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Rheumatic Diseases Clinics of North... Aug 2018A variety of conditions mimicking rheumatologic syndromes may be associated with an underlying malignancy. Therefore, distinguishing these syndromes from more common,... (Review)
Review
A variety of conditions mimicking rheumatologic syndromes may be associated with an underlying malignancy. Therefore, distinguishing these syndromes from more common, nonparaneoplastic rheumatologic conditions can be perplexing. Some autoimmune conditions and the medications used for their management can be associated with increased future risk of malignancy. Some cancers can directly involve the musculoskeletal structures, whereas others present with systemic manifestations at sites away from the tumor and its metastases. Better awareness and timely recognition of these associations may lead to earlier cancer detection and, it is hoped, better long-term survival.
Topics: Autoimmunity; Diagnosis, Differential; Early Detection of Cancer; Humans; Paraneoplastic Syndromes; Rheumatic Diseases
PubMed: 30001783
DOI: 10.1016/j.rdc.2018.03.003 -
Neurology Jan 2021To test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurologic syndromes we retrospectively reviewed clinical, radiologic, and... (Observational Study)
Observational Study
OBJECTIVE
To test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurologic syndromes we retrospectively reviewed clinical, radiologic, and serologic features of 32 patients with concomitant paraneoplastic spinal cord and peripheral nervous system involvement.
METHODS
Observational study investigating patients with myeloneuropathy and underlying cancer or onconeural antibody seropositivity.
RESULTS
Among 32 patients with paraneoplastic myeloneuropathy, 20 (63%) were women with median age 61 years (range 27-84 years). Twenty-six patients (81%) had classified onconeural antibodies (amphiphysin, n = 8; antineuronal nuclear antibody [ANNA] type 1 [anti-Hu], n = 5; collapsin response mediator protein 5 [CRMP5] [anti-CV2], n = 6; Purkinje cell cytoplasmic antibody type 1 [PCA1] [anti-Yo], n = 1; Purkinje cell cytoplasmic antibody type 2 [PCA2], n = 2; kelch-like protein 11 [KLHL11], n = 1; and combinations thereof: ANNA1/CRMP5, n = 1; ANNA1/amphiphysin, n = 1; ANNA3/CRMP5, n = 1). Cancer was confirmed in 25 cases (onconeural antibodies, n = 19; unclassified antibodies, n = 3; no antibodies, n = 3). Paraneoplastic myeloneuropathies had asymmetric paresthesias (84%), neuropathic pain (78%), subacute onset (72%), sensory ataxia (69%), bladder dysfunction (69%), and unintentional weight loss >15 pounds (63%). Neurologic examination demonstrated concomitant distal or asymmetric hyporeflexia and hyperreflexia (81%), impaired vibration and proprioception (69%), Babinski response (68%), and asymmetric weakness (66%). MRI showed longitudinally extensive (45%), tract-specific spinal cord T2 hyperintensities (39%) and lumbar nerve root enhancement (38%). Ten of 28 (36%) were unable to ambulate independently at last follow-up (median 24 months, range 5-133 months). Combined oncologic and immunologic therapy had more favorable modified Rankin Scale scores at post-treatment follow-up compared to those receiving either oncologic or immunologic therapy alone (2 [range 1-4] vs 4 [range 2-6], < 0.001).
CONCLUSIONS
Paraneoplastic etiologies should be considered in the evaluation of subacute myeloneuropathies. Recognition of key characteristics of paraneoplastic myeloneuropathy may facilitate early tumor diagnosis and initiation of immunosuppressive treatment.
Topics: Adult; Aged; Aged, 80 and over; Autoantibodies; Female; HEK293 Cells; Humans; Male; Middle Aged; Paraneoplastic Polyneuropathy; Paraneoplastic Syndromes, Nervous System; Retrospective Studies
PubMed: 33208548
DOI: 10.1212/WNL.0000000000011218 -
Schmerz (Berlin, Germany) Dec 2022Paraneoplastic syndromes (PS) are rare disorders with often complex clinical manifestations that occur in association with a tumor without being triggered by direct... (Review)
Review
Paraneoplastic syndromes (PS) are rare disorders with often complex clinical manifestations that occur in association with a tumor without being triggered by direct tumor invasion or compression. They arise from tumor secretions of hormones, peptides or cytokines or from immune cross-reactivity between malignant and healthy tissue. They are categorized into endocrine, neurological, dermatological, rheumatological, and hematological PS. The PS most commonly occurs in small cell lung carcinoma but also in association with other respiratory tract tumors, gynecological tumors, and hematological malignancies. The PS can precede a tumor diagnosis, therefore timely diagnosis can improve the prognosis of a malignant disease. The diagnostics are based on the clinical presentation as well as diagnostic methods depending on the underlying pathogenesis. The most important treatment approach involves the best possible treatment of the tumor and a targeted treatment is only sometimes possible. This review focuses on the clinically most frequently encountered PS.
