Visceral Involvement Research Articles

Pembrolizumab Plus Gemcitabine in the Subset of Triple-Negative Advanced Breast Cancer Patients in the GEICAM/2015-04 (PANGEA-Breast) Study.

Simple SummaryAdvanced triple-negative breast cancer (TNBC) remains an extremely challenging situation in oncology, where new therapeutical strategies are desperately needed. Immunotherapy has opened a window of opportunity in this setting, with some promising results with chemo-immunotherapeutic schedules based on anti-PD1/PD-L1 agents, especially in the PD-L1-positive cohort. However, there is certainly room for improvement; thus, new schemes that could potentially boost synergism against cancer cells must be explored. This work analyzes the effects of combination therapy with anti-PD1 (pembrolizumab) and gemcitabine, specifically in the TNBC cohort of the PANGEA-Breast trial. Patients included in this study were not selected by PD-L1 status, and most of them were also heavily pretreated, which could explain the modest objective response rate of 15% achieved. Complementary translational subanalyses, focused on T infiltrating lymphocytes, myeloid-derived suppressor cells, and PD-L1 were accomplished.The PANGEA-Breast trial evaluated a new chemo-immunotherapeutic combination that would synergistically induce long-term clinical benefit in HER2-negative advanced breast cancer patients. Treatment consisted of 21-day cycles of 200 mg of pembrolizumab (day 1) plus gemcitabine (days 1 and 8). The primary objective was the objective response rate (ORR). The tumor infiltrating lymphocytes (TILs) density and PD-L1 expression in tumor, and the myeloid-derived suppressor cells (MDSCs) level in peripheral blood, were analyzed to explore associations with treatment efficacy. Considering a two-stage Simon’s design, the study recruitment was stopped after its first stage as statistical assumptions were not met. A subset of 21 triple-negative breast cancer (TNBC) patients was enrolled. Their median age was 49 years; 15 patients had visceral involvement, and 16 had ≤3 metastatic locations. Treatment discontinuation due to progressive disease (PD) was reported in 16 patients. ORR was 15% (95% CI 3.2–37.9). Four patients were on treatment >6 months before PD. Grade ≥3 treatment-related adverse events were observed in 8 patients, where neutropenia was the most common. No association was found between TILs density, PD-L1 expression or MDSCs levels and treatment efficacy. ORR in TNBC patients also did not meet the assumptions, but 20% were on treatment >6 months.

Clinical and Laboratory Profile of People Living with HIV/AIDS with Oral Kaposi Sarcoma.

The aim of this article was to evaluate the clinical and laboratory profile of people with oral Kaposi's sarcoma (KS) associated with AIDS (KS-AIDS), followed-up at a public university hospital in Salvador, Bahia, Brazil, in the past 10 years. We identified patients diagnosed with KS-AIDS, presenting oral manifestation from January 2007 to December 2017. We searched, in the hospital information systems, the patient demographics, diagnostic data, treatment, image studies, and oral photographic records. Of the 39 cases of KS-AIDS identified at the institution, 14 (22.8%) presented oral lesions. There was a predominance of black men, with a mean age of 32.5 years. Most cases (85.1%) manifested signs of KS simultaneously with the diagnosis of HIV infection, with extremely low initial CD4 T cell counts (average of 52.6 cells/mm2) and visceral involvement (64.3%). The palate (32.1%) and gingiva (21.4%) were the most affected oral sites. Histologically, the tumors exhibited proliferation of spindle cells between vascular clefts and extravasated erythrocytes. Oral KS-AIDS was frequent in young black adult men, with severe immunosuppression and high viral load counting, mostly with lesions manifested in the same period of diagnosis of infection by the HIV.

Plasma Neurofilament Light (NfL) in Patients Affected by Niemann-Pick Type C Disease (NPCD).

