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Cutaneous angiosarcoma (CAS) is a rare soft-tissue sarcoma of vascular endothelial origin. Paclitaxel (PTX) and docetaxel (DTX) are used as systemic chemotherapy; however, chemoresistance often occurs in CAS. Switching one taxane to the other (i.e., PTX to DTX, or vice versa) is an option when the first taxane is no longer effective in malignant cancers such as ovarian or breast cancer. However, the efficacy of the same strategy in CAS has not been reported. Herein, we report the clinical response of switching one taxane-based chemotherapy to the other in CAS patients with resistance to the first taxane. Twelve CAS patients were included for analyses. In all patients, the median overall survival from the start of the first taxane treatment was 29.0 months (range, 6.47-58.5). During the first taxane, the median PFS for all patients was 5.96 months (1.81-47.1). Similarly, the median (range) PFS for all patients during the second taxane was 5.87 months (1.60-18.2). Furthermore, the median OS was 22.7 months (PTX to DTX) and 39.5 months (DTX to PTX) (p = 0.307). The median PFS during the first taxane was 5.14 (PTX to DTX) and 12.5 months (DTX to PTX), respectively (p = 0.380). The median PFS during the second taxane was 3.5 (PTX to DTX) and 7.1 months (DTX to PTX), respectively (p = 0.906). The objective response rate, defined as the sum of complete response (CR) and partial response (PR) rates, was 16.7%. The disease control rate, defined as the sum of CR, PR, and stable disease rates, was 50%. The frequency of adverse events during the second taxane was the same between the two groups (p > 0.999). Our report suggests that CAS patients could benefit from the second taxane treatment if the tumor is resistant to the first taxane., (© 2023 Japanese Dermatological Association.)

Purpose of Review: Radiation-associated angiosarcoma is a cutaneous aggressive tumor that is very rare and it represents a specific entity poorly studied in literature. It requires new therapeutic opportunity., Recent Findings: The complete surgical resection with negative margins is the mainstay treatment of localized treatment, even though it is difficult to reach in case of diffuse cutaneous infiltration. Adjuvant re-irradiation may improve local control with no benefit demonstrated on survival. Many systemic treatments can be efficient not only in metastatic setting but also in neoadjuvant setting in case of diffuse presentation. These treatments have never been compared to each other; the most efficient regimen remains to be determined, and a high heterogeneity of treatment is observed, even between sarcoma reference centers., Summary: Immune therapy represents the most promising treatment under development. At the time of building clinical trial to assess the efficacy of immune therapy, the lack of randomized studies prevents the identification of a strong and consensual reference arm treatment. Given the rarity of the disease, only international collaborative clinical trials may have a chance to include enough patients to draw any conclusion and so will have to counteract the heterogeneity of management., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Aim: Post-radiation angiosarcoma is an iatrogenic event seen in the setting of breast cancer treatment. Histopathologically, there are morphologic variants of angiosarcoma that mimic benign entities, including the capillary lobule variant of post-radiation angiosarcoma. We present the largest case series to date of this histopathologic variant of post-radiation angiosarcoma., Methods and Results: Cases of the capillary lobule variant of post-radiation angiosarcoma from institutional/consultation archives from 2008 to June 2022 were reviewed. For inclusion, tumors had to occur in irradiated skin and exhibit a multi-lobular proliferation of tightly packed capillary-like vessels, as previously described in this variant. Prior ancillary studies were also reviewed. Eight cases met the criteria. All occurred in women treated with radiation for breast cancer (median age 75 years). All cases had similar findings, including a multi-lobular proliferation of tightly packed vessels, infiltrative cords, and atypical single endothelial cells. A conventional angiosarcoma pattern was also seen in five cases. All cases tested were positive for vascular markers (CD31, CD34, and/or ERG) and MYC. MYC amplification was shown by FISH in all cases tested. Smooth muscle actin (SMA) was positive in pericytes in the capillary lobules in all five cases tested and areas of conventional angiosarcoma in two of three cases., Conclusions: The capillary lobule variant of angiosarcoma is a rare and therefore potentially under-recognized variant of post-radiation angiosarcoma. The lobular architecture and SMA positivity may mimic benign vascular proliferations. Careful attention to histopathologic features and ancillary tests may facilitate accurate diagnosis., (© 2022 The Authors. Journal of Cutaneous Pathology published by John Wiley & Sons Ltd.)

