Abstract
POEMS syndrome is a rare paraneoplastic syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The diagnosis of this disease is often challenging due to its rarity and its multisystemic involvement. Isolated adrenal tuberculosis is also a rare disease which is also difficult to diagnose. Therefore it is incredibly challenging to diagnose a patient with POEMS syndrome when he has isolated adrenal tuberculosis. In this report, we describe a 43-year-old man suffering from POEMS syndrome with right adrenal tuberculosis. His symptoms were very complicated and initially we misdiagnosed him with endocrinal disturbances and the adverse effects of antituberculosis drugs. It was not until we accumulated enough evidences of POEMS syndrome that we could make the final diagnosis. To the best of our knowledge, this is the first report of POEMS syndrome with isolated adrenal tuberculosis.
Highlights
POEMS syndrome is a rare paraneoplastic syndrome whose acronym represents some of the defining features of this disease, including polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
The therapy of POEMS is mainly classified into two major parts: the first part is targeting the plasma cell clone, which includes radiation, chemotherapy, autologous peripheral blood stem cell transplantation (ASCT); the second part is targeting the rest of this syndrome, such as the elevated vascular endothelial growth factor (VEGF) [8]
The computed tomography (CT) scanning, MRI (Magnetic Resonance Imaging) and histopathological examination of the suspected adrenal lesions are of great help in diagnosis [10,11]
Summary
POEMS syndrome is a rare paraneoplastic syndrome whose acronym represents some of the defining features of this disease, including polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The other important features involve papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated vascular endothelial growth factor (VEGF), and abnormal pulmonary function [1]. The syndrome is a manifestation of an underlying plasma cell disorder and its pathogenesis currently remains unclear [2]. The therapy of POEMS is mainly classified into two major parts: the first part is targeting the plasma cell clone, which includes radiation, chemotherapy, autologous peripheral blood stem cell transplantation (ASCT); the second part is targeting the rest of this syndrome, such as the elevated VEGF [8]. Isolated adrenal tuberculosis is a rare disease. The computed tomography (CT) scanning, MRI (Magnetic Resonance Imaging) and histopathological examination of the suspected adrenal lesions are of great help in diagnosis [10,11]
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