POEMS Syndrome with Biclonal Gammopathy: A Rare Association.

Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Here, we are describing the cases of a 60-year-old lady, presented with bilateral pedal oedema and pericardial effusion and peripheral neuropathy. She also had hepatosplenomegaly, hyperpigmented rash and hypothyroidism and hyperparathyroidism. The serum protein electrophoresis and the immunofixation electrophoresis revealed two distinct monoclonal bands, immunoglobulin IgG kappa and IgA lambda. There was a mild increase in plasma cells and sclerotic bone lesion in pelvis. The POEMS syndrome is generally associated with lambda light chain restriction. The presence of biclonal gammopathy involving kappa and lambda is a rare manifestation. The pathogenic or prognostic role of different paraprotein is not known. Further studies are required to delineate such effect.

Adrenocortical carcinoma presenting as a rupture and extensive retroperitoneal haemorrhage.

Adrenocortical carcinoma (ACC) is an extremely rare tumour. We are reporting a 45-year-male patient who had a history of sudden severe worsening epigastric pain and fullness in abdomen, with giddiness. The radiological investigations showed a large right suprarenal mass with extensive destruction and retroperitoneal haemorrhage, with extra capsular, periportal and liver metastases. Exploratory laparotomy was done for excisions of mass and surrounding tissue. On histopathological examination, diagnosis was given as Adreno Cortical Carcinoma with capsular, vascular, and soft tissue nodular involvement.