Opinion Statement: Cutaneous angiosarcoma is a rare and invasive malignant tumor. For localized cAS patients, wide-margin excision was recommended. Due to the latent local invasion characteristic of cAS, we suggest preoperative and postoperative radiotherapy to nearly all patients. Recently, there is growing interest in using neoadjuvant chemotherapy and/or radiotherapy as part of a combination therapy regimen, which may allow some patients to undergo potentially less disabling surgery. For metastatic cAS patients with unresectable tumors and who refuse surgery, radical radiotherapy or chemoradiotherapy may be an option. Paclitaxel was recognized as the first-line treatment. For tumors resistant to taxanes, emerging medications such as targeted agents and immunotherapy are also under investigation., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: Angiosarcoma secondary to post-irradiation and lymphedema is rare, but it is aggressive with a poor prognosis. It is essential to understand these patients' clinical features and distinguish them from benign diseases or other malignant tumors., Patient Concerns: Three patients who had radiotherapy for cancer treatment and chronic lymphedema admitted to the hospital with specific skin lesions at upper or lower extremities., Diagnosis: Excisional biopsies revealed prominent, highly atypical cells with a vasoformative area, composed of atypical, large epithelioid cells with vesicular nuclei, prominent nucleoli, and mitoses. Immunohistochemistry revealed diffuse expression of endothelial cell markers suggestive of angiosarcoma., Interventions: One patient had shoulder disarticulation with wide excision with adjuvant radiotherapy and chemotherapy and other 2 discontinued the treatment., Outcomes: After the treatment, one patient was transferred to rehabilitation department for shoulder disarticulation prosthesis fitting without recurrence sign for 1 year. Two patient refused further treatment and was lost to follow-up., Conclusion: In cases of patients with irratiation and chronic lymphedema, clinical findings suggestive of angiosarcoma, biopsy and imaging studies should be performed as soon as possible., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Angiosarcomas are ubiquitous neoplasms involving both cutaneous and soft tissue and visceral locations. Accumulating biomolecular evidences suggest that cutaneous angiosarcomas are distinctive entities with molecular, clinical and pathological peculiarities. Despite several ongoing clinical trials with promising therapeutic agents, the prognosis of cutaneous angiosarcomas is dismal and survival still rely on early diagnosis and surgery. An accurate diagnosis and the knowledge of the underlying molecular landscape are therefore essential to improve the prognosis. We detail the molecular, clinical, dermoscopic, morphological and prognostic features of cutaneous angiosarcoma. Although the molecular landscape of cutaneous angiosarcoma is not completely understood, accumulating evidences suggest that there are characteristic molecular alterations including dysregulation of angiogenesis and several complex molecular pathways. Secondary cutaneous angiosarcomas, arising in correlation with chronic lymphedema and ionizing radiation, have different molecular hallmarks, which are also leading to the first diagnostic applications. The diagnosis of cutaneous angiosarcoma may be challenging, as well-differentiated forms can be hard to distinguish from benign and low-grade vascular neoplasms, while poorly differentiated forms can be easily confounded with other non-vascular high-grade neoplasms. An accurate and early diagnosis, which is mandatory to ensure the best survival for the patients, is mainly based on morphological hallmarks., (Copyright © 2020 Elsevier Inc. All rights reserved.)

