A rare case report of breast sarcoma and synchronous thymoma in a 60-year-old woman.

This case report aims to describe the clinical presentation, imaging findings, histopathological features and therapeutic approach of a patient diagnosed with coexisting breast sarcoma and thymoma. A 64-year-old woman presented with a palpable lump in her left breast, and subsequent imaging studies (ultrasound, mammography, and MRI) revealed breast sarcoma, a rare and aggressive subtype of breast cancer. At the same time, the MRI revealed the presence of a thymoma. A multidisciplinary approach involving surgeon, breast specialist and oncologist is essential for optimal management and favorable outcomes in patients with this rare diagnosis.

Multidisciplinary Management of Phyllodes Tumours and Breast Sarcoma: A Cross-sectional Survey of Clinical Practice across the UK and Ireland.

AIMS: Phyllodes tumours and breast sarcomas are uncommon tumours and their rarity poses significant challenges in diagnosis and management. This cross-sectional study was conducted to evaluate the multidisciplinary clinical practice for these tumours across the UK and Ireland, with the aim of identifying gaps in knowledge and providing direction for establishing national guidelines. MATERIALS AND METHODS: An international survey was adapted and circulated to breast and/or sarcoma surgeons and oncologists in the UK and Ireland through national organisations. Multidisciplinary team (MDT) responses were analysed anonymously. RESULTS: Twenty-eight MDTs participated in this study, predominately from high-volume units (85.5%). Although only 43% of the surveyed units were part of a trust that holds a sarcoma MDT, 68% of units managed malignant phyllodes and angiosarcoma, whereas 64.5% managed soft-tissue sarcoma of the breast. Across all subtypes, axillary surgery was recommended by 14-21% of the MDTs and the most recommended resection margins for breast surgery were 'no tumour on ink' in benign phyllodes (39%) and 10 mm in the remaining subtypes (25-29%). Immediate breast reconstruction was supported by 11-18% of MDTs for breast sarcoma subtypes, whereas 36% and 32% advocated this approach in benign and borderline phyllodes tumours, respectively. Adjuvant radiotherapy and chemotherapy were recommended by up to 29% and 11% of the MDTs, respectively. CONCLUSION: The results of this study demonstrate a wide variation in clinical practice across the surveyed MDTs. As only 28 MDTs participated in our study, with under-representation from low-volume units, our results might be an underestimation of the variability in practice across the UK and Ireland. This multi-institutional study sheds light on controversial aspects in the management of phyllodes tumours and breast sarcoma, identifies the need for national guidelines to inform best practice, and calls for the centralisation of the management of breast sarcoma within specialist centres.

Rare case of radiotherapy-induced angiosarcoma (RIAS) after conservative and radical treatment of breast cancer: About two cases and review of the literature.

INTRODUCTION AND IMPORTANCE: Angiosarcoma of the breast is a rare malignant tumour of endothelial origin. It is characterised by a high degree of malignancy and a polymorphous clinical and radiological presentation, a source of diagnostic error and delay. It has a very poor prognosis. Mammary angiosarcoma is a rare but formidable complication of radiotherapy. The specificity of this observation is that we are presenting two clinical cases of different surgical management of breast cancer who suffered the same complication from radiotherapy. CASE PRESENTATION: We report two cases of Radiotherapy-induced angiosarcoma (RIAS) in two patients with a history of breast cancer one treated by conservative surgery and radiotherapy and the other by radical surgery and radiotherapy both patients were operated. CLINICAL DISCUSSION: Radiotherapy-induced angiosarcoma (RIAS) is a rare complication of radiotherapy. The increasing use of conservative treatment of breast cancer, which combines surgery with radiotherapy and chemotherapy, can rarely be complicated by breast sarcoma. CONCLUSION: The natural history of radiation-induced angiosarcoma is more or less rapid, with death occurring in the setting of metastatic spread after a median survival of 24 months. The quality of the surgical procedure is a prognostic factor.

