Malignant phyllodes tumor and primary breast sarcoma; distinct rare tumors of the breast

Abstract

Background Malignant phyllodes (MP) and primary breast sarcomas (PBS) are rare neoplasms with overlapping histopathologic features. We compared overall survival (OS) and estimated the association of surgery and therapies with OS. Methods We utilized the National Cancer Database (2004–2016). Patients without surgery, unknown surgery, or margins, or Stage IV disease were excluded. Kaplan–Meier curves and Cox proportional hazards models were used to estimate unadjusted and adjusted OS, respectively. Results A total of 3209 (59.5%) MP, and 2185 (40.5%) PBS were identified. Despite a larger median tumor size in MP (46 vs. 40 mm PBS, p < 0.001), lumpectomy rate was higher for MP (52.9% vs. 27.0% PBS, p < 0.001). Compared to MP, PBS patients more frequently received radiation (28.9% vs. 24%), and chemotherapy (28.1% vs. 4%), both p < 0.001. Unadjusted OS was lower for PBS (57% vs. 85% MP, log‐rank p < 0.001). PBS (vs. MP) had persistently worse survival (hazard ratio [HR]: 1.98, 95% confidence interval [CI]: 1.69–2.31) after adjustment. Receipt of adjuvant therapies was not associated with OS (either neoplasm); however, lumpectomy was associated with improved OS (vs. mastectomy) for both PBS (HR: 0.59, 95% CI: 0.50–0.75) and MP (HR: 0.65, 95% CI: 0.53–0.81). Positive margins had no association with OS for MP (HR: 1.09, 95% CI: 0.75–1.60), but was associated with worse survival for PBS (HR: 2.35, 95% CI: 1.82–3.02). Discussion We found significant survival differences between MP and PBS, with PBS having a consistently worse OS. Our findings support surgery as the mainstay of treatment for both tumor types and suggest that lumpectomy may be a reasonable option for select patients without compromising outcomes.

Primary Breast Sarcoma: A Two-Centre Analysis and Review of Literature

“…Moreover, the margin in a patient with leiomyosarcoma from our cohort was 0.1 cm only and she is still without progression 617 days after surgery. In accordance with other authors, we conclude that negative margin achievement in patients with primary breast sarcoma is a cornerstone of surgical therapy and is connected with a better prognosis, but the benefit of larger safe margins remains unclear [5, 8, 14, 21, 42].…”

“…Fujita et al [41] performed a literature review of case reports on margin status and local recurrence risk in leiomyosarcomas with the recommendation of at least 3 cm negative margin in mastectomy and 2 cm in breast-conserving surgery. On the other hand, few authors presented margin status as a prognostic factor without the recommendation of safety margins [5, 8, 14, 21]. Pandey et al [5] published an analysis with margin status as a prognostic factor distinguished as positive or negative (without cut-off) as statistically significant.…”

“…The mastectomy used to be a standard for therapy in all patients with primary breast sarcoma, but the survival analysis between mastectomy and breast-conserving surgery did not reveal a better prognosis for patients with more radical therapy [11][12][13][14]. The indication of breastconserving surgery in primary breast sarcoma is nowadays comparable to breast cancerlarger tumours should be treated by mastectomy because of unsatisfactory cosmetic effect and negative margin achievement.…”

A case report of a patient with ductal carcinoma and a malignant phyllodes tumor in situ in 2 separate breasts

“…Since metastases to the axillary lymph nodes are uncommon, axillary dissection was not done routinely. [23,24] However, the debate over mastectomy vs breast-conserving surgery continues to this day. Cases with MPT should be selectively treated according to tumor size, according to a review of the Surveillance, Epidemiology, and End Results 1902 data (2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009).…”

Current clinical practice in the management of phyllodes tumors of the breast: an international cross-sectional study among surgeons and oncologists

“…They entail a broad range of pathological and clinical features and therefore regarded as a spectrum of fibroepithelial neoplasms rather than a single entity. For individuals with malignant phyllodes tumors the prognosis is poor when metastasis occur [ 3 ]. In contrast, long-term prognosis for borderline phyllodes tumors is more favorable, and mortality from benign tumors are close to zero.…”