Treatment Of Paraneoplastic Syndromes Research Articles

Paraneoplastic syndromes in rheumatology

Paraneoplastic syndromes in rheumatology are agroup of canonical rare rheumatic diseases with musculoskeletal involvement that occur in close temporal and causal association with malignancies. Knowledge of these possibly enables aprognostically relevant early diagnosis of the underlying malignant disease. In the era of immune checkpoint inhibitor treatment, there are first indications of an increase in the incidence and severity of paraneoplastic syndromes, so that they are becoming of increasing importance for the practicing rheumatologist. These nine syndromes, paraneoplastic arthritis, palmar fasciitis and polyarthritis, remitting seronegative symmetrical synovitis with pitting edema, pancreatic panniculitis with polyarthritis, paraneoplastic vasculitis, cancer-associated myositis, hypertrophic osteoarthropathy (Marie-Bamberger), eosinophilic fasciitis and tumor-induced osteomalacia, usually occur with characteristic courses and sometimes pathognomonic clinical manifestations, which are presented in this article accompanied by the rational use of a diagnostic algorithm for tumor detection. With frequently disappointing therapeutic response to glucocorticoids, nonsteroidal antirheumatic drugs and immunosuppressants, treatment of the underlying malignant disease represents the crucial step in the treatment of paraneoplastic syndromes.

Paraneoplastic syndrome associated with gynaecological malignancy: a review of the evidence

Key content Paraneoplastic syndrome (PNS) is a clinical manifestation of both benign and malignant tumours. Symptoms are not attributable to direct organ involvement of the cancer nor as a therapeutic adverse effect; instead, they are a result of hormones, cytokines or growth factors released by the tumour, or an immunological response. Paraneoplastic syndromes can affect any body system, so can cause myriad potential symptoms. These clinical manifestations often pre‐date those of the underlying disease process. The incidence of PNS attributable to gynaecological tumours is increasing, resulting in considerable morbidity in those affected. There is an overall lack of awareness of PNS among clinicians; this, combined with wide‐ranging signs and symptoms, creates an opportunity for diagnostic difficulty and therapeutic delay. Learning objectives To raise awareness among clinicians to aid earlier diagnosis and treatment of paraneoplastic syndromes.

A case of membranous glomerulopathy associated with lung cancer and review of the literature.

Membraneous nephropathy (MN) is the most commonly occurring nephrotic syndrome in adults as well as the most common paraneoplastic nephropathy associated with solid tumors, and it is mostly associated with gastrointestinal system and lung carcinomas. Accurate diagnosis is important as the treatment of paraneoplastic glomerulonephritis is very varied from that of idiopathic ones. In the current report, a case of a patient that was referred with proteinuria and edema and was diagnosed with lung cancer, and responded markedly to treatment of malignancy, with improvement of MN, is presented. Active cancer is present in all patients with paraneoplastic MN. In numerous patients, the paraneoplastic MN and cancer diagnoses are made within one year of each other. The treatment of paraneoplastic syndromes is usually associated with the treatment of primary malignancy. There are conflicting data on which treatment modality is more suitable. In conclusion, further studies are required in order to determine the actual incidence of cancer in patients with nephropathy, explain the physiopathological association between cancer and nephropathy and to determine the most suitable treatment approaches.

Understanding Paraneoplastic Syndromes in Athletes

The understanding of paraneoplastic syndromes has improved over the last several years. These disorders are brought about by tumor secretion of substances that can alter hormonal function and create immune cross-reactivity with normal tissues. This, in turn, can alter normal metabolic pathways resulting in paraneoplastic syndromes. The athlete must maintain an awareness of their body because these syndromes affect diverse organ systems. Commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumors, and gastroenterological malignancies. These cancers most notably affect the neuromuscular, rheumatologic, mucocutaneous, hematologic, renal, and endocrine systems. Effective diagnosis and treatment of paraneoplastic syndromes most often require appropriate diagnosis and treatment of the underlying malignancy. This review focuses on the most commonly encountered paraneoplastic syndromes, so athletes and those who care for them can be aware of the potential risk of an occult or recurrent malignancy.

8 - Neuroendocrine tumours that secrete biologically active peptides and amines

Summary The neuroendocrine tumours are a closely related family including foregut carcinoids, medullary carcinomas of the thyroid, islet-cell and some other pancreatic carcinomas, oat cell tumours of the lung, malignant epithelial thymomas, and the phaeochromocytoma-neuroblastoma group. They are named neuroendocrine because they share certain morphological and secretory features of the neural, amine-secreting tissues (such as autonomic ganglia) as well as certain features of the peptide-secreting glandular tissues (the classical endocrine tissues). In addition to their common histological, cytochemical and ultrastructural features, they have a tendency to secrete similar biologically active products (biogenic amines and peptide hormones of low molecular weight), a common embryogenesis from neuroectoderm, and a tendency to produce clinical syndromes characterized by vasomotor crises and secretory diarrhoea. Knowledge of these factors permits correct diagnoses which then dictate further therapeutic, diagnostic, or genetic interventions. Even if it is not always possible to tell an islet cell tumour from a carcinoid, the identification of a neuroendocrine neoplasm will suggest that certain particular peptides should be sought which might either serve as tumour markers or as indicators for the presence of specific paraneoplastic syndromes. With this group of neoplasms, the identification and treatment of paraneoplastic syndromes is particularly important. Since these tumours tend to be relatively indolent, the paraneoplastic syndromes may be crucial in determining the quantity and quality of life of the patient. Thus, diarrhoea, flushing, hyponatraemia, ectopic ACTH syndrome, hypoglycaemia, hyperglycaemia and fulminant peptic ulcer disease should be sought in each patient. Some of these tumours tend to occur as components of familial tumour diatheses — particularly the three types of multiple endocrine neoplasia. Thus, when appropriate, there should be a careful search for tumours that are likely to co-exist in the patient and in members of his or her family. Such a search is often rewarded with an early diagnosis of a potentially lethal disease at a stage at which it can be cured. The appearance of one of the paraneoplastic syndromes associated with these tumours should always alert the physician to the necessity to search for an occult neuroendocrine tumour; the nature of the syndrome should permit the physician to predict the locations that should be examined most carefully. For example, in patients with ectopic ACTH syndrome, neoplasms are most likely to be found in the lungs, thyroid, thymus, adrenal medulla, and foregut; a barium enema or an intravenous pyelogram is much less likely to demonstrate a tumour. In contrast, the presence of hypercalcaemia (when due to ectopic parathormone secretion) or erythrocytosis suggests that the primary tumour might be found in the liver or kidney but less likely in the locations characteristic of neuroendocrine tumours. The story of the neuroendocrine tumours is far from completed. As new information becomes available it will be necessary to constantly revise and update the concepts outlined in this chapter. For the present, viewing the neuroendocrine tumours as a closely related family with each of the specific neoplasms considered as a variation within a spectrum seems to provide the most rational approach currently available to the diagnosis and clinical management of patients having these tumours.