Factors associated with unsatisfactory cosmetic results in oncoplastic surgery.

Introduction: Oncoplastic surgery (OS) has expanded the indications for breast-conserving surgery associated with an adequate aesthetic result. However, few studies have described the factors associated with unsatisfactory cosmetic outcomes from this surgical modality. Materials and methods: This is a cross-sectional prospective study that included patients undergoing breast-conserving surgery (BCS) with or without OS. The patients self-evaluated the cosmetic results of the breasts posttreatment and had them photographed. The photos were analyzed by BCCT.core. Individual and treatment factors (local and systemic) for all patients were evaluated. These factors were dichotomized according to the use of OS and to the cosmetic result (satisfactory and unsatisfactory). Categorical variables were tested for association with surgical outcome using the chi-square test while numerical variables using the Mann-Whitney U test. Variables with p <0,2 were selected for multivariate analysis. Results: Of the 300 patients evaluated, 72 (24,0%) underwent OS. According to the patient self-evaluations, an unsatisfactory cosmetic result from OS was significantly associated with younger age at diagnosis, higher body mass index (BMI) at the time of evaluation, larger tumor size and greater weight of the surgical specimen. According to the BCCT.core, only the laterality of the tumor (left) was significantly associated with an unsatisfactory cosmetic result. In logistic regression, considering OS as a control variable, the risk of an unsatisfactory outcome according to patient self-evaluation was related to the tumor ≥ T2 odds ratio (OR) 1,85 (1,027-3,34) and age at diagnosis < 40 [OR 5,0 (1,84-13,95)]. However, according to the software, the variables were associated with an increased risk of an unsatisfactory outcome were the time interval between surgery and evaluation [OR 1,27 (1,16-1,39)], the presence of lymphedema [OR 2,97 (1,36-6,46)], surgical wound infection [OR 3,6 (1,22-11,16)], tumor location on the left side [OR 3,06 (1,69-5,53)], overweight [OR 2,93 (1,48-5,8)] and obesity [OR 2,52 (1,2-5,31)]. Conclusion: There is no standard methodology for breast cosmesis evaluation, which influences the factors associated with unsatisfactory results. Younger patients and those with increased BMI, left breast cancer and extensive resections tend to present with unsatisfactory cosmetic results when OS is performed.

Breast reconstruction for locally advanced breast cancer previously submitted to mastectomy and an ipsilateral thoracoabdominal dermofat (ITADE) flap.

Thoracoabdominal (TA) flaps are a good option for primary closure of small and medium defects after mastectomy for locally advanced breast tumours. Although they have a higher rate of necrosis than myocutaneous flaps, they can be easily performed by breast surgeons. Few studies on this procedure have been reported, and we have been unable to identify any prior publications reporting breast reconstruction with TA flaps.

Oncoplastic surgery for Paget's disease of the breast.

Introduction: Paget's disease of the breast (PDB) is a rare nipple entity associated with multifocality. Due to its location, resection of the entire nipple-areolar complex is necessary. Historically central quadrantectomy and mastectomy have the surgical treatments of choice. The feasibility of oncoplastic breast surgery (OBS) for PDB is unknown. Methods: This was a retrospective study performed in a Brazilian oncological hospital. We evaluated the factors related to the performance of OBS in PDB. In addition, the impact of OBS on local recurrence and survival was analysed. Comparisons were made between groups using the chi-square test, Mann-Whitney U test, and Kaplan-Meier method. To assess the impact factor of the variables on the performance of OBS, logistic regression was performed. Results: Eighty-five patients were evaluated. OBS was performed in 69.4% (n=59), and of these, 16 (27.2%) were symmetrized with contralateral surgery. Mastectomy without reconstruction was performed in 28.3% of the patients. The primary procedure performed was mastectomy with reconstruction (n=38; 44.7%), and the preferential technique for immediate reconstruction was skin-sparing mastectomy with prosthesis; for late reconstruction, the preferred technique was using the latissimus dorsi. Breast conserving-surgery was performed in 27.0% (n=23), primarily using the plug-flap technique (OBS). Age was associated with the use of OBS; as patients aged 40-49 exhibited a higher rate of OBS (p = 0.002; odds ratio 3.22). OBS did not influence local recurrence (p=1.000), overall survival (p=0.185), or cancer-specific survival (p=0.418). Conclusion: OBS improves options related to surgical treatment in PDB without affecting local recurrence or survival rates.