We report a radiation-associated angiosarcoma(RAAS)of the breast, which is a rare but important complication after breast-conserving surgery(BCS)and radiotherapy(RT)for breast cancer. A7 2-year-old woman had undergone BCS for invasive ductal carcinoma of the right breast(pT2pN1M0, StageⅡB), followed by RT of 50 Gy; she was treated with doxifluridine and anastrozole for 5 year. She noticed a bloody cutaneous bulla in the right breast 64 months later, and the skin lesions gradually expanded. She was brought to our clinic for the treatment of massive bleeding from the skin lesions. Ulcer biopsy revealed cutaneous AS(cells were CD31[+], CD34[+], VEGF[-], and VEGF-R[+]). She underwent mastectomy and latissimus dorsal flap surgery. She died of local recurrence and liver metastasis 13 months later. RAAS is rare, but it should be considered in patients with skin lesions, such as erosion and bloody bulla, after BCS and RT for breast cancer. To our knowledge, only 12 cases of RAAS, including the present case, have been reported in Japan, and we reviewed the Japanese RAAS cases in comparison with those reported in the Western literature.

Background: Chronic lymphoedema is classically complicated by recurring episodes of cellulitis. Degeneration to the angiosarcoma form (Stewart-Treves syndrome) is much less common. It occurs mainly in the upper limbs following surgery or radiotherapy for mammary neoplasia. Herein we report a rare case of Stewart-Treves syndrome (STS) of the lower limb as a complication of congenital lymphoedema., Patients and Methods: A 69-year-old woman treated for bilateral lower-limb oedema present for 30years developed painful necrotic lesions in her left lower limb. A diagnosis of angiosarcoma was made based on biopsy of a nodular lesion. Since surgical excision was precluded by the extent of the lesions, chemotherapy was initiated with paclitaxel 175mg/m 2 every 21days. The outcome was rapidly unfavourable and the patient died at her home during the third course of treatment., Discussion: Angiosarcoma is an extremely rare complication of primary lymphoedema; treatment is poorly codified and the prognosis is unfavourable., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Introduction and Objectives: Primary cutaneous angiosarcoma is one of the most aggressive skin tumors and carries a very poor prognosis. Its initially indolent clinical presentation explains the frequently late diagnosis that, together with its typically multifocal pattern and poor delimitation, often makes surgery difficult. The low incidence of primary cutaneous angiosarcoma means that few large single-center series have been published. We review the clinical and pathologic characteristics of cutaneous angiosarcomas treated in our hospital, looking for prognostic factors and for possible diagnostic traits that could facilitate early diagnosis., Material and Methods: This was a retrospective observational study including all patients diagnosed with cutaneous angiosarcoma in Instituto Valenciano de Oncología in Valencia, Spain between January 2000 and December 2015. We recorded 16 clinical parameters, including age, sex, type of angiosarcoma, site, size, and time since diagnosis, and 8 histopathologic parameters., Results: We identified 16 patients (11 women and 5 men) with cutaneous angiosarcoma. Their mean age was 67 years (median, 71 years). The most common site was the trunk (10 cases), followed by the head and neck (5 cases). The mean size of the tumor was 10cm (median, 6.5cm). Fourteen patients underwent surgical excision. Six of the 16 patients were alive at the end of the study, after a mean follow-up period of 42.5 months., Conclusions: The major determinants of survival among patients with cutaneous angiosarcoma are tumor size and patient age. Other characteristics associated with a poor prognosis were infiltration of deep planes (muscle), a predominantly solid histologic pattern, and a larger number of mitoses., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