(1) Background: Niemann–Pick type C disease (NPCD) is an autosomal recessive lysosomal storage disorder caused by mutations in the NPC1 or NPC2 genes. The clinical presentation is characterized by visceral and neurological involvement. Apart from a small group of patients presenting a severe perinatal form, all patients develop progressive and fatal neurological disease with an extremely variable age of onset. Different biomarkers have been identified; however, they poorly correlate with neurological disease. In this study we assessed the possible role of plasma NfL as a neurological disease-associated biomarker in NPCD. (2) Methods: Plasma NfL levels were measured in 75 healthy controls and 26 patients affected by NPCD (24 NPC1 and 2 NPC2; 39 samples). (3) Results: Plasma NfL levels in healthy controls correlated with age and were significantly lower in pediatric patients as compared to adult subjects (p = 0.0017). In both pediatric and adult NPCD patients, the plasma levels of NfL were significantly higher than in age-matched controls (p < 0.0001). Most importantly, plasma NfL levels in NPCD patients with neurological involvement were significantly higher than the levels found in patients free of neurological signs at the time of sampling, both in the pediatric and the adult group (p = 0.0076; p = 0.0032, respectively). Furthermore, in adults the NfL levels in non-neurological patients were comparable with those found in age-matched controls. No correlations between plasma NfL levels and NPCD patient age at sampling or plasma levels of cholestan 3β-5α-6β-triol were found. (4) Conclusions: These data suggest a promising role of plasma NfL as a possible neurological disease-associated biomarker in NPCD.

P15 A Juvenile idiopathic arthritis mimic

P15 A Juvenile idiopathic arthritis mimic

Fulvestrant-Palbociclib vs Letrozole-Palbociclib as Initial Therapy for Endocrine-Sensitive, Hormone Receptor–Positive, ERBB2-Negative Advanced Breast Cancer

The cyclin-dependent kinase 4 and 6 inhibitor palbociclib in combination with letrozole has become a standard first-line treatment for patients with endocrine-sensitive, hormone receptor-positive, ERBB2-negative advanced breast cancer. Meanwhile, the antiestrogen fulvestrant was shown to be superior to anastrozole in the absence of cyclin-dependent kinase 4 and 6 inhibition for this patient population. To assess whether fulvestrant is superior to letrozole when combined with palbociclib in the first-line scenario. In this international, randomized, open-label, phase 2 clinical study conducted from July 30, 2015, to January 8, 2018, patients with hormone receptor-positive, ERBB2-negative advanced breast cancer with no prior therapy in the metastatic setting and endocrine-sensitive criteria were recruited from 47 centers in 7 countries. Data were analyzed from February 11 to May 15, 2020. Patients were randomly assigned (1:1 ratio) to receive palbociclib with either fulvestrant or letrozole. Stratification factors were type of disease presentation (de novo vs recurrent) and the presence of visceral involvement (yes vs no). The primary end point was investigator-assessed progression-free survival determined by Response Evaluation Criteria in Solid Tumors, version 1.1. A total of 486 women (median age, 63 years [range, 25-90 years]; 3 Asian women [0.6%]; 4 Black women [0.8%]; 461 White women [94.9%]; 18 women of unknown race [3.7%]) were randomized (243 to fulvestrant-palbociclib and 243 to letrozole-palbociclib). Median investigator-assessed progression-free survival was 27.9 months (95% CI, 24.2-33.1 months) in the fulvestrant-palbociclib group vs 32.8 months (95% CI, 25.8-35.9 months) in the letrozole-palbociclib group (hazard ratio, 1.13; 95% CI, 0.89-1.45; P = .32). This result was consistent across the stratification factors. No significant differences were observed in objective response rate (46.5% vs 50.2%) and 3-year overall survival rate (79.4% vs 77.1%) for fulvestrant-palbociclib and letrozole-palbociclib, respectively. Grade 3-4 adverse events were comparable among treatment groups, and no new safety signals were identified. No treatment-related deaths were reported. Although fulvestrant-palbociclib demonstrated significant antitumor activity, this randomized clinical trial failed to identify an improvement in progression-free survival with this regimen over letrozole-palbociclib in patients with endocrine-sensitive, hormone receptor-positive, ERBB2-negative advanced breast cancer. ClinicalTrials.gov Identifier: NCT02491983.