Genetic Ancestry of 1127 Brazilian Breast Cancer Patients and Its Correlation With Molecular Subtype and Geographic Region.

PURPOSE: Breast cancer molecular subtypes show significant differences in different ethnic groups in the United States, but no study has evaluated genetic ancestry in breast cancer in Brazilian women. METHODS: Breast cancer patients from distinct parts of Brazil were evaluated. Molecular subtypes were determined by immunohistochemistry. Genetic ancestry was evaluated using a panel of 46 AIMs (ancestry informative markers), which classified genetic ancestry as European, African, Asian, and Amerindian. PCR products were subjected to capillary electrophoresis and analyzed using GeneMapper 4.0 software. Ancestry was evaluated with Structure v.2.3.3 software. Ancestry was tested for correlations with geographic region and molecular subtype. The chi-square test and ANOVA with Bonferroni adjustment were applied. RESULTS: Genetic ancestry and clinical data were evaluated in 1127 patients. Higher rates of self-reported white ethnicity, European ancestry, and HER-2- luminal tumors were identified in the South region, which may influence age at diagnosis and result in a higher rate of early tumors. Conversely, higher rates of African ancestry in the North and Northeast regions, self-reported nonwhite ethnicity, HER-2+ tumors, and triple-negative tumors were noted. Triple-negative and HER-2+ tumors were associated with higher advanced and metastatic disease rates at diagnosis, with triple-negative tumors being more frequent in young women. CONCLUSION: Differences in genetic ancestry, self-reported ethnicity, and molecular subtype were found between Brazilian demographic regions. Knowledge of these features may contribute to a better understanding of age at diagnosis and the molecular distribution of breast cancer in Brazil.

Lymph node microcalcifications after neoadjuvant chemotherapy: A therapeutic response marker.

Lymph node microcalcifications are rare events, and when they are accompanied by neoplasia, they usually seem to be associated with a metastatic condition. We present a case of a patient with breast cancer and lymph node microcalcifications undergoing neoadjuvant chemotherapy (NCT). A change in the calcification pattern towards becoming coarse was observed. Calcification represented a marker of axillary disease, and it was resected after NCT. This is the first report of a patient with lymph node microcalcification undergoing NCT. We observed a change in the calcification format, which facilitated lymph node sentinel identification. Pathological evaluation indicated metastatic disease.

Phase III study of the European Organisation for Research and Treatment of Cancer Quality of Life cancer survivorship core questionnaire.

PURPOSE: The purpose of this study is to develop a European Organisation for Research and Treatment of Cancer Quality of Life Group (EORTC QLG) questionnaire that captures the full range of physical, mental, and social health-related quality of life (HRQOL) issues relevant to disease-free cancer survivors. In this phase III study, we pretested the provisional core questionnaire (QLQ-SURV111) and aimed to identify essential and optional scales. METHODS: We pretested the QLQ-SURV111 in 492 cancer survivors from 17 countries with one of 11 cancer diagnoses. We applied the EORTC QLG decision rules and employed factor analysis and item response theory (IRT) analysis to assess and, where necessary, modify the hypothesized questionnaire scales. We calculated correlations between the survivorship scales and the QLQ-C30 summary score and carried out a Delphi survey among healthcare professionals, patient representatives, and cancer researchers to distinguish between essential and optional scales. RESULTS: Fifty-four percent of the sample was male, mean age was 60 years, and, on average, time since completion of treatment was 3.8 years. Eleven items were excluded, resulting in the QLQ-SURV100, with 12 functional and 9 symptom scales, a symptom checklist, 4 single items, and 10 conditional items. The essential survivorship scales consist of 73 items. CONCLUSIONS: The QLQ-SURV100 has been developed to assess comprehensively the HRQOL of disease-free cancer survivors. It includes essential and optional scales and will be validated further in an international phase IV study. IMPLICATIONS FOR CANCER SURVIVORS: The availability of this questionnaire will facilitate a standardized and robust assessment of the HRQOL of disease-free cancer survivors.

Evaluation of upper limb lymphoedema and diagnostic accuracy of bioimpedance spectroscopy. A comprehensive validation in a Brazilian population.

