Frantz's Tumor: A Papillary and Cystic Tumor of the Pancreas
in Girls
Takuji TodaniC Katsumasa Shimada, Yasuhiro Watanabe, Akira
Toki, Tadashi Fujii, Naoto Urushihara
J Pediatr Surg 23: 116-121, 1988
Four girls with Frantz's tumor, a papillary and cystic
tumor of the pancreas, are studied and discussed in comparison with 112
cases in the literature, including 58 Japanese cases. The neoplasms occur
predominantly in girls and young women. Up to recently, the tumors have
possibly been misclassified as nonfunctioning islet cell tumor or carcinoma,
acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma
according to microscopic findings and frequently have been managed with
aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated
and the cut surfaces show characteristically solid and hemorrhagic-necrotic
patterns. Ultrasonography and CT scan are the most useful tools for the
diagnosis. The neoplasms usually behave like a very low-grade malignancy,
so that complete removal is the treatment of choice for the tumor arising
anywhere in the pancreas. Immunochemical and electron microscopic studies
can differentiate Frantz's tumor from other neoplasms and also suggest that
the tumors originate from primordial cells or multipotential stem cells
capable of differentiating into both exocrine and endocrine lines.
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