A rare case of epithelioid angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis. We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall's integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery. To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm. Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.

Anti-SSA Ro52 and anti-Ro60 autoantibodies: association with clinical phenotypes.

OBJECTIVES: Anti-SSA autoantibodies can be differentiated according to their antigenic target proteins as anti-Ro60 (60 kDa) or anti-Ro52 (52 kDa). Anti-SSA(Ro60) antibodies are clearly associated with connective tissue diseases (CTD), but the clinical significance of anti-SSA(Ro52) antibodies remains unclear. The aim of the present study was to analyse the disease phenotype of patients with anti-Ro52 and/or anti-Ro60 antibodies. METHODS: A multicentre, cross-sectional study was carried out of positive anti-Ro52 and/or Ro60 antibodies patients followed at 10 Rheumatology centres from January 2018 until December 2021. Patients were categorised into 3 groups: group 1 (Ro52+/Ro60-); group 2 (Ro52-/Ro60+); group 3 (Ro52+/Ro60+). Antinuclear antibodies were evaluated by indirect immunofluorescence assay and further screened for anti-extractable nuclear antigen (ENA) antibodies. Demographicsand clinical data were compared between the 3 groups, by patients' medical chart review. Univariate analysis was performed and subsequently logistic regression was used to identify intergroup differences and calculate the odds ratio with a 95% confidence interval (95% CI). RESULTS: We included 776 patients [female: 83.1%; median age: 59 (46-71) years]. Groups 1, 2, and 3 comprised 31.1%, 32.6%, and 36.3% of the patients, respectively. Anti-Ro52 antibody alone was more frequently associated with non-rheumatic diseases, older age, and men (p<0.05). Among patients with CTD, the diagnosis of systemic lupus erythematosus is 3 and 2 times more prevalent in groups 2 and 3, respectively, than in group 1 [OR 2.8 (95% CI 1.60, 4.97), p<0.001; OR 2.2 (95% CI 1.28, 3.86), p<0.01]. In group 2, the diagnosis of undifferentiated CTD is more frequent than in the other groups. Group 1 was more frequently associated with inflammatory myositis than group 2 [OR 0.09 (95% CI 0.01, 0.33), p<0.001] or group 3 [OR 0.08 (95% CI 0.01, 0.29), p<0.001]. Group 1 was also more frequently associated with arthritis (p<0.01), interstitial lung disease (p<0.01), and myositis (p<0.01). CONCLUSIONS: Anti-Ro52+ antibody alone is frequently found in patients with non-rheumatic diseases. In addition, anti-Ro52+ antibody is also prevalent in patients with CTD and associated with clinical phenotypes that are different from anti-Ro60+ antibody.

The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register.

AIMS: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort. METHODS: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022. RESULTS: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125). CONCLUSIONS: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.

Predictors of cardiac involvement in idiopathic inflammatory myopathies.

Objectives: Idiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. This work aimed to find predictors of cardiac involvement in IIM. Methods: Multicenter, open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without cardiac involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities, and/or premature coronary artery disease were considered. Results: 230 patients were included, 163 (70.9%) of whom were females. Thirteen patients (5.7%) had cardiac involvement. Compared with IIM patients without cardiac involvement, these patients had a lower bilateral manual muscle testing score (MMT) at the peak of muscle weakness [108.0 ± 55.0 vs 147.5 ± 22.0, p=0.008] and more frequently had oesophageal [6/12 (50.0%) vs 33/207 (15.9%), p=0.009] and lung [10/13 (76.9%) vs 68/216 (31.5%), p=0.001] involvements. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/11 (27.3%) vs 9/174 (5.2%), p=0.026]. In the multivariate analysis, positivity for anti-SRP antibodies (OR 104.3, 95% CI: 2.5-4277.8, p=0.014) was a predictor of cardiac involvement, regardless of sex, ethnicity, age at diagnosis, and lung involvement. Sensitivity analysis confirmed these results. Conclusion: Anti-SRP antibodies were predictors of cardiac involvement in our cohort of IIM patients, irrespective of demographical characteristics and lung involvement. We suggest considering frequent screening for heart involvement in anti-SRP-positive IIM patients.

