Peritoneal sarcomatosis due to undifferentiated pleomorphic sarcoma: A case report and review of the literature

Undifferentiated pleomorphic sarcoma (UPS), which was previously known as malignant fibrous histiocytoma (MFH), rarely presents in the abdomen, and sarcomatosis due to UPS has not yet been reported in the literature. Here, we present a 62-year-old man who had abdominal sarcomatosis due to UPS with a poor prognosis.

Three years follow-up report of two diffuse connective tissue disease cases caused by SARS-CoV-2 infection / 中国热带医学

@#Abstract: To report on two patients with Coronavirus Disease 2019 (COVID-19) combined with diffuse connective tissue disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection followed for nearly 3 years, in order to understand the long-term effects on the patients' immune system. Both patients were male, aged 81-82 years, and were hospitalized with fever on January 29, 2020 and February 10, 2020, respectively. Both were diagnosed with COVID-19 after positive SARS-CoV-2 polymerase chain reaction (PCR) tests. After receiving anti-infection treatment, cough suppressants, ex‐pectorants, and symptomatic supportive treatment, their body temperature returned to normal and two consecutive PCR tests were negative for SARS-CoV-2, and they were discharged from hospital. However, due to recurring fevers and varying degrees of rheumatic disease-related symptoms, both patients were readmitted to the hospital, indicating the presence of positive auto‐ antibodies and organ involvement. One patient recovered from COVID-19 with recurrent fever, joint pain, muscle aches and subcutaneous nodules, and was subsequently diagnosed with undifferentiated connective tissue disease. The other patient developed recurrent fever, mouth ulcers and rash after recovery from COVID-19 and was subsequently diagnosed with anti neutro phil cytoplasm antibody (ANCA)-associated vasculitis (AAV). The patient was treated with glucocorticoids and immunosuppres sive drugs and the symptoms resolved rapidly and subsequent laboratory and imaging examinations showed stable condition. However, due to self-termination of medication, their symptoms quickly relapsed, and further treatment with glucocorticoids and immunosuppressive agents resulted in sustained stability of their condition. The erythrocyte sedimentation rate and hyper‐sensitive C-reactive protein remained within normal limits, and lung CT scans showed stable lesions with partial absorption.SARS-CoV-2 infection may have long-term effects on patients' immune systems, leading to abnormal immune responses and diffuse connective tissue disease. This suggests that regular follow-up observation of immune system-related diseases may be necessary for elderly patients with COVID-19.

Analysis of pregnancy outcomes, disease progression, and risk factors in patients with undifferentiated connective tissue disease / 北京大学学报(医学版)

OBJECTIVE@#To investigate the fetal and maternal outcomes, risk factors of disease progression and adverse pregnancy outcomes (APOs) in patients with undifferentiated connective tissue disease (UCTD).@*METHODS@#This retrospective study described the outcomes of 106 pregnancies in patients with UCTD. The patients were divided into APOs group (n=53) and non-APOs group (n=53). The APOs were defined as miscarriage, premature birth, pre-eclampsia, premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), postpartum hemorrhage (PPH), and stillbirth, small for gestational age infant (SGA), low birth weight infant (LBW) and birth defects. The differences in clinical manifestations, laboratory data and pregnancy outcomes between the two groups were compared. Logistic regression analysis was performed to analyze the risk factors for APOs and the progression of UCTD to definitive CTD.@*RESULTS@#There were 99 (93.39%) live births, 4 (3.77%) stillbirths and 3 (2.83%) miscarriage, 20 (18.86%) preterm delivery, 6 (5.66%) SGA, 17 (16.03%) LBW, 11 (10.37%) pre-eclampsia, 7 (6.60%) cases IUGR, 19 (17.92%) cases PROM, 10 (9.43%) cases PPH. Compared with the patients without APOs, the patients with APOs had a higher positive rate of anti-SSA antibodies (73.58% vs. 54.71%, P=0.036), higher rate of leukopenia (15.09% vs. 3.77%, P=0.046), lower haemoglobin level [109.00 (99.50, 118.00) g/L vs. 124.00 (111.50, 132.00) g/L, P < 0.001].Multivariate Logistic regression analysis showed that leucopenia (OR=0.82, 95%CI: 0.688-0.994) was an independent risk factors for APOs in UCTD (P=0.042). Within a mean follow-up time of 5.00 (3.00, 7.00) years, the rate of disease progression to a definite CTD was 14.15%, including 8 (7.54%) Sjögren's syndrome, 4 (3.77%) systemic lupus erythematosus (SLE), 4 (3.77%) rheumatoid arthritis and 1 (0.94%) mixed connective tissue disease. Multivariate Cox proportional risk regression analysis showed that Raynaud phenomenon (HR=40.157, 95%CI: 3.172-508.326) was an independent risk factor for progression to SLE.@*CONCLUSION@#Leukopenia is an independent risk factor for the development of APOs in patients with UCTD. Raynaud's phenmon is a risk factor for the progression of SLE. Tight disease monitoring and regular follow-up are the key measures to prevent adverse pregnancy outcomes and predict disease progression in UCTD patients with pregnancy.