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

Introduction: Primary breast sarcoma is a very rare malignant type of breast tumours with an incidence of 0.1% of all primary breast malignancies. Methods: We present a retrospective analysis of the case series from two hospitals in the Czech Republic with a review of the diagnostic and treatment approach to primary breast sarcomas with an analysis of published prognostic factors. Results: Eleven patients were included in the study, 9 women and 2 men. Statistical evaluation revealed that tumour size (p = 0.1964), grade (p = 0.1667), margin distance (p = 0.5403), mitotic activity (p = 0.8577), or age (p = 0.7822) were not prognostic factors in our cohort. Conclusion: The analysis did not prove any of the factors, such as age, tumour size, grade, or mitotic activity, to be statistically significant prognostic factors. Based on the literature review, the most common published prognostic factors are tumour size, margin status, and grade, but the results are ambiguous.

Clinico-demographic characteristics and outcomes of radiation-induced sarcomas (RIS): a CanSaRCC study.

Background: Radiation-induced sarcomas (RIS) tend to have aggressive behaviour and because of their rarity, the most appropriate management for these malignancies is uncertain. Objectives: Using the Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) database, a national sarcoma registry, we aimed to investigate prognostic factors and outcomes for RIS. Design: Retrospective study of RIS patients treated from 1996 to 2021 at three Canadian centres. Methods: RIS was defined as a sarcoma arising in a previously irradiated field following a 3+ year latency period, whose histology was distinct from the initially irradiated tumour. Clinicopathologic and treatment-related information was extracted from the CanSaRCC database. Overall survival (OS) was defined as the time from RIS diagnosis to death from any cause. Response rate (RR) to neoadjuvant chemotherapy (NACT) was based on physician assessment. Time-to-event analyses were estimated using the Kaplan-Meier method, with Cox regression for multivariate analysis. We considered a two-tailed p-value of <0.05 as statistically significant. Results: One hundred seven tumours met the criteria for RIS and were divided into three subgroups: breast angiosarcoma (BAS, n = 54), osteosarcoma (OST, n = 16), and other soft-tissue sarcomas (STS, n = 37). Patients were mostly female (n = 85, 79%), treated initially for breast carcinomas (n = 54, 50.5%), and diagnosed with high-grade tumours (n = 61/71, 86%). None had evidence of synchronous metastasis. Patients with OST were younger (median age: 48 years, p < 0.001), and BAS had the shortest latency interval (8 versus 18 years for OST/STS, p < 0.001). Most patients underwent surgery, 76% (n = 76/100) R0; 24% (n = 26) received radiation therapy, mostly (n = 15, 57.7%) neoadjuvant. Among those receiving chemotherapy, 30 (75%) underwent NACT; among patients with documented response assessment, the RR was 68% (n = 17/25), being even higher in the BAS population (89.5%, n = 13/17). Median OS was 53 months (95% CI 34-101), with a 5-year OS of 47.6%; larger tumour size, high histologic grade and older age were independent prognostic factors for worse OS. Conclusion: Surgery is standard, and NACT might be useful to downsize large lesions, especially in BAS patients. Raising RIS awareness is fundamental to promoting appropriate management and fostering research through multi-institutional collaborations.

Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Turning the Magnifying Glass on Rare and Aggressive Entities.

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.

Breast Sarcomas-How Different Are They from Breast Carcinomas? Clinical, Pathological, Imaging and Treatment Insights.

Breast sarcoma (BS) is a very rare and poorly studied condition. This has led to a lack of studies with a high level of evidence and to low efficacy of current clinical management protocols. Here we present our experience in treating this disease in the form of a retrospective case series study including discussion of clinical, imaging, and pathological features and treatment. We also compare the main clinical and biological features of six cases of BS (phyllodes tumors were excluded) with a cohort of 184 patients with unilateral breast carcinoma (BC) from a previous study performed at our institution. Patients with BS were diagnosed at a younger age, presented no evidence of lymph node invasion or distant metastases, had no multiple or bilateral lesions, and underwent a shorter length of hospital stay versus the breast carcinoma group. Where recommended, adjuvant chemotherapy consisted of an anthracycline-containing regimen, and adjuvant external radiotherapy was delivered in doses of 50 Gy. The comparison data obtained from our BS cases and the ones with BC revealed differences in diagnosis and treatment. A correct pathological diagnosis of breast sarcoma is essential for the right therapeutic approach. We still have more to learn about this entity, but our case series could add value to existing knowledge in a meta-analysis study.

