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BMC Cancer Aug 2023Giant breast malignant phyllodes tumor or sarcoma (GBPS) are rare entities with diameter larger than 10 cm and variously histological pleomorphisms. This disease poses...
BACKGROUND
Giant breast malignant phyllodes tumor or sarcoma (GBPS) are rare entities with diameter larger than 10 cm and variously histological pleomorphisms. This disease poses a significant threat to the quality of life of individuals, and its prognosis remains unclear. This study aimed to explore the differential diagnosis, treatment, and prognosis of GBPS in a real-world retrospective cohort.
METHODS
We collected GBPS (diameter > 10 cm, n = 10) and BPS (diameter ≤ 10 cm, n = 126) from patients diagnosed with sarcoma or malignant phyllodes tumor between 2008 and 2022. We analyzed clinical characteristics, histological status, treatment, and local recurrence using the Fisher's exact test between GBPS (diameter > 10 cm) and BPS (diameter ≤ 10 cm) cohort. We described overall survival (OS) and disease-free survival (DFS) using Kaplan-Meier curves and identified risk factors for local recurrence using logistic regression. The tumor size, age at diagnosis, and differential immunohistochemistry markers of breast sarcoma or phyllodes tumor to determine the prognosis of GBPS.
RESULTS
In our retrospective analysis of breast malignancies, we identified 10 cases of GBPS and 126 cases of BPS, corresponding to a GBPS prevalence of 0.17% (10/6000). The median age was 38.5 years (inter-quartile range, IQR: 28.25-48.5 years). During the follow-up of period (median: 80.5 months, IQR: 36.75-122 months), the local recurrence (LR) rate was 40% and 20.6%, respectively. Clinical characteristics of young age (HR:2.799, 95%CI -00.09276-0.017, p < 0.05) and cytological characteristics of marked stromal atypia (HR:0.88, 95% CI 0.39-1.40, p < 0.05) were risk factors for the poor prognosis of GBPS by COX regression model analysis. The Kaplan-Meier curves of GBPS 5-year disease-free survival (DFS) and overall survival (OS) were 31.5 months and 40 months, respectively, and were not associated with adjuvant radiation or chemotherapy.
CONCLUSION
We recommend mastectomy with a clear surgical margin as the preferred treatment for GBPS. Age and stromal atypia are significantly associated with recurrence. Adjuvant radiation therapy is advised; however, there was no improvement in overall survival. There is no consensus on the effectiveness of adjuvant chemotherapy and genetic methods, highlighting the need for further research into this aggressive tumor. We recommend a multidisciplinary approach involving a dedicated team for the management of GBPS.
Topics: Humans; Adult; Female; Phyllodes Tumor; Retrospective Studies; Breast Neoplasms; Quality of Life; Mastectomy; Sarcoma; Soft Tissue Neoplasms
PubMed: 37635229
DOI: 10.1186/s12885-023-11279-2 -
Revista Espanola de Enfermedades... Oct 2023We present the case of a 72-year-old woman with a history of right radical mastectomy due to the Phyllodes Tumor. Two months later, she went to the emergency room for...
We present the case of a 72-year-old woman with a history of right radical mastectomy due to the Phyllodes Tumor. Two months later, she went to the emergency room for intestinal occlusion. Analytically, she had anemia and thrombocytopenia. The abdominal tomography noticed signs of occlusion of the small bowell and injury of 5cm at the pericaecal level. Urgent exploratory laparotomy was performed and objectified distal ileum attached to the cecum with signs of irreversible ischemia, so an ileocaecal resection was performed with ileo-colonic anastomosis. In the postoperative period she presented paralytic ileus and paraneoplastic syndrome with bicytopenia refractory to corticosteroid treatment. To rule out abdominal complications, abdominal tomography was performed on the 5th day post-op that confirmed the integrity of the anastomosis and absence of signs of mechanical occlusion. The patient's evolution was unfavorable, went on to exitus on the 14th day. The anatomopathological study diagnosed metastasis of malignant Phyllodes Tumor at the ileal level.
