Radiation-Associated Angiosarcoma of the Breast: The State of the Art of a Rare and Aggressive Disease.

INTRODUCTION: Considering the increasing number of conservative surgeries (quadrantectomies) for primary breast carcinoma, especially in the early stages, often followed by adjuvant radiotherapy, the incidence of radiation-associated angiosarcoma (RAS) is expected to rise in the coming decades, and it will represent a clinical and therapeutic challenge, as limited data are available due to the rarity and heterogeneity of the disease. Though the prognosis of these patients is poor, a number of clinical and pathological factors can be evaluated to better understand the course of RAS. The aim of this systematic review is to explore the available clinical-pathological, therapeutic, and prognostic data regarding RAS to evaluate its occurrence, diagnosis, treatment, and outcomes. METHOD: RAS clinical data were identified by a systematic review conducted in five different databases (Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane). Only RAS works published in English, with access to the full manuscript text, and with clear results, were considered as eligible. RESULTS: We considered 52 papers comprising 319 RAS cases. The patient age at diagnosis ranged from 37 to 93 years, with most cases occurring from 5 to 10 years after breast irradiation. The most common clinical presentation was an aggressive development of macules, papules, or erythematous-violaceous skin discoloration at the site of previous radiation treatment for primary breast carcinoma. Complete surgical excision appeared to be the first-line treatment of the disease, in some cases followed by adjuvant local and/or systemic therapies. Despite different treatments, local recurrence rates ranged from 40% to 90%, leading to negative outcomes and poor prognosis for patients. CONCLUSION: Although the literature is limited and the data are heterogeneous and contentious, our review aims to highlight the importance of early diagnosis, multimodal treatment, and long-term follow-up of RAS in order to limit and prevent the aggressiveness of this neoplasm.

Use of neoadjuvant paclitaxel in breast angiosarcoma-Impact on surgical resection and response rates.

OBJECTIVE: Breast angiosarcoma is a tumor that can arise as a primary breast tumor or in association with prior radiation therapy. Angiosarcomas are uniquely sensitive to paclitaxel. This study evaluated the impact neoadjuvant paclitaxel (NAC) therapy has on surgical outcomes, tumor recurrence, and survival in breast angiosarcomas. METHODS: Patients with angiosarcoma of the breast, either primary or radiation-associated, were identified from a prospective institutional database. Patients receiving NAC were compared to those treated with upfront surgery. Clinical and pathological variables were compared using Student's t-test or Fisher's exact test, differences in survival were calculated using Kaplan-Meier methods. RESULTS: Twenty-four patients with angiosarcoma of the breast were identified, 10 with primary angiosarcoma and 14 with radiation-associated angiosarcoma. Twelve patients received NAC, 6 of each with primary angiosarcoma or radiation-associated angiosarcoma. Of these 12 patients, 11 had a margin negative resection (91%) of which, nine had a complete pathological response on surgical pathology. Of the 12 surgery-first patients, four (n = 4/12, 33%) had positive surgical margins, two of the four underwent reoperation. With a median follow-up of 16 months, four NAC patients had recurrence (33%) compared to six patients in the surgery-first group (58%) (p = 0.41). While not statistically significant, NAC patients had a 33% less risk of recurring compared to surgery-first patients ([hazard ratio =0.67 (95% confidence interval 0.16-2.72; p = 0.6]). CONCLUSION: NAC for breast angiosarcoma may be associated with high rates of complete pathological response and margin-negative resection. However, this did not impact overall survival. Future prospective control studies and longer follow-up periods are warranted to understand the impact on recurrence and survival.

Renal pelvis metastasis following surgery for breast angiosarcoma: a case report and literature review.