History In 2004, this woman received a diagnosis of invasive mammillary carcinoma, tubular variant, strongly positive for estrogen and progesterone receptors. Her lesion was found at screening mammography performed at an outside institution when she was 59 years old. She underwent partial mastectomy, with partial axillary node dissection and sentinel node mapping. A 0.6 × 0.5 cm Nottingham grade 1 infiltrating ductal carcinoma was removed from the right upper outer quadrant, margins were free of tumor, and there was no angiolymphatic invasion. The six dissected lymph nodes were negative for malignancy. Her surgical history was otherwise unremarkable. Her medical history was positive for hypercholesterolemia and depression. Pertinent family history included breast cancer in both her mother and her sister. Given the patient's age, tumor size, lack of nodal involvement, and clear surgical margins, she met recommended MammoSite criteria, and she underwent accelerated partial breast radiation. She subsequently received 340 cGy of radiation twice a day for a total dose of 3400 cGy in 10 administrations in February 2005. Accelerated partial breast radiation treatment was completed in February 2005, and she received subsequent routine care. Prior to 2014, the only postoperative complication was a chronic radiation bed seroma, which required periodic percutaneous drainage. She did not develop postsurgical lymphedema. In December 2013, 9 years after accelerated partial breast radiation treatment, she experienced progressive painful pruritic breast fullness, skin dimpling, and skin discoloration of the mastectomy scar and radiation bed. She sought medical care in January 2014 after she noticed a periareolar ulcerating skin plaque, more noticeable nipple retraction, and new onset of retroareolar aching. At physical examination ( Fig 1 ), there was generalized periareolar erythema, dimpling, firmness, and fixation involving the central breast and right upper outer quadrant. There was more conspicuous retraction of the nipple when compared with that seen at prior examinations. Nipple discharge was not present. There was a 1-cm periareolar ulcerating skin plaque. The only discrete palpable finding was lumpectomy bed seroma. There was no palpable axillary adenopathy. [Figure: see text] A diagnostic mammogram was obtained and compared with the most recent studies available. Ultrasonography (US) and magnetic resonance (MR) imaging were performed. Her most recent mammogram, obtained 3 months earlier in September 2013, reported Breast Imaging Reporting and Data System (BI-RADS) category 2 findings (ie, stable postoperative benign findings).

Objective: Cutaneous radiation therapy (RT)-associated breast angiosarcoma (AS) is a rare consequence of breast RT associated with poor outcomes. Previous small case series have documented high recurrence rates and poor survival. We reviewed our experience and focused on the impact of conservative versus radical resections., Methods: Data for patients with RT-associated breast AS evaluated at our institution from 1993 to 2015 who underwent surgery were reviewed., Results: Seventy-six women were diagnosed with RT-associated breast AS at a median 85 months from surgery for invasive breast carcinoma or ductal carcinoma in situ. Thirty-eight underwent mastectomy/wide excision with partial skin resection ("conservative") and 38 underwent resection of all or nearly all previously irradiated skin plus mastectomy ("radical"). The radical group (vs the conservative group) more often had multifocal disease (80% vs 56%, P = 0.04), chemotherapy for AS (58% vs 22%, P < 0.01), margin-negative resection (100% vs 73%, P < 0.01), reconstructive surgery (100% vs 13%, P < 0.01), and re-operation (16% vs 3%, P = 0.04). Five-year crude cumulative incidences of local recurrence and distant metastasis for radical versus conservative groups were 23% versus 76% (P < 0.01) and 18% versus 47% (P = 0.02), respectively. Five-year disease-specific survival (DSS) for radical versus conservative groups was 86% versus 46% (P < 0.01), respectively. On multivariable analysis, age, radicality of surgery, and margin were predictive of DSS., Conclusions: For patients with RT-associated breast AS, radical resection was associated with reduced recurrence rates and improved DSS. Although margin was predictive of DSS, multifocality calls into question the reliability of negative margin assessment.

More and better accessibility to screening and increasing women's knowledge about the main symptomatic breast cancer signs result in detection of this neoplasm at an early stage, allowing a conservative surgical treatment. Breast conserving therapy (BCT) due to breast carcinoma involves the use of adjuvant radiotherapy which carries a risk the secondary radiation-induced malignancy. Angiosarcoma of the breast is an extremely rare radiation-induced malignant tumor following breast conserving therapy (BCT), first described by Schmidt in 1887. In this article the authors presented the case of a 56-year-old woman who was admitted to the hospital because of diagnosed angiosarcoma of the right breast after breast conserving therapy (BCT) with the Sentinel Node Biopsy (SNB) and adjuvant radiotherapy of her breast carcinoma 5 years earlier. The patient had a simple mastectomy performed and on the second day after surgery was discharged home., Key Words: Angiosarcoma, Breast cancer, Radiation therapy, Surgery, Treatment.

