plus infiltrated organs. No downsizing hormonal treatment was offered preoperative. Main outcome measures were disease free survival, recurrence pattern and salvage. Extensive pelvic and perineal tumor resection is tough and meticulous but straightforward with minor (Clavien-Dindo Classification (CDC) grade I & II) early and delayed morbidity. Resection extends to hysterectomy, vaginectomy and vascular resection. Histopathology plus immunohistochemistry showed 9-aggressive angiomyxoid tumors (AA), 2-fibromatosis and 2-neurofibromas (NF). Margins of resection are all adequate (R0) except for 2 (Resection margin 1 (R1)). After 50-month median follow up, 8/13 total series (61.5%) were surviving free of disease. 4/9 of AA (44%) had local perineal and/or pelvic recurrences (13–37 months) and all were amenable to curative salvage resections; while, 1/2 patients with fibromatosis died of disseminated peritoneal relapses. No systemic metastases are noticed. Extensive tumors meticulous surgery with experienced pelvic dissection and resection could offer alone long term cure even after recurrences with minor morbidity in a good percent. Fertility sparing resection is not oncologically safe because of the frequent uterine and ovarian invasions.