Renal metastasis of breast angiosarcoma is rare. This article reports the medical records of a patient diagnosed with breast angiosarcoma who underwent radical mastectomy and was found to have multiple lung metastases 3 years after surgery and renal pelvic metastasis 4 years after surgery. The patient underwent robot-assisted laparoscopic radical nephroureterectomy and sleeve resection of the intramural segment of the ureter, and postoperative pathology and immunohistochemical staining confirmed the diagnosis of renal pelvic metastasis of breast angiosarcoma. The patient received anlotinib for lung metastases following surgery and was followed up for 4 months after surgery. Currently, the patient has symptoms of coughing and hemoptysis but no other discomfort. The diagnosis and treatment of this rare malignant tumor remain challenging.

Latissimus Dorsi Flap Revisited: Coverage of Large Chest Wall Defects Following Mastectomy for Locally Advanced Breast Carcinoma and Angiosarcoma.

BACKGROUND:  The latissimus dorsi myocutaneous flap (LDMF) remains a significant tool in the armamentarium of the oncoplastic breast surgeon. Despite declining popularity for reconstruction, owing to the increasing use of implants and free flaps, it still has an important role in certain salvage situations and as a flap to cover large defects. We report our experience with the use of LDMF for immediate coverage of large mastectomy defects when options are limited. METHODS:  Retrospective series of prospectively collected patient records. Patient and tumour characteristics, length of stay, and post-operative and oncologic outcomes are reported. Patients with angiosarcoma were discussed at tertiary sarcoma centres as per national guidelines. Operations were carried out by oncoplastic breast surgeons. The case series was approved by the institutional information governance department in line with institutional requirements for patient data sharing. All patients provided written consent for photography. Descriptive statistics were used to report findings. Median (IQR) was used for continuous variables. RESULTS:  Six women were included, with a median age of 62.5 years, from December 2019 to October 2022. Follow-up ranged from 15 to 49 months. Median tumour size was 72.5 (16.25) mm. Four patients had locally advanced breast carcinoma (LABC), and two had breast angiosarcoma. The donor site and chest wall defects were closed primarily in all cases. Median length of stay was three nights. All mastectomy wounds healed without issues and any delay to their adjuvant treatment. One patient had a minor latissimus dorsi (LD) donor site wound breakdown managed conservatively. Three patients had adjuvant radiotherapy after surgery. Four patients, one after high-grade angiosarcoma and three after aggressive breast carcinoma, had a locoregional recurrence or distant metastases and succumbed within 20 months of surgery. CONCLUSION:  The LDMF can be a reliable option for the primary closure of large post-mastectomy wounds. Its use can lead to timely wound healing, allowing patients to undergo adjuvant treatment without delay. However, the overall oncologic outcomes in patients with LABC and angiosarcoma are poor due to the underlying aggressive tumour biology. Long-term outcomes are to be interpreted with caution due to the small number of patients with diverse pathologic features.

Angiosarcoma of the breast: A review.

Breast angiosarcoma is a rare and highly aggressive malignancy with a poor prognosis. It can occur spontaneously or be associated with factors such as radiation therapy or chronic lymphedema. The etiology and pathogenesis of this disease are still unclear, the clinical symptoms and imaging findings lack specificity, and the pathological morphology is diverse, which is easy to be confused with other diseases. There is no clear guideline for surgical treatment. Although the optimal surgical approach remains unclear, the ultimate goal is surgical excision with optimal margins, which remains the primary method of treatment. In clinical practice, the choice of the surgical approach should be made by considering the tumor size, pathological type, and patient preferences. In clinical practice, the selection of surgical methods should be carried out with comprehensive consideration of tumor size, pathological types and patients' wishes. There is no clear consensus on whether radiotherapy and chemotherapy should be carried out after surgery, and its optimal program and efficacy are uncertain. This article reviews the etiology, clinical manifestations, pathological features, imaging findings, treatment, prognosis and other aspects of breast angiosarcoma, so as to strengthen clinicians' overall understanding of this disease and avoid missed diagnosis and misdiagnosis.

Post-Irradiation Breast Angiosarcoma: All the Possible Treatments and Electrochemotherapy. Case Report and Literature Review.

