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

Objectives: To assess long-term results of surgical resections of the sporadic extra-large pelvi-perineal soft tissue tumors. Design: Retrospective series of patients referred to the National Cancer Institute 2001-2022. Setting: Tertiary care cancer university hospital in Egypt. Population: 13 patients (3 males) averaged age 57 years, had large 29 cm (18-38) pelvi perineal tumors (10 to the paraanal ischiorectal spaces & 3 vulval) plus upper intra-abdominal extensions in 9 cases. Methods: The entire underwent combined open abdomino-perineal approach to widely en masse resect tumors plus infiltrated organ. No downsizing hormonal treatment was offered preoperative. Main Outcome Measures: Disease free Survival, recurrence characters and salvage Results: Extensive pelvic and perineal tumor resection is tough and meticulous but straightforward with minor (CDC grade I&II) early and delayed morbidity. Resection extends to hysterectomy, vaginectomy and vascular resection. Histopathology plus immunohistochemistry show 9-aggressive angiomyxoid tumors (AA), 2-fibromatosis and 2-neurofibromas. Margins of resection are all adequate (R0) except for 2 (R1). After 50-months 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 (13-27 m) and were amenable to curative salvage resections; while, 1/2 of patients with fibromatosis died of disseminated peritoneal relapses. No systemic metastases are noticed. Conclusions: Extensive 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.