Clinical analysis of 60 children with anaplastic large cell lymphoma in a single center

Objective: To summarize the clinical features, treatment outcome and prognostic factors of childhood anaplastic large cell lymphoma (ALCL). Methods: Clinical data of 60 newly diagnosed and biopsy-proven ALCL pediatric patients (≤18 years of age) at Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine from January 2010 to December 2018 were collected. All patients were treated with the Chinese Children Cancer Group-B cell-non-Hodgkin Lymphoma 2010 (CCCG-BNHL-2010) regimen. Overall survival (OS), event free survival (EFS) and progression free survival (PFS) rates were calculated by the Kaplan-Meier method. Univariate analysis was performed with Log-Rank test to find factors of poor prognosis. Results: Among 60 ALCL patients included in the current study, 39 were males and 21 females, the age of onset was 7.9 (1.2-16.7) years. Among all cases, 43 (72%) had B syndrome (any of the following: fever, drenching, weight loss). Forty-nine (82%) cases had lactate dehydrogenase (LDH) levels<2 times upper limit of normal (ULN) and 11 (18%) cases had LDH levels 2-<4 times ULN. The distribution of stages was stage Ⅰ,Ⅱ,Ⅲ, and Ⅳ in 2% (1/60), 5% (3/60), 92% (55/60), and 2% (1/60) of patients, respectively. Of 58 cases who had results of anaplastic lymphoma kinase (ALK) immunohistochemical staining, 53 (91%, 53/58) cases were positive. Visceral involvement was observed in 12 patients (20%). The 4-year OS and EFS rates were (88±4)% and (76±6)% for the entire group, respectively. Univariate analysis for gender, B symptoms, LDH level, ALK expression, clinical stage and visceral involvement showed that only LDH level correlated with an inferior OS rate (χ²=6.571, P=0.010) while not correlated with EFS rate. No independent risk factor for disease progression or recurrence was found by Logistic regression. Up to the last follow-up, 44 cases were continuously at complete remission state, and their follow-up time was 50 (13-119) months. Of 13 (23%) cases experienced disease progression or relapse, 3 cases abandoned treatment, 2 cases progressed to death, 8 cases received second line or salvage treatment (6 survived at last follow-up). For post progression or relapse cases, the 2-year OS and PFS rates were (60±16)% and (16±14)%, respectively. The treatment related death occurred in 3 cases (5%) and all of them were due to severe infection during the chemotherapy. Conclusions: The efficacy of CCCG-BNHL-2010 regimen in the treatment of children with ALCL was good. However, the safety needs to be improved as the treatment-related mortality in the present study was slightly higher. Efficient second line or salvage treatment can achieve cure in pediatric patients post progression or recurrence. LDH ≥2 times ULN was associated with worse prognosis.

S1735 A Rare Case of Immunosuppression-Induced Kaposi’s Sarcoma With Dermatologic and Visceral Involvement

S1735 A Rare Case of Immunosuppression-Induced Kaposi’s Sarcoma With Dermatologic and Visceral Involvement

Abdominal tuberculosis: Clinical profile and outcome.

Tuberculosis (TB) is common form of communicable disease in India. Abdominal TB is one of the most common yet misdiagnosed forms of extrapulmonary TB. It is missed due to its similarity to other conditions such as Crohn's disease and nonspecific clinical presentation. Medical records of 317 patients who were diagnosed with abdominal TB from August 2015 to December 2020 were reviewed retrospectively from our prospectively maintained database. Among 317 patients, 167 (52.7%) were male. Median age of presentation was 45 (8-85) years. Luminal involvement was seen in most of the patients (n = 157, 49.5%), followed by peritoneal (n = 63, 19.8%), mixed (n = 42, 13.2%), solid visceral (n = 30, 9.4%), and nodal (n = 25, 7.8%) involvement. Two hundred and sixty-one (82.3%) showed complete response. Seven (2.2%) patients died and 5 (1.6%) patients lost to follow-up. Median duration of treatment was 28 (25-52) weeks. Drug-induced liver injury was identified in 30 (9.5%) patients. Median follow-up duration was 32 (1-70) months. Abdominal TB is quite a diagnostic challenge due its vague clinical symptoms, nonspecific radiological features, and poor sensitivity and specificity of diagnostic tests. Hence, clinicians should have a high index of suspicion to diagnose and treat this treatable yet lethal condition promptly. Most cases respond very well to medical management and a small fraction requires surgical intervention if diagnosed early.