Breast angiosarcoma is a rare malignancy, accounting for less than 1% of all soft tissue cancers. It comprises primitive and secondary subtypes, such as radiogenic breast angiosarcoma (RAS). Despite multimodal treatment, angiosarcomas represent an incurable disease for many patients and a significant cause of deterioration in their quality of life. Surgery is a cornerstone in management, but high recurrence rates are reported. Electrochemotherapy (ECT) is a practicable locoregional treatment for patients with advanced angiosarcoma as part of a multimodal therapeutic strategy. The palliative benefits of ECT include optimal patient compliance, good local hemostasis control, and positive local responses. Since only 22 cases are described in the literature, we reported a rare case of RAS treated with ECT after a multidisciplinary approach, including Next Generation Sequencing (NGS). A literature review on the feasibility of ECT in RAS management was also performed.

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.

Radiation-Associated Breast Angiosarcoma.

Radiation-associated breast angiosarcomas are rare following breast-conserving surgery. These angiosarcomas are considered adverse events associated with radiation therapy and are characterized by a high risk of both local and distant recurrence, even after complete resection. Despite this, there is currently no established standard treatment for them. The patient was a 70-year-old woman who had breast-conserving surgery for right breast cancer 10 years before presentation. She was followed up for 10 years after receiving 50 Gy of residual breast irradiation and an aromatase inhibitor for 5 years. During follow-up, a painless purplish area with induration, measuring 10 cm by 5.4 cm, was noted on the right nipple. A skin biopsy confirmed hemangiosarcoma. Treatment included surgery with a 2.0 cm margin from the area, followed by skin excision and total mastectomy. A final diagnosis of radiation-associated breast angiosarcoma was made. Radiation-associated breast angiosarcoma is a rare disease with a poor prognosis that lacks standard treatment. An aggressive skin biopsy should be considered when skin findings such as purpura are seen after breast cancer radiotherapy, as in this case.

Radiation-induced angiosarcoma of the breast: a case report.

Introduction: Breast angiosarcoma is a rare, aggressive tumour affecting adult women. It can occur in two forms, primary form and secondary forms or radiation-induced breast angiosarcoma affecting patients with history of breast or chest radiotherapy. Case presentation: The authors report a new case of breast angiosarcoma in 52-year-old women, with history of invasive ductal carcinoma, and reporting a discoloration of her breast skin. The patient did undergo a mastectomy of right breast and adjuvant chemotherapy. Conclusion/discussion: Surgery with total excision associated or not to adjuvant chemotherapy remains the treatment of choice in breast angiosarcoma.

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.

Primary breast angiosarcoma in a postmenopausal woman: A case report.

INTRODUCTION AND IMPORTANCE: Primary angiosarcoma of the breast is a rare malignancy with an associated poor prognosis due to its high rates of reoccurrence and metastasis It is most common in females presenting in their 3rd and 4th decades with no evidence of hormonal dependency. Cases usually present with rapidly progressing non-tender breast lump. CASE PRESENTATION: A 75-year-old female referred for triple assessment of a rapidly enlarging right breast lump. The patient underwent multiple investigations for work-up and to aid in the diagnosis of a moderately differentiated angiosarcoma of the breast. CLINICAL DISCUSSION: Diagnostic dilemmas remain due to the non-specific findings on standard radiological investigations and high false-negative results on core-biopsy. Consideration should be made in those with a high clinical suspicion for magnetic resonance imaging and excisional biopsy. Although limited research, first line management of those without metastatic disease is radical surgery. CONCLUSION: Breast primary angiosarcoma is a rare entity in post-menopausal women that should be considered in the differentials of breast lumps. Standardized information is limited though current management includes local control with radical surgery. The role for neoadjuvant therapy, adjuvant radiotherapy or chemotherapy is still unclear.

A case report of primary angiosarcoma of the breast.