Radiation-Induced Sarcomas of the Breast: A Review of a 20-Year Single-Center Experience.

Background Radiation-induced sarcomas (RISs) are histologically proven sarcomas within or around a previously irradiated site, per Cahan's criteria. RIS incidence is higher in breast cancer compared to other solid cancers and the prognosis remains poor given limited treatment options. This study aimed to review 20-year experience with RISs at a large tertiary care center. Methodology Using our institutional cancer registry database, we included patients meeting Cahan's criteria diagnosed between 2000 and 2020. Patient demographics, oncologic treatment, and oncologic outcomes data were collected. Descriptive statistics were used to describe demographic data. Oncologic outcomes were assessed using the Kaplan-Meier method. Results A total of 19 patients were identified. The median age at RIS diagnosis was 72 years (range = 39-82 months), and the median latency period for the development of RIS was 112 months (range = 53-300 months). All patients underwent surgery, three patients received systemic therapy, and six patients received re-irradiation as salvage treatment. The median follow-up time was 31 months (range = 6-172 months) from the diagnosis of RIS. Overall, five patients had local recurrence, and one patient developed distant metastases. The median time to progression was seven months (range = 4-14 months). The progression-free survival (95% confidence interval (CI)) at two years was 56.1% (37.4-84.4%). At follow-up two years after the diagnosis of sarcoma, the overall survival (95% CI) was 88.9% (75.5-100%). Conclusions While breast RIS remains rare, when managed in a large tertiary care center, overall survival outcomes appear favorable. A significant proportion of patients recur locally after maximal treatment and require salvage therapy to improve outcomes. These patients should be managed in high-volume centers where multidisciplinary expertise is available.

Incidence and Outcome of Breast Sarcomas in England (2013-2018): An analysis from the National Cancer Registration and Analysis Service.

BACKGROUND: Breast sarcomas (BS) are rare cancers originating from mesenchymal breast tissue with a paucity of national population level data detailing their incidence and outcomes. METHODS: We performed an analysis of data collected by National Cancer Registration and Analysis Service (NCRAS) for patients diagnosed with BS between 2013 and 2018. Chi-square test was used to compare groups. Overall survival (OS) was calculated by Kaplan-Meier. Specialist sarcoma centres (SSC) were defined as centres with a sarcoma multidisciplinary team (MDT). RESULTS: There were 684 patients with BS (357 malignant phyllodes tumours [PTs], 238 vascular tumours, 93 other morphology) with a median age of 64 (range 14-96); 187 (27%) had received breast radiotherapy for a prior malignancy; 633 (92%) had resection of the tumour within 12 months of diagnosis. Five-year OS was 82%, 54% and 48% in patients with PT, vascular tumours and other sarcomas, respectively, and 55% for those with radiation-induced BS. Patients managed within SSC more frequently had a biopsy prior to surgery 83% versus 72%, p < 0.05) and were less likely to require multiple operations (26% versus 41%, p < 0.05). Tumour stage and grade data were not available. CONCLUSION: This is the first population series evaluating incidence and outcomes for BS. Patients treated at non-specialist sarcoma centres (NSSCs) are less likely to have a biopsy prior to surgery and more likely to require multiple operations. Based on these observational data, we would recommend all BS are discussed at a sarcoma MDT meeting early in their pathway and surgery to be considered at SSC where possible.

Primary and Secondary Breast Sarcoma: Clinical and Pathological Characteristics, Prognostic Factors, and Nomograms for Predicting Survival.

