Screening for Tumours in Paraneoplastic Syndromes

Titulaer, Maarten J.; Soffietti, Riccardo; Dalmau, Josep; Gilhus, Nils Erik; Giometto, Bruno; Graus, Francesc; Grisold, Wolfgang; Honnorat, Jérôme; Tanasescu, Radu; Vedeler, Christian A.; Voltz, Raymond; Verschuuren, Jan J.

doi:10.1002/9781444346268.ch21

Cited by 10 publications

(17 citation statements)

References 108 publications

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“…Benign teratomas have also been associated with paraneoplastic syndromes, including paraneoplastic limbic encephalitis (6-10), opsoclonus-myoclonus syndrome (11), paraneoplastic polyarthritis (12) and autoimmune hemolytic anemia (13). Titulaer et al (14) performed a screen for tumors in paraneoplastic syndromes to aid the early detection of tumors.…”

Section: A B Discussionmentioning

confidence: 99%

Adult dermatomyositis associated with benign ovarian teratoma: A case report

Qin

Mao

et al. 2016

Oncology Letters

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Abstract.A 27-year-old female patient presented with a 3-month history of bilateral orbital and facial edema accompanied by skin erythema and heliotrope rash. The left lesion was more critical than the right. Limb muscles were occasionally sore. On physical examination, scattered hemorrhagic rashes were observed on the patient's face and neck. Upon laboratory testing, creatine kinase (CK) was markedly elevated at 1,543.2 U/l, while CK-MB isoenzyme (27.4 U/l), α-hydroxybutyric dehydrogenase (188.4 U/l) and aspartate aminotransferase (65.3 U/l) were marginally elevated. The patient was diagnosed with dermatomyositis due to the dermatological features and elevated CK. Cancer marker analysis revealed positivity for carbohydrate antigen 125 (68.15 U/ml). Magnetic resonance imaging revealed thickening of the soft tissue of the left eyelid; furthermore, concurrent long T1-and T2-weighted signals (fat saturation) were compatible with inflammatory infiltration. Non-enhanced computed tomography identified that the right accessory region (right ovary and fallopian tube) had a cystic mass with a significant fat component and thickening of the anterior uterine wall. Gynecological ultrasound findings indicated an ovarian teratoma (8.7x7.8x9.3 cm) and uterine myoma (3.6x3.1 cm). The patient's dermatological symptoms and laboratory results were significantly relieved one week after surgical removal of the teratoma with the aid of hydrocortisone, methylprednisolone and methotrexate therapy. IntroductionDermatomyositis is a type of idiopathic inflammatory myopathy (1). Bohan and Peter (1) proposed five major criteria for the diagnosis dermatomyositis and another idiopathic inflammatory myopathy, polymyositis, in 1975: Progressive symmetrical weakness, muscle-biopsy evidence, including muscle fiber swelling, absence of striations and muscle fibers revealing vacuolar degeneration, elevated enzymes in skeletal muscle serum, abnormal electromyogram and dermatological features. Bohan and Peter (1) also suggested five subsets of myositis: Polymyositis, dermatomyositis, dermatomyositis associated with neoplasia, child dermatomyositis associated with vasculitis and polymyositis or dermatomyositis with associated collagen-vascular disease. Subsequent to this, Saoud et al (2) recognized an additional subset: Amyopathic dermatomyositis. Furthermore, Callen (3) previously reported that dermatomyositis is associated with malignant tumors. Teratomas are a type of germ cell tumor comprising well-differentiated tissues and 3 germ cell layers: Ectoderm, mesoderm and endoderm. They are divided into mature (benign) and immature (malignant) teratomas, and dermatomyositis may be associated with malignant or benign tumors. The current study presents a case of dermatomyositis accompanied by a benign ovarian teratoma. Case reportA 27-year-old female patient with a 3-month history of bilateral orbital and facial edema accompanied by skin erythema and rash accepted treatment at the Department of Rheumatology of the Affiliated Hospital of Qingdao Universit...