Background: Breast Angiosarcoma can be divided into primary breast angiosarcoma (PBA) and second breast angiosarcoma (SBA). PBA is rare malignant breast cancer with poor outcomes. PBA usually occurs in women between 30-40 years old. PBA does not have a specific clinical manifestation. Clinically, PBA presents with a rapidly enlarging breast mass and skin involvement with skin color changes. Ultrasonography of PBA can be hypoechoic or hyperechoic, or mixed disordered areas. Microscopically, PBA can be classified into three grades according to the degree of differentiation, and the grade is related to the prognosis. And PBA can also express vascular endothelial markers. The main treatment for PBA is surgery, especially mastectomy. There are other treatments, such as chemotherapy and radiotherapy, but their effectiveness needs further confirmation. Targeted drugs may be helpful. Case Description: A 32-year-old woman with main clinical presentation of a rapidly growing mass located in the upper inner quadrant of the right breast with skin involvement. PBA was diagnosed with first extended local resection, and then the patient underwent a second right mastectomy. The patient is now undergoing chemotherapy. Conclusions: Since this is a rare form of breast cancer, we report this case to raise the attention of breast surgeons to avoid misdiagnoses.

Angiosarcoma of the Breast: Overview of Current Data and Multimodal Imaging Findings.

Angiosarcoma of the breast is a rare breast cancer, which can arise de novo (primary breast angiosarcoma, PBA) or as a secondary malignancy (secondary breast angiosarcoma, SBA) as a result of a biological insult. In the latter case, it is usually diagnosed in patients with a previous history of radiation therapy following a conserving treatment for breast cancer. Over the years, the advances in early diagnosis and treatment of breast cancer, with increasing use of breast-conserving surgery and radiation therapy (instead of radical mastectomy), brought about an increased incidence of the secondary type. PBA and SBA have different clinical presentations and often represent a diagnostic challenge due to the nonspecific imaging findings. The purpose of this paper is to review and describe the radiological features of breast angiosarcoma, both in conventional and advanced imaging to guide radiologists in the diagnosis and management of this rare tumor.

Low Radiologic Sensitivity in Detecting Radiation-Associated Breast Angiosarcoma (RAS).

Due to its rarity, literature pertaining to radiation-associated breast angiosarcoma (RAS) remains sparse, with most studies focusing on retrospective review. Of more significant concern is the ambiguity of screening recommendations and modalities used to detect RAS, with current guidelines focusing on yearly mammographic imaging for women who underwent lumpectomy with radiation. Unfortunately, routine post-cancer screening has demonstrated low sensitivity in detecting RAS, often mistaking it for benign changes in roughly half of cases. We present an 83-year-old woman initially diagnosed with stage 1 invasive ductal carcinoma of the left breast who underwent a lumpectomy followed by radiation with 6040 cGy. Five years after her initial diagnosis, the patient noticed a suspicious lesion which then led her to undergo multiple modalities of imaging that described benign features. After continued concern, a biopsy was taken that demonstrated RAS of the left breast within the irradiated site. The patient underwent further radiation and declined surgical intervention. Routine screening with mammography and ultrasonography following breast radiation treatment are not sensitive modalities in detecting RAS. High-risk patient groups treated with greater than 0.5 Gy of radiation with concerning physical features 2-10 years after treatment should undergo MRI with biopsy at the initial concern to rule out angiosarcoma. Benign findings on imaging with patients in these groups should also consider biopsy.

Primary angiosarcoma of breast: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Primary angiosarcoma of breast (PAB)3 is a rare and aggressive malignancy with limited published literature. This article aims to disclose the diagnosis and treatment of this case, review previous case reports, and provide clinical experience to breast surgeons. CASE PRESENTATION: A 36-year-old Asian female presented with a diffuse mass growing rapidly in her left breast. The ultrasonography (USG)4 suspected granulomatous mastitis. The core needle biopsy (CNB)5 confirmed the diagnosis of breast angiosarcoma (AS).6 She underwent mastectomy without axillary lymph node dissection (ALND)7 followed by adjuvant chemotherapy. About 11 months after mastectomy, the patient had bone metastasis. CLINICAL DISCUSSION: PAB is a rare vascular neoplasia, characterized by aggressive patterns, poor prognosis, and high malignant potential. It is difficult to diagnose or differentiate only by clinical or imaging examination. Biopsy and immunohistochemical staining are the most reliable method. Mastectomy is the most common treatment. CONCLUSION: PAB is a rare and malignant cancer. We should pay attention to the diffuse progressive masses in the breast of young female, and perform MRI and biopsy if necessary. Mastectomy is the only treatment known that has proven to benefit these patients. There are no evidence-based guidelines regarding treatment.

