BackgroundHead and neck paragangliomas are rare tumours and can arise as a part of inherited syndromes. Their association with thymic tumour is not well known.Case descriptionThis report describes a female patient who presented with right sided neck paragangliomas. The histology of the tumour was consistent with paraganlioma. Few years later her MRI scan of the chest revealed presence of an anterior mediastinal mass that corresponded to the location of the thymus. Review of her previous scans showed that the mass was present all along and had gradually increased in size. Patient developed symptoms including fatigue, dyspnoea, migratory polyarthritis, Raynaud’s phenomenon and erythema nodosum. She had sternotomy and excision of mediastinal mass. The histology was consistent with cortical thymoma (WHO type B2) and she had radiotherapy. After treatment her constitutional symptoms improved. Her paraganglioma susceptibility genes are negative.Discussion and evaluationTo our knowledge this is only the second case report in the literature of coexistence of carotid body tumour and thymoma. The first case reported was bilateral carotid body tumour, thyroid gland adenoma and thymoma. This case also highlights the importance of long term surveillance, multidisciplinary management and being aware of associated pathologies in patients with isolated paraganglioma.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome due to underlying plasma cell disorder. It belongs to group of plasma cell dyscrasias such as multiple myeloma, waldenstrom’s macroglobulinemia, castleman disease and monoclonal gammopathy of undetermined significance. The term POEMS syndrome was first coined in 1980 by Bardwick. In the last 10 years clinical criteria to diagnose POEMS syndrome have been elaborated to avoid overlap with other haematological disorders. Due to disease rarity there is very few case series and case reports described in literature. This is a haematological disease which frequently presents with peripheral neuropathy but endocrine dysfunction can be a presenting or an associated feature. We report two cases of POEMS syndrome who presented with endocrinopathy as a predominant feature with literature review of this syndrome
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