Head and Neck Langerhans Cell Histiocytosis in Children

Langerhans Cell Histiocytosis (LCH) is a sporadic myeloid neoplasm with proliferation of dendritic cells.1 In the United States, LCH has an estimated annual incidence of approximately 1 per million children.2 Controversy exists regarding management of LCH in head and neck: debridement, chemotherapy, or both. Few reports describe LCH in the OMS literature. The purpose of this study was to present experience with LCH of head and neck in children.This was a retrospective review of children with LCH at Children's Healthcare of Atlanta from 2009 to 2020. A patient was included when he or she: 1) was 18 years old or younger, 2) had LCH in the head and neck, and 3) had complete medical records. Medical records were reviewed for demographic information, lesion characteristics, and clinical presentations. Radiographic images were reviewed for specific findings characteristic of LCH (e.g., osteolytic changes, lymphadenopathy). Treatment regimen (debridement, debridement with chemotherapy, or chemotherapy only) and length of follow-up were recorded. The outcome variable was disease reactivation defined as: 1) a new LCH lesion or 2) an increase in size/recurrence of a lesion after treatment. Data were collected using a standardized collection form. Descriptive statistics were calculated.Overall, 67 patients (42 males) with a mean age of 6.5 years old met inclusion criteria. According to clinical, radiographic, and histological features, there were 3 presentations of LCH in the head and neck: 1) lesions in 1 system without CNS risk (i.e., single system without CNS risk, SS-), 2) lesions in 1 system with CNS risk (i.e., single system with CNS risk, SS+), or 3) multisystem (> 2 systems, MS) presentations. As a group, length of follow-up was on average 3 years (3 months-10.5 years).There were 24 patients (17 males) with an average age of 10 years old (19 months to 18 years) with SS- LCH. Lesions appeared as persistent local swelling (n = 18, 75%) and/or pain (n = 8, 33.3%). Lesions were predominately located in calvaria (n = 15, 62.5%) and mandible (n = 7, 29.2%). Radiographically, most patients had osteolytic changes (n = 18, 78.3%). Patients were treated via debridement (n = 16, 72.7%), chemotherapy (n = 5, 22.7%), or debridement with chemotherapy (n = 2, 9.1%). Three patients (12.5%) experienced reactivation.There were 30 patients (18 males) with an average age of 6 years old (9 month to 15 years) with SS+ LCH. Patients presented with persistent local swelling (n = 21, 70%), recurrent ear infection (n = 5, 16.7%), localized pain (n = 4, 13.3%), or systemic symptoms (n = 2, 6.5%). Radiographic findings were osteolytic changes (n = 24, 77.4%) and/or soft-tissue mass (15, 48.4%). All except 2 patients completed treatment consist of debridement (n = 1, 3.3%), chemotherapy (n = 17, 56.7%), or debridement with chemotherapy (n = 11, 36.7%). One patient (3.3%) who was treated with chemotherapy had disease reactivation.Thirteen patients (9 males) with an average age of 2 years old (1 day to 7 years) had MS LCH. LCH was found in skin (n = 9, 69.2%), lymphatic system (n = 6, 46.2%), and at least 1 bone (range 1-5). In this group, patients presented with extracranial visceral organ involvement on imaging (e.g., hepatosplenomegaly, pulmonary nodules). Patients were treated with chemotherapy (n = 12, 92.3%) or debridement with chemotherapy (n = 1, 7.7%). Six patients (40%) experienced reactivation, and 1 patient passed away during treatment.There are 3 separate categories of LCH in the head and neck. Oral and maxillofacial surgeons who provide care to children would benefit from approaching head and neck LCH based on these categories. A referral to hematology/oncology is necessary for appropriate diagnosis and management.

