Infrequent Pelvic Non-Visceral Soft Tissue Mesenchymal Tumors: Surgical Techniques for En Bloc Resection and Long-Term Surveillance

Nassar OAH, Fahim MI and Farahat IG

Department of Surgical Oncology, National Cancer Institute, Cairo University, Egypt Department of Surgical Pathology, National Cancer Institute, Cairo University, Egypt

^*Correspondance to: Omaya Abdul Hameed Nassar 

 PDF  Full Text DOI: 10.25107/2474-1647.3689

Abstract

Purpose: To assess long-term resections consequences for the sporadic large nonvisceral pelvic soft tissue tumors in a designed series referred to the National Cancer Institute 1998-2020. Main outcome measures are disease free survival, recurrence pattern and salvage. Patients and Methods: Thirty-one patients (17 females and 14 males) averaged 48 years presented with average 21 cm tumors including 17 (55%) extensions (10-paraanal ischiorectal spaces, 3-vulva, 3-gluteal region (sciatic notch) and 1-femoral triangle) plus 21 (68%) upper abdominal growths. Immunohistochemistry showed Aggressive Angiomyxoid tumors AA (10), fibromatosis (6), Peripheral Nerve Sheath Tumors PNST (6), Solitary Fibrous Tumors SFT (3), leiomyosarcoma (2), liposarcoma (2), one monophasic synovial sarcoma and one undifferentiated sarcoma. Abdominal approach was combined with special perineal incisions to widely en masse resect tumors (primary/ recurrence) plus infiltrated viscera. Results: Pelvic and perineal tumor resection extended to the viscera in 23 (74%) with 77% (R0) and low morbidity (CDC grade I-III). Following 50-m median surveillance, 22/31 (71%) were disease free with relapses in 4/10 of AA (40%) as local perineal and/or pelvic recurrences, amenable to curative salvage resections; while, 4/6 with fibromatosis died of repeated recurrences (19 m-33 m), only 1/3 with SFT died after 21-m, single PNST had resectable local relapse while other 5 were disease free, one liposarcoma patient had resectable recurrence and 2/2 of leiomyosarcomas had systemic spread. Undifferentiated sarcoma patient died of recurrence 24 month; meanwhile, synovial sarcoma male patient was disease free. Conclusion: Combined approaches enable en block resection and offer alone safe long-term disease-free survival in a reliable percent even for recurrences.

Keywords

Pelvic soft tissue tumor resection; Pelvi-perineal tumor; Vulval tumor; Aggressive angiomyxoma; Pelvic fibromatosis; Pelvic PNST; Pelvic SFT; Pelvic soft tissue sarcoma

Cite the article

Nassar OAH, Fahim MI, Farahat IG. Infrequent Pelvic Non-Visceral Soft Tissue Mesenchymal Tumors: Surgical Techniques for En Bloc Resection and Long-Term Surveillance. Clin Surg. 2024; 9: 3689..