Carcinoma of the Pancreas with Endocrine Component in Childhood: A Case Report

Ichijima, Kunio; Akaishi, Kyoji; Toyoda, N.; Kobashi, Yoichiro; Ueda, Yoshimichi; Matsuo, Shuji; Yamabe, Hirohiko

doi:10.1093/ajcp/83.1.95

Cited by 44 publications

(20 citation statements)

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“…The cells of the latter structures are thought to be epithelial in nature, and some authors described signs of keratinization in these "squamoid corpuscles" [5,13]. In our case we found neither keratinization nor positivity for a number of epithelial markers, such as CAM 5.2, KL1, Lu 5, CK7, CK14, CK19, CK20 and EMA.…”

Section: Discussioncontrasting

confidence: 72%

“…The age at diagnosis in these children ranged from birth to 8 years [2,3,4,5,6,10,11,12,13,14,23,24,25,26,29,30]. The only exception was a 37-year-old man who developed a metastasizing pancreatoblastoma [28].…”

Section: Discussionmentioning

confidence: 99%

“…4D), they could easily be distinguished from "squamoid corpuscles". Neuroendocrine cells have also been described in some pancreatoblastomas, but were never identified in "squamoid corpuscles" [4,13,30].…”

Section: Discussionmentioning

confidence: 99%

“…Pancreatoblastoma in infants appear to have a good prognosis if adequately treated [3,4,10,13,14,29]. Whether this is also true for the pancreatoblastomas occurring in adults is unclear.…”

Section: Discussionmentioning

confidence: 99%

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Pancreatoblastoma in an adult: its separation from acinar cell carcinoma

Hoorens¹,

Gebhard²,

Kraft

et al. 1994

Vichows Archiv A Pathol Anat

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Pancreatoblastomas are rare tumours, which usually occur in childhood. Here we describe a pancreatoblastoma in a 39-year-old woman. The tumour was located in the tail of the pancreas and consisted of cells forming well-differentiated acinar structures and scattered solid components ("squamoid corpuscles"). Immunocytochemically, the acinar components were positive for pancreatic enzymes and pancreatic stone protein, while the cells of the "squamoid corpuscles" lacked these markers. There was no p53 overexpression nor any mutation at codon 12 of the Ki-ras oncogene. The main differential diagnosis of this tumour was acinar cell carcinoma, because both tumours have a number of features in common (scattered solid components, positivity for pancreatic enzymes, lack of p53 overexpression and of Ki-ras mutation). Findings which distinguished the pancreatoblastoma and separated it from acinar cell carcinoma were the negativity of the solid components ("squamoid corpuscles") for neuroendocrine markers and their very weak keratin positivity. As the patient is alive and well 30 months after tumour resection, this pancreatoblastoma also differs in biology from the usual acinar cell carcinoma.

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Section: Discussioncontrasting

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Pancreatoblastoma in infants appear to have a good prognosis if adequately treated [3,4,10,13,14,29]. Whether this is also true for the pancreatoblastomas occurring in adults is unclear.…”

Section: Discussionmentioning

confidence: 99%

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Pancreatoblastoma in an adult: its separation from acinar cell carcinoma

Hoorens¹,

Gebhard²,

Kraft

et al. 1994

Vichows Archiv A Pathol Anat

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“…The latter represents the major differential diagnosis of pancreatoblastoma. The histogenesis of both tumours remains uncertain: acinar and ductal derivations have been proposed, and occasionally endocrine features have been noted in either tumour [2, 9]. As a possible explanation of these findings, each tumour is believed to derive from primitive cells of pancreatic differentiation [2, 10].…”

Section: Discussionmentioning

confidence: 99%

Pancreatoblastoma: Ultrastructural and Image DNA Cytometric Analysis

et al. 2001

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Background: Since the first description of a pancreatoblastoma as a malignant pancreatic tumour of childhood in 1957, approximately 60 cases have been reported. We present the symptoms, pathology, and therapy of this rare tumour in a 17-year-old girl. Case Report: The patient initially presented with upper gastro-intestinal bleeding. During laparotomy a 16-cm (diameter) tumour was recognized in the pancreatic tail. Open biopsy and radical resection, followed by histological and immunohistochemical examinations, confirmed the diagnosis of a pancreatoblastoma. The results of single-cell DNA cytometry underlined the low DNA grade of malignancy of the primary tumour. In spite of adjuvant chemotherapy with cisplatin and adriamycin, the patient returned 22 months later with many hepatic and peritoneal metastases measuring up to 5 cm in diameter. Peritoneal tumour debulking and an extended hemihepatectomy were performed as a palliative treatment. Unfortunately, the patient died 18 months later from further tumour progression. Conclusions: A review of the literature reveals that a pancreatoblastoma in childhood has to be considered malignant, but usually shows a favourable prognosis in contrast to pancreatic neoplasms in adult patients. The treatment of choice is radical resection. Adjuvant chemotherapy or radiotherapy should be considered because of the metastatic potential of the tumour.

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