Plexiformer fibrohistiozytischer Tumor. Fallbericht: Ein seltener niedrig-maligner Tumor mit vorwiegendem Befall der oberen Extremität

A male child aged nine months was admitted due to a slow growing, painless resistance at the palmar aspect of the proximal phalanx of the index finger. The intraoperative aspect of the tumour showed a yellow to white softtissue mass with signs of infiltration. After special histopathological stainings, the diagnosis of a plexiform fibrohistiocytic tumour was confirmed. In literature almost 100 cases were reported since first description by Enzinger and Zhang in 1988 4 . In this paper, 41 of 65 described cases showed tumour localization in the upper limb. This is the second case ever published in Germany and the first with localisation to the hand. There is very little clinical experience with this tumour entity. Local recurrence, lymphatic and pulmonal metastases with lethal outcome in rare cases are described. In the presented case, after secondary radical excision of the tumour, the patient has been free of local recurrence and actually in complete remission for 14 months.