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A Journal on Internal Medicine

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Minerva Medica 2008 June;99(3):341-5

language: English

Right leg swelling as primary presentation of metastatic Merkel cell carcinoma

Noto R. 1, Giaquinta A. 1, Alessandria I. 1, Soma P. 2, Latteri S. 3, Grasso G. 4, Fraggetta F. 4

1 Istituto di Medicina Interna Università degli Studi di Catania Azienda Ospedaliera Cannizzaro, Catania, Italia
2 Unità Operativa Clinicizzata di Chirurgia Plastica Azienda Ospedaliera Cannizzaro, Catania, Italia
3 Divisione Clinicizzata di Chirurgia d’Urgenza e Pronto Soccorso Azienda Ospedaliera Cannizzaro, Catania, Italia
4 Unità Operativa di Anatomia Patologica Azienda Ospedaliera Cannizzaro, Catania, Italia


Merkel cell carcinoma (MCC) is a rare malignant cutaneous neuroendocrine tumour with an aggressive behaviour and frequent regional lymph node and distant metastases. It mostly occurs in old patients and the commonest sites are the skin of the head, neck and the extremities. Typically, the primary tumour presents as a fast-growing, painless, reddish nodule with an iceberg-like effect, broadening in the depth. Although the pathogenesis of MCC remains largely unknown, ultraviolet radiation and immunosuppression are likely to play a significant pathogenetic role. The authors describe an unusual case of MCC clinically presenting as lymphedema on the right leg due to an inguinal lymphonodal metastasis. Although extensive investigations were performed the authors were unable to discover the cutaneous primary tumor. The authors examine the etiopathogenesis and hypothesis of this rare tumour and describe the clinical differential diagnosis. They suggest that clinical features together with imaging studies and morphological and immuno-histochemical findings are important for the correct diagnosis.

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