Thoracic SMARCA4-deficient undifferentiated tumor-pathological diagnosis and combined immune checkpoint inhibitor treatment / 北京大学学报(医学版)

We explored clinicopathological features and treatment strategies for thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT). Thoracic SMARCA4-UT is a new entity recently acknowledged in the 2021 edition of World Health Organization Classification of Thoracic Tumors, and doctors are relatively unfamiliar with its diagnosis, treatment, and prognosis. Taking a case of SMARCA4-UT treated in Peking University First Hospital as an example, this multi-disciplinary discussion covered several hot issues on diagnosing and treating thoracic SMARCA4-UT, including histological features, immu- nohistochemical and molecular phenotype, immune checkpoint inhibitor (ICI) therapy, and pathological assessment of neoadjuvant therapy response. The patient was an older man with a long history of smoking and was admitted due to a rapidly progressing solid tumor in the lower lobe of the right lung. Histologically, tumor cells were epithelioid, undifferentiated, diffusely positive for CD34, and partially positive for SALL4.The expression of BRG1 protein encoded by SMARCA4 gene was lost in all of tumor cells, and next-generation sequencing(NGS)confirmed SMARCA4 gene mutation (c.2196T>G, p.Y732Ter). The pathological diagnosis reached as thoracic SMARCA4-UT, and the preoperative TNM stage was T1N2M0 (ⅢA). Tumor proportion score (TPS) detected by immunohistochemistry of programmed cell death 1-ligand 1 (PD-L1, clone SP263) was 2%. Tumor mutation burden (TMB) detected by NGS of 1 021 genes was 16. 3/Mb. Microsatellite detection showed the tumor was microsatellite stable (MSS). Neo-adjuvant therapy was implemented with the combined regimen of chemotherapy and ICI. Right lower lobectomy was performed through thoracoscopy after the two weeks' neoadjuvant. The pathologic assessment of lung tumor specimens after neoadjuvant therapy revealed a complete pathological response (CPR). The post-neoadjuvant tumor TNM stage was ypT0N0M0. Then, five cycles of adjuvant therapy were completed. Until October 2022, neither tumor recurrence nor metastasis was detected, and minimal residual disease (MRD) detection was negative. At present, it is believed that if BRG1 immunohistochemical staining is negative, regardless of whether SMARCA4 gene mutation is detected, it should be classified as SMARCA4-deficient tumors. SMARCA4-deficient tumors include a variety of carcinomas and sarcomas. The essential criteria for diagnosing SMARCA4-UT includes loss of BRG1 expression, speci-fic histological morphology, and exclude other common thoracic malignant tumors with SMARCA4-deficiency, such as squamous cell carcinoma, adenocarcinoma and large cell carcinoma. SMARCA4-UT is a very aggressive malignant tumor with a poor prognosis. It has almost no targeted therapy mutations, and little response to chemotherapy, but ICI is currently the only effective drug. The successful diagnosis and treatment for this case of SMARCA4-UT should enlighten significance for various kinds of SMARCA4-deficient tumors.