Angiosarcoma of the skin and breast is a known complication of chronic lymphedema following mastectomy or external radiation therapy for breast cancer. We report a 68-year-old woman who presented with a 2.5 cm violaceous plaque on the skin of the right breast and a 3 cm mixed mass of the same breast by ultrasound 9 years after MammoSite® balloon brachytherapy.  Biopsy of the skin lesion and the breast mass showed an infiltrating high grade angiosarcoma.  The tumor cells in the skin and breast showed immunohistochemical reactivity for C-MYC.  A total mastectomy confirmed the presence of high grade angiosarcoma in the skin and parenchyma of the breast and radiation changes in the breast parenchyma.  Surgical margins were considered negative.  The patient had cutaneous recurrence of angiosarcoma three months after the mastectomy.  There have been only two other case reports in the literature of angiosarcoma on the skin following MammoSite® therapy. The c-myc mutation has been shown to be a specific mutation for angiosarcoma following radiation treatment. It is not found in atypical vascular lesions following irradiation or angiosarcoma unrelated to radiation treatment.

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

A woman with postmastectomy lymphoedema presented with purple-red nodules on the right upper arm. Histopathology of a skin biopsy showed a cutaneous angiosarcoma. Angiosarcoma arising in an oedematous arm is called the Stewart-Treves syndrome and is a rare and late complication of breast cancer treatment.

This review gives an overview of radiotherapy-induced malignant skin tumors as described in the present medical literature. Basal cell carcinomas are the most frequent post-radiation malignant skin tumors; however, specific incidence ratios are few and show ratios of 2%. Squamous cell carcinomas are briefly discussed, followed by post-radiation sarcomas. Most cases of post-radiation cutaneous sarcomas are angiosarcoma, malignant fibrous histiocytoma, leiomyosarcoma and fibrosarcoma. In cases of radiotherapy for breast cancer, angiosarcomas are the most frequently found malignant sarcomas worldwide (incidence 0.5%) in the irradiated area. We present 192 cases of angiosarcomas after radiotherapy for breast cancer. Also, the atypical vascular lesion, a benign vascular skin lesion occurring after radiotherapy, and the important differential diagnosis of angiosarcoma will be presented and discussed. Other skin tumors supposedly related to radiotherapy are occasionally published and summarized in this review. Because most radiation-induced malignant tumors occur many years after the initiation of radiotherapy and incidences are low, we suggest good instruction of patients regarding self control of the skin rather than a yearly follow-up.

We present a case of chronic lymphoedema that progressed to Stewart-Treves syndrome in a 63-year-old woman with a previous modified radical mastectomy, associated lymph node dissection, chemotherapy and radiotherapy. While producing stabilisation of most cutaneous lesions initially, chemotherapeutic treatment of the angiosarcoma did not prevent subsequent metastasis and patient death. We urge vigilance and regular follow-up appointments for patients following a mastectomy with chronic lymphoedema to facilitate prevention or early treatment of this devastating syndrome.