Lymphoedema is a complication of breast cancer treatment. Its early diagnosis is related to a good prognosis for lymphoedema treatment. The bioimpedance spectroscopy (BIS) evaluates changes in extracellular fluid. The objective of our study was to evaluate the validity, agreement and accuracy of BIS in the diagnosis of breast cancer-related lymphoedema in a Brazilian population. Methods: This is a prospective, cross-sectional study of a convenience sample of 462 women who underwent surgical treatment for breast cancer (mastectomy or breast-conserving treatment). The validity, agreement and accuracy were performed comparing BIS (lymphoedema index (L-DEX) ≥ 6.5 or 10) with volumetry by water displacement, which is the gold standard for evaluating lymphoedema. Receiver operating characteristic curve was performed. Additionally, other methods like perimetry and indirect volumetry of the upper limbs were compared with water displacement volumetry (direct volumetry), and the BIS were compared with subjective evaluation. Results: Considering L-DEX ≥ 10 the sensitivity of the BIS was 44.1%, specificity 95.4%, positive predictive value (PPV) was 70.7%, negative predictive value (NPV) was 87% and kappa was 0.459. The BIS with L-DEX ≥ 6.5, the sensitivity, specificity, PPV, NPV and kappa were 57%, 88.5%, 55.8%, 89% and 0.452, respectively. Area under curve was 0.724 and a possible cut-off point of L-DEX ≥ 7.35 with sensitivity of 57%, specificity of 90.7% and kappa value = 0.489. Conclusion: Although BIS was significantly associated with the subjective evaluation of lymphoedema, it showed low sensitivity and agreement and moderate correlation when used as a method for diagnosing the condition. Thus, it is not the most valid method for evaluating lymphoedema. In addition, it was not the most accurate method when compared with other objective evaluation tools. Public health resources are scarce and must be used consciously. The knowledge that BIS is not a more accurate method than other, lower-cost instruments allows for better targeting of these resources.

HPV-positive cervical squamous cell carcinoma metastasis to the breast, mimicking primary tumor.

INTRODUCTION: Metastatic disease to the breast is a rare condition, with contralateral breast metastasis being the most common primary site. CASE PRESENTATION: We present the case of a patient who underwent treatment for an HPV positive squamous cell carcinoma (SCC) of the cervix who, during follow-up, complained of a nodule in her left breast. Anatomopathological results indicating squamous carcinoma, which was not able to be differentiated from breast metaplastic carcinoma. Resection of the lesion was carried out, confirming carcinoma with squamous cell differentiation with negativity for GCDFP-15, mammaglobin, p63 and SOX10, but with positivity for p16 and for high risk HPV, confirming a single metastatic lesion of cervical carcinoma. DISCUSSION/CONCLUSION: In the presence of SCC in the breast, the differential diagnosis may consider the presence of primary lesion, metaplastic carcinoma with squamous cell differentiation or metastatic disease. The use of markers such as p63, SOX10 and p16, may help for a definitive diagnosis.

Breast-conserving surgery with the geometric compensation/split reduction technique. Indications, oncologic safety and cosmesis. A cohort series and systematic review of the literature.

BACKGROUND: The Geometric Compensation Technique (GCT) and the Split Reduction Technique (SRT) enables breast conserving surgery (BCS) in selected patients with breast cancer initially candidates to mastectomy. METHODS: Observational study of patients with breast cancer who underwent GCT consecutively treated. Evaluated retrospectively: indications, clinical characteristics, surgical features and recurrences. Cosmesis were evaluated prospectively by the BCCT.core software, Harris/Harvard and Garbay scales. Descriptive statistics were performed, chi-square test was used to compare aesthetic outcomes; Kappa and Weighted Kappa test was used to assess agreement between the postoperative evaluations; Kaplan-Meier model for follow-up and recurrence. A systematic review was carried out using PRISMA methodology. RESULTS: Thirty-six patients were evaluated, 26 (72.2%) with medium/large breasts with or without ptosis, seven (19.4%) with small breasts with or without ptosis, a profile undergoing GCT not previously identified in the literature. The mean tumor clinical size was 3.65 ± 1.59 cm. 34 (94.4%) patients underwent GCT. Mean follow-up time was 36.6 ± 16.8 months, with no local recurrences. According to BCCT.core, the postoperative aesthetic was good in 17 (51.5%) patients and 11 (33.3%) was reasonable. In the systematic review, 3.584 articles were evaluated, 20 articles were selected, 243 patients undergoing GCT were found, with several indications for BCS instead of mastectomy, with high rates of free margins, low recurrence and good aesthetic outcomes. CONCLUSION: GCT, an oncologically safe and aesthetically satisfactory option, has been extended to patients with small and medium breasts with ptosis, large tumors to breast volume or in unfavorable resection sites, initially candidates for mastectomy.

