Florid Pseudosarcomatous Polypoid Fibroplasia: An Unusual Cutaneous Manifestation of Chronic Lymphedema

Bizarre, reactive fibroblastic proliferations associated with localized lymphedema may arise with chronic condom catheter use. We report a unique presentation of innumerable, cutaneous nodules occurring in chronic lymphedema of the right lower extremity due to remote pelvic lymph node dissection and radiation therapy for squamous carcinoma of the uterine cervix. Histologically, the cutaneous nodules consisted of several bizarre, enlarged polygonal to stellate, multinucleated fibroblasts having variably vacuolated cytoplasm in a background of markedly edematous dermis with prominent vascular ectasia. Nuclear hyperchromasia, scattered mitotic figures and pleomorphism in the context of chronic lymphedema led to an original diagnosis of malignancy. However, on histologic re-review, close correlation with imaging studies revealed complete continuity of massive subcutaneous lymphedema with the overlying skin nodules. The presence of diffuse reactive changes of edema and ectasia associated with multinucleated fibroblasts enables recognition of this pseudosarcomatous lesion. To our knowledge, this is the first report of extreme, multiple, cutaneous polypoid lesions comprising bizarre fibroblasts that simulate sarcoma and occur in the setting of chronic lymphedema. Awareness of this entity is critical, as it is easily misdiagnosed as sarcoma complicating lymphedema. We propose the descriptive term florid pseudosarcomatous polypoid fibroplasia for such extreme lesions.