S2857 DRESS Syndrome Potentially Induced by Carbamazepine and Triggered by CMV Viremia

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe adverse drug reaction that manifests with fever, rash with visceral organ involvement and hematological abnormalities. It can occur due to many etiological factors, here we present a case of DRESS that is potentially induced by carbamazepine and triggered by CMV viremia. Case Description/Methods: A 53-year-old male with a history of DM, neuropathy and HTN who presented for acute liver failure (ALF). He endorsed a mild rash after his 1st dose of mRNA COVID vaccine, with full resolution. After receiving his 2nd dose, he developed a diffuse rash with nausea, vomiting, fever and malaise within 72 hrs. He denied supplements, alcohol. Labs were remarkable for eosinophilia, hyponatremia, AST/ALT greater than 10x the ULN, ALP 567, Tbil 8.3, INR 1.9, ammonia 94. Autoimmune panel, drug toxicology screen, viral hepatitis panel with work up including varicella, HSV, EBV, HEV were negative except CMV IgG reactive with low CMV viremia 34 IU/ML. He was initially suspected to have COVID vaccine induced ALF due to temporal association. However, on review, he reported that he was started on carbamazepine for neuropathy ∼7wks prior to initial presentation. He was also evaluated for liver transplant due to jaundice and coagulopathy. Pt met RegiSCAR criteria for DRESS syndrome and was started on steroids. Liver biopsy showed liver injury with resolving eosinophilia not specific. Pt showed significant improvement in liver enzymes and clinical symptoms after 6 days of high dose PO steroids;he was eventually discharged on steroid taper. Discussion: DRESS syndrome has a latency period of about 2-8weeks between drug initiation and onset of symptoms. Although pathogenesis is unknown, it involves two mechanisms- drug-specific immune response and reactivation of human Herpesviridae with a subsequent antiviral response. We hypothesize that DRESS was induced by initiation of carbamazepine and triggered by CMV infection. This case indicates prompt diagnosis and steroids can be lifesaving in this rare presentation of DRESS as ALF. There are case reports of influenza vaccine triggering DRESS. In our case vaccine initially appear to be responsible for ALF, but on review was likely a bystander since mRNA vaccine does not have viral particles like influenza vaccine. Hence this case also highlights the importance of detailed history and complete lab evaluation before correlating organ injury to new mRNA COVID vaccine.

Femoral Cartilage Thickness in Patients with Systemic Sclerosis and Its Relation to Vitamin D

Abstract Background Systemic sclerosis (SSc) is a connective tissue disease characterized by different degrees of skin fibrosis and visceral organ involvement. The etiology of SSc remains obscure; the disease appears to be the result of a multistep and multifactorial process, including immune system alterations, under the influence of genetic and exogenous factors. The aim of this work is to study levels of vitamin D in relation to the femoral cartilage thickness (FCT) in patients with SSc and to analyze the associations between the (FCT), vitamin D levels, SSc- disease severity score. Patients and methods This is a cross sectional study which included 40 adult systemic sclerosis patients diagnosed according to ACR/EULAR (2013) classification criteria of systemic sclerosis. Patients were categorized into two groups according to vitamin D sufficiency; Group I: n = 14, sufficient vitamin D (level ≥ 30 ng/ml), Group II: n = 26, insufficient vitamin D (level &amp;lt; 30 ng/ml). Serum levels of 25-hydroxyvitamin D (25[OH] D) were assessed by (ELISA). The thickness of femoral articular cartilage was measured by muscloskeletal ultrasound. Three measurements were taken from each knee: lateral femoral condyle (LFC), femoral intercondylar area (ICA) and medial femoral condyle (MFC). Results The majority of patients (60%) had thin femoral cartilage of knee joint, we found that (35%) of patients had sufficient vitamin D level while (65%) of patients had insufficient vitamin D level. We compared the insufficient vitamin D level group with sufficient vitamin D level group according to femoral cartilage thickness, and concluded that vitamin D level related to femoral cartilage thickness at left medial condylar area and left lateral condyle, meanwhile we found no relation between disease severity and cartilage thickness or vitamin D level. There was significant relation between vitamin D sufficiency and sex of studied patients more in females, also showed significant inverse correlation between parity in females and femoral cartilage thickness at Rt intercondylar area and Rt medial condyle. There was highly significant statistical relation between disease severity grades and proximal muscle weakness among studied patients. Conclusion Vitamin D level has significant relation with femoral cartilage thickness in SSc patients in two of six of measured cartilage areas and proximal muscle weakness was associated with vitamin D insufficiency and disease severity.