Evaluation of Effectiveness of Doxycycline as Empirical Therapy for Treatment in Acute Undifferentiated Febrile Illnesses in Routine Clinical Practice: A Retrospective, EMR-based, Real-world Study

Background: Tetracyclines, in particular doxycycline, are recommended for the treatment of patients with acute undifferentiated febrile illness (AUFI); however, real-world studies are scarce. Methods: This retrospective, multicenter, observational study reviewed electronic medical records (April 2018 to March 2021) of adult patients (outpatient and inpatient departments [OPD and IPD]) with AUFI, treated with doxycycline monotherapy (doxycycline group) or doxycycline in combination with other antimicrobials (combination therapy group), from 7 tertiary hospitals and clinics in India. Results: Overall, 473 patients were included; 73.8% and 26.2% patients were prescribed doxycycline alone or in combination with other antimicrobials, respectively. Defervescence was achieved in 65.6% and 57.3% patients, respectively at the second (8-14 days) follow-up visit. Clinical cure rate for symptomatic resolution varied between 89.6% and 100% in OPD settings. Time taken from treatment initiation to defervescence was 3.51 ± 3.16 days for the doxycycline group and 3.46 ± 3.07 days for the combination therapy group. Both groups showed improvements in body temperature in OPD settings (84.2% and 84.5%) as well as IPD settings (97.4% and 94.1%). Adverse events in OPD patients in both groups were nausea (7.8% and 8.7%), anorexia (1.6% and 33.0%) and dyspepsia (1.6% and 67.9%). Conclusion: Doxycycline appears to be a promising candidate for treating patients with AUFI due to its demonstrated real-world effectiveness and safety profile.

Lymphoepithelial carcinoma of the maxillary sinus: A case report

Lymphoepithelioma-like carcinoma or lymphoepithelial carcinoma (LEC) of the maxillary sinus is an extremely rare malignancy. It is a rare type of undifferentiated carcinoma, consisting of atypical epithelial cells surrounded by reactive lymphoplasmacytic infiltrate. The paper reports the case of a 76-year-old male patient, with complain of swelling on the hard palate and epistaxis. Biopsy from the hard palate mass showed features consistent with LEC, with contrast-enhanced computed tomography nasopharynx and neck showing soft-tissue lesion in the right maxillary sinus. The patient is currently under treatment with external beam radiotherapy.

Seasonal, Regional and Demographic Trends in Patients with Acute Undifferentiated Fever in Northern India

Introduction : Acute Undifferentiated Fever (AUF) has a myriad of etiologies most common of which are Vector Borne Diseases in a country like India. The etiology and diagnosis of AUF is driven by the regional disease burden, seasonality of infectious diseases, spectrum and severity of disease, availability of diagnostics and access to health care facilities. Methods : A twelve-month prospective study was conducted at our centre to determine the commonly occurring causes of AUF. Monthly incidence of each of the common etiologies was noted and a graph was plotted to understand the seasonal distribution of each disease. Other parameters including age, gender, occupation and regional distribution were also studied. Results : The mean age (±SD) of the population in the present study was 33.16 (±15.88) years. Our study established that Scrub Typhus, Dengue fever and malaria were the most common reasons for AUF. The maximum incidence of AUF was between the months of June to October, peaking in the month of August. Urban population, younger age and males were more affected by AUF. Haryana followed by Punjab and Chandigarh had the highest regional burden of disease. Conclusion : It is imperative that we understand the seasonal and regional trends of AUF so that we can plan the resource allocation. Identifying the population at risk and timely intervention at Community level may help to reduce disease transmission.

A Comparative Study of Clinical Presentation of Scrub Typhus Patients between Pre and Post Monsoon Clusters in a Tertiary Care Hospital in Kolkata

Background : Scrub typhus is an endemic zoonoses, caused by Orientia (formerly Rickettsia) tsutsugamushi and transmitted through the chiggers of the genus Leptotrombidium. Humans are accidental hosts and are infected through the vector mites during a blood meal. Scrub typhus is a reemerging cause of Acute Undifferentiated Fever in India as well as Worldwide. Previously considered a seasonal disease with distribution mainly in the rural areas, cases are being increasingly recognized in the Metropolitan Cities and beyond the Monsoon months. Objective : In this study, we present a comparative clinical data of Scrub Typhus cases presenting during the wet Postmonsoon months (August to November) with those presenting during the dry summer months (April-May) in a Tertiary Care Teaching Hospital in Kolkata

Etiology and clinical spectrumofacuteundifferentiated fever illness in patients in atertiary care hospital Sheri Kashmir institute of medicalscience Soura, Jammu and Kashmir

