ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Aneonate delivered to a nondiabetic mother at 30 weeks’ gestation experienced symptoms of severe hypoglycemia within hours of birth. The neonate required a continuous high-glucose infusion rate to maintain euglycemia. Based on the finding of an inappropriately elevated plasma insulin level concurrent with low glucose levels, the diagnosis of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) was rendered. Medical treatment was ineffective in preventing hypoglycemia, necessitating surgical intervention. A 90% pancreatectomy was performed when the neonate was 16 days old. After surgery, the neonate recovered fully but later had insulin-dependent diabetes develop. Results of genetic studies were unremarkable. Histologic, immunohistochemical, and electron microscopic examinations of the pancreas demonstrated findings consistent with the diagnosis of the diffuse type of nesidioblastosis. The literature on nesidioblastosis is reviewed, and current theories regarding its origin, significance, and relationship to PHHI are discussed.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Lymphoplasmacytic sclerosing pancreatitis (LPSP), also known as nonalcoholic, duct destructive chronic pancreatitis or autoimmune pancreatitis, increasingly is recognized as a form of pancreatitis associated with autoimmune and fibroinflammatory lesions. The process often simulates pancreatic carcinoma because of the tumor-like nature of the fibrosis and the co-existence of jaundice caused by bile duct involvement. A case is reported of a 51-year-old woman with a mass in the tail of the pancreas with histologic features of LPSP exhibiting fibrotic pseudotumor formation. Although there were no autoimmune or fibroinflammatory diseases in her history, after distal pancreatectomy the patient experienced bilateral periorbital fibroinflammatory lesions (consistent with inflammatory pseudotumor of the orbit) and subsequently had recurrence of the process in the head of the pancreas accompanied by jaundice. The jaundice responded to steroid therapy. The histologic features in this case were characteristic of LPSP, with dense periductal inflammation rich in lymphocytes and plasma cells, ductal epithelial damage, focally dense fibrosis, and perivascular inflammatory cell aggregates (periphlebitis). The possibility of nonoperative therapy for LPSP can be considered, although reliable preoperative diagnosis currently requires a compelling clinical history of associated conditions.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

The cytologic separation of inflammatory and reparative atypia seen in pancreatitis from atypical cells in aspirates of well-differentiated pancreatic carcinoma can be challenging. Aspirates of pancreatitis can be cellular because of the presence of numerous inflammatory cells with necrotic debris in the background. Both pancreatic