PubMed: 37882213
DOI: 10.17235/reed.2023.9958/2023 -
Cancers Aug 2023Breast sarcomas (BSs), phyllodes tumors (PTs), and desmoid tumors (DTs) are rare entities that arise from connective tissue. BSs can be classified as either primary or... (Review)
Review
Breast sarcomas (BSs), phyllodes tumors (PTs), and desmoid tumors (DTs) are rare entities that arise from connective tissue. BSs can be classified as either primary or secondary, whether they develop de novo or after radiation exposure or lymphedema. PIK3CA seems to play an important common role in different BS. Malignant PTs show similar behavior to BSs, while DTs are locally aggressive but rarely metastasize. BSs usually present as unilateral, painless, rapidly growing masses with rare nodal involvement. The diagnosis should be based on magnetic resonance imaging and a core needle biopsy. Staging should comprise a chest computed tomography (CT) scan (except for benign PT and DT), while abdominal and pelvic CT scans and bone scans should be added in certain subtypes. The mainstay of treatment for localized BS is surgery, with margin goals that vary according to subtype. Radiotherapy and chemotherapy can be used as neoadjuvant or adjuvant approaches, but their use in these settings is not standard. Advanced BS should be treated with systemic therapy, consistent with recommendations for advanced soft tissue sarcomas of other topographies. Given the rarity and heterogeneity of these entities, multidisciplinary and multi-institutional collaboration and treatment at reference centers are critical.
PubMed: 37568749
DOI: 10.3390/cancers15153933 -
Clinics and Practice Apr 2021Phyllodes tumor (PT) of the breast, particularly malignant phyllodes tumor (mPT), is a rare fibroepithelial neoplasm. A complex diagnosis is based on pathologic,... (Review)
Review
INTRODUCTION
Phyllodes tumor (PT) of the breast, particularly malignant phyllodes tumor (mPT), is a rare fibroepithelial neoplasm. A complex diagnosis is based on pathologic, radiologic, and clinical findings, with controversies about what is the best therapeutic strategy.
OBJECTIVE
Our objective was to provide an overview of the clinical, pathologic, and therapeutic aspects of this rare tumor.
CONCLUSIONS
mPT is a rare presentation of breast cancer and a challenge in clinical practice. A multidisciplinary approach should take into account some aspects like pathogenic mutations and hereditary syndromes. Oncologic surgery is the fundamental approach, and the use of adjuvant therapies is still controversial due to the lack of clinical trials. Treatment recommendations should be individualized according to patient risk and preferences. Prospective studies are fundamental to clarifying the best treatment for these tumors.
PubMed: 33917271
DOI: 10.3390/clinpract11020030 -
Case Reports in Oncology 2021Phyllodes tumor of the breast is an infrequently encountered fibroepithelial neoplasm, which accounts for 0.3-1% of all tumors. Few case reports have described the... (Review)
Review
Phyllodes tumor of the breast is an infrequently encountered fibroepithelial neoplasm, which accounts for 0.3-1% of all tumors. Few case reports have described the occurrence of giant phyllodes tumor. To our knowledge, about 20% of phyllodes tumors would be considered giant benign. Complete surgical excision is the standard of care for giant benign phyllodes tumors; axillary lymph node metastasis is rare, and dissection should be limited to patients with pathologic evidence of tumor in the lymph nodes. We report the case of a 40-year-old Mexican woman with giant mammary tumor who underwent a right total mastectomy. The pathology results showed a benign phyllodes tumor 4,857 g in weight and 40.2 × 36.3 × 15 cm in size. We do not suggest adjuvant radiation therapy for patients with benign phyllodes tumors that are widely excised. A review of the pertinent literature was performed.
PubMed: 33776693
DOI: 10.1159/000510741 -
NPJ Breast Cancer Apr 2022Malignant phyllodes tumors (PT) are rare aggressive fibroepithelial neoplasms with high metastatic potential and lack effective therapy. We established a patient-derived...