Molecular techniques are increasingly important in the practice of surgical pathology. In soft tissue tumors, there are a number of tumors with recurring cytogenetic abnormalities. Knowledge of these abnormalities has furthered our understanding of these tumors and has also allowed development of molecular techniques to aid in the diagnosis. This review will focus on mesenchymal tumors with specific cytogenetic abnormalities that may present as a superficial tumor of the dermis or subcutis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

In recent years, a number of new soft tissue tumor entities have been described that occur in the skin only, or that also occur in other sites but form clinically and pathologically distinct subsets when arising in the skin and subcutaneous tissue. These include a variety of mesenchymal lineages and have variable malignant potential, although superficial malignant soft tissue tumors generally have a more favorable outcome than their more deeply located counterparts. This article reviews the clinical and pathologic features and differential diagnoses of atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelioma, myopericytoma, and perivascular epithelioid cell tumor., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Radiation-induced angiosarcomas are uncommon adverse sequelae related to treatment of tumors. Early diagnosis and treatment are key to successful management., Purpose: The purpose of this case study is to describe the clinical characteristics of radiation-induced angiosarcomas., Materials and Methods: We retrospectively reviewed the medical literature using PubMed, searching the terms angiosarcoma, breast, post, radiation, and treatment. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented., Results: Approximately one hundred patients have been diagnosed with radiation-induced angiosarcomas. The condition presents within the radiation field, approximately six years after initial treatment. We describe the dramatic efficacious response of our patient's angiosarcoma to adjuvant chemotherapy both preoperatively (gemcitabine and docetaxel) and postoperatively (gemcitabine and docetaxel followed by ifosfamide and adriamycin)., Conclusion: We recommend that new skin lesions within or adjacent to radiation ports should be considered for biopsy. Also, for lesions that are larger, ill-defined, or both, several sites should be sampled to ensure an accurate diagnosis and to prevent the possibility of a false negative interpretation.

Background: Cutaneous radiation-associated angiosarcoma of the breast (CRAASBr) is a rare complication of radiation therapy (RT) administered for primary breast cancer treatment. Although case series have provided clinical and histological descriptions of this disease, to our knowledge, none have identified trends in presentation and treatments that may contribute to outcomes., Methods: Demographic, clinical, histopathologic, and outcomes data for all patients presenting with CRAASBr for treatment or consultation at our institution from 1987 to 2009 were reviewed., Results: We identified 33 patients (median age at CRAASBr presentation 71.3 years, range 43.1-87.2 years; median latency period 73.5 months, range 39.6-148.5 months). The most common presentation was breast skin ecchymosis (55 %). In four patients, initial biopsy demonstrated atypical vascular lesions suspicious for, but not diagnostic of, angiosarcoma. All patients underwent mastectomy. Median local recurrence-free survival (LRFS), recurrence-free survival (RFS), and overall survival (OS) rates were 18.2, 13.0, and 48.5 months, respectively. Patients who underwent resection of all irradiated breast skin as part of the mastectomy trended toward a better median LRFS (80.8 vs. 10.0 months, p = 0.065), RFS (72.6 vs. 10.0 months, p = 0.098), and OS (not achieved vs. 29.0 months, p = 0.054)., Conclusions: CRAASBr is a potentially devastating consequence of RT for breast cancer, with poor LRFS, RFS, and OS rates. Patients with ecchymotic skin lesions require biopsy. Atypical vascular lesions require careful evaluation to rule out CRAASBr. If the diagnosis is confirmed, radical surgery encompassing both the breast parenchyma and the at-risk radiated skin should be performed.

We report a case of cutaneous angiosarcoma occurring on the leg of a 97-year-old Japanese woman. Considering the patient's age and general condition, she was treated with electron beam irradiation, which led to the almost complete disappearance of the tumour. Because cutaneous angiosarcoma is an aggressive tumour with a high propensity for local recurrence and distant metastases, therapy preferably involves a multimodal approach. However, monotherapy with radiation may be effective in some cases of cutaneous angiosarcoma., (© 2011 The Authors. Australasian Journal of Dermatology © 2011 The Australasian College of Dermatologists.)

