Neurology

Update: Atypical presentation of a midgut neuroendocrine tumor originally diagnosed as eosinophilic fasciitis




Here, we report an unusual clinical presentation of a neuroendocrine tumor (NET) that was initially diagnosed, treated, and reported in this journal as a case of eosinophilic fasciitis (EF).1 EF usually presents with the abrupt onset of symmetrical tenderness, erythema, and swelling of the extremities, followed by induration of the overlying skin and subcutaneous tissue. EF can be associated with a peripheral eosinophilia, hypergammaglobulinemia, and an elevated ESR. Histopathology findings include marked thickening of the fascia between the subcutis and muscle with dense infiltration by lymphocytes and plasma cells. Because of the rarity of this disease, there is no agreed therapeutic strategy. Corticosteroids remain the mainstay of treatment and may be combined with an immunosuppressive drug such as methotrexate.

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