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RATIONALE: POEMS syndrome is a rare paraneoplastic syndrome, manifesting with progressive distal polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes, which has multisystem manifestations and chronic cytokine overproduction. Pituitary abscess is also a rare life-threatening disease that could lead to panhypopituitarism. We report a case of pituitary abscess with high suspicion of POEMS syndrome, which to our knowledge has not been reported previously. PATIENT CONCERNS: The co-presentation of pituitary abscess and POEMS-like syndrome is rare and its clinical manifestations are complex, making it easy to miss diagnosis and misdiagnosis. Long-term disease management and follow-up is crucial under this condition. DIAGNOSIS: The patient was admitted to outside hospital due to headache, impaired consciousness, and fever since April 2024. Initial test results suggested neuroinfection. Endocrine abnormalities revealed diabetes insipidus and panhypopituitarism, including central hypocortisolism, hypogonadism, and hyperprolactinemia. Pituitary-enhanced MRI revealed abnormal pituitary signals on T1- and T2-weighted images, with peripheral rim enhancement noted after gadolinium injection. Further examination revealed lymphadenopathy, polyneuropathy, skin changes, monoclonal gammopathy (lamda chain, M-protein), ascites, meeting the diagnostic criteria for POEMS syndrome. Bone marrow core biopsy showed no malignancy. Despite the normal VEGF level, the patient was diagnosed with pituitary abscess and possibly POEMS syndrome due to other clinical presentations. INTERVENTIONS: The patient underwent transsphenoidal resection of the pituitary gland lesions, antibiotic treatment and hormone replacement therapy with hydrocortisone and desmopressin. OUTCOMES: At 9 months of follow-up, the patient experienced recurrent fever, headache, and diabetes insipidus after discontinuation of hormone therapy. LESSONS: The diagnosis of complex comorbidities is based on the combination of clinical manifestations and laboratory tests, multidisciplinary and comprehensive assessment are of great significance for this disease.

BACKGROUND: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes (POEMS) syndrome is a rare paraneoplastic disorder driven by an underlying plasma cell dyscrasia. While renal involvement is common, it typically manifests after neurological or dermatological symptoms. Chronic glomerulonephritis-like manifestations as the sole initial feature for a prolonged period are exceptionally rare and can lead to delayed diagnosis. CASE PRESENTATION: A 38-year-old woman presented with a 1-year history of foamy urine and a 1-week history of lower limb muscle pain. She had previously been treated with irbesartan for presumed chronic glomerulonephritis. Two months prior to admission, she developed lower limb numbness, followed by progressive muscle pain. Physical examination revealed cervical lymphadenopathy and left-sided cardiomegaly. Laboratory investigations demonstrated proteinuria, mild renal impairment, and a serum monoclonal immunoglobulin A (IgA)-λ protein on immunofixation electrophoresis. Nerve conduction studies confirmed severe demyelinating polyneuropathy. Imaging revealed pericardial effusion, splenomegaly, and bilateral renal enlargement. Following multidisciplinary consultation, subsequent investigations revealed markedly elevated serum vascular endothelial growth factor (VEGF) and patchy, slightly high-density shadows in bilateral iliac bones on abdominal computed tomography (CT), which supported the definitive diagnosis of POEMS syndrome. At follow-up, the patient was initiated on lenalidomide and dexamethasone therapy and is currently undergoing autologous stem cell transplantation at a tertiary care center, showing significant symptomatic improvement. CONCLUSION: In patients with an unexplained chronic glomerulonephritis-like presentation that is refractory to standard therapy, POEMS syndrome should be considered in the differential diagnosis, even in the absence of typical skin or bone changes. Early serum immunofixation and VEGF assessment are critical for avoiding a delayed diagnosis.

POEMS is an uncommon paraneoplastic plasma cell disorder known with the expansion of polyneuropathy, organomegaly, endocrine abnormalities, M-band, and skin changes. We report a patient presenting with monoarthritis and weight loss for 5 months, who was treated empirically for tuberculous arthritis and later developed debilitating axonal polyneuropathy due to delayed diagnosis. POEMS/Plasma cell dyscrasias rarely involve joints, and this case highlights this unique presentation of POEMS syndrome. Bone biopsy from the affected joint revealed the diagnosis. Chronic large-joint monoarthritis with systemic symptoms, mimicking tuberculous arthritis, and polyneuropathy is a unique combination-so clinicians should consider an underlying plasma cell dyscrasia/POEMS to avoid delayed diagnosis.

INTRODUCTION: POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome is a rare paraneoplastic disorder driven by proinflammatory cytokines, particularly vascular endothelial growth factor (VEGF), which promotes multisystemic angiogenesis and inflammation. It is frequently misdiagnosed as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) due to overlapping features. CASE REPORT: A 63-year-old woman was referred for presumed CIDP unresponsive to IVIG and steroids. Her clinical course was notable for 100-pound weight loss, papilledema, skin changes, thrombocytosis, thromboembolisms, hemi-diaphragmatic paralysis, organomegaly/lymphadenopathy, and negative bone marrow and lymph node biopsies. POEMS was diagnosed based on repeat electrodiagnostic testing, elevated VEGF and prolactin, and nerve/muscle biopsies showing endomysial edema and angiogenesis. Notably, no monoclonal protein (M-protein) was identified. Treatment with lenalidomide and dexamethasone resulted in significant clinical improvement. CONCLUSION: Paraprotein-negative POEMS syndrome should be considered in refractory CIDP, especially in the presence of skin changes, endocrinopathy, papilledema, and thrombocytosis. VEGF levels and nerve/muscle biopsy findings are critical diagnostic tools in patients with high clinical suspicion who lack monoclonal gammopathy. Muscle biopsy may increase the diagnostic yield by revealing characteristic vascular proliferation, a feature previously described in nerve biopsies of patients with POEMS syndrome.