Clinical characteristics and prognosis of cystic degeneration in retroperitoneal schwannoma: A retrospective study of 79 patients.

Abstract

Diagnosis of retroperitoneal schwannoma (RS), especially cystic RS, is frequently missed or delayed owing to its rarity, location, nonspecific symptoms, and similarities with other tumors on various imaging modalities. This study aimed to determine associations between clinical, radiological, and histopathologic features and outcome. Seventy-nine patients with pathologically confirmed RS who underwent tumor resection between June 2010 and June 2020 were retrospectively reviewed and analyzed. Patients were stratified into three groups according to degree of tumoral cystic degeneration. Cystic degeneration was significantly associated with multiple foci (p=0.025), calcification (p=0.012), and hemorrhage (p=0.000), but not size (p=0.08), high Ki-67 (p=0.094), malignancy (p=0.115; prevalence of cystic degeneration in the benign and malignant groups were 53.9% vs 100%), rough margin (p=0.162), or irregular shape (p=0.369). Malignant RS was significantly associated with multiple lymph nodes enlargement (p=0.034). Tumor size, margins, shape, or/and multiplicity did not significantly differ between benign and malignant tumors. No recurrence occurred in patients with benign RS (mean follow-up, 45 months). All malignant tumors recurred; mean time to recurrence was 11.4months (mean follow-up, 33 months). Since RS is misdiagnosed mostly as malignancy and diagnosis is often delayed, a suspicion is necessary for diagnosis when atypical features are present. In RS, cystic degeneration was not associated with tumor size, Ki-67, or malignancy; however, it was significantly associated with multiple foci, calcification, and hemorrhage. Cystic degeneration and related factors are useful for the diagnosis of RS. Malignant RS should be considered when a mass involves multiple lymph nodes. Margins, morphology, and size are not associated with malignancy. Pathological tumor type, tumor location, and adjacent anatomic structures are associated with outcome.