Paraneoplasia

OBJECTIVES: A timely diagnosis is imperative for curing cancer. However, in patients with rheumatic musculoskeletal diseases (RMDs) or paraneoplastic syndromes, misleading symptoms frequently delay cancer diagnosis. As metabolic remodelling characterises both cancer and RMD, we analysed if a metabolic signature can indicate paraneoplasia (PN) or reveal concomitant cancer in patients with RMD. METHODS: Metabolic alterations in the sera of rheumatoid arthritis (RA) patients with (n=56) or without (n=52) a history of invasive cancer were quantified by nuclear magnetic resonance analysis...

Dermatomyositis (DM) is a rare autoimmune disease with involvement of skin and muscle that is classified as an idiopathic inflammatory myopathy. In addition to cutaneous lesions as well as weakness and atrophy of muscles, the heart and lungs are the major affected organs. DM occurs in association with malignant tumors in 20% of affected adults. The pathogenesis of the disease is not completely understood. DM is a multifactorial disease influenced by genetic, environmental and immunological factors...

Necrobiotic xanthogranuloma is a rare disease that is part of the non-Langerhans cell histiocytoses. It is characterized by yellowish skin lesions, which are typically periorbitally localized. Extracutaneous manifestations of all organs are possible and can cause potentially life-threatening complications. The disease also belongs to the facultative paraneoplasias and is often associated with paraproteinemia. These aspects should be considered regarding further diagnostics. Due to the rarity of the disease, there are no standardized guidelines for therapy so far...

Thymic carcinomas are exceedingly rare and very aggressive malignancies of the anterior mediastinum. While thymomas exhibit a high association with paraneoplastic syndromes, these phenomena are a rarity in thymic carcinomas. In general, acanthotic syndromes such as acroceratosis neoplastica and acanthosis nigricans maligna are commonly observed as paraneoplastic phenomena in patients with carcinomas. In contrast, psoriasis vulgaris, another acanthotic disease, rarely occurs as a paraneoplasia. We report the case of a 36-year-old patient with progressive thymic carcinoma (undifferentiated carcinoma, T3N2M1a) and paraneoplastic psoriasis occurring ten months before the initial diagnosis of the carcinoma...

Malignant tumors trigger a complex network of inflammatory and wound repair responses, prompting Dvorak's characterization of tumors as "wounds that never heal."1 Some of these responses lead to profound defects in blood clotting, such as disseminated intravascular coagulopathy (DIC), which correlate with poor prognoses.2 , 3 , 4 Here, we demonstrate that a new tumor model in Drosophila provokes phenotypes that resemble coagulopathies observed in patients. Fly ovarian tumors overproduce multiple secreted components of the clotting cascade and trigger hypercoagulation of fly blood (hemolymph)...

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PURPOSE: Anti-N-methyl-D-aspartate receptor encephalitis (NMDA-R) is the most common among autoimmune encephalitis caused by antibodies developed against surface antigens. It was first identified in 2007 as presenting with seizures, autonomic dysfunction and movement disorders, in addition to acute or subacute onset of neuropsychiatric symptoms. Multifocal, nonspecific white matter lesions can be seen on cranial magnetic resonance imaging (MRI). Since paraneoplasia is usually found in the etiology, malignancy should be screened in patients with NMDA-R encephalitis...

BACKGROUND: Non-schistosomiasis-associated squamous cell carcinoma of the urinary bladder is less common in the Western world. Limited information on its possible paraneoplastic syndromes exists. Leukocytosis tends to commonly be regarded by clinicians as an indication of sepsis, rather than a feature of paraneoplasia, potential surrogate marker for recurrence, and prognostic marker. Accompanying hypercalcemia may be missed entirely. CASE PRESENTATION: A 66-year-old Caucasian man presented with visible painless hematuria and symptomatic hypercalcemia...

OBJECTIVE AND DESIGN: The hallmark of type 2 inflammation is eosinophilia and/or high IgE serum levels, mostly in atopic dermatitis. Nevertheless, many dermatoses may present similar findings. Our aim is to explore the biological and clinical spectrum of cutaneous manifestations involving tissue and/or systemic eosinophilia, and distinct serum levels of IgE, where atopic dermatitis or other primary allergic eczema, not always is the definitive diagnosis. MATERIALS/METHODS: A total of 37 scientific papers were enrolled in this narrative review...

Hypereosinophilic syndrome is characterized by chronic eosinophil overproduction, resulting in multiple organ damages due to eosinophil infiltration and mediator release. According to the etiology, we distinguish between myeloproliferative disorders, parasitic infections, solid tumors, T-cell lymphomas and idiopathic forms. In our case report, the 49-year-old man was hospitalized with weight loss, leg edema and tachycardia. In his laboratory tests increased biliary obstructive parameters as well as extreme leukocytosis and eosinophilia had been highlighted...

