Bilateral diffuse uveal melanocytic proliferation(BDUMP)is a rare paraneoplastic ocular syndrome,often associated with significant vision loss and poor prognosis.BDUMP is typically linked to systemic malignancies,incl...Bilateral diffuse uveal melanocytic proliferation(BDUMP)is a rare paraneoplastic ocular syndrome,often associated with significant vision loss and poor prognosis.BDUMP is typically linked to systemic malignancies,including ovarian,lung,pancreatic,and colorectal cancers.Here,we reported a case of BDUMP in a 59-year-old male patient who had been misdiagnosed with a nonfunctional adrenal adenoma.This man,with a history of diabetes mellitus and high blood pressure,complained of progressive visual loss in both eyes.He was diagnosed with BDUMP after undergoing comprehensive ophthalmic assessments at our eye center.After being referred to the urology department,he was initially diagnosed with a right nonfunctional adrenal adenoma because his renin-aldosterone levels and serum potassium levels were normal.However,three months later,the adrenal mass rapidly increased in size,and liver metastasis was confirmed.Despite normal tumor marker levels,a histopathological diagnosis ultimately confirmed adrenocortical carcinoma.This case underscores the importance of considering underlying malignancies in patients with BDUMP and highlights the necessity for early systemic evaluation,including imaging studies and multidisciplinary consultations.Timely diagnosis of underlying cancers may improve patient outcomes.It is worth noting that normal hormone levels and a tumor small-sized cannot be used as conclusive evidence that the tumor is a nonfunctional adrenal adenoma;patients with BDUMP should be closely followed up to identify the primary malignancy.展开更多
The paraneoplastic retinopathies are a heterogeneous group of disorders with significant visual consequences occuring in the setting of a systemic malignancy.These conditions may be characterized by the presence of an...The paraneoplastic retinopathies are a heterogeneous group of disorders with significant visual consequences occuring in the setting of a systemic malignancy.These conditions may be characterized by the presence of antiretinal antibodies and may predate or follow the diagnosis of an underlying malignancy.Herein I review the clinical findings,pathophysiology,laboratory testing and management of the paraneoplastic retinopathies:cancer-associated retinopathy(CAR),melanoma-associated retinopathy(MAR),bilateral diffuse uveal melanocytic proliferation(BDUMP)and paraneoplastic vitelliform maculopathy(PVM).The pathophysiology of the paraneoplastic retinopathies varies from molecular mimicry resulting in anti-retinal antibody production(CAR,MAR)to relases of soluble factors either by the primary tumor(BDUMP)and/or immune system in response to the primary tumor(PVM)which result in retinal and/or retinal pigment epithelium dysfunction.For each condition,structural and functional multimodal retinal testing can be helpful in establishing the diagnosis.Treatment for the paraneoplastic retinopathies involves a combination of treating the underlying malignancy plus additional local and/or systemic immunosuppressive agents though no systemic therapeutic protocols exist.Despite these interventions,the retinopathy may be progressive or the retinopathy may be a harbinger of poor survival.Nevertheless,prompt diagnosis may help identify an underlying malignancy earlier and may thus improve cancer-related survival.展开更多
基金supported by the National Natural Science Foundation of China(81770971).
文摘Bilateral diffuse uveal melanocytic proliferation(BDUMP)is a rare paraneoplastic ocular syndrome,often associated with significant vision loss and poor prognosis.BDUMP is typically linked to systemic malignancies,including ovarian,lung,pancreatic,and colorectal cancers.Here,we reported a case of BDUMP in a 59-year-old male patient who had been misdiagnosed with a nonfunctional adrenal adenoma.This man,with a history of diabetes mellitus and high blood pressure,complained of progressive visual loss in both eyes.He was diagnosed with BDUMP after undergoing comprehensive ophthalmic assessments at our eye center.After being referred to the urology department,he was initially diagnosed with a right nonfunctional adrenal adenoma because his renin-aldosterone levels and serum potassium levels were normal.However,three months later,the adrenal mass rapidly increased in size,and liver metastasis was confirmed.Despite normal tumor marker levels,a histopathological diagnosis ultimately confirmed adrenocortical carcinoma.This case underscores the importance of considering underlying malignancies in patients with BDUMP and highlights the necessity for early systemic evaluation,including imaging studies and multidisciplinary consultations.Timely diagnosis of underlying cancers may improve patient outcomes.It is worth noting that normal hormone levels and a tumor small-sized cannot be used as conclusive evidence that the tumor is a nonfunctional adrenal adenoma;patients with BDUMP should be closely followed up to identify the primary malignancy.
文摘The paraneoplastic retinopathies are a heterogeneous group of disorders with significant visual consequences occuring in the setting of a systemic malignancy.These conditions may be characterized by the presence of antiretinal antibodies and may predate or follow the diagnosis of an underlying malignancy.Herein I review the clinical findings,pathophysiology,laboratory testing and management of the paraneoplastic retinopathies:cancer-associated retinopathy(CAR),melanoma-associated retinopathy(MAR),bilateral diffuse uveal melanocytic proliferation(BDUMP)and paraneoplastic vitelliform maculopathy(PVM).The pathophysiology of the paraneoplastic retinopathies varies from molecular mimicry resulting in anti-retinal antibody production(CAR,MAR)to relases of soluble factors either by the primary tumor(BDUMP)and/or immune system in response to the primary tumor(PVM)which result in retinal and/or retinal pigment epithelium dysfunction.For each condition,structural and functional multimodal retinal testing can be helpful in establishing the diagnosis.Treatment for the paraneoplastic retinopathies involves a combination of treating the underlying malignancy plus additional local and/or systemic immunosuppressive agents though no systemic therapeutic protocols exist.Despite these interventions,the retinopathy may be progressive or the retinopathy may be a harbinger of poor survival.Nevertheless,prompt diagnosis may help identify an underlying malignancy earlier and may thus improve cancer-related survival.
