scholarly journals Characteristics of dermatomyositis patients with and without associated malignancy

Author(s):  
Julia Lauinger ◽  
Kamran Ghoreschi ◽  
Sebastian Volc

2018 ◽  
Vol 1 ◽  
pp. 3
Author(s):  
Joshua S Agranat ◽  
Yoshihiro Yonekawa

Iris pigment epithelial (IPE) cysts are a subset of iris cysts that arise from the IPE. They are spontaneously erupting epithelial-lined cavities that are found in various anatomic locations of the iris, including the iris pupillary margin, midzone, periphery, and free floating in the vitreous or anterior chamber. We report the case of an asymptomatic 13-year-old boy with an incidental finding of a dislodged anterior chamber cyst diagnosed on routine examination. Modern multimodal image analysis of the cyst including anterior segment optical coherence tomography and ultrasound biomicroscopy (UBM) was utilized to characterize the microstructural anatomy of the lesion. The patient was managed conservatively without complications. Cysts of the IPE typically do not affect vision or ocular health and can be monitored and observed after ascertaining no associated malignancy. Initial diagnostic investigation can include UBM and anterior segment optical coherence tomography. Intervention should be reserved only for cases where the cyst growth leads to obstruction of the visual axis and/or other secondary complications.



2021 ◽  
pp. 000313482110318
Author(s):  
Aaron J. Arroyave ◽  
Alan W. Good ◽  
Andrew J. Ward ◽  
Amila L. Orucevic ◽  
James M. McLoughlin

Lynch syndrome (LS) is a common genetic syndrome characterized by pathogenic mutations of DNA mismatch repair genes resulting in a hereditary predisposition to cancer. While typically associated with colonic and endometrial cancer, LS additionally influences the development of many other malignancies. The Amsterdam II and Revised Bethesda Guidelines are the established clinical criteria for diagnosing LS. These guidelines are based on the most general characteristics of LS and do not address specific characteristics of the less commonly LS-associated malignancies. For individuals that present initially with a non-colon and non-endometrial malignancy, recommendations and guidelines on when to consider screening for LS are limited. Therefore, it is essential that clinicians are familiar with distinct LS-associated patient- and tumor-specific characteristics, especially of the less common LS-associated cancers, so that LS’s diagnosis is not missed. In this review article, we focus on extra-colonic and extra-endometrial LS-associated cancers, paying particular attention to any established or currently investigated cancer features that help raise suspicion for LS and potentially lead to its earlier diagnosis. This review will also discuss current guidelines specific to each LS-associated malignancy.



2001 ◽  
Vol 125 (1) ◽  
pp. 141-145
Author(s):  
Rajal B. Shah ◽  
Min W. Lee ◽  
Alvaro A. Giraldo ◽  
Mahul B. Amin

Abstract Context.—We have observed intraluminal crystalloid morphology in seminal vesicles that is superficially similar to that seen in prostate neoplasia, but found little information on such morphology in the literature. Design.—Two hundred fifty-three prostate specimens (163 needle biopsies, 75 radical prostatectomies with prostate carcinoma, 11 prostates from autopsy, and 4 cystoprostatectomies without prostate carcinoma) were examined for seminal vesicle secretions, which were categorized as (a) dense platelike inspissated, (b) fluidlike, (c) crystalloid morphology, and (d) absent. Histochemical stains (periodic acid–Schiff with and without diastase, Alcian blue at pH 2.5, and mucicarmine) were performed to characterize the nature of secretions. Results.—Proteinaceous secretions were identified in 82% of seminal vesicles examined. Of these, 61% had predominantly dense, platelike, inspissated secretions, 15% had predominantly fluidlike secretions, and 24% had predominantly crystalloid morphology. Although in some cases the crystalloid morphology resembled that of prostatic intraluminal crystalloids, the seminal vesicle crystalloids differed in that they were invariably multiple, had curved edges, and had varied forms (elliptical, cylindrical, rodlike, and rectangular). Seventy-one percent of seminal vesicle crystalloids were associated with dense, platelike, inspissated secretions and appeared to be created by fracturing within platelike secretions. There was no relationship between seminal vesicle crystalloid morphology and associated malignancy in the prostate gland, as it was seen in 24% of cases with prostate carcinoma and 25% of cases without prostate carcinoma (P = 1.0000). Fluidlike secretions were positive for Alcian blue (pH 2.5) and mucicarmine, whereas dense platelike secretions and crystalloid morphology were negative for Alcian blue (pH 2.5) and mucicarmine. Conclusions.—Seminal vesicle secretions are fairly common and, when fluidlike, are composed of acid mucopolysaccharides. Inspissation of secretions appears to be associated with loss of acidity, presumably resulting in dense platelike secretions and crystallization. Awareness of both the crystalloid morphology in seminal vesicle tissue and the distinguishing features from prostatic crystalloids may be important while interpreting prostate needle biopsies in which seminal vesicle epithelium may be confused for prostate carcinoma because of a small acinar morphology with accompanying cytologic atypia and crystalloid morphology.



2018 ◽  
Vol 4 (9) ◽  
pp. 944-946 ◽  
Author(s):  
Patricia M. Richey ◽  
Janet A. Fairley ◽  
Mary S. Stone


Rheumatology ◽  
2018 ◽  
Vol 58 (4) ◽  
pp. 650-655 ◽  
Author(s):  
Alexander Oldroyd ◽  
Jamie C Sergeant ◽  
Paul New ◽  
Neil J McHugh ◽  
Zoe Betteridge ◽  
...  