Analysis of RPL37A, MTSS1, and HTRA1 expression as potential markers for pathologic complete response and survival.

BACKGROUND: Non-metastatic locally advanced breast carcinoma (LABC) treatment involves neoadjuvant chemotherapy (NCT). We evaluated the association of clinical-pathological data and immunoexpression of hormone receptors, HER2 and Ki67, and new biomarkers, RPL37A, MTSS1 and HTRA1, with pathological complete response (PCR) or tumour resistance (stable disease or disease progression), disease-free survival (DFS) and cancer-specific survival (CSS). METHODS: This is a retrospective study of 333 patients with LABC who underwent NCT. Expression of MTSS1, RPL37A and HTRA1/PRSS11 was evaluated by immunohistochemistry in TMA slides. Cutoff values were established for low and high tumour expression. ROC plotter evaluated response to NCT. Chi-square test for factors related to PCR, and Kaplan-Meier test and Cox model for factors related to DFS and CSS were prformed. RESULTS: The mean follow-up was 70.0 months and PCR rate was 15.6%. At 120 months, DFS rate was 32.5% and CSS rate was 67.1%. In multivariate analysis, there was an association between: (1) necrosis presence, intense inflammatory infiltrate, ER absence, HER2 molecular subtype and high RPL3A expression with increased odds of PCR; (2) lymph node involvement (LNI), high Ki67, low RPL37A and high HTRA1 expression with increased risk for NCT non-response; (3) LNI, high proliferation, necrosis absence, low RPL37A and high HTRA1 expression with increased recurrence risk; (4) advanced LNI, ER negative tumours, high HTRA1, low RPL37A expression and desmoplasia presence with higher risk of cancer death. CONCLUSION: RPL37A is a potential biomarker for response to NCT and for prognosis. Additional studies evaluating HTRA1 and MTSS1 prognostic value are needed.

Modified External Oblique Myocutaneous Flap for Repair of Postmastectomy Defects in Locally Advanced Breast Tumors: A Cohort Series Associated with a Systematic Review of Literature.

BACKGROUND: Locally advanced breast tumors (LABT) are situations of difficult resolution in clinical practice. External oblique myocutaneous flap (EOMF) is an option, but there are few studies in the literature on its use. METHODS: This was a retrospective, cohort institutional study of patients with LABT who were undergoing mastectomy combined with the use of modified-EOMF (M-EOMF). Preoperative indications and conditions, factors associated with surgery, time to radiotherapy, local recurrence, and survival were assessed. A systematic review of the literature also was performed to evaluate the use of EOMF. RESULTS: Over the 10-year period, 17 patients underwent M-EOMF closure. The mean duration of surgery was 251 min, and extensive skin area was resected (mean 468 ± 260 cm2). Four patients developed local recurrence. The actuarial survival at 36 months was 48.3%. Using PRISMA statement, among 115 articles evaluated from 3 databases, 8 articles were selected, in which 146 patients underwent EOMF. EOMF are associated with low postoperative complications with 8.9% skin necrosis. The M-EOMF allowed the resection of larger areas than other flaps described in the literature but is associated with skin necrosis. CONCLUSIONS: M-EOMF has the advantages of not requiring a change in the patient's position for the closure of large areas. It is thus an acceptable option for chest wall reconstruction in tumors at the limit of resectability.

miRNA expression profiling of hereditary breast tumors from BRCA1- and BRCA2-germline mutation carriers in Brazil.

BACKGROUND: MicroRNAs (miRNAs) are small non-coding RNAs involved in post-transcriptional gene expression regulation and have been described as key regulators of carcinogenesis. Aberrant miRNA expression has been frequently reported in sporadic breast cancers, but few studies have focused on profiling hereditary breast cancers. In this study, we aimed to identify specific miRNA signatures in hereditary breast tumors and to compare with sporadic breast cancer and normal breast tissues. METHODS: Global miRNA expression profiling using NanoString technology was performed on 43 hereditary breast tumors (15 BRCA1, 14 BRCA2, and 14 BRCAX), 23 sporadic breast tumors and 8 normal breast tissues. These normal breast tissues derived from BRCA1- and BRCA2- mutation carriers (n = 5) and non-mutation carriers (n = 3). Subsequently, we performed receiver operating characteristic (ROC) curve analyses to evaluate the diagnostic performance of differentially expressed miRNAs. Putative target genes of each miRNAs considered as potential biomarkers were identified using miRDIP platform and used for pathway enrichment analysis. RESULTS: miRNA expression analyses identified several profiles that were specific to hereditary breast cancers. A total of 25 miRNAs were found to be differentially expressed (fold change: > 2.0 and p < 0.05) and considered as potential biomarkers (area under the curve > 0.75) in hereditary breast tumors compared to normal breast tissues, with an expressive upregulation among BRCAX cases. Furthermore, bioinformatic analysis revealed that these miRNAs shared target genes involved in ErbB, FoxO, and PI3K-Akt signaling pathways. CONCLUSIONS: Our results showed that miRNA expression profiling can differentiate hereditary from sporadic breast tumors and normal breast tissues. These miRNAs were remarkably deregulated in BRCAX hereditary breast cancers. Therefore, miRNA signatures can be used as potential novel diagnostic biomarkers for the prediction of BRCA1/2- germline mutations and may be useful for future clinical management.

