Angiosarcoma: a 10-year retrospective study from a high-volume UK regional referral centre.

PURPOSE: Angiosarcoma (AS) is a rare malignancy with considerable heterogeneity seen in its aetiology, anatomical location, and clinicopathological behaviour. Diagnosis is often delayed and prognosis poor. The purpose of this study was to perform a retrospective review of all cases of AS over 10 years at a high-volume regional UK referral centre. METHODS/PATIENTS: We reviewed all cases of AS discussed at the sarcoma multidisciplinary meetings of University Hospitals Birmingham NHS Foundation Trust from September 2013 to August 2023. Demographic and clinicopathologic features at diagnosis, approaches to treatment, and outcomes were compared between four AS subtypes. RESULTS: A total of 130 cases were identified. The median age at diagnosis was 71 years, with the majority being female (78%). The most common AS subtype was radiation-induced AS (RIAS) (n = 72; 55%), followed by primary cutaneous (n = 28; 22%), primary non-cutaneous (n = 25; 19%), and AS secondary to lymphoedema (n = 5; 4%). Metastases were present at diagnosis in 18% of patients. Treatment was with surgery in the majority of patients (71%). The median survival for the cohort was 30 months (95% CI 20-40), although this differed significantly by AS subtype (p < 0.001), ranging from 5 months in primary non-cutaneous AS to 76 months in RIAS. CONCLUSION: RIAS is the most common AS subtype, with surgery the only potentially curative treatment modality. Overall prognosis varies significantly by subtype. An international consensus on classification of AS subtypes is required to allow meaningful comparisons across studies and/or a prospective multi-centre registry.

Benign osseous metaplasia of the breast infiltrated by invasive pleomorphic lobular carcinoma: A case report.

INTRODUCTION AND IMPORTANCE: Benign osseous metaplasia (BOM) is a rare entity, with only few cases reported in the breast. Here we present an unusual case of pleomorphic lobular carcinoma of the breast infiltrating BOM, discuss potential mimics and review the literature. CASE PRESENTATION: An 86 year-old female presented with right breast lump for two weeks. Clinical examination revealed a palpable mass, associated with skin tethering and nipple inversion. Mammography and ultrasound showed a densely calcified lesion associated with parenchymal distortion. Core biopsy confirmed malignancy and the patient underwent mastectomy and sentinel lymph node biopsy. Histological assessment showed a 45 mm mass of benign bone trabecula infiltrated by invasive grade 2 lobular carcinoma of classic and pleomorphic types with nodal positivity (2/2). The patient received adjuvant radiotherapy to chest wall and axilla for 3 months. She remains well on aromatase inhibitors after 9 months of follow up. CLINICAL DISCUSSION: Few cases of breast BOM have been reported in the literature commonly in association with benign lesions such as fibroadenomas. So far, only two cases associated with invasive classic lobular carcinoma have been reported in the literature. The main differential is metaplastic (mesenchymal/ matrix producing) carcinoma, in which the osseous component is malignant and the cancer if often of a high grade, basal phenotype. CONCLUSION: We present the first case of BOM of the breast associated with invasive pleomorphic lobular carcinoma. Awareness of the entity and distinction from metaplastic carcinoma and malignant phyllodes with heterologous element are important to ensure appropriate patient management.

Constitutively Enhanced Lymphatic Pumping in the Upper Limbs of Women Who Later Develop Breast Cancer-Related Lymphedema.