The Role of Imaging in the Diagnosis of Primary and Secondary Breast Angiosarcoma: Twenty-Five-Year Experience of a Provincial Cancer Institution.

BACKGROUND: Breast angiosarcoma may arise spontaneously (primary breast angiosarcoma (PBA)) or may arise secondary to a biological insult, such as radiation therapy (secondary breast angiosarcoma (SBA)). We evaluated the imaging findings of patients diagnosed with PBA and SBA within the province of British Columbia, Canada. MATERIALS AND METHODS: This was a multi-center, retrospective study of patients diagnosed with PBA and SBA over a 25-year period. Patients were identified via a provincial database which registers all cases of sarcoma. Patients diagnosed with histologically proven PBA and SBA were eligible for inclusion. Multimodal breast imaging reviewed included mammography, ultrasound, magnetic resonance imaging, and computed tomography. RESULTS: Thirteen patients were diagnosed with PBA and 22 patients were diagnosed with SBA. The median (interquartile range (IQR)) age of patients diagnosed with PBA (45.5 years (19.7 years)) was less than that of patients diagnosed with SBA (75.8 years (13.8 years), P < .001). Patients diagnosed with PBA (90.9%) were more likely to present with a parenchymal mass clinically and radiographically than those with SBA (28.6%, P < .002). Patients diagnosed with SBA (71.4%) were more likely to present with cutaneous findings than patients diagnosed with PBA (0.0%, P < .05). Without specific clinical context, the imaging findings of PBA and SBA were observed to be non-specific. CONCLUSION: This is the only study which evaluated the imaging findings of patients diagnosed with PBA and SBA within a large, defined geographical area. Given non-specific imaging findings, awareness of the disease and clear and timely communication between radiologists and clinicians is required to ensure appropriate diagnosis and management.

The Complex Management of the Breast Angiosarcoma: A Retrospective Study.

BACKGROUND/AIM: Breast angiosarcoma is a rare and aggressive disease with a poor prognosis. Two subtypes have been identified: primary angiosarcoma (PBA) and secondary breast angiosarcoma (SBA). In this retrospective analysis, we describe and compare our institute experience with the data existing in the literature. MATERIALS AND METHODS: We included in our analysis 29 patients who received a diagnosis of PBA or SBA between 2006 and 2019. RESULTS: All patients received surgery as frontline treatment, but only 6 patients underwent to adjuvant treatment. Neoadjuvant chemotherapy was administered 2 patients. The preferred chemotherapeutic regimen was taxanes with or without gemcitabine and associated with anthracyclines. A lower median RFS and OS were reported in patients with PBA compared to those with SBA, but the difference observed was not statistically significant. Patients with PBA had a lower median age at the diagnosis (38 vs. 75). CONCLUSION: In our analysis, we have shown a lower median RFS and OS in patients with PBA compared with those with SBA, and a significantly younger age at diagnosis in patients affected by PBA.

Breast Angiosarcoma: Imaging Features With Histopathologic Correlation.