BACKGROUND: Breast sarcoma is one of the rare types of breast tumors with different features and outcomes compared to carcinoma. Our study aims to describe the clinical and pathological characteristics of primary breast sarcoma (PBS) and secondary breast sarcoma (SBS) along with determining prognostic factors and developing nomograms for predicting survival. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) Program, female patients diagnosed with breast sarcoma between 1975 and 2016 were identified. Cox regression was used to evaluate the association between survival and clinical features. RESULTS: Out of 1334 included patients, 816 had PBS and 518 had SBS. PBS had a significantly better overall survival than SBS with median survival months of 107 for PBS and 45 for SBS. The primary tumor site did not have a significant impact on the survival of SBS. Cox regression showed worse survival of PBS patients who were > 60 years (HR 3.04, 95% CI 2.46-3.74) and had tumor size > 50 mm (HR 2.01, 95% CI 1.61-2.51). Being not married was associated with worse survival of PBS (HR 1.29, 95% CI 1.06-1.56) and SBS (HR 1.50, 95% CI 1.19-1.90). Surgery was associated with better survival of PBS (HR 0.60, 95% CI 0.42-0.85) and SBS (HR 0.46, 95% CI 0.31-0.68). The C-indexes of created nomograms were 0.73 for PBS and 0.69 for SBS. CONCLUSION: Age and size were the most important prognostic factors for the survival. Surgery was associated with better survival. However, radiation and chemotherapy did not show significant improvement in survival.

A 53-year-old female with a large breast sarcoma: A case report from Hong Kong.

Breast sarcomas are very rare and are aggressive tumors associated with a poor prognosis. We present a case of a 53-year-old female who presented to the hospital after noticing a palpable mass in the right breast. Mammography, ultrasound, and MRI investigations were done. The patient was treated with radical mastectomy combined with both neoadjuvant and adjuvant chemoradiotherapy. Undifferentiated pleomorphic breast sarcoma was later diagnosed.

Large mirror brain metastases from primary undifferentiated sarcoma of the breast: case report and review of the literature.

Primary breast sarcomas are rare high-grade tumors with a reported incidence of <1% of breast malignancies. Its dissemination to the CNS is exceptional and only one is found in the literature. The authors described the case of a 22-year-old female with history of a breast undifferentiated sarcoma that present with two large bilateral retrolenticular brain metastases. Both lesions were excised in the same procedure and she underwent adjuvant therapy. She died 24 months after surgery. Despite being aggressive lesions, aggressive treatment of primary breast sarcomas including brain metastases excision, should be considered in order to improve overall survival.

Clinical and Molecular Insights of Radiation-Induced Breast Sarcomas: Is There Hope on the Horizon for Effective Treatment of This Aggressive Disease?

Radiation-induced breast sarcomas (RIBS) are rare entities representing <1% of all primary breast malignancies, limiting most reports to small retrospective case series. They constitute a heterogeneous group of neoplasms, with high-grade angiosarcoma being the most common subtype. Other sarcoma histotypes, such as undifferentiated pleomorphic sarcoma and leiomyosarcoma, can also be identified. Radiation-induced breast angiosarcoma (RIBA) has an incidence of approximately 0.1% after breast-conserving therapy and arises mainly from the dermis of the irradiated breast. MYC gene amplification is highly indicative of secondary breast angiosarcomas. Their clinical presentation often mimics benign port-radiation lesions, leading to a delay in diagnosis and a lost window of opportunity for cure. Surgery with negative margins is the mainstay of treatment of localized RIBS. In the case of angiosarcoma, technical difficulties, including multifocality, infiltrative margins, and difficulty in assessing tumor margins, render surgical treatment quite challenging. A limited number of studies showed that adjuvant radiation therapy reduces local recurrences; therefore, it is proposed by many groups for large, high-grade tumors. Chemotherapy has been evaluated retrospectively in a small subset of patients, with some evidence supporting its use in angiosarcoma patients. Approximately half of patients with RIBA will show local recurrence. In the advanced setting, different therapeutic options are discussed in the review, including chemotherapy, antiangiogenic therapy, and immunotherapy, whereas the need for further research on molecular therapeutic targets is pointed out.