BACKGROUND: It has previously been shown that the lymph drainage rate in both upper limbs is greater in women destined to develop breast cancer-related lymphedema (BCRL) than in those who do not develop BCRL, indicating a constitutive predisposition. We explored constitutive differences further by measuring the maximum lymphatic pump pressure (Ppump) and the rate of (99m)Tc-Nanocoll transport generated by the contractile upper limb lymphatics before and after breast cancer surgery in a group of women who were followed for 2 years to determine their eventual BCRL or non-BCRL status. METHODS AND RESULTS: Ppump and tracer transport rate were measured by lymphatic congestion lymphoscintigraphy in the ipsilateral upper limb in 26 women pre- and post-breast cancer surgery. BCRL occurred in 10/26 (38.5%) cases. Ppump in the women who later developed BCRL (40.0 ± 8.2 mmHg) was 1.7-fold higher than in those who did not develop BCRL (23.1 ± 10.8 mmHg, p = 0.001). Moreover, the rate of lymph tracer transport into the forearm was 2.2-fold greater in the women who later developed BCRL (p = 0.052). Surgery did not significantly reduce Ppump measured 21 weeks postsurgery, but impaired forearm tracer transport in pre-BCRL women by 58% (p = 0.047), although not in those who did not develop BCRL. CONCLUSIONS: Women destined to develop BCRL have higher pumping pressures and lymph transport, indicating harder-working lymphatics before cancer treatment. Axillary lymphatic damage from surgery appears to compromise lymph drainage in those women constitutively predisposed to higher lymphatic pressures and lymph transport.

Challenges in the management of pleomorphic lobular carcinoma in situ of the breast.

BACKGROUND: Pleomorphic Lobular Carcinoma in Situ (PLCIS) is a pathological variant of Lobular Carcinoma in Situ (LCIS) with distinct features. Since first described over a decade ago there are only few papers published about this condition. METHODS: Medline and Pubmed based literature overview was done with the aim of describing the different histopathological, radiological and clinical features of this pathological entity to highlight the different clinicopathological presentations and modalities of treatment described. RESULTS: PLCIS has different biological features when compared to LCIS. It is more likely to be associated with invasive disease and the immuno-histochemical profile shows it is less likely to be ER and PR positive with higher positivity of HER2, Ki-67and p53. It has been suggested that PLCIS should be treated more aggressively than LCIS and surgically excised in similar fashion to DCIS. CONCLUSION: PLCIS is a more aggressive variant of LCIS that needs to be managed differently. Surgical excision with clear margins is advised. Further adjuvant treatments have been described in the literature with little evidence to support their use.

Percutaneous ultrasound-guided thrombin injection for the treatment of iatrogenic femoral artery pseudoaneurysms.

PURPOSE: To audit our experience with ultrasound-guided thrombin injection for the treatment of iatrogenic femoral artery pseudoaneurysms. METHODS: A retrospective study of 85 consecutive patients undergoing percutaneous ultrasound-guided thrombin injection of post-catheterization femoral pseudoaneurysms during the period January 2002 to May 2007. RESULTS: Pseudoaneurysms had a mean maximum diameter of 3.3 cm (range 1.0-7.6 cm) and a mean neck width of 3.4mm (range 1.0-7.0mm). No statistically significant correlation existed between maximum diameter and neck width (Kendall's rank correlation tau b=-0.09, p=0.5). The median dose of thrombin injected was 425 U (range 100-1500 U). The procedure resulted in complete sac thrombosis in 81 (95%) patients. Seventy-nine pseudoaneurysms thrombosed immediately after one injection, whereas two required a second thrombin injection. There were no procedural complications. The maximum diameter of the pseudoaneurysm was predictive of procedural success (Wilcoxon's rank sum test, p=0.001) and of the 5 patients with a pseudoaneurysm measuring ≥6 cm, ultrasound-guided thrombin injection was unsuccessful in 4 (4/5 versus 0/80, p<0.0001, Fisher's exact test). Three of these necessitated implantation of a stent-graft, whereas one required repeated thrombin injection and coil placement. In contrast, the pseudoaneurysm neck width did not seem to relate to the success of the procedure. CONCLUSION: Percutaneous ultrasound-guided thrombin injection of is a quick, effective and safe treatment for iatrogenic femoral pseudoaneurysms. For larger pseudoaneurysms, although it is worth attempting more than one thrombin injection, endovascular repair may eventually be required.