Breast angiosarcoma is a rare malignancy of endothelial origin that can be categorized as primary angiosarcoma (PAS) or secondary angiosarcoma (SAS) based on etiology. Primary angiosarcoma typically affects younger women with no known risk factors, whereas SAS of the breast typically develops in older women who have undergone breast cancer treatment. There are two types of SAS, one that develops in the setting of chronic lymphedema and one that develops as a radiation-associated neoplasm after breast-conserving therapy (BCT). Clinically, PAS often presents as a palpable mass that may be rapidly growing, whereas SAS presents with skin changes such as erythematous plaques or nodules or with areas of skin discoloration. Mammographically, the appearance of PAS can be nonspecific and may be obscured by the dense tissue that is characteristic of the young patient population it typically affects. Cases of mammographically occult PAS have been visible at US and MRI. Mammography and US have been found to be less sensitive than MRI for the diagnosis of secondary radiation-associated angiosarcoma. Angiosarcomas, both PAS and SAS, are graded, depending on degree of differentiation, as low, intermediate, or high grade. Endothelial markers such as ERG and CD31 immunohistochemical stains are used to support the diagnosis of angiosarcomas. In this article, we review the clinical presentation, imaging findings, associated histopathology, and treatment of primary and secondary breast angiosarcoma.

Primary Breast Angiosarcoma: Comparative Transcriptome Analysis.

Primary breast angiosarcoma, with de novo appearance and not associated with exposure to radiation or lymphedema, is a rare pathology representing less than 0.05% of the neoplasms related to this organ. The pathology is characterized by its aggressiveness, poor prognosis, and difficulties in its differential diagnosis. This article reports the case of a 55-year-old white woman with no family history of cancer, with a rapidly growing mass in the left mammary gland that ulcerates and bleeds. It is confirmed as primary breast angiosarcoma by immunostaining in the tumor tissue for CD31, CD34, and FLI-1. In addition, a sample of neoplastic and healthy tissues is collected from the patient for RNA sequencing; the results are contrasted with a tissue sample from a patient with Luminal A subtype of breast cancer, as well as data from other cases of angiosarcoma available in public databases. These findings revealed a genetic profile associated with the immune and inflammatory response in the patient's sample when compared to available angiosarcoma data; these molecular patterns are consistent with other recent studies. Due to the rarity of the disease, the studies carried out on each patient contribute to the expanding knowledge of the etiology and molecular pathways that are still partially known and continue to be the subject of research. Aside from a comparative transcriptome study, this article aims to provide an update on the state of knowledge about this disease.

A clinicopathologic and immunohistochemical study of primary and secondary breast angiosarcoma.

BACKGROUND: We aimed to study the clinicopathologic and immunohistochemical (IHC) (CD117, c-Myc, and p53) characteristics, and overall survival of primary and secondary breast angiosarcoma (BAS). METHODS: This was a retrospective study of BAS cases diagnosed between 1997 and 2020 at our institution. Hematoxylin and eosin-stained slides were reviewed for tumor morphology, margin status, and lymph node metastasis. CD117, p53, D2-40, CD31, and c-Myc IHC stains were performed on 11 viable tissue blocks. Additional clinical information was obtained from the electronic medical records. RESULTS: Seventeen patients with BAS were identified. Of these, five (29%) were primary and 12 (71%) were secondary BAS, respectively. The median age at diagnosis for primary BAS was 36 years. The median age at diagnosis for secondary BAS was 67 years. The median time to secondary BAS development following radiotherapy was 6.5 years (range, 2 to 12 years). There was no significant difference between primary and secondary BAS in several histopathologic parameters examined, including histologic grade, necrosis, mitotic count, lymph node metastasis, and positive tumor margins. There was also no difference in CD117, p53, D2-40, CD31, and c-Myc expression by IHC between primary and secondary BAS. During a median followup of 21 months, primary BAS had two (40%) reported deaths and secondary BAS had three (25%) reported deaths. However, this difference in survival between both groups was not statistically significant (hazard ratio, 0.51; 95% confidence interval, 0.09 to 3.28; p = .450). CONCLUSIONS: BAS is a rare and aggressive disease. No histologic, IHC (CD117, c-Myc, and p53), or survival differences were identified between primary and secondary BAS in this study.