Malignant Gastric Carcinoid and Paraneoplastic Cerebellar Degeneration

Balducci, Genoveffa; Frontoni, Marco; Bocchetti, Tommaso; Angelini, Daniele; Giacomo, Giovanni Di; Ziparo, Vincenzo

doi:10.1080/110241599750007766

Cited by 10 publications

(3 citation statements)

References 21 publications

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“…There are various cases in the literature describing patients with neuroendocrine carcinoid tumors and neurological symptomes with the tumors being localized in the stomach [ 24 ] or the bronchial system [ 25 , 26 ]. In the majority of these cases the neurological affections were due to the hormones produced by the carcinoid or by the metastases [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic brainstem encephalitis in a patient with exceptionally long course of a metastasized neuroendocrine rectum neoplasm

et al. 2014

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BackgroundParaneoplastic neurological syndromes (PNS) have frequently been described in patients with lung or breast cancer. However, some reports also described a correlation to carcinoid tumors, probably triggered via the excessive release of hormones.Case presentationWe report the case of a 40-year-old woman that was diagnosed with a neuroendocrine neoplasm (NEN) of the rectum and multiple synchronous liver metastases ten years ago. She initially responded well to transarterial chemoembolization (TACE), resulting in prolonged disease stabilization. However, ten years after initial diagnosis the patient developed unspecific neurological symptoms that could not be classified by standard neurological diagnostic work-up. Special laboratory analysis revealed a high titer of anti-Ri (ANNA-2), a well-characterized antibody that is associated with paraneoplastic neurologic syndromes. The patient’s symptoms improved markedly after a 5-day-course of high-dose glucocorticoid therapy. To our knowledge, this is the first report of a Ri-positive PNS in a patient with hormone-negative rectal NEN.ConclusionPNS can complicate the patient’s clinical course, response to treatment, impact prognosis and even be interpreted as metastatic spread. However, owing to their rarity, the knowledge of these syndromes is very helpful in order to be able to provide evidence-based diagnostic and therapeutic approaches.

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Section: Discussionmentioning

confidence: 99%

Paraneoplastic brainstem encephalitis in a patient with exceptionally long course of a metastasized neuroendocrine rectum neoplasm

et al. 2014

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“…The onset of neurological manifestations usually precedes cancer diagnosis and may lead to earlier cancer detection and treatment [1,2]. Although most studies focus on small-cell lung cancer (SCLC)-associated paraneoplastic neurological syndromes [3,4], diverse neurological manifestations have been described accompanying other neuroendocrine neoplasms (NENs), including "high-risk" paraneoplastic neurological phenotypes (e.g., Lambert-Eaton myasthenic syndrome, limbic encephalitis or rapidly progressive cerebellar syndrome) [5][6][7][8][9][10][11] and other classical presentations of neurological autoimmunity (e.g., brainstem encephalitis, myelopathy) [12][13][14][15][16][17][18][19][20]. A single patient may have multiple neural autoantibodies, the patient's serological profile reflects the antigenic makeup of the underlying tumor [21] and may herald tumor recurrence or predict better tumor survival [3,[21][22][23][24][25][26][27].…”

Section: Introductionmentioning

confidence: 99%

Neurological autoimmunity in patients with non‐pulmonary neuroendocrine neoplasms: clinical manifestations and neural autoantibody profiles

Mangioris,

Halfdanarson,

Lennon

et al. 2024

Euro J of Neurology

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Background and purposeParaneoplastic neurological autoimmunity is well described with small‐cell lung cancer, but information is limited for other neuroendocrine neoplasms (NENs).MethodsAdult patients with histopathologically confirmed non‐pulmonary NENs, neurological autoimmunity within 5 years of NEN diagnosis, and neural antibody testing performed at the Mayo Clinic Neuroimmunology Laboratory (January 2008 to March 2023) were retrospectively identified. Control sera were available from patients with NENs without neurological autoimmunity (116).ResultsThirty‐four patients were identified (median age 68 years, range 31–87). The most common primary tumor sites were pancreas (nine), skin (Merkel cell, eight), small bowel/duodenum (seven), and unknown (seven). Five patients received immune checkpoint inhibitor (ICI) therapy before symptom onset; symptoms preceded cancer diagnosis in 62.1% of non‐ICI‐treated patients. The most frequent neurological phenotypes (non‐ICI‐treated) were movement disorders (12; cerebellar ataxia in 10), dysautonomia (six), peripheral neuropathy (eight), encephalitis (four), and neuromuscular junction disorders (four). Neural antibodies were detected in 55.9% of patients studied (most common specificities: P/Q‐type voltage‐gated calcium channel [seven], muscle‐type acetylcholine receptor [three], anti‐neuronal nuclear antibody type 1 [three], and neuronal intermediate filaments [two]), but in only 6.9% of controls. Amongst patients receiving cancer or immunosuppressive therapy, 51.6% had partial or complete recovery. Outcomes were unfavorable in 48.3% (non‐ICI‐treated) and neural autoantibody positivity was associated with poor neurological outcome.DiscussionNeurological autoimmunity associated with non‐pulmonary NENs is often multifocal and can be treatment responsive, underscoring the importance of rapid recognition and early treatment.

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“… 1 Paraneoplastic neurological syndromes associated with carcinoid tumors are rare, which include Lambert-Eaton myasthenic syndrome, limbic encephalitis, and autonomic dysfunction. 1 Balducci et al 2 described a case of paraneoplastic cerebellar degeneration associated with a malignant gastric carcinoid. Paraneoplastic cerebellar degeneration is commonly associated with small-cell lung carcinomas, breast and ovarian carcinomas, and lymphomas.…”

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confidence: 99%