Background:Current study aimed at s to delineate the etiology and clinical parameters associated with AUFIpresenting toemergency department in atertiary care hospital.Methods:This wasa prospectivehospital based study carried out atemergency medicine, SKIMS hospital, SouraKashmir, IndiaJuly2017 toaugust 2018. Patients with acute undifferentiated fever were enrolled. Descriptive statistics were calculated in terms of mean盨D for continuous variables like age of the patients and duration of fever, Frequency and percentage were used to analyse categorical variables such as causes of fever and gender, while as descriptive analysis was calculated in terms of mean盨D for continuous variables like ageof the patients and duration of fever.Results:Totalnumbers of patients included were 174, among these 112 (64.3%) were males and 62 (35.6%) were females. Most patientswere diagnosedenteric fever (N=59, 33.9%)followed byUTI (N=25, 14.3%) dengue (N=12, 6.8%) and malaria (N=8, 4.5%)while rest of cases were associated with other viral illnesses (N=70, 40.5%)based on clinicalbasisand inconclusive laboratory results.Conclusions:Enteric fever was found to be the most commoncause of acute undifferentiated fever followed by dengue andother viral illnesses, althoughcauses and clinic spectrumof AUFI is varied.

Analysis of imaging characteristics of undifferentiated carcinoma with pancreatic osteoclast-like giant cell / 中华消化杂志

Objective:To investigate the imaging characteristics of computed tomography (CT) and magnetic resonance imaging (MRI) of undifferentiated carcinoma with pancreatic osteoclast-like giant cell (UPC-OGC).Methods:From April 2015 to November 2019, at Zhongshan Hospital, Fudan University, 11 pathologically confirmed UPC-OGC patients who received upper abdominal CT or MRI before surgery and with complete clinical and pathological data were retrospectively included. The imaging characteristics of CT and MRI were analyzed, which included lesion location, number, shape, size, boundary, plain scan and enhancement features, adjacent tissue invasion and metastasis. Independent sample t test was used for statistical analysis. Results:The tumor lesions of 11 patients with UPC-OGC were all single, and the maximum diameter of lesion was (4.84±2.96) cm (ranged from 2.00 to 12.80 cm). The lesions of 7 patients with UPC-OGC were located in the head of pancreas, 2 located in the body of pancreas, 1 located in the tail of pancreas and 1 located in the junction of body and tail of pancreas. The lesion shapes of 3 patients with UPC-OGC were round, and the lesion shapes of 8 patients were oval with lobulation. The lesion boundaries of 8 patients with UPC-OGC were clear and the lesion boundaries of 3 patients were unclear. Seven patients with UPC-OGC were examined by plain and enhanced CT scan. Plain CT scan showed that the density of solid area of the tumor was similar to that of normal pancreatic parenchyma ((37.14±6.10) HU vs. (43.14±4.55) HU), and the difference was not significant ( t=-2.85, P=0.097). Contrast-enhanced CT scan in arterial phase showed that the degree of enhancement in solid area of the tumor was weaker than that of normal pancreatic parenchyma ((67.29±12.79) HU vs. (90.43±9.81) HU), and the difference was statistical significant ( t=-4.10, P=0.004), while contrast-enhanced CT scan showed that in venous phase the solid area of the tumor continued to strengthen and the degree of enhancement was similar to that of normal pancreatic parenchyma ((84.71±15.30) HU vs. (79.57±10.73) HU), and the difference was not significant ( t=0.38, P=0.535). Both CT and MRI enhanced scans showed uneven enhancement of the lesions, the degree of enhancement of solid component in arterial phase was slightly weaker than that of normal pancreatic parenchyma and the marginal and internal separation were progressively enhanced, and the degree of enhancement in the venous phase and balanced phase was slightly higher than that of the normal pancreatic parenchyma or similar to that of the normal pancreas. Conclusions:The imaging of CT and MRI of UPC-OGC have certain characteristics, which are helpful for the diagnosis and identification of the disease.

