Pancreatoblastoma. Histopathologic Criteria Based upon a Review of Six Cases

Abstract

So-called infantile carcinoma of the pancreas was highlighted as an entity in 1959 by Frantz in the first series of the Armed Forces Institute Pathology fascicle [1]. This particular case had originally been reported by Becker [2] two years previously. Since that date and until the present, the histogenesis of this particular tumor, if indeed it is a single entity, has been controversial. The two principal theories have been a tumor of exocrine derivation versus a nonfunctioning islet-cell neoplasm. In 1971, Frable and co-workers [3] described a tumor in the pancreas of a 4-year old female with histologic patterns including nests, cord-like structures, solid foci with a squamous appearance and a delicate stroma with thin-walled vascular channels. The ultrastructural features were quite suggestive of a ductal origin with acinar cell differentiation. Microvillous projections and membrane-bound granules were some of the more notable findings. These authors designated the tumor ‘carcinoma of the pancreas, infantile type.’ In 1974, two pancreatic tumors in children were presented at the 20th Annual Meeting of the Japanese Pathological Society [4] with similar features as those reported by Frable et al. The term ‘pancreatoblastoma’ was proposed to indicate the primitive or blastomatous nature of the tumor in line with the better known solid embryonic neoplasms of childhood, i.e., nephroblastoma, neuro-blastoma and hepatoblastoma. Other authors have similarly equated the pancreatoblastoma and infantile type of pancreatic carcinoma [5, 6].