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Section: A B Discussionmentioning

confidence: 99%

Adult dermatomyositis associated with benign ovarian teratoma: A case report

Qin

Mao

et al. 2016

Oncology Letters

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“…The European Federation of Neurological Societies has published guidelines on the approach to cancer screening in patients with classic paraneoplastic antibodies. 25 Antibody profile, age, sex, and cancer risk factors are predictive of the probability of cancer detection. 5,25 Investigations should initially include targeted investigations according to antibody and risk factors (e.g., chest computed tomography [CT] in a smoker with ANNA-1), followed by further testing if the initial screen is negative.…”

Section: Investigationsmentioning

confidence: 99%

“…25 Antibody profile, age, sex, and cancer risk factors are predictive of the probability of cancer detection. 5,25 Investigations should initially include targeted investigations according to antibody and risk factors (e.g., chest computed tomography [CT] in a smoker with ANNA-1), followed by further testing if the initial screen is negative. First-line tests to be considered include CT thorax, abdomen, or pelvis; esophagoduodenostomy; colonoscopy; mammography; and/or PAP smear (for women); digital rectal exam and/or testicular ultrasound (for men).…”

Section: Investigationsmentioning

confidence: 99%

“…Neurologic manifestations often precede cancer signs and symptoms. 25 The portion of the tumor tissue that has cross-reactivity with neural cell components is the inciting antigen. Small-cell carcinoma of lung and breast adenocarcinoma are particularly immunogenic.…”

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confidence: 99%

“…Small-cell carcinoma of lung and breast adenocarcinoma are particularly immunogenic. 1,22,23,25 Antibodies commonly coexist; this may be useful to guide the search for malignancy. 5 For example, Purkinje cell antibody-1 (PCA-1) antibody is highly suggestive of breast or ovarian malignancy.…”

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Paraneoplastic and Autoimmune Encephalopathies

2013

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Immune-mediated encephalitis is an increasingly recognized cause of neurologic dysfunction including behavioral change, psychosis, movement disorders, seizures, autonomic instability, and coma. Associated antineuronal antibodies are of two main subtypes, those targeting neuronal cell surface antigens, which are pathogenic, and nonpathogenic antibodies targeting intracellular antigens. Antibody identification aids in screening for underlying cancers and prediction of outcome. Cancer is found most commonly with antibodies targeting intracellular neural components. Certain cancers, such as small-cell lung carcinoma, and breast and ovarian cancer are particularly immunogenic. When cancer is detected, oncologic treatment should be followed with immunotherapy. Nonpathogenic antibody disorders respond poorly to treatment, whereas pathogenic antibodies predict a favorable response to immune treatment. If no cancer is identified, then ongoing surveillance is recommended for 5 years after detection of most antineuronal antibodies.

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Update on Pediatric Opsoclonus Myoclonus Syndrome

Hero

Schleiermacher

2013

Neuropediatrics

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Opsoclonus myoclonus syndrome (dancing eye syndrome) is a rare paraneoplastic syndrome characterized by opsoclonus, myoclonus, and ataxia, usually accompanied by behavioral abnormalities. In adults, opsoclonus myoclonus syndrome has been reported in association with different types of cancer; whereas in children, the syndrome may be associated with neuroblastic tumors. Although a direct proof is lacking, the syndrome is assumed to be of autoimmune origin. The treatment is corticosteroid based with the addition of other immunosuppressive or immunomodulating drugs if intensification seems necessary. Because of the rarity of the disease, international collaborations as well on research as on therapeutic strategies are urgently needed. A European consortium just started a trial for this rare condition.

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Screening for Tumours in Paraneoplastic Syndromes

Cited by 10 publications

References 108 publications

Adult dermatomyositis associated with benign ovarian teratoma: A case report

Adult dermatomyositis associated with benign ovarian teratoma: A case report

Paraneoplastic and Autoimmune Encephalopathies

Update on Pediatric Opsoclonus Myoclonus Syndrome

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