Malignant phyllodes tumors (PT) are rare aggressive fibroepithelial neoplasms with high metastatic potential and lack effective therapy. We established a patient-derived xenograft (PDX) and cell line model (designated MPT-S1) of malignant PT which demonstrated clinical response to pazopanib. Whole exome sequencing identified somatic mutations in TP53, RB1, MED12, and KMT2D. Immunohistochemistry and genomic profiles of the tumor, PDX and cell line were concordant. In keeping with clinical observation, pazopanib reduced cell viability in a dose-dependent manner and evoked apoptosis, and led to significant abrogation of in vivo tumor growth. Whole transcriptomic analysis revealed that pazopanib decreased expression of genes involved in oncogenic and apoptosis signaling. We also observed decreased expression of ENPP1, with known roles in cancer invasion and metastasis, as well as STING pathway upregulation. Accordingly, pazopanib induced micronuclei formation, and evoked phospho-TBK1 and PD-L1 expression. In an additional cohort of malignant PT (n = 14), six (42.9%) showed comparable or higher levels of ENPP1 relative to MPT-S1, highlighting its potential role as a therapeutic target. In conclusion, we established MPT-S1, a new PDX and cell line model, and provided evidence for the clinical efficacy of pazopanib in malignant PT.
PubMed: 35365682
DOI: 10.1038/s41523-022-00413-1 -
Cureus Apr 2024In this comprehensive study, we present an exceptionally rare case characterized by the occurrence of multi-recurrent asynchronous bilateral malignant phyllodes tumors....
In this comprehensive study, we present an exceptionally rare case characterized by the occurrence of multi-recurrent asynchronous bilateral malignant phyllodes tumors. Phyllodes tumors, known for their rapid growth, originate within the stromal tissue of the breast and predominantly manifest as benign entities. Our case stands out as an extraordinary anomaly, not only due to its bilateral malignant nature but also owing to the manifestation of a multi-recurrent pattern on both sides. This unprecedented presentation underscores the complexity and heterogeneity of malignant phyllodes tumors, necessitating further in-depth investigation to unravel the underlying mechanisms driving their aggressive behavior and to explore innovative therapeutic strategies aimed at optimizing patient outcomes and prognosis.
PubMed: 38738143
DOI: 10.7759/cureus.57936 -
F1000Research 2024Phyllodes tumor is a rare fibroepithelial neoplasm of the breast, which is classified histologically as benign, borderline, or malignant. Accurate preoperative diagnosis...
BACKGROUND
Phyllodes tumor is a rare fibroepithelial neoplasm of the breast, which is classified histologically as benign, borderline, or malignant. Accurate preoperative diagnosis allows the correct surgical planning and reoperation avoidance.
OBJECTIVE
To describe the clinical presentation and radiologic features of phyllodes tumors and differentiate between benign and non-benign (borderline and malignant) groups.
METHODS
A retrospective study of 57 patients with a diagnosis of phyllodes tumor who had preoperative imaging (mammography, ultrasound, or CT chest) and histological confirmation. The data was collected from 1 June 2011 to 30 September 2021. The imaging features of the phyllodes tumors were described according to the 5th edition of the ACR BI-RADS lexicon. For comparing between two groups, the student t-test, Wilcoxon rank sum test, Chi-square test, and Fisher's exact test were used for statistical analyses. The logistic regression analysis was calculated for non-benign phyllodes tumor prediction.
RESULTS
From 57 patients, the pathologic results were benign for 43 cases and non-benign phyllodes tumors for 14 cases. There was no differentiation of mammographic and CT features between benign and non-benign groups. Non-benign phyllodes tumors had the statistical significance of menopausal status, entire breast involvement, tumor size larger than 10 cm, and heterogeneous echo on univariable analysis. After multivariable analysis, menopausal status (odd ratios=13.79, p=0.04) and presence of vessels in the rim (odd ratios=16.51, p=0.019) or absent vascularity (odd ratios=8.45, p=0.047) on doppler ultrasound were significantly increased possibility of non-benign phyllodes tumor.