Health-Related Quality of Life Issues Experienced by Thoracic and Breast Sarcoma Patients: A Rare and Understudied Group.

Thoracic and breast sarcomas constitute a rare subgroup within the sarcoma population. There is limited knowledge about their health-related quality of life (HRQoL) and a valid disease-specific HRQoL instrument is lacking. This qualitative study aimed to investigate the HRQoL issues experienced by a small group of thoracic and breast sarcoma patients. Semi-structured interviews with 19 thoracic and four breast sarcoma patients were conducted and thematically analysed. Physical issues mentioned by both groups were fatigue, sleep disturbances, pain, wound infections, and symptoms related to chemotherapy and radiotherapy. Tightness in the back and restrictions in performing tasks above arm height were specific physical issues for breast sarcoma patients, whereas respiratory problems were only mentioned by thoracic sarcoma patients. Body image issues, changes in mood, fear of recurrence, and living with uncertainty were important mental health issues for both subgroups. Social issues in both groups included challenges in work and relationships, financial difficulties, loss of independence, and limitations in social activities. The identified physical, mental, and social health challenges can significantly impact thoracic and breast sarcoma patients' HRQoL. Results of this qualitative study will guide personalised supportive care for breast and thoracic sarcoma patients and help in determining the best possible HRQoL measurement strategy for sarcoma patients with different primary sarcoma locations.

Breast Recurrence of Acute Myeloid Leukemia After Bone Marrow Transplantation: A Case Report About Myeloid Sarcoma of the Breast.

Myeloid sarcoma of the breast is a rare malignancy, can be seen after bone marrow transplantation. Although there are no specific features for this malignancy which is difficult to diagnose, some common features draw attention in the published case reports. Since there is no consensus on the treatment of myeloid sarcoma of the breast, we aimed to explain our own diagnosis and treatment methods in this case report.

Case Report of a Breast Mass with Three Types of Malignant Tumors-Squamous Cell Carcinoma, Invasive Ductal Carcinoma, and Breast Sarcoma.

Breast malignancy comprises malignant tumors originating from epithelial tissue and breast sarcoma arising from mesodermal tissues. Despite heterogeneity in tumors, most malignant tumors of the breast are composed of a single type of tumor. Here, we report a patient with breast tumor consisting of three different types of malignancies-squamous cell carcinoma, invasive ductal carcinoma, and high-grade breast sarcoma.

A rare case report of breast sarcoma.

Malignant tumors that originate from the mesenchymal tissue of the mammary gland, known as breast sarcomas, are very rare and can be divided into 2 types: primary and secondary (therapy-related development). Breast sarcomas are aggressive tumors associated with a poor prognosis. Treatment options include the coordination of surgery, chemotherapy, and radiotherapy. We present a case of a 51-year-old female who presented to the hospital after noticing a palpable mass in the left breast and bloody nipple discharge. These symptoms lasted for more than 4 months. Postoperative histopathology revealed an undifferentiated pleomorphic breast sarcoma. After 8 months of treatment, the patient experienced metastasis to the brain and lungs.

Primary stromal sarcoma of breast: A case report and literature review.

INTRODUCTION: Primary breast sarcoma (PBS) was first described in 1887 as a rare heterogeneous neoplasm arising from the mesenchymal tissue of the mammary gland accounting for less than 5% of all body soft-tissue sarcomas and less than 0.1% of all malignant tumors of the breast. CASE PRESENTATION: A 31-year old lady presented with left breast mass which she felt four years before during which the mass increased in size from 1 to 6 cm in the largest diameter, diagnosed clinically as a benign fibroadenoma without any further cytological or histopathological confirmation. Histopathological examination of the excised breast mass reveals undifferentiated, primary stromal sarcoma of the breast (PSSB), which was followed by mastectomy three weeks later with reconstructive breast surgery with a total duration of follow-up of 3 years thereafter. This is the first case of PSSB reported in Jordan. DISCUSSION/CONCLUSION: PSSB is the generic term given to malignant breast tumors thought to arise from the specialized mesenchymal stroma of the breast but lacking an epithelial component with a phylloides pattern. PSSB is difficult to diagnose preoperatively due to its rarity and inadequate imaging methods to establish an exact diagnosis. The histology of the patient mass may be the leading factor for the management of these tumors. Even in very young patients, a progressively growing breast mass should alert the clinician to investigate for malignancy and verify the results by biopsy. Surgery with adequate resection margins represents the only potentially curative modality with prognostic significance. Adjuvant chemotherapy and radiotherapy are not very beneficial. The prognosis is dismal for patients with lymph node involvement and the size of the tumor has a lesser bearing on the outcome.