Abstract Objectives To characterize the 10 year relationship between anti-transcriptional intermediary factor 1 antibody (anti-TIF1-Ab) positivity and cancer onset in a large UK-based adult DM cohort. Methods Data from anti-TIF1-Ab-positive/-negative adults with verified diagnoses of DM from the UK Myositis Network register were analysed. Each patient was followed up until they developed cancer. Kaplan–Meier methods and Cox proportional hazard modelling were employed to estimate the cumulative cancer incidence. Results Data from 263 DM cases were analysed, with a total of 3252 person-years and a median 11 years of follow-up; 55 (21%) DM cases were anti-TIF1-Ab positive. After 10 years of follow-up, a higher proportion of anti-TIF1-Ab-positive cases developed cancer compared with anti-TIF1-Ab-negative cases: 38% vs 15% [hazard ratio 3.4 (95% CI 2.2, 5.4)]. All the detected malignancy cases in the anti-TIF1-Ab-positive cohort occurred between 3 years prior to and 2.5 years after DM onset. No cancer cases were detected within the following 7.5 years in this group, whereas cancers were detected during this period in the anti-TIF1-Ab-negative cases. Ovarian cancer was more common in the anti-TIF1-Ab-positive vs -negative cohort: 19% vs 2%, respectively (P < 0.05). No anti-TIF1-Ab-positive case <39 years of age developed cancer, compared with 21 (53%) of those ≥39 years of age. Conclusion Anti-TIF1-Ab-positive-associated malignancy occurs exclusively within the 3 year period on either side of DM onset, the risk being highest in those ≥39 years of age. Cancer types differ according to anti-TIF1-Ab status, and this may warrant specific cancer screening approaches.



AIDS ◽  
2018 ◽  
Vol 32 (3) ◽  
pp. 404-406
Author(s):  
Christopher J.A. Duncan ◽  
Fraser G. Charlton ◽  
Mark Bower ◽  
David A. Price


2010 ◽  
pp. 3692-3698
Author(s):  
John H. Stone

Polymyositis and dermatomyositis are two types of idiopathic inflammatory myopathy. The pathological findings in polymyositis suggest an HLA class I-restricted immune response mediated by cytotoxic T cells; dermatomyositis appears to be associated with humorally mediated destruction of muscle-associated microvasculature. Clinical features—polymyositis is characterized by symmetrical painless proximal muscle weakness that develops slowly, usually over weeks to months, and typically associated with significant elevation of serum creatine kinase and other muscle enzymes. The pattern of muscle involvement in dermatomyositis is clinically indistinguishable from that of polymyositis, but with cutaneous manifestations including Gottron’s sign, heliotrope rash, erythema, ‘mechanic’s hands’, periungual abnormalities, and calcinosis cutis. Extra-muscular features include interstitial lung disease (30% of cases), aspiration pneumonia, and associated malignancy (polymyositis 9%, dermatomyositis 15%)....



Author(s):  
Clarissa Pilkington ◽  
Liza McCann

Juvenile polymyositis and dermatomyositis are inflammatory myopathies that affect muscle. Dermatomyositis also affects skin, and can have many extramuscular manifestations. Inflammatory myopathies are uncommon in childhood, with dermatomyositis occurring more than polymyositis. For this reason, published research has concentrated on juvenile dermatomyositis. The spectrum of disease severity ranges from mild cases that can recover completely without treatment, to multisystem inflammation that can be fatal. Treatments have improved over the decades, reducing mortality from 30% before the era of steroids, to less than 1% in the present day. Juvenile cases of dermatomyositis differ from those seen in adulthood, without tendency for associated malignancy, and a far greater incidence of calcinosis. Calcinosis can be deposited as small calcinotic lumps or as sheets of calcinosis. It is very difficult to treat and causes extensive morbidity, and depending on where the calcinosis is deposited, it can cause severe disability or even death. Over the last decade, international collaborative work has concentrated on developing disease activity and assessment tools for both adult and juvenile forms of myositis. This will enable more subjective study of these rare diseases in multinational cohort studies, and enable clinical trials to investigate drug treatments. This work led to the first international double-blind placebo controlled trial of treatment in both adults and children with dermatomyositis (using rituximab as the drug). Further international collaboration has led to the development of core outcome variables, a definition of disease flare, and classification criteria.





Blood ◽  
2020 ◽  
Vol 136 (19) ◽  
pp. 2175-2187
Author(s):  
Jungang Chen ◽  
Navneet Goyal ◽  
Lu Dai ◽  
Zhen Lin ◽  
Luis Del Valle ◽  
...  

Abstract Primary effusion lymphoma (PEL) is an aggressive malignancy with poor prognosis even under chemotherapy. Kaposi sarcoma–associated herpesvirus (KSHV), one of the human oncogenic viruses, is the principal causative agent. Currently, there is no specific treatment for PEL; therefore, developing new therapies is of great importance. Sphingolipid metabolism plays an important role in determining the fate of tumor cells. Our previous studies have demonstrated that there is a correlation between sphingolipid metabolism and KSHV+ tumor cell survival. To further develop sphingolipid metabolism-targeted therapy, after screening a series of newly synthesized ceramide analogs, here, we have identified compounds with effective anti-PEL activity. These compounds induce significant PEL apoptosis, cell-cycle arrest, and intracellular ceramide production through regulation of ceramide synthesizing or ceramide metabolizing enzymes and dramatically suppress tumor progression without visible toxicity in vivo. These new compounds also increase viral lytic gene expression in PEL cells. Our comparative transcriptomic analysis revealed their mechanisms of action for inducing PEL cell death and identified a subset of novel cellular genes, including AURKA and CDCA3, controlled by sphingolipid metabolism, and required for PEL survival with functional validation. These data provide the framework for the development of promising sphingolipid-based therapies against this virus-associated malignancy.



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