Standardized diagnosis and treatment of undifferentiated connective tissue disease and mixed connective tissue disease / 中华内科杂志

Undifferentiated connective tissue disease (CTD) usually refers to patients who are presented with certain symptoms and signs related to CTD, and positive serological evidence of autoimmune diseases but don′t fulfill any of the classification criteria for a certain CTD. Mixed CTD refers to patients who are presented with one or more clinical manifestations such as hand swelling, synovitis, myositis, Raynaud′s phenomenon, and acrosclerosis. Patients with mixed CTD always have high-titer anti-nuclear antibodies (ANA) of speckled pattern and high-titer anti-U 1 ribonuclear protein (RNP) antibody in serum, while with negative anti-Sm antibody. The update of diagnosis and treatment of undifferentiated CTD and mixed CTD lags behind other established CTD. There is a lack of evidence from randomized controlled trials or guidelines/recommendations on the treatment of undifferentiated CTD or mixed CTD. At present, the conventional therapy is mainly adopted according to the specific clinical manifestations of the disease. The standardized diagnosis and treatment of undifferentiated CTD and mixed CTD were drafted by the Chinese Rheumatology Association based on the previous guidelines and the progress of available evidence, so as to improve the management of these patients in China.

Carbon ion radiotherapy for unresectable primary undifferentiated pleomorphic sarcoma of the 11th thoracic spine: a case report / Journal of Rural Medicine

Objective: Primary undifferentiated pleomorphic sarcoma (UPS) of the bone is rare. However, the common sites are the knee and proximal femur and humerus, while spinal involvement is rare. We report a case of primary UPS of the 11th thoracic vertebra, where corpectomy would have been difficult and extensive, treated with carbon ion radiotherapy.Case report: A 76-year-old man presented with an osteolytic tumor of the 11th thoracic vertebra on plain computed tomography (CT). The spinal cord was compressed and displaced posteriorly by the tumor on magnetic resonance imaging (MRI), and extraosseous extension was observed. An incisional biopsy was performed, and primary UPS of the 11th thoracic vertebra was diagnosed pathologically. Total en bloc spondylectomy was considered to be challenging because of the extraosseous extension and the patient’s age; thus, carbon ion radiotherapy (70.4 GyE / 32 fraction) was performed. Denosumab (120 mg) was administered subcutaneously every four weeks. No adjuvant chemotherapy was administered. Four years post-treatment, imaging revealed a compression fracture of the 11th thoracic vertebra, but there was no recurrence.Conclusion: Despite a poor prognosis and an aggressive course of UPS of the spine, the tumor continues to be controlled without local recurrence four years after carbon ion radiotherapy.

A Long-Term Survival Case after Successful Five-Fold Surgical Intervention for Recurrent Undifferentiated Pleomorphic Sarcoma in the Left Atrium / 日本心臓血管外科学会雑誌

Primary cardiac undifferentiated pleomorphic sarcoma is rare and is associated with very poor survival. We report a case of a 45-year-old female who presented with dyspnea on effort, in whom an echocardiographic exam showed a large mass in the left atrium and the tumor resection was performed. The pathological diagnosis of the resected tumor was undifferentiated pleomorphic sarcoma which subsequently recurred. The patient needed four re-surgeries, and chemotherapy with Pazopanib was performed. A long-term survival of 5 years after the initial surgery was achieved.

Research advances on undifferentiated connective tissue disease in pregnant women / 中华全科医师杂志

Undifferentiated connective tissue disease (UCTD) has clinical and laboratory characteristics of connective tissue disorders, but it can not meet the diagnostic criteria of any specific disease. Pregnancy complicated with UCTD is one of the most common rheumatic diseases in women of childbearing age. UCTD will increase the risk of adverse events of pregnancy, such as spontaneous abortion, preeclampsia, fetal growth restriction, small for gestational age,recurrent implantation failure and so on. Pregnancy may lead to recurrence, aggravation and even serious complications of UCTD; multidisciplinary monitoring and appropriate treatment can improve the pregnancy outcome.

Sociological interpretation in general practice clinical thinking / 中华全科医师杂志

There are a large number of atypical symptoms and undifferentiated diseases in general practice, in which sociological factors are deeply involved. So to incorporate social factors into clinical thinking and to interpret disease from the perspective of the patient′s social life will enrich the multidisciplinary medical model and holistic care in general practice. This article shares author′s clinical experience through introducing typical cases, in which the patient′s social life and behavior characteristics played certain roles in disease development. The article also explores a new way from the sociological perspectives to interpret the complex symptoms that are difficult to be explained clinically by biomedicine or psychology.