Canine dermal haemangiosarcoma (HSA) is believed to have a better prognosis compared to HSA in other organs, but outcome has only been reported in a small number of dogs. The purpose of this study was to assess outcome and prognostic factors in a larger cohort of dogs with dermal HSA. Clinical data was collected retrospectively for 94 dogs and histopathology was reviewed in 53 dogs. Median overall survival time was 987 days. Dogs of predisposed breed with ventral location and histologic solar changes had longer survivals. Loco-regional recurrence occurred in 72/94 (77%) dogs. Predisposed breeds with ventral location and multiple masses were more likely to develop recurrence. Non-predisposed breeds with invasive tumours were more likely to develop metastasis. Results suggest that dogs with solar-induced dermal HSA may have high recurrence rates, but prolonged survivals. Dogs with non-solar tumours may be at increased risk for metastasis and shorter survival., (© 2011 Blackwell Publishing Ltd.)

Background: Post-radiation atypical vascular lesions of the skin display clinical and morphological overlap with well-differentiated angiosarcomas, and correct diagnosis may be difficult., Patients and Methods: We studied clinical, histological and immuno-histochemical aspects (CD31, CD34, D2-40 and VEGFR-3) of eight post-radiation atypical vascular lesions comparatively with three post-radiation angiosarcomas., Results: All patients were female and received radiation therapy for breast carcinoma. On average, atypical vascular lesions occurred 4.3 years after radiation therapy and presented as small papulonodules or erythematous plaques. The clinical course after simple excision was benign. Histologically, they were relatively circumscribed lesions and showed slit-like vessels dissecting dermal collagen in all cases. On average, angiosarcomas occurred 5 years after radiation therapy and presented as more extensive lesions with a more aggressive clinical course. The lesions showed histological overlap with atypical vascular lesions, but were poorly circumscribed, with deeper invasion, cytological atypia and mitosis. Although the immuno-histochemical profiles were similar, expression of VEGFR-3 was greater in two cases of angiosarcoma., Conclusion: Post-radiation atypical vascular lesions are benign lesions that display clinical, histological and immuno-phenotypic overlap with well-differentiated angiosarcoma, and diagnosis requires good clinicopathological correlation. VEGFR-3 may be useful for differential diagnosis, as well as amplification of the MYC gene., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Cutaneous angiosarcoma or lymphangiosarcoma represents an uncommon aggressive tumor known to arise on a background of chronic lymphedema secondary to various etiologies, principally following surgery or irradiation. There have been rarely reported cases of angiosarcoma following infective conditions that eventuate with lymphatic stasis. We report a case of angiosarcoma arising after 33 years within a background of filariasis. Awareness of this association can lead to early diagnosis and appropriate treatment of this potentially fatal malignant tumor., (Copyright © 2011 John Wiley & Sons A/S.)

Postradiation cutaneous vascular lesions after treatment of breast carcinoma comprise a heterogeneous group of benign, atypical, and malignant lesions and are best regarded as points along a morphological spectrum. We analyzed a series of cutaneous angiosarcomas after treatment of breast cancer in comparison with control cases and cases of atypical vascular lesions with special emphasis on the expression and amplification of MYC. The 66 cases were divided into control cases (5), cases in which a slight vascular proliferation was seen after radiotherapy of breast cancer (12), cases of atypical vascular lesions after radiotherapy (16), cases of postradiation cutaneous angiosarcomas (25), and cases of angiosarcomas of skin and soft tissues unrelated to radiotherapy (8). None of the control cases (2 M, 3 F, 20-76 years), of cases showing slight vascular proliferation, dermal fibrosis and inflammation after radiotherapy of breast cancer (12 F, 48-79 years), of cases of atypical vascular lesions after radiotherapy (16 F, 29-81 years), and of cases of angiosarcomas of skin and soft tissues unrelated to radiotherapy (3 M, 5 F, 25-92 years) showed an amplification of MYC by FISH analysis. In striking contrast, in all cases of postradiation cutaneous angiosarcomas (25 F, 46-95 years), MYC amplification was found by FISH analysis in a variable number of counted nuclei. Immunohistochemically, strong positive nuclear staining for MYC and prox-1 was seen in cases of postradiation cutaneous angiosarcoma, whereas control cases and cases of atypical vascular proliferation after radiotherapy were negative for MYC, and stained only focally positive for prox-1 in a number of cases. In conclusion, the presence of MYC amplification represents an important additional diagnostic tool in the distinction of postradiation cutaneous angiosarcomas from atypical vascular lesions after radiotherapy. Immunohistochemical stainings for MYC are useful for mapping of these lesions and for careful tumor margin control.