Not a neuroendocrine tumor: A case of hepatocellular carcinoma in ectopic liver tissue in the pancreas.

Hepatocellular carcinoma (HCC) accounts for most of the hepatic neoplasms and can also occur in ectopic liver tissue. We present a case of a 55-year-old male complaining of weight loss. The imaging studies reported a 2.9 cm nodule in the pancreatic body, with a neuroendocrine tumor diagnosis by cytology. A corpo-caudal pancreatectomy was performed. Pathology showed a well-differentiated HCC developed in ectopic liver tissue with free margins and no lymph node metastases. HCC presenting in ectopic liver tissue is rare. In this case, the preoperative study did not establish the diagnosis, warranting the need for suspicion of this neoplasm.

Perioperative management of disease-modifying antirheumatic drugs and other immunomodulators.

OBJECTIVES: We aim to summarize the relevant evidence and provide guidance for perioperative management of disease-modifying antirheumatic drugs (DMARDs) and other immunomodulators used in the treatment of the various inflammatory rheumatic diseases in patients submitted to elective surgery. METHODS: This is a review article directed towards clinical practice, based on recent literature available in PubMed database, as well as guidelines published by Rheumatology Societies. RESULTS: Treatment with conventional DMARDs (methotrexate, hydroxychloroquine, sulfasalazine and leflunomide) can be continued perioperatively; targeted synthetic DMARDs should be suspended at least 3 to 7 days before surgery, depending on the drug, and restarted 3-5 days after the procedure, while biologic DMARDs should be withheld a dosing cycle prior to surgery and resumed at least 14 days after the procedure, with evidence of complete wound healing. In the case of Systemic Lupus Erythematosus (SLE), one should consider the severity of the condition to make the decision about discontinuing immunomodulators (mycophenolate mofetil, azathioprine, cyclosporine, or tacrolimus) as these should be continued in severe SLE because of the increased risk of life-threatening flares. The usual dose of glucocorticoids should be continued perioperatively; however, elective procedures with high-risk surgical site infection should be postponed in patients under ≥20 mg/day prednisone or equivalent until the inflammatory process is controlled with the minimum effective dose. CONCLUSIONS: The perioperative management of patients with rheumatic disease under DMARDs or other immunomodulators is challenging but vital for achieving the best outcome possible. A multidisciplinary approach agreed upon by the anesthesiologist, surgeon and rheumatologist is the best strategy for success.

Predictive factors of non-sentinel lymph node disease in breast cancer patients with positive sentinel lymph node. / Factores predictivos de metástasis en ganglios no centinela en el cáncer de mama con ganglio centinela positivo.

INTRODUCTION: Management of positive sentinel lymph node biopsy (SLNB) in breast cancer remains a matter of debate. Our aim was to evaluate the incidence and identify predictive factors of non-sentinel lymph node metastases. METHODS: Retrospective review of all cN0 breast cancer patients treated between January 2013 and December 2017, with positive SLNB that were submitted to ALND. RESULTS: Of the 328 patients included, the majority of tumors were cT1 or cT2, with lymphovascular invasion in 58.4% of cases. The mean isolated nodes in SLNB was 2.7, with a mean of 1.6 positive nodes, 60.7% with extracapsular extension. Regarding ALND, a mean of 13.9 nodes were isolated, with a mean of 2.1 positive nodes. There was no residual disease in the ALND in 50.9% of patients, with 18.9% having ≥ four positive nodes. In the multivariate analysis, lymphovascular invasion, extracapsular extension in SLN, largest SLN metastases size (>10 mm) and ratio of positive SNL (> 50%) were independent predictors of non-sentinel lymph node metastases. These four factors were used to build a non-pondered score to predict the probability of a positive ALND after a positive SLNB. The AUC of the model was 0.69 and 81% of patients with score = 0 and 65.6% with score = 1 had no additional disease in ALND. CONCLUSION: The absence of non-sentinel lymph node metastases in the majority of patients with 1-2 positive SLN with low risk score questions the need of ALND in this population. The identified predictive factors may help select patients in which ALND can be omitted.