PORCELAIN PLEURA: A RARE CASE OF METASTATIC CHONDROSARCOMA WITH EXTENSIVE PLEURAL CALCIFICATION

TOPIC: Disorders of the Pleura TYPE: Fellow Case Reports INTRODUCTION: Calcified pleural lesions have both benign and malignant etiologies. Benign lesions can originate from previous trauma, prior pleural infections, hemothorax, or asbestos exposures, and are often focal and limited1,2. Malignant lesions often involve metastatic disease from mesenchymal tumor origin, such as osteosarcoma, chondrosarcoma, or mesothelioma1,3. We describe a case of a patient with metastatic chondrosarcoma manifesting with extensive calcification of the parietal and visceral pleura. CASE PRESENTATION: A 67 year old man was referred to pulmonary for a new subcarinal soft tissue mass on chest CT. He had been previously diagnosed with chondrosarcoma in 2016 and underwent treatment via a radical right proximal humerus excision and shoulder arthroplasty, with final pathology revealing grade 2 conventional chondrosarcoma, mixed hyaline and myxoid, without metastatic foci.Screening chest CT in 2020 noted a new, partially calcified subcarinal soft tissue mass and adjacent lymphadenopathy with moderate hypermetabolism on a positron emission tomography scan (PET/CT). Bronchoscopy with endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) subcarinal lymph node and adjacent posterior mass confirmed metastatic disease.He subsequently developed rapidly progressive dyspnea on exertion over the following month. Repeat chest CT revealed extensive development of circumferential nodularity and calcification of the right visceral and parietal pleura with a large pleural effusion. Thoracentesis improved dyspnea despite limited reexpansion of the lung and noted loculation of the effusion. Pleuroscopic evaluation of the complicated space was undertaken to remove loculations and optimize placement of an indwelling pleural catheter (IPC) for his malignant effusion. Despite difficulty with trocar insertion into the calcified pleural space, placement of the IPC was successful with improvement in his dyspnea. DISCUSSION: Metastatic pleural involvement of chondrosarcoma is rare, and can occur in the setting of direct extension in locoregional disease or hematogenous spread from other sites of primary involvement. Metastatic disease has been noted to be more prevalent in the dedifferentiated and mesenchymal subtypes4. Manifestations of metastatic pleural involvement are typically pleural calcifications that can be profound in the rapid evolution and extent of calcified involvement. Management is often palliative, as in this case, with drainage of any associated pleural effusions and attempts to mitigate accompanying dyspnea. CONCLUSIONS: This case of metastatic chondrosarcoma with extensive visceral and parietal pleural involvement is rare and infrequently noted in the literature. Palliative management with thoracentesis and IPC placement was complicated by the pervasive pleural calcification that prevented ultrasound imaging and made access to the pleural space difficult and dangerous. REFERENCE #1: Sureka B, Thukral BB, Mittal MK, Mittal A, Sinha M. Radiological review of pleural tumors. Indian J Radiol Imaging. 2013;23(4):313-320 DISCLOSURES: No relevant relationships by Robert Browning, source=Web Response, value=Grant/Research Support Removed 04/29/2021 by Robert Browning, source=Web Response No relevant relationships by Robert Browning, source=Web Response, value=Consulting fee Removed 04/29/2021 by Robert Browning, source=Web Response No relevant relationships by Robert Browning, source=Web Response, value=Consulting fee Removed 04/29/2021 by Robert Browning, source=Web Response No relevant relationships by Sean McKay, source=Web Response No relevant relationships by Philip Mullenix, source=Web Response No relevant relationships by Caitlin Nickens, source=Web Response No relevant relationships by John Shumar, source=Web Response

