Gruber-Frantz tumor: a very rare pathological condition in children

Solid pseudopapillary tumor of the pancreas or Gruber-Frantz tumor is a rare pathological entity. Its incidence is even lower in children. This neoplasm has low malignant potential and with adequate surgical treatment has a very good prognosis. We present the case of a 15-year-old girl admitted in our department for an intra-abdominal tumor, diagnosed incidentally on ultrasonography after a minor trauma. The CT scan revealed an important mass, apparently localized in the liver. Intraoperative assessment found the tumor originating from the pancreatic head (12 cm in diameter), well defined, with reddish-brown color, well-vascularized and heterogeneous consistency. It was completely removed without sacrificing other tissues. The postoperative evolution was uneventful. The microscopic feature of the tumor, using Hematoxylin and Eosin staining was characteristic. Immunohistochemistry confirmed the diagnosis. Four years postoperative follow-up showed no recurrence. Only several hundred solid pseudopapillary tumors of the pancreas are described in the literature. In children, the incidence of this tumor is extremely rare. Some investigators believe it is originated from pluripotent primordial stem cells. The clinical picture is non-specific. Despite its rarity, this type of tumor should be taken into account in the differential diagnosis of abdominal masses in children, especially in girls. The diagnosis is made only histologically. Complete surgical excision is curative.