Comparison of long-term outcomes between endoscopic submucosal dissection and surgical resection for early gastric cancer with undifferentiated histology / 中华胃肠外科杂志

Objective: Endoscopic submucosal dissection (ESD) of undifferentiated early gastric cancer (UD-EGC) remains controversial due to high positive rate of horizontal and vertical resection margins and the risk of lymph node metastasis. The purpose of this study was to compare long-term outcomes of patients with UD-EGC undergoing ESD versus surgery. Methods: This study was a retrospective cohort study. Inclusion criteria: (1) patients with early gastric cancer undergoing ESD or surgical resection; (2) histological types included poorly differentiated adenocarcinoma, poorly differentiated adenocarcima with signet ring cell carcinoma, and signet ring cell carcinoma; (3) no lymph node metastasis or distant metastasis was confirmed by preoperative CT and endoscopic ultrasonography. Exclusion criteria: (1) previous surgical treatment for gastric cancer; (2) synchronous tumors; (3) death with unknown cause; (4) additional surgical treatment was performed within 1 month after ESD. According to the above criteria, clinical data of patients with UD-EGC who received ESD or surgery treatment in Cancer Hospital of Chinese Academy of Medical Sciences from January 2009 to December 2016 were collected. After further comparing the clinical outcomes between the two groups by 1:1 propensity score matching, 61 patients in the ESD group and 61 patients in the surgery group were finally included in this study. The disease-free and overall survivals were analyzed by Kaplan-Meier method. Results: All patients in the two groups completed operations successfully. In the ESD group, the median operation time was 46.3 (26.5, 102.3) minutes, 61 cases (100%) were en-bloc resection, and 57 cases (93.4%) were complete resection. Positive margin was found in 4 (6.6%) patients, of whom 2 were positive in horizontal margin and 2 were positive both in horizontal and vertical margins. In the surgery group, only 1 case had positive horizontal margin and no positive vertical margin was observed. There was no significant difference in the positive rate of margin between the two groups (P>0.05). Median follow-up time was 59.8 (3.0, 131.5) months. The follow-up rate of ESD group and surgery group was 82.0% (50/61) and 95.1% (58/61), respectively. The 5-year disease-free survival rate in ESD group and surgery group was 98.2% and 96.7%, respectively (P=0.641), and the 5-year overall survival rate was 98.2% and 96.6%, respectively (P=0.680). In the ESD group, 1 patient (1.6%) had lymph node recurrence, without local recurrence or distant metastasis. In the surgery group, 1 case (1.6%) had anastomotic recurrence and 1 (1.6%) had distant metastasis. Conclusion: ESD has a sinilar long-term efficacy to surgery in the treatment of UD-EGC.

Enfermedad indiferenciada del tejido conectivo con progresión a esclerosis sistémica / Undifferentiated disease of the connective tissue with progression to systemic sclerosis

La enfermedad indiferenciada del tejido conectivo es una condición de etiología desconocida que comparte características clínicas, patológicas y de laboratorio de varias colagenosis, sin cumplir los criterios del Colegio Americano de Reumatología para el diagnóstico de una enfermedad reumática específica y muchos pacientes evolucionan a condiciones definidas a lo largo del tiempo tales como Lupus, Esclerosis sistémica progresiva, Enfermedad de Sjögren entre otros. Antecedentes: Linfoma Hodgkin diagnosticado desde 2012 para lo cual recibió múltiples esquemas de quimioterapia. Las muestras de ganglio y médula ósea se habían enviado al laboratorio de Inmunopatologia de la Universidad de Stanford y allí no se apreciaron hallazgos compatibles con enfermedad linfoproliferativa. Enfermedad actual: Mujer de 27 años de edad con cuadro clínico de 1 mes de evolución, caracterizado por edema blando en miembros inferiores acompañado de edema palpebral matutino; concomitantemente presenta aumento de temperatura intermitente sin patrón especifico y dolor osteomuscular generalizado con limitación para la deambulación. Se ingresa. Al examen físico, regulares condiciones clínicas. En la piel se aprecia engrosamiento cutáneo importante. Se realizó biopsia cutánea y los hallazgos fueron compatibles con Esclerosis Sistémica(AU)

