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Clinical and histological findings of pancreatic carcinoma in a 6-year-old boy are reported. Gradual change of histological appearance of the tumor during his course of 3 years and elevation of serum alpha-1-fetoprotein (AFP) are documented. Two biopsy specimens showed immature histological appearance compatible with pancreatoblastoma, and autopsy material showed well-differentiated adenocarcinoma with distinct ductal and acinar differentiation. Electron microscopy demonstrated zymogen-like granules in the apical portion of the neoplastic cells. Immunoperoxidase method demonstrated AFP in the neoplastic cells in addition to alpha-1-antitrypsin. Literature of pancreatic tumor in the young was reviewed, and characteristics of this case were discussed. ACTA PATHOL. JPN. 35 : 1543–1554, 1985.

A case of pancreatoblastoma in a 4-year-old boy is presented. This tumor was studied by histochemical and ultrastructural techniques and was found to have both exocrine and endocrine components. This analysis and a review of the literature helps to further define this entity, which has a significantly better prognosis than other pancreatic malignancies.

The association of pancreatoblastoma and Wiedemann-Beckwith syndrome has not been noted previously. In this report we describe a child with Wiedemann-Beckwith syndrome who had a pancreatoblastoma resected on day 27 of life. He is also the first reported case of Wiedemann-Beckwith syndrome in a black baby.

We present a male newborn (weight 4000 g) who died at age 12 days with a clinical history of persistent hypoglycemia and polycythemia. Clinical examination disclosed somatic hemihypertrophy (left s...

Based on histopathological examination of 264 exocrine pancreatic tumours (167 autopsy and 97 surgical) from the files of the Institute of Pathology, University of Hamburg, over a 15-yr period (1966-1980), a histogenetic classification is proposed. In addition to the more common neoplasms this also includes rarer and more recently defined entities. Of the 264 tumours, 250 were of duct origin, 10 acinar and four of uncertain histogenesis. Ductal adenocarcinoma, subdivided into a well-differentiated and a poorly-differentiated type, was most frequent (81.1%), followed by its variants: pleomorphic giant cell carcinoma 5.3%, adenosquamous carcinoma 3.8%, and mucinous carcinoma 1.1%. All these had a poor prognosis. Serous cystadenoma (1.1%), mucinous cystic tumour (1.5%) and intraductal papilloma (0.8%), which were rare tumours and mostly apparent in surgical material, proved to be benign or of only latent malignancy. The group of tumours of acinar cell origin consisted of the solid and cystic tumour (2.7%) with favourable prognosis and the acinar cell carcinoma (1.1%). No pancreatoblastoma was observed. The pleomorphic carcinomas of the small cell type (1.5%) were classed as tumours of uncertain histogenesis.

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.

Two cases of infantile carcinoma of the pancreas were diagnosed as pancreatoblastoma based on the morphogenesis of the tumors. These encapsulated tumors adhered to the head of the pancreas and to the descending portion of the duodenum. Histologic examination revealed an organoid structure made up of cords or nodules of squamoid cells with elongated nuclei arranged in a parallel fasciculating pattern (squamoid corpuscles), surrounding tubular structures of columnar epithelial cells and intermediate light cell masses with little differentiation. Electron microscopy revealed zymogen-like granules and well developed granular endoplasmic reticulum in the cytoplasm. There were no detectable islet cells in tumor tissue. Both of these tumors could be derived from the ventral pancreas and be isolated by the lack of communication with the duct of Wirsung. As the duct of Santorini was patent, extirpation of these organoid tumors would not influence secretion of pancreatic juice. Considering the favorable prognosis after extirpation of these tumors, they should be differentiated from the usual adenocarcinoma of the pancreas occurring in adults.

A case of primary pancreatic carcinoma confirmed by postmortem examination in a 15-year-old girl is presented. The tumor was studied by light and electron microscopy and an indirect immunoperoxidase technique. Some of the tumor cells contained eosinophilic, PAS-positive, diastase-resistant granules. Electron microscopy revealed large electron-dense granules resembling zymogen granules. The granules seen in the islet cell tumors were not demonstrated by electron microscopy and immunoperoxidase technique. These findings suggest that the tumor is of duct cell origin with some differentiation toward the acinar cells. The tumor appears to belong to the so-called "pancreatoblastoma".