CONCLUSIONS
Menopausal status and presence of vessels in the rim or absent vascularity on Doppler ultrasound were important predictors for the diagnosis of non-benign phyllodes tumor.
Topics: Humans; Phyllodes Tumor; Female; Adult; Middle Aged; Breast Neoplasms; Retrospective Studies; Tomography, X-Ray Computed; Mammography; Aged; Young Adult
PubMed: 38845824
DOI: 10.12688/f1000research.145872.2 -
Medicine Nov 2023The present study aimed to explore the association between immunohistochemical markers and phyllodes tumor (PT). The retrospective case control study included biopsies...
The present study aimed to explore the association between immunohistochemical markers and phyllodes tumor (PT). The retrospective case control study included biopsies from patients with PT who underwent surgical treatment, and patients with fibronenoma (FA), diagnosed in our hospital from October 2014 to May 2021. Differences in microscopic histopathological characteristics and expressions of common immunohistochemical markers (CD10, cluster of differentiation 117 marker, cluster of differentiation 34 marker, tumor protein P53, cell proliferation antigen) for different grades of PT and FA were analyzed. A total of 69 patients were enrolled, of them 34 with PT (12 with benign PT, 13 with borderline PT, and 9 with malignant PT) and 35 with FA. With the increase of tumor malignancy, significant enlargement trend was noted; for FA, most tumor boundaries were well-defined, the stromal distribution was homogeneous, the stromal cellularity was small. In contrast for PT, as the degree of malignancy increased, tumor boundary gradually became ill-defined and the stromal distribution was heterogeneous; stromal cellularity and stromal overgrowth had increased significantly (All P < .05). Multivariate analysis showed that among other markers only CD10 expression (OR = 0.67, 95%CI: -0.88, 2.22, P < .05) was independently associated with PT. The study showed that in addition to histological features, CD10 expression was independently associated with PT and has a potential to be used as a differentiation marker.
Topics: Humans; Female; Phyllodes Tumor; Retrospective Studies; Case-Control Studies; Stromal Cells; Breast Neoplasms
PubMed: 37933030
DOI: 10.1097/MD.0000000000035677 -
Modern Pathology : An Official Journal... Jan 2021Fibroepithelial lesions of the breast, comprising the fibroadenoma and phyllodes tumour, are a unique group of neoplasms that share histological characteristics but... (Review)
Review
Fibroepithelial lesions of the breast, comprising the fibroadenoma and phyllodes tumour, are a unique group of neoplasms that share histological characteristics but possess different clinical behaviour. The fibroadenoma is the commonest benign breast tumour in women, while the phyllodes tumour is rare and may be associated with recurrences, grade progression and even metastasis. The diagnosis of fibroadenoma is usually straightforward, with recognised histological variants such as the cellular, complex, juvenile and myxoid forms. The phyllodes tumour comprises benign, borderline and malignant varieties, graded using a constellation of histological parameters based on stromal characteristics of hypercellularity, atypia, mitoses, overgrowth and the nature of tumour borders. While phyllodes tumour grade correlates with clinical behaviour, interobserver variability in assessing multiple parameters that are potentially of different biological weightage leads to significant challenges in accurate grade determination and consequently therapy. Differential diagnostic considerations along the spectrum of fibroepithelial tumours can be problematic in routine practice. Recent discoveries of the molecular underpinnings of these tumours may have diagnostic, prognostic and therapeutic implications.
Topics: Breast; Breast Neoplasms; Diagnosis, Differential; Female; Fibroadenoma; Humans; Neoplasm Grading; Neoplasm Recurrence, Local; Neoplasms, Fibroepithelial; Phyllodes Tumor
PubMed: 32461622
DOI: 10.1038/s41379-020-0583-3