Impact of the 8th edition of the AJCC TNM classification on gastric cancer prognosis-study of a western cohort.

INTRODUCTION: The 8th edition of the American Joint Committee on Cancer (AJCC) TNM classification for gastric cancer introduced changes, mainly in stage III, with the incorporation of the pN3 sub-classification in the final staging group. The goal was to compare the 7th and 8th editions to evaluate the discriminative capacity of the new edition. METHODS: This study was a retrospective review of patients with gastric cancer treated with surgery in 2013 and 2014. RESULTS: We analysed 310 patients, with a median age of 66 years and out of which 55.5% were male. The most commonly performed surgery was subtotal gastrectomy (n = 158; 51%), with a median of 30 lymph nodes removed. With a median follow-up of 39.5 months, the 1- and 3-year overall survival (OS) was 82% and 59%, respectively. In stage III (n = 115), there was stage migration in 40 cases (34.8%), with upstage in 11 cases and downstage in 29 cases. In this group, there was a statistically significant difference in OS between N3a and N3b patients (p = 0.002), as well as a statistically significant difference in OS between stages IIIA, IIIB and IIIC when the 8th edition was applied (p = 0.001), which was not verified with the 7th edition (p = 0.057). In multivariate analysis, both extracapsular extension and N classification from TNM were independent prognostic factors (p = 0.033 and p = 0.024, respectively). CONCLUSION: The 8th edition of the AJCC TNM classification allows for a better prognostic refinement, namely in the new stage III groups after the stratification of lymph node disease in N3a and N3b. Factors that evaluate the biological behaviour of the disease remain excluded from this edition, such as extracapsular extension, which had a prognostic impact in our series.

Physical abuse against elderly persons in institutional settings.

INTRODUCTION: People over 65 years old are expected to be an increasing group exposed to abuse. Despite the well-studied intra-familial abuse, institutional abuse still lacks a proper understanding about its determinants and characteristics. AIM: The general objective of this study is to provide a better knowledge about physical abuse against elderly people in institutional settings, in order to contribute to a timely detection, correct forensic diagnosis and prevention of these cases. METHODS: A retrospective study was conducted through the analysis of forensic medical exams performed in the North Forensic Medical Services of Portugal, between 2004 and 2013, to elderly persons allegedly victims of physical abuse in an institutional setting by a caregiver (n = 59). RESULTS: All the alleged cases occurred in nursing homes and in most of them (93.2%) the charges were against the institution and not focussing on a particular individual. The alleged victims were mainly female (79.7%), 75 years or older (75.9%), presenting a severe disability (55.9%) and 47.2% being unable to communicate. No injuries or post-traumatic pain were found in 55.9% of the cases to support the charge of physical abuse. Only in 6.8% of the cases were the forensic medical findings suggestive of physical abuse and, although this was not the object of the examination, 69.1% were considered suggestive or highly suggestive of neglect. A statistically significant association was found between the alleged victim's degree of disability and the occurrence of neglect (p = 0.003). CONCLUSION: The sample's size seems to be underestimated, probably due to lack of detection and/or reporting. The condition of these persons, mainly related with their inability to perceive abusive behaviours and/or to disclose them (mostly by physical and/or mental disability), as well as their reluctance to press charges due to fear of reprisal, affects significantly the detection and diagnosis of physical abuse, particularly in whom injuries are not obvious. In anticipation to the rapid ageing of the population, it is urgent to analyse and understand this emerging issue so that social policies and regulation may be developed, in an effort to protect the elderly, as well as to make improvements in the professionals' skills.