A case of pancreatoblastoma, arising in the tail of the pancreas with metastases to the right radius, in an 8-year-old boy is reported. The serum alpha-fetoprotein (AFP) level was over 13 times the normal value before surgery, but returned to normal after removal of the primary tumor. Furthermore, AFP was detected in tumor tissue by immunohistochemistry. This case, representing the second primary non-germ cell pancreatic neoplasm in a child producing elevated serum AFP, supports the use of serum AFP in diagnosing this lesion.

The authors report a case of recurrent paacreatoblastoma in a 3-year-old girl who was successfully treated with radiotherapy. The patient had two local tumor recurrences before radiation treatment; the second recurrence was managed with radiation alone. Computerized tomography scan at the completion of radiotherapy showed dramatic tumor regression. Six weeks after radiotherapy was finished exploratory laparotomy was performed; multiple biopsies showed no evidence of tumor. The patient is currently disease-free 2 years after completion of radiotherapy. This case suggests a role for radiation therapy in the management of children with paacreatoblastoma.

We describe a boy with recurrent pancreatoblastoma who developed Cushing's syndrome due to inappropriate adrenocorticotrophic hormone secretion.

The literature describes 15 cases of congenital pancreatoblastoma (PB): 5 had prenatal diagnosis, none had metastases at diagnosis, 7 were associated with Beckwith–Wiedemann syndrome (BWS). In 13 cases resection was radical, while in 2 there were macroscopic residues. Only one patient underwent chemotherapy after distant recurrence. All children are alive except one who died because of problems related to BWS. Our goal is to describe the approach adopted in an infant with congenital PB treated in our center. After a prenatal third semester diagnosis of abdominal anechoic lesion, the radiological investigations (ultrasound, MRI) performed at birth described a cystic lesion of unclear nature. We proceeded to laparoscopic exploration, transformed into open approach after the detection of a lesion located in the body of the pancreas; this lesion was resected, preserving the head and tail of pancreas. The histological diagnosis showed a completely excised PB. After excluding metastatic lesions, we decided to perform only careful follow-up without chemotherapy. The follow-up at 12 months is negative. Although PB is a malignant tumor that requires a multidisciplinary treatment, the congenital cases seem to have a less aggressive biological behavior. The treatment, therefore, in case of complete resection, could be only surgical, followed by a careful follow-up. These forms are often associated with congenital BWS, but in our case the patient did not have the typical characteristics of the syndrome.

Twelve cases of pancreatoblastoma, 7 males and 5 females, were 5 years of age in average and showed an organoid structure consisting of acinar differentiation with squamoid corpuscles. Seven cases died, but 5 lived after surgery. On the contrary, 17 cases of papillary cystic tumor, ductuloacinar adenoma of the pancreas, one male and 16 females, were 21 years of age in average and disclosed a monomorphous pseudopapillary pattern of ductuloacinar type of tumor cells with some degenerative and granulomatous changes. All of the cases had suffered no recurrence after resection. Immunohistochemically, strong positivity for alpha 1-antitrypsin may be associated with the autodigestive process and limited growth of this tumor. Electron microscopically, both tumors showed centroacinar and acinar types of tumor cells. Both tumor cells frequently contained well-developed RER, zymogen-like granules, and annulate lamellae. The ductular or acinar lumina were clear in pancreatoblastoma, but not in ductuloacinar adenoma due to degeneration. Though both tumors disclosed contiguous histogenesis, pancreatoblastoma should be differentiated from ductuloacinar adenoma, based upon the young age, almost equal sex ratio, unfavorable prognosis, high serum AFP level in three measured cases, an organoid structure containing frequent mitotic figures, and the invasive growing margin.

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].