Potential biomarkers of ductal carcinoma in situ progression.

BACKGROUND: Ductal carcinoma in situ is a non-obligate precursor of invasive breast carcinoma and presents a potential risk of over or undertreatment. Finding molecular biomarkers of disease progression could allow for more adequate patient treatment. We aimed to identify potential biomarkers that can predict invasiveness risk. METHODS: In this epithelial cell-based study archival formalin-fixed paraffin-embedded blocks from six patients diagnosed with invasive lesions (pure invasive ductal carcinoma), six with in-situ lesions (pure ductal carcinoma in situ), six with synchronous lesions (invasive ductal carcinoma with an in-situ component) and three non-neoplastic breast epithelium tissues were analyzed by gene expression profiling of 770 genes, using the nCounter® PanCancer Pathways panel of NanoString Technologies. RESULTS: The results showed that in comparison with non-neoplastic tissue the pure ductal carcinoma in situ was one with the most altered gene expression profile. Comparing pure ductal carcinoma in situ and in-situ component six differentially expressed genes were found, three of them (FGF2, GAS1, and SFRP1), play a role in cell invasiveness. Importantly, these genes were also differentially expressed between invasive and noninvasive groups and were negatively regulated in later stages of carcinogenesis. CONCLUSIONS: We propose these three genes (FGF2, GAS1, and SFRP1) as potential biomarkers of ductal carcinoma in situ progression, suggesting that their downregulation may be involved in the transition of stationary to migrating invasive epithelial cells.

Brachial plexus schwannoma mimicking advanced breast carcinoma.

BACKGROUND: Schwannoma is an extremely rare benign tumour of the peripheral nervous system; its association with breast cancer is sporadic, and its association with the brachial plexus is extremely rare. CASE DESCRIPTION: The authors report a case of a patient with breast cancer associated with nodulation in the left supraclavicular fossa, and due to the clinical and radiological features, it was considered metastatic lymph node disease. The patient underwent neoadjuvant chemotherapy, with partial response of the breast. Surgical treatment included resection of the supraclavicular nodule, which was found to be a supraclavicular fossa schwannoma. The correct diagnosis, influences the radiotherapeutic planning. The unusual presentation led to diagnostic confusion in the present case, a fact that changed the breast treatment. CONCLUSION: The best of our knowledge it is the third description of brachial plexus schwannoma associated with breast cancer and the first with a synchronous association. The knowledge of this pathology and its potential to alter treatment justify the reporting of the present case.

Factors associated with return to work in breast cancer survivors treated at the Public Cancer Hospital in Brazil.

PURPOSE: To evaluate the impact of return to work on the quality of life of breast cancer patients and to identify factors related to nonreturn to work. METHODS: An observational, cross-sectional study was performed in breast cancer survivors who had worked before their breast cancer diagnosis. We evaluated factors related to return to work (patient perspective, disease, and work), EORTC quality of life questionnaires (general: EORTC QLQ-C30; and breast cancer-specific: EORTC QLQ-BR23), the Shoulder Pain and Disability Index (SPADI), and the Anxiety and Depression Scale (HADS). Half of the patients underwent a physical therapy examination (shoulder goniometry, hand dynamometry, and limb volume). Univariate and multivariate analysis were performed. RESULTS: We included 304 patients, 163 of whom underwent physiotherapy evaluation. Approximately 54.0% (164) of the patients returned to work after treatment. The women who returned to work presented lower age, higher education levels, higher incomes, and smaller initial tumor size. The women who returned to work had higher scores related to body image and sexual function, lower scores in relation to disability and pain, and lower scores related to anxiety and depression. In the multivariate model to evaluate nonreturn to work, pretreatment variables were age, education level, and clinical staging. Sequelae related to loss of strength increased the risk of nonreturn to work. CONCLUSION: Return to work was influenced by age, education level, previous activity types, axillary treatment, and physical sequelae related to loss of hand strength. Breast cancer treatment decreased the women's work capacity. Return to work improved the patients' quality of life.

