Paraneoplastic neurological syndrome due to burned-out testicular tumor showing hot cross-bun sign

Ishikawa, Hidehiro; Kawada, Natsuko; Taniguchi, Akira; Odachi, K.; Mizutani, Atsushi; Asahi, Momoyo; Tomimoto, Hidekazu

doi:10.1111/ane.12469

Cited by 24 publications

(19 citation statements)

References 17 publications

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“…During the clinical evaluation of our case series, the first two patients were initially listed as having the lymphoproliferative syndrome and the third as having pulmonary metastasis of unknown origin. In various revised reports, due to various symptoms, the diagnostic work was also directed to pathology different from that of GCT [10–12, 14, 21, 24, 44, 54–57].…”

Section: Discussionmentioning

confidence: 99%

Spontaneous testicular tumor regression: case report and historical review

Astigueta

Abad-Licham

Agreda³

et al. 2018

ecancer

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Spontaneous regression of a primary testicular germ-cell tumour (GCT), over time known as ‘Burned out’, ‘Shrinking Seminoma’, ‘pT0’, ‘Burnout’ or ‘Spontaneous Regression’, is an uncommon, generally metastatic phenomenon, which may present elevated tumour markers and a suspicious testicular ultrasound image. The histological study of the testicle demonstrated morphological changes of complete or partial tumour regression and found fibrous scarring and other characteristic changes of this phenomenon, which in some cases include vestiges of GCT.There are few publications on testicular GCT tumour regression and those that exist present limited data on the biology of the disease and its etiopathogenesis. This entity was recently recognised in the latest edition of the World Health Organization’s (WHO) Classification of Tumours.We present our clinical, imaging, laboratory, cytohistological and management experience, as well as a historical review of the literature.

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Section: Discussionmentioning

confidence: 99%

Spontaneous testicular tumor regression: case report and historical review

Astigueta

Abad-Licham

Agreda³

et al. 2018

ecancer

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“…3–5 Patients have been described who have seminoma-associated paraneoplastic encephalitis but do not have IgG autoantibodies to Ma2 or to any other antigen and do not have specific immunofluorescence staining patterns on central nervous system tissue obtained from mice. 6–8…”

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confidence: 99%

Kelch-like Protein 11 Antibodies in Seminoma-Associated Paraneoplastic Encephalitis

Mandel‐Brehm¹,

et al. 2019

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SUMMARY A 37-year-old man with a history of seminoma presented with vertigo, ataxia, and diplopia. An autoantibody specific for kelch-like protein 11 (KLHL11) was identified with the use of programmable phage display. Immunoassays were used to identify KLHL11 IgG in 12 other men with similar neurologic features and testicular disease. Immunostaining of the patient’s IgG on mouse brain tissue showed sparse but distinctive points of staining in multiple brain regions, with enrichment in perivascular and perimeningeal tissues. The onset of the neurologic syndrome preceded the diagnosis of seminoma in 9 of the 13 patients. An age-adjusted estimate of the prevalence of autoimmune KLHL11 encephalitis in Olmsted County, Minnesota, was 2.79 cases per 100,000 men.

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“…Paraneoplastic syndrome secondary to metastatic germ cell neoplasm from a “burned-out” testicular tumor is a rare occurrence ( 7 , 8 ), but this case underscores the importance of a high clinical index of suspicion for testicular malignancy in men with symptoms of progressive cerebellar ataxia and limbic encephalitis.…”

Section: Discussionmentioning

confidence: 86%

“…The cause of primary lesion regression is not clear and might be due to ischemia or an immune process ( 6 ). Paraneoplastic syndromes have been described in association with germ cell tumors; however, such a finding in the presence of a “burned out tumor” is an extremely rare occurrence with only two case reports to date to the best of our knowledge ( 7 , 8 ). In one of them, diagnosis was only achieved after 4 years despite an extensive workup.…”

Section: Discussionmentioning

confidence: 99%

Metastatic “Burned Out” Seminoma Causing Neurological Paraneoplastic Syndrome—Not Quite “Burned Out”

et al. 2018

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A 44-year-old man presented with cerebellar ataxia and limbic encephalitis and was ultimately diagnosed with metastatic germ cell neoplasm resulting from a “burned out” primary testicular tumor. The patient had progressive ataxia, leading to a thorough investigation for infectious, autoimmune, metabolic, and malignant causes of acquired cerebellar ataxia that revealed no significant findings. Testicular sonography demonstrated a possible right testicular lesion that was not confirmed on radical inguinal orchiectomy. F18-FDG positron emission tomography/computerized tomography scan revealed a solitary retroperitoneal lesion, concerning for metastatic disease but not amenable to percutaneous biopsy. A robotic retroperitoneal lymph node dissection was performed and pathology revealed a CD117-positive metastatic seminoma leading to appropriate germ cell tumor-directed chemotherapy. After completing chemotherapy and during 1 year of follow-up, there has been a gradual improvement of the patient’s neurological manifestations.

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Paraneoplastic neurological syndrome due to burned-out testicular tumor showing hot cross-bun sign

Abstract: Seminoma can cause PNS showing 8th cranial nerve palsy, cerebellar, and brainstem atrophy with hot cross-bun sign on MRI study. Extensive screening for onconeural antibodies was negative and thereby suggested that unknown antibodies worked for both antitumor immunity and induction of PNS.

Cited by 24 publications

References 17 publications

Spontaneous testicular tumor regression: case report and historical review

Spontaneous testicular tumor regression: case report and historical review

Kelch-like Protein 11 Antibodies in Seminoma-Associated Paraneoplastic Encephalitis

Metastatic “Burned Out” Seminoma Causing Neurological Paraneoplastic Syndrome—Not Quite “Burned Out”

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