Rare and changeable as a chameleon: paraneoplastic syndromes in renal cell carcinoma

Hegemann, Miriam; Kroeger, Nils; Stenzl, Arnulf; Bedke, Jens

doi:10.1007/s00345-018-2215-9

Cited by 24 publications

(22 citation statements)

References 50 publications

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“…Renal cell carcinoma is not usually associated with paraneoplastic neurological syndromes (PNS), and its association with autoimmune encephalitis is rare (7). Few cases with limbic encephalitis in association with renal cell carcinoma have been reported (5), but no autoimmune antibodies in serum or CSF were detected in these cases, so this is the first case of NMDAR encephalitis associated with renal cell carcinoma, which was cured with nephron-sparing surgery.…”

Section: Discussionmentioning

confidence: 88%

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated With Clear Cell Renal Carcinoma: A Case Report

Yang

et al. 2020

Front. Oncol.

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Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a cause of autoimmune encephalitis and is characterized by epileptic seizures, psychosis, and consciousness impairments. It mostly affects young adults with ovarian cancers. We herein reported a case of anti-NMDAR encephalitis associated with clear cell renal carcinoma. Case Presentation: A 54-year-old male with headache for 1 week and mood and behavioral changes for 3 days was presented, but his clinical presentation and poor response to antiviral treatment did not support a diagnosis of viral encephalitis. Positive anti-NMDAR antibodies in serum and cerebrospinal fluid confirmed autoimmune encephalitis. A subsequent evaluation revealed a paraneoplastic etiology of a renal mass, and this was then resected and pathologically confirmed as clear cell renal carcinoma. The patient's symptoms showed improvement after resection of the mass. The patient relapsed 6 months after discharge, and the symptoms completely disappeared after treatment with corticosteroids and intravenous immunoglobulin. Conclusion: Our findings suggested that NMDAR encephalitis might be associated with clear cell renal carcinoma. When patients present with unexplained seizures, neuropsychiatric disorder, or other brain symptoms, clinicians should be careful with paraneoplastic neurological disorders. Early diagnosis and treatment of primary tumors might show improvement.

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Section: Discussionmentioning

confidence: 88%

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated With Clear Cell Renal Carcinoma: A Case Report

Yang

et al. 2020

Front. Oncol.

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“…While 20% of patients diagnosed with RCC present with PNS, another 10%-40% develop PNS over the course of their disease [2]. There are 15 different types of PNS that have been described in the context of RCC, contributing to its historical reference as the "internist's tumor", including: constitutional symptoms, hypercalcemia, hypertension, polycythemia, hepatic dysfunction, hyperglycemia, amyloidosis, anemia, neuromyopathy, vasculopathy, and coagulopathy [5,6]. Hypercalcemia is the most common PNS in patients with RCC [5].…”

Section: Discussionmentioning

confidence: 99%

“…As PNS mostly resolve with appropriate treatment of the underlying malignancy, the gold standard in the treatment of localized RCC PNS is surgical removal of the primary. Resolution with oncologic treatment is considered an important part in the confirmation that it is truly a paraneoplastic process [8], and persistence of PNS following appropriate oncologic treatment is considered a poor prognostic factor and can signal recurrence of malignancy [6].…”

Section: Discussionmentioning

confidence: 99%

Stereotactic Radiotherapy in the Treatment of Paraneoplastic Vasculitis in Oligometastatic Renal Cell Carcinoma

et al. 2021

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Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT.

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“…As many as 10% to 40% of patients with renal cell carcinoma have paraneoplastic syndromes. 9 However, endocrine or neuroendocrine effects rather than neurologic symptoms are often present in these patients. These paraneoplastic syndromes include high blood pressure, anemia, fever, weight loss, cachexia, polycythemia, abnormal liver function, hypercalcemia, high blood sugar, rapid blood sedimentation, neuromuscular disease, amyloidosis, excessive breast disease, and coagulopathies.…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic limbic encephalitis cured with nephron-sparing surgery in a patient with clear cell renal cell carcinoma: a case report

et al. 2019

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Paraneoplastic limbic encephalitis (PLE) in association with clear cell renal cell carcinoma has never been reported in China. We herein describe a 54-year-old man with a 1-week history of headache and a 3-day history of psychiatric symptoms. Slight nuchal rigidity was found by physical examination. Imaging studies of the head were normal. Blood and cerebrospinal fluid antibody testing were both positive for N-methyl-D-aspartic acid antibodies. Subsequent contrast-enhanced computed tomography revealed a 5.2- × 4.2-cm left kidney mass with intense enhancement. Emergent laparoscopic nephron-sparing surgery was successfully performed. Immunohistochemistry revealed clear cell renal cell carcinoma. The patient’s status improved after the surgery, and he was discharged after 1 month of hospitalization. At the 6-month follow-up, magnetic resonance imaging showed no recurrence, and the patient was living independently. This case indicates the potential effect of nephron-sparing surgery in the treatment of PLE. Tumor screening should be performed early in patients with suspected PLE. Early surgical resection of the primary tumor can improve patients’ prognosis.

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Rare and changeable as a chameleon: paraneoplastic syndromes in renal cell carcinoma

Abstract: Paraneoplastic syndromes are often unrecognized but are important biomarkers in RCC. Further research into the underlying pathomechanisms of PNS may improve our understanding of the RCC tumour biology and is urgently needed.

Cited by 24 publications

References 50 publications

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated With Clear Cell Renal Carcinoma: A Case Report

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated With Clear Cell Renal Carcinoma: A Case Report

Stereotactic Radiotherapy in the Treatment of Paraneoplastic Vasculitis in Oligometastatic Renal Cell Carcinoma

Paraneoplastic limbic encephalitis cured with nephron-sparing surgery in a patient with clear cell renal cell carcinoma: a case report

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