A rare etiology of a large tumoral mass of the breast - Case report primary leiomyosarcoma and osteosarcoma of the breast.

Breast sarcomas are a rare group of malignant tumors accounting for less than 1% of all malignant neoplasms of the breast and fewer than 5% of all sarcomas. We report a case of an 87-year-old caucasian female who recurred to the emergency department with complaints of a painful mass of the left breast with purulent discharge. Observation revealed a volumous mass in the inferior quadrants of the breast, ill defined, with petrous consistency, areas of necrosis, and inflammatory signs. She was admitted to Surgery ward for further study and therapy of a probable inflammatory tumor of the breast. Magnetic resonance image was obtained, raising suspicion on papillary carcinoma and classified the breast as BIRADS5. Microbiological and cytological exams of the exudate were negative. An incisional biopsy of the tumoral mass was also obtained, and the patient discharged while waiting for surgery. Histological exam and immunohistochemical essay were compatible with leiomyosarcoma. Left mastectomy was performed and the patient was discharged with no morbidities on the 5th day after surgery. Histological exam of mastectomy piece showed a metaplastic carcinoma, with osteosarcomatous and focal leiomyosarcomatous differentiation. The lesion was classified as pT4N0M0 and subsequent radiotherapy was performed. Twenty months after surgery the patient was being followed-up on Oncology and Senology consultations and remained asymptomatic. Consensus on ideal management of this diseases is still on debate. Some authors defend the treatment of this entity in a similar way to sarcoma of the breast. More studies are needed to better understand this entity.

A need for clarity on surgical management of breast sarcoma: Scottish sarcoma network guidelines and regional audit.

Currently, there are no comprehensive breast sarcoma guidelines in the UK. There is therefore a need for guidelines to clarify surgical management, which we have based on data from our regional audit, current evidence, and consensus between West of Scotland Breast Cancer and Scottish Sarcoma Managed Clinical Networks. Methods and results: From 2007 to 2019, 46 patients were treated with breast sarcoma in the West of Scotland. Sarcoma Centre versus Peripheral Hospitals: Incomplete excision rate was 0% at sarcoma centre and 50% at peripheral hospitals (p = 0.0002, Odds Ratio 43). For angiosarcoma, 0% positive margin at the sarcoma centre versus 62.5% at the peripheral unit (p = 0.0036, odds ratio 39.3). Tumours treated at the sarcoma centre were larger than those treated at peripheral hospitals (92.5 versus 39.7 mm, p = 0.0009). WLE (wide local excision) versus mastectomy: Out of eight WLE patients, seven (87.5%) had positive margins, with 6 of these patients proceeding to mastectomy (i.e. 75% WLE patients ultimately had a mastectomy). The positive margin rate was significantly higher in WLE (87.5%) than in mastectomy (10.3%) (p = 0.0001, odds ratio 60.7). Survival: No difference was noted between the sarcoma centre and peripheral hospitals for overall survival (p = 0.43), stratified for tumours <5 cm (p = 0.16), and disease-free survival (p = 0.45). Conclusions: Our data strongly suggest that specific guidelines are needed for breast sarcoma, and that managing these patients according to breast carcinoma protocols in peripheral hospitals is sub-optimal. We recommend centralisation of breast sarcoma patient care to a specialist sarcoma centre, with WLE not recommended as a firstline surgical option given both the high rates of incomplete excision and subsequent need for completion mastectomy.