NOD2-associated granulomatous autoinflammatory syndromes – a short update for clinicians

NOD2 (nucleotide-binding oligomerization domain-2), a pattern recognition receptor, is involved in innate immune defense against pathogens, intestinal mucosal barrier integrity, gut microbiota composition, autophagy, immune homeostasis and inflammation. NOD2 mutations have been associated primarily with childhood diseases (Blau syndrome and sarcoidosis with early-onset, monogenic Crohn’s disease), but also with adult diseases (NOD2-associated autoinflammatory syndrome – NAID, also called Yao syndrome etc.). Intermediate forms between Blau syndrome and NAID have also been described. Blau’s disease and early-onset sarcoidosis are the familial and the sporadic forms respectively of a dominantly inherited rare monogenic autoinflammatory disease. Blau’s syndrome starts in early childhood, evolving with non-caseating granulomatous arthritis with prominent tenosynovitis, dermatitis with a “bronzed”, maculo-papular or scaly skin rash, and intermittent fever. Periodic fever, generalized lymphadenopathy, and granulomatous visceral involvement may be present. Therapy consists in systemic steroids, immunosuppressants and biologic drugs. In adults the NAID or Yao’s syndrome evolves with bouts of systemic inflammation with lower limbs swelling, tenosynovitis and non-erosive arthritis, fever and dermatitis. We discuss the differential diagnosis with other granulomatous diseases. Increased awareness is necessary regarding these rare diseases which may alter the quality of life or lead to disability, in order to improve their prognosis.

Less Known Gastrointestinal Manifestations of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Systematic Review of the Literature.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life threatening severe cutaneous drug reaction. Most patients develop eosinophilia, a rash, a fever, lymphadenopathy and variable visceral organ involvement 2–6 weeks following exposure to the inciting medication. Unlike other severe cutaneous drug reactions, internal organ involvement that leads to high mortality is a unique feature of DRESS syndrome. While the liver is the most common internal organ involved, literally every other visceral organ can be affected in this syndrome. The lesser-known gastrointestinal manifestations of this syndrome include esophagitis, gastritis, enteritis, colitis, pancreatitis and a late autoimmune sequela due to pancreatic injury such as fulminant type 1 diabetes mellitus, autoimmune type 1 diabetes mellitus and type 2 diabetes mellitus. While these entities are less common, they are associated with equally severe complications and adverse patient outcomes. In this review, we synthetize data on these rare manifestations using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The liver, the most common visceral organ involved, has been described as part of DRESS elsewhere and is not included in the scope of this article.

Diagnosis of Early Mycosis Fungoides.

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphomas, generally has a favorable clinical course. Early MF typically presents erythematous patches and/or plaques and lasts for many years without affecting the life expectancy. Only limited cases progress to develop skin tumors, with subsequent lymph nodes and rarely visceral organ involvement. One of the clinical problems in early MF is the difficulty in differentiating the disease from benign inflammatory disorders (BIDs), such as atopic dermatitis, chronic eczema, and psoriasis. In some MF cases, clinical and pathological findings are similar to those of BIDs. However, the accurate diagnosis of early MF is quite important, as inappropriate treatment including immunosuppressants can cause unfavorable or even fatal outcomes. This article focuses on general methods and novel tools for diagnosis of early MF.