The purpose of this study was to investigate the surgical management of radiation-associated cutaneous breast angiosarcoma with an emphasis on surgical margins and choice of reconstruction. Nine cases of angiosarcoma were identified in patients earlier treated with radiotherapy for breast cancer. Breast angiosarcoma was diagnosed a median of 5.25 years following radiotherapy. Median age at diagnosis was 60 years. Surgical treatment consisted of radical mastectomy (four cases), simple mastectomy (two cases) and wide local excision (three cases). Defect reconstruction involved three latissimus dorsi flap reconstructions and four skin grafts. Clear histological margins were achieved in all cases. Median follow-up was 81 months. Six patients were alive and disease-free at the end of the study period. Aggressive surgical resection with wide margins is essential to reduce local recurrence and improve survival., (Copyright © 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

A significant proportion of angiosarcoma arises in the setting of chronic lymphedema, often following surgery for breast carcinoma (Stewart-Treves syndrome). Massive localized lymphedema (MLL), a relatively recently described and rare pseudotumor most often occurring in morbidly obese patients, is thought to be the result of derangements in lymphatic drainage secondary to excessive adipose tissue. Only a very small number of cases of angiosarcoma have been reported in patients with MLL. We reviewed the clinical and pathological features of five patients with angiosarcoma arising in this unusual clinical setting. The tumors arose in three women and two men, who were in the age range from 36 to 81 years (median: 52 years). All patients were or had recently been morbidly obese (mean body mass index = 47.2 kg/cm(2) ; range:37.6-68.5 kg/cm(2) ) and had long-standing soft tissue changes of MLL. The tumors arose in the thigh (two cases), lower leg (two cases) and abdomen/proximal thigh (one case) and ranged from 6 to 28.5 cm in size (mean: 15.9 cm). All tumors were conventional high-grade angiosarcomas and microscopically exhibited an admixture of vasoformative, spindled and epithelioid features. Microscopic changes of MLL, including dermal fibrosis, expansion of interlobular fibrous septa and lymphangiectasia, were identified in all cases. Clinical follow-up (four cases; mean: 13.8 months; range: 2-32 months) showed one patient to be dead of disease, one patient to be dead from therapy-related complications and two patients to be alive without disease. One case was too recent for meaningful clinical follow-up. We conclude that lymphedema secondary to obesity should be recognized as a significant risk factor for the development of angiosarcoma. The clinical and pathological features of angiosarcoma arising in this distinctive clinical setting appear to be identical to those of other lymphedema-associated angiosarcomas. With the continuing epidemic of obesity in Western societies, the incidence of obesity-related angiosarcoma is probably to increase. Consequently, it is critical that lymphedematous areas in obese patients be carefully examined for clinical evidence of early angiosarcoma, with the hope that timely diagnosis may lead to improved patient outcome., (Copyright © 2011 John Wiley & Sons A/S.)

A 54-year-old woman presented a peri-areolar nodule located in the skin of the right breast. Clinical examination showed a 6 x 5 cm exophytic, lobed, ulcerated, and bleeding nodule. The patient reported that the tumor had grown gradually over a period of 3 months. The patient had been diagnosed 8 years prior to presentation with infiltrating ductal carcinoma of the right breast (pT2NO). This tumor was treated with partial mastectomy (conservative surgery) and lymph node dissection, then subsequently received 30 tangent field radiotherapy sessions to the breast for a total dose of 45 Gy. The rest of her cutaneous exam was normal. There was no family history of any similar tumor.