Összefoglaló. A nyálkahártya-pemphigoid különleges formája a paraneoplasiás pemphigoid, amely primer malignus betegséghez társul; lefolyása szokatlanul gyors, és a klasszikus immunszuppresszív terápiákkal szemben rezisztens lehet. Közleményünkben három eseten keresztül mutatjuk be a paraneoplasiás pemphigoid megjelenését, diagnosztikáját és a terápiás kihívásokat. A diagnózist a kórelőzményi adatok és az immunfluoreszcens vizsgálatok segítik. A terápiás célkitűzés a progresszió lassítása immunszuppresszív kezeléssel, amely a zajló onkológiai kezelés mellett kontraindikált lehet...

Multiple myeloma is a lymphoproliferative disease, which rarely presents with skin involvement or associated symptoms. Better awareness of these dermatological presentations is required for early diagnosis and to guide the patient towards appropriate therapy. We report on a patient with diffuse filiform hyperkeratosis and immunoglobulin-associated vasculitis in a severe progression of a known myeloma.

A variety of well-characterized cutaneous paraneoplastic syndromes (PNS) are diagnosed during internal malignancies; however, the spectrum of keratinocyte skin neoplasms (KSC) related to PNS is still obscure. The aim of the present review is to compile and evaluate the literature data on PNS associated with a keratinocyte skin neoplasm (KSC). Employing Pubmed, MEDLINE was searched for KSC-associated PNS reports. Forty relevant entries were assembled, reporting a total of 41 PNS cases associated with a KSC (34 male)...

Cancer patients often die from symptoms that manifest at a distance from any tumor. Mechanisms underlying these systemic physiological perturbations, called paraneoplastic syndromes, may benefit from investigation in non-mammalian systems. Using a non-metastatic Drosophila adult model, we find that malignant-tumor-produced cytokines drive widespread host activation of JAK-STAT signaling and cause premature lethality. STAT activity is particularly high in cells of the blood-brain barrier (BBB), where it induces aberrant BBB permeability...

There is a large gap between the deep understanding of mechanisms driving tumour growth and the reasons why patients ultimately die of cancer. It is now appreciated that interactions between the tumour and surrounding non-tumour (sometimes referred to as host) cells play critical roles in mortality as well as tumour progression, but much remains unknown about the underlying molecular mechanisms, especially those that act beyond the tumour microenvironment. Drosophila has a track record of high-impact discoveries about cell-autonomous growth regulation, and is well suited to now probe mysteries of tumour - host interactions...

A thymoma belongs to rather rare tumors, although it holds the first place among primary mediastinal tumors in adults. The biological characteristics of thymomas are considerably divergent. The tumors are usually asymptomatic at the begining, later due to a local spread, symptoms from the infiltration or compression of adjacent structures arise, they metastasize rarely. Thymomas are often associated with various autoimmune or endocrinologic diseases. The authors present case of the patient with less common paraneoplasia - autoimmune hemolytic anemia (AIHA)...

In recent years, an association between granuloma annulare and the occurrence of malignant cutaneous lymphomas in the sense of a facultative paraneoplasia has been observed several times. The aim of the present work is to provide an overview of the currently available literature on granuloma annulare as well as an analysis of its association with cutaneous lymphomas. Using the example of two patients with granuloma annulare and associated cutaneous lymphomas, we would like to sensitize clinically active dermatologists and dermatopathologists to the possible association between these two diseases...

In contrast to the obligatory paraneoplasias, which are usually rare, diseases such as Sweet's syndrome, thrombophlebitis or herpes zoster are more common. But they are less frequently associated with neoplasia. The risk of overlooking an associated neoplasm is therefore greater. In this article, prototypic diseases are presented with their clinical appearance, possible pathogenesis and treatment options; this is accompanied by raising awareness of potential associations with cancer. The emphasis on the distinct features of the neoplasm-associated forms should enable more reliable detection of these variants and hopefully contribute to an earlier diagnosis of associated neoplasms...

Neutrophilic febrile dermatosis (NFD) is a rare paraneoplastic syndrome that may be found in patients with head and neck cancer. NFD may appear before the neoplasia and may only concern the dorsal faces of the hands. This article reports the NFD findings of a patient with pharyngeal cancer, which was developed 2 years after the occurrence of NFD. The development of NFD in patient with alcohol and tobacco consumption should lead otolaryngologists and dermatologists to suspect head and neck malignancy. In cases of normal otolaryngological examination, patients have to be followed...

BACKGROUND: Hyperplasia-associated cutaneous paraneoplasia is an important differential diagnosis in everyday clinical practice. An early diagnosis of the underlying tumor disease can significantly improve the patient's prognosis. PATHOGENESIS: Hyperplasia is probably mainly cytokine-mediated. The primary tumor and/or the metastases release growth factors and transcription factors which, via epidermal growth factors, lead to hyperproliferation of keratinocytes. ACANTHOSIS NIGRICANS MALIGNA: Symmetrical mainly intertriginous hyperpigmentation with partially verrucous hyperplasia and lichenification mostly in association with gastric adenocarcinoma...