Peritoneal Metastases: Their Associated Imaging Features

Peritoneal Metastases (PM) detection remains a challenge even with modern imaging. Knowing imaging features of abnormal findings frequently associated with PM is of interest to improve PM detection. Although ascites is a common imaging finding of PM, the presence of ascites alone, even in patients with known cancer, is not enough to diagnose PM. The peritoneum should be read as an own organ with careful analysis of the ligament (e.g. falciform and hepatoduodenal ligament), the mesos and the omenta. Indirect manifestations of visceral peritoneal involvement is a segment of small bowel fixed to the parietal peritoneum, the appearance of blockage of free circulation of ascites, plurisegmental bowel obstruction and clumped bowel that is a strong predictor of diffuse involvement of the visceral peritoneum by a high grade tumor. Ovarian and umbilical metastases are frequently associated with PM in particular in digestive cancers. Moreover, ovarian metastases has been shown to be less responsive to chemotherapy than other metastases and should not be chosen as a target lesion for RECIST assessment. The presence of cardiophrenic angle lymph nodes also increases the possibility of metastatic spread in peritoneum. Finally, the most common PM mimickers include colonic diverticulum, mesenteric lymph nodes, splenosis implants, fat necrosis and postoperative changes after cytoreductive surgery and HIPEC.

1543P Gemcitabine in classic Kaposi’s sarcoma: A pilot study

Kaposi’s Sarcoma (KS) is a rare multifocal neoplasm of lymphatic endothelium-derived cells, associated with infection by human herpes virus-8 (also called KS herpes virus). Four clinical subtypes are recognized: classic, endemic, epidemic (HIV-related), and iatrogenic. Classic KS typically occurs in elderly people of specific areas, such as the Mediterranean; it usually featured by skin lesions, often at lower limbs, without visceral involvement, and has a chronic course that requires systemic chemotherapy for locally aggressive extensive disease.

Mycosis fungoides and Sézary syndrome.

SummaryMycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T‐cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor‐cell burden (Sézary cells) in the peripheral blood consistent with advanced‐stage disease and a poor prognosis. In early‐stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early‐stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin‐directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health‐related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.

Diffuse vertebral metastases from glioblastoma with vertebroepidural diffusion: A case report and review of the literature

Background: The occurrence of extraneural metastasis in patients diagnosed with glioblastoma (GBM) is rare with an estimated incidence ranging from 0.4% to 2.0%. Short clinical history is believed to be a possible explanation of the paucity of such cases. Furthermore, to date, only few papers describe cases of vertebral metastases from GBM without evidence of synchronous visceral involvement.Case Description: The authors report on the case of a 46-year-old woman presenting with a history of surgically treated GBM who developed multiple metastases located in the posterior laminae and vertebral bodies with a single dural metastasis at D6-D8 level 5 years after the initial diagnosis. Total-body computed tomography did not show signs of either intracranial recurrence or visceral involvement. Postoperative pathological examination confirmed the diagnosis of the World Health Organization-2016 Grade IV GBM metastases.Conclusion: From a clinical point of view, the awareness of the possibility of spinal and vertebral metastasis from intracranial GBM is crucial. The present case demonstrates that distant dissemination from the primary tumor is possible despite the absence of intracranial recurrence.

LB713 Proteomic identification of new diagnostic biomarkers of early-stage Cutaneous Mycosis Fungoides

Primary cutaneous T-cell lymphoma (CTCL) is responsible for two-thirds of cutaneous lymphoma cases. Mycosis fungoides (MF), the most common subtype of CTCL comprises approximately 60% of CTCLs. Due to the similar clinical features of MF and inflammatory diseases such as eczema and psoriasis2,3, early-stage MF can easily be misdiagnosed as chronic inflammatory dermatoses, posing a diagnostic challenge to the dermatologist. Early-stage MF is characterized by a favorable prognosis with long-term survival similar to or slightly lower than that of age-matched healthy people, with a 5-year survival between 88% and 100%. In contrast, advanced-stage MF shows aggressive progression, and the median survival time of patients with lymph node and visceral involvement is only 13 months. Therefore, it is important to achieve early diagnosis to improve prognosis, yet there are few specific biomarkers for the early diagnosis and prognosis of MF. In this study, we described the pathological features of MF during the early and advanced stages through proteomics technology, providing clues for the pathogenesis of MF as well as biomarkers for malignant tumors in the early stage. More importantly, diagnostic biomarkers of early-stage MF were identified by comparing the proteomic characteristics of early-stage MF and inflammatory diseases, with the goal of preventing delayed therapy due to misdiagnosis.