Undifferentiated connective tissue disease is a condition of unknown etiology that shares clinical, pathological and laboratory characteristics of several collagenopathies that do not meet the criteria of the American College of Rheumatology for the diagnosis of a specific disease; a large number of patients evolve to conditions defined over time such as Lupus, Systemic Sclerosis, Sjogren's Disease, among others. Past history: Hodgkin lymphoma was diagnosed since 2012 for which she received multiple chemotherapy schemes. A gland biopsy was sent to the Stanford University, as well as a bone marrow sample, and lymphoma was discarded. Present history: this 27-year-old female consulted for edema in lower limbs present during one month, accompanied by eyelid edema in the mornings; also fever without a specific pattern, myalgias and arthralgias. On physical examination, the skin was thickened and limb edema was present. A skin biopsy was performed, and the findings were consistent with Systemic Sclerosis. The patient is receiving cyclophosphamide and Azathioprine and leading her normal life(AU)

Supradesnivel del segmento ST como forma de presentación atípica de un tumor cardíaco: rol diagnóstico de la multimodalidad de imagen / ST segment elevation as an atypical presentation of a cardiac tumor: diagnostic role of a multimodal imaging approach / Elevação do segmento ST como apresentação de tumor cardíaco: papel diagnóstico da multimodalidade de imagem

Los tumores cardíacos malignos son neoplasias poco frecuentes que pueden presentarse de diversas formas, lo que dificulta su diagnóstico. La ecocardiografía y la resonancia magnética cardíaca son técnicas fundamentales para el diagnóstico, la caracterización y la evaluación de su extensión tumoral. La identificación de la línea tumoral es esencial al iniciar un tratamiento oncológico dirigido. Si bien el "estándar de oro" para este fin es el estudio anatomopatológico (obtenido por biopsia o resección quirúrgica), en los casos en que esto no es posible, la resonancia magnética cardíaca es la técnica no invasiva que proporciona un mejor abordaje diagnóstico. El tratamiento de elección es la resección quirúrgica y el pronóstico, en general, es malo. Presentamos el caso de una paciente con un tumor cardíaco de presentación clínica atípica, en la que un abordaje imagenológico multimodal aporta información clave y complementaria para el diagnóstico y la caracterización tisular.

Malignant cardiac tumors are rare neoplasms that can present in various forms, making their diagnosis difficult. Echocardiography and cardiac magnetic resonance imaging are fundamental techniques for the diagnosis, characterization, and evaluation of tumor extension. Identification of the tumor line is essential when initiating targeted cancer therapy. Although the "gold standard" for this purpose is the pathological study (obtained by biopsy or surgical resection), in cases where this is not possible, cardiac resonance is the non-invasive technique that provides a better diagnostic approach. The treatment of choice is surgical resection and the prognosis is generally poor. We present the case of a patient with an atypical clinical presentation, in which a multimodal approach provides key and complementary information for tumor diagnosis and tissue characterization.

Os tumores cardíacos malignos são neoplasias raras que podem se apresentar de várias formas, dificultando seu diagnóstico. A ecocardiografia e a ressonância magnética cardíaca são técnicas fundamentais para o diagnóstico, caracterização e avaliação da extensão tumoral. A identificação da linha do tumor é essencial ao iniciar a terapia direcionada do câncer. Embora o "padrão ouro" para esse fim seja o estudo patológico (obtido por biópsia ou ressecção cirúrgica), nos casos em que isso não seja possível, a ressonância cardíaca é a técnica não invasiva que proporciona melhor abordagem diagnóstica. O tratamento de escolha é a ressecção cirúrgica e o pronóstico geralmente é ruim. Apresentamos o caso de uma paciente com apresentação clínica atípica, em que a abordagem multimodal fornece informações essenciais e complementares para o diagnóstico do tumor e caracterização do tecido.

Undifferentiated embryonal sarcoma of liver in a 9-year-old: case report

The embryonal sarcoma of the liver is an extremely malignant, aggressive and infrequent condition of the liver, predominantly affecting children in the age groups of four to ten. The disease manifests with fever, abdominal pain or mass, nausea and laboratory findings suggestive of liver cell injury. The diagnosis can be made using tumor markers, imaging, immunohistochemistry and histopathology. The multimodal management protocol focuses the use of surgical resection, chemotherapy and radiation to significantly improved survival rates and quality of life in these patients