Nine acinar cell carcinomas of the pancreas, 2 pancreatoblastomas, 16 solid-cystic (papillary-cystic) tumors, and 20 ductal adenocarcinomas were immunocytochemically investigated using antisera against four pancreatic enzymes (alpha-amylase, lipase, trypsinogen, chymotrypsinogen), four pancreatic hormones, neuron specific enolase (NSE), alpha-1-antitrypsin (AAT), carcinoembryonic antigen (CEA), and CA 19–9. Lipase, trypsinogen, and chymotrypsinogen, but no alpha-amylase were detected in all acinar cell carcinomas and pancreatoblastomas. In contrast, solid-cystic tumors (SCT) were negative for pancreatic enzymes but 2 of 16 stained with NSE. No neuroendocrine granules or pancreatic hormones could be demonstrated. AAT was found in all tumors except ductal adenocarcinomas, which stained with CEA and CA 19–9. The study established pancreatic enzymes (except alpha-amylase) as immunocytochemical markers for acinar cell carcinomas and pancreatoblastomas. There is as yet no marker specific for SCT, which would elucidate the obscure histogenetic origin and phenotypic differentiation of these tumors. Cancer 59:729-747, 1987.

The macroscopic, light microscopic and ultrastructural findings in a case of pancreatoblastoma in a neonate are presented. No evidence for exocrine or endocrine secretion could be found by histochemistry, immunocytochemistry or electron microscopy. However, enzyme histochemical techniques to localize activity of 'glucose-6-phosphatase', acid phosphatase, esterase and esteroprotease supported both a pancreatic origin for the tumour and a biphasic pattern of differentiation.

A 3-week-old infant presenting with jaundice and an abdominal mass was found to have juvenile adenocarcinoma of the pancreas (pancreatoblastoma). A pancreato-duodenectomy was performed, and the child is alive, well, and free of disease at age 4 years. The operative management and surgical technique are discussed.

I. Selected Topics.- 1. Role of natural killer (NK) cells in resistance to tumors.- 2. Clonal evolution and childhood tumors.- 3. Cancer cell differentiation.- 4. Clinical significance of chromosome abnormalities in childhood and adult leukemia.- II. Pancreatic Malignancies.- 5. Genetic aspects of endocrine neoplasia.- 6. Tumors of the exocrine pancreas in childhood.- 7. Pre-operative evaluation of pancreatic tumors.- 8. Surgical therapy in pancreatic malignancies in children.- 9. Pancreatic carcinoma in children in Japan-Review of the Japanese literature.- 10. Pancreatoblastoma. Histopathologic criteria based upon a review of six cases.- 11. Solid and papillary epithelial neoplasms of the pancreas.- 12. Ectopic Cushing's syndrome in an adolescent.- 13. Low grade papillary pancreatic neoplasm in an adolescent female.- 14. Cancer of the pancreas in children: Experience at the M.D Anderson Hospital and Tumor Institute.- 15. A case of metastatic insulinoma in an adolescent girl.- 16. Experience with endocrine and exocrine neoplasias in children.- 17. Childhood rare endocrine tumors.- 18. Overview of childhood pancreatic tumors.

Context Pancreatoblastoma is a rare neoplasm in adults with a total of only 24 cases that have been reported in the literature. Adult pancreatoblastomas are large tumors and majority are larger than 8 cm at the time of diagnosis. Metastasis is seen in 26% of adults and usually involves the liver and then the lymph nodes. Metastasis is usually observed in cases where the primary tumor measures more than 10 cm. Pancreatoblastoma is named after its resemblance to fetal pancreatic tissue in the seventh week of life. The presence of squamoid corpuscles with a morular appearance is the most characteristic feature of the tumor. Pancreatoblastomas can have mixed features of both endocrine and exocrine cells; however, acinar differentiation is the most prevalent feature. Case report We present a case of a 27-year-old female with a 3.6 cm pancreatoblastoma with metastasis to the liver and lungs as well as to the breast. This case has several distinguishing features from previously reported cases. Such widespread metastasis is unusual given the small size of the primary tumor. Also, metastasis to the breast from a pancreatoblastoma has been previously undescribed in literature. The histological features in our case of pancreatoblastoma were atypical, characterized by the absence of acinar component, supported by the lack of staining for both trypsin and lipase in the tumor, which has not been described in literature. Additionally, the nests of squamous cells in this tumor had a pilomatricoma like morphology as opposed to the morular appearance of the squamoid corpuscles seen in classical cases. Conclusion Pancreatoblastoma can have an atypical clinical picture and a small primary with extensive metastasis to unusual sites may present a diagnostic challenge. Given its rarity, a high index of suspicion is required to correctly diagnose this condition. The histology reported on this case is unique and has not been reported in the literature., JOP. Journal of the Pancreas, Vol 13, N° 3 (2012): May - p. 252-329