Late locoregional complications associated with adjuvant radiotherapy in the treatment of breast cancer: Systematic review and meta-analysis.

This systematic review with meta-analysis addressed late locoregional complications associated with adjuvant radiotherapy (RT) in breast cancer. Among 2120 titles, ten comparative studies in patients undergoing surgery vs surgery and radiotherapy reporting complications were evaluated. RT was associated with an increased risk of capsular contracture and decreased the mobility of the upper limb. A borderline association of lymphedema risk using RT was noted in the random-effects model but was significant in the fixed-effects model.

CAIX is a predictor of pathological complete response and is associated with higher survival in locally advanced breast cancer submitted to neoadjuvant chemotherapy.

BACKGROUND: Locally advanced breast cancer often undergoes neoadjuvant chemotherapy (NAC), which allows in vivo evaluation of the therapeutic response. The determination of the pathological complete response (pCR) is one way to evaluate the response to neoadjuvant chemotherapy. However, the rate of pCR differs significantly between molecular subtypes and the cause is not yet determined. Recently, the metabolic reprogramming of cancer cells and its implications for tumor growth and dissemination has gained increasing prominence and could contribute to a better understanding of NAC. Thus, this study proposed to evaluate the expression of metabolism-related proteins and its association with pCR and survival rates. METHODS: The expression of monocarboxylate transporters 1 and 4 (MCT1 and MCT4, respectively), cluster of differentiation 147 (CD147), glucose transporter-1 (GLUT1) and carbonic anhydrase IX (CAIX) was analyzed in 196 locally advanced breast cancer samples prior to NAC. The results were associated with clinical-pathological characteristics, occurrence of pCR, disease-free survival (DFS), disease-specific survival (DSS) and overall survival (OS). RESULTS: The occurrence of pCR was higher in the group of patients whith tumors expressing GLUT1 and CAIX than in the group without expression (27.8% versus 13.1%, p = 0.030 and 46.2% versus 13.5%, p = 0.007, respectively). Together with regional lymph nodes staging and mitotic staging, CAIX expression was considered an independent predictor of pCR. In addition, CAIX expression was associated with DFS and DSS (p = 0.005 and p = 0.012, respectively). CONCLUSIONS: CAIX expression was a predictor of pCR and was associated with higher DFS and DSS in locally advanced breast cancer patients subjected to NAC.

Unsuspected Stewart-Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart-Treves syndrome.

BACKGROUND: Stewart Treves-Syndrome (STS) was first characterized as angiosarcoma in the homolateral limb of a patient with breast cancer and lymphedema. Now, other conditions represent STS. It's a rare condition. The diagnosis is easier in the presence of single or multiple purple nodules. Even though other dermatological aspects have been reported, no study has grouped its characteristics. AIM: Evaluate the dermatological characteristics of classical STS (c-STS). METHODS AND RESULTS: We report a patient with chronic lymphedema with a history of recurrent erysipelas that rapidly developed multiple papules in the superior limb. It was initially diagnosed as bullous erysipelas, but unsatisfactory evolution led to biopsy, which demonstrated an unsuspected epithelioid angiosarcoma. We have also performed a review of dermatologic aspects of c-STS using PubMed and Lilacs databases. PICTOS methodology and PRISMA flow chart were considered. The main dermatological aspects associated with c-CTS were summarized. Using a systematic evaluation from 109 articles, 29 were selected and 44 patients were described to whom we added one case. The mean time with lymphedema was 10 years. Of the patients analyzed, 97.2% were female; 95.6% were submitted to radical mastectomy; 81.2% presented with multiple lesions, 67.4% of the lesions were reported as nodules or tumors, 53.4% were purple, 33.4% were associated with an ecchymotic halo, and 33.4% were ulcerated lesions. CONCLUSION: When evaluating patients with chronic lymphedema with new dermatological abnormalities, clinical suspicion, or unfavorable evolution, the knowledge of clinical signs is important for diagnosis, and a biopsy must be considered. Papules associated with erythematous-wine color and bluish hematoma aspect must raise clinical suspicion.