Topics: Humans; Lambert-Eaton Myasthenic Syndrome; Paraneoplastic Syndromes; Prognosis
PubMed: 36260170
DOI: 10.1007/s00482-022-00669-3 -
The Pan African Medical Journal 2017Paraneoplastic dermatoses are a spectrum of cutaneous manifestations which may precede, coincide with or follow the diagnosis of cancer. Our study aims to remind...
Paraneoplastic dermatoses are a spectrum of cutaneous manifestations which may precede, coincide with or follow the diagnosis of cancer. Our study aims to remind clinicians that desquamative erythrodermia is a form of paraneoplastic dermatosis which may occur during hematologic malignancies. Hence the importance of a complete assessment to identify a neoplastic process in the presence of these clinical signs and especially when they are suspect.
Topics: Aged; Hematologic Neoplasms; Humans; Male; Paraneoplastic Syndromes; Skin Diseases
PubMed: 28748019
DOI: 10.11604/pamj.2017.27.18.12273 -
Journal of the American Academy of... Jul 2024Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with... (Review)
Review
Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed.
Topics: Humans; Pemphigus; Paraneoplastic Syndromes; Bronchiolitis Obliterans; Autoimmune Diseases; Multiple Organ Failure; Male; Autoantibodies; Female; Risk Factors
PubMed: 37597771
DOI: 10.1016/j.jaad.2023.08.020 -
Magyar Onkologia Jun 2023The term paraneoplastic syndrome refers to the conditions when tumor-related symptoms are not caused by the size, invasion or metastasis of a tumor, but due to soluble...
The term paraneoplastic syndrome refers to the conditions when tumor-related symptoms are not caused by the size, invasion or metastasis of a tumor, but due to soluble mediators produced or an immune reaction induced by a tumor. Paraneoplastic syndromes occur in about 8% of all malignant tumors. Hormone-related paraneoplastic syndromes are termed paraneoplastic endocrine syndromes. In this short synopsis, the main clinical and laboratory characteristics of the most important paraneoplastic endocrine syndromes are presented including humoral hypercalcemia, the syndrome of inappropriate ADH secretion, ectopic ACTH syndrome. Two very rare diseases, paraneoplastic hypoglycemia and tumor-induced osteomalatia are also briefly presented.
Topics: Humans; Paraneoplastic Endocrine Syndromes; Paraneoplastic Syndromes; Autoantibodies; Rare Diseases
PubMed: 37314070
DOI: No ID Found -
The Israel Medical Association Journal... Oct 2018
Topics: Autoantibodies; Humans; Opsoclonus-Myoclonus Syndrome; Paraneoplastic Syndromes, Nervous System
PubMed: 30324785
DOI: No ID Found -
The American Journal of the Medical... Sep 2021Thymic neoplasms are rare and may run an indolent course. Among them, thymic epithelial carcinoma is exceptional as it may be presented with extensive local invasion and... (Review)
Review
Thymic neoplasms are rare and may run an indolent course. Among them, thymic epithelial carcinoma is exceptional as it may be presented with extensive local invasion and distant metastases. There is a wide spectrum of autoimmune/paraneoplastic syndromes associated with thymic tumors including autoimmune diseases, some of which may precede the diagnosis of thymoma. This article describes a 37-year-old woman with metastatic malignant thymoma and a combination of manifestations from different organs. Vitiligo, Raynaud's phenomenon and anti-centromere antibodies were preceded while eosinophilia, interstitial lung disease, rash, thickening of the skin and asymptomatic cryoglobulinemia were diagnosed concomitantly with the neoplasm. We have reviewed the literature and found only twenty case reports with a cluster of three or more autoimmune/paraneoplastic syndromes in the same patient but none with this unique constellation of disorders. The diversity of thymoma's clinical presentation and laboratory/histological features may cause diagnostic dilemmas and therapeutic challenges.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 33961845
DOI: 10.1016/j.amjms.2021.04.012 -
Anais Brasileiros de Dermatologia 2018Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory...
Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory bowel diseases, vaccination, medications and neoplasms. Hematologic malignancies are the diseases most related to paraneoplastic Sweet's syndrome, but this clinical entity can also be found occasionally in some solid tumors, including genitourinary tract tumors. We report a rare case of paraneoplastic Sweet's syndrome associated with the diagnosis of cervical cancer.
Topics: Diagnosis, Differential; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Sweet Syndrome; Uterine Cervical Neoplasms
PubMed: 30066769
DOI: 10.1590/abd1806-4841.20187353 -
Journal of the American Academy of... Jul 2024In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic... (Review)
Review
In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic criteria, investigative work-up, and management are reviewed. PNP/PAMS is a rare autoimmune blistering disorder associated with high morbidity and mortality. Recognizing PNP/PAMS's key features and its diagnostic criteria is critical in initiating appropriate work-up. Evaluating PNP/PAMS requires knowledge of its findings on histopathology, direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay. Lastly, treatments for PNP/PAMS are reviewed with suggestions based on case reports and expert opinions in the literature.
Topics: Humans; Pemphigus; Paraneoplastic Syndromes; Autoimmune Diseases; Multiple Organ Failure; Immunosuppressive Agents; Male
PubMed: 37714216
DOI: 10.1016/j.jaad.2023.08.084