Chuanyan Mao

Papillary cystic and solid tumors of the pancreas: A pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature

BACKGROUND The papillary cystic and solid tumor of the pancreas is rare. It occurs predominantly in young women, and most present a benign behavior. The pathogenesis of this tumor has attracted a number of investigations but remains unclear. METHODS We present three patients with this tumor and a review of 289 others from the worlds literature, a total of 292 cases. On the basis of the analyses of the clinical and pathologic features from the reported cases, the pathogenesis of this unusual tumor has been further explored. RESULTS Ninety percent of the patients were female, with a mean age of 23.9 years. The tumors were usually quite large with a mean diameter of 10.3 cm. Ninety-two percent of these tumors were totally or partially cystic. Rupture of the capsule resulted in hemoperitoneum in eight cases, five of which were without any identifiable cause. Forty-three tumors (14.7%) have been recognized as malignant. The overall prognosis has been excellent and an aggressive approach to resection is indicated. CONCLUSIONS The results of immunohistochemical staining and electromicroscopy were rather diverse, but most, including the current cases, support the hypothesis that the tumor originates from pleuripotential embryonic stem cells. Thus the term pancreatic embryonic tumors seems preferable to papillary cystic and solid tumor of the pancreas to delineate the origin of the tumor and to reflect some of its biologic characteristics.

Pancreatitis associated with neuroendocrine (islet cell) tumors of the pancreas

BACKGROUND The association of pancreatitis with neuroendocrine tumors of the pancreas is uncommon, whereas its association with exocrine pancreatic cancer is well recognized. Since the latter, but not the islet cell tumor, is thought to originate within the ductal system, it is not surprising that pancreatitis is less likely to result from the islet cell tumor. The senior author has recently noted that the first recognizable indication of an islet cell carcinoma may be one or more discrete attacks of acute pancreatitis. METHODS AND RESULTS Acute pancreatitis, resulting from an islet cell tumor, has been observed in five patients, in one of whom it had become chronic. In four of the patients, the tumor was malignant. A review of the English language literature reveals 14 such patients with pancreatitis, and 2 others have been noted in other languages (a total of 21 patients). The tumor was malignant in 15 of the 21 patients (71%). Although the development of pancreatitis was found to result from the obstruction of the duct in most patients, at least three seemed unrelated. In 12 of 21 cases (57%), an acute attack of pancreatitis, usually recurrent, was the initial syndrome, in contradistinction to pancreatitis resulting from exocrine tumors, which has usually been chronic in nature. CONCLUSION Pancreatitis, particularly acute pancreatitis, may result from an islet cell tumor. Although unusual, its occurrence may be the first signal of the presence of the tumor. In our experience, obstruction of the pancreatic duct by the islet cell carcinoma appears to be the important etiologic factor.

Carcinoma of the Ampulla of Vater and Mesenteric Fibromatosis (Desmoid Tumor) Associated with Gardner's Syndrome : Problems in Management

To date, 29 patients with Gardners syndrome have been identified who developed carcinoma of the ampulla of Vater, and many others have been identified with rnesenteric fibromatosis (desmoid tumor), but there have been only four cases of Gardners syndrome with both ampullary carcinoma and mesenteric fibromatosis reported in the world literature. This article focuses on the complications and major problems in management resulting from the simultaneous occurrence of these two known features of Gardners syndrome: carcinoma of the ampulla of Vater and fibromatosis (desmoid tumor) of the intestinal mesentery.

Glucagonoma Associated with Calculous Pancreatitis

A Patient with a glucagonoma associated with calculous pancreatitis is reported. The authors know of only one other such patient. Because the calculous pancreatitis developed both upstream and downstream from the tumor, it cannot be attributed to obstruction of the pancreatic duct by the tumor. Continued critical appraisal is needed to determine whether the association of the glucagonoma and pancreatitis is etiologically related or merely coincidental.

Letter to the editor cytosolic estrogen and progesterone receptors in exocrine pancreatic adenocarcinoma

ConclusionThe results of sex-hormone therapy have been negative or inconclusive, but the proposed interactions of the hormones on normal or malignant acinar cells continue to open new horizons. To date, however, neither the standard estrogen/progesterone receptor assays nor the therapeutic use of tamoxifen demonstrates any benefit to the patient with pancreatic exocrine adenocarcinoma. Nevertheless, the disease is so devastating that consideration of a limited trial of orchiectomy, oophorectomy, or even adrenalectomy might be justified. From the hormonal aspects of these cancers, believed to be mainly of ductal origin, perhaps a study of the relationship of secretin or even cholecystokinin metabolism to the cancer might open new horizons.

Case report solitary true cyst of the pancreas in an adult

SummaryA 35-yr-old woman was presented to the hospital because of nausea 20-30 min after eating, associated with weight loss. A CT scan revealed a cystic lesion in the neck of the pancreas. At operation, a 3-cm diameter cyst was excised from the pancreas. Endoscopic retrograde pancreatography (ERP) was used intraoperatively to identify the transected pancreatic duct, after which a Roux-Y pancreaticojejunostomy was performed. Histologically, the cyst proved to be unilocular and was lined with cuboidal epithelium. The natural history of this lesion is unknown as only four previous case reports of unilocular true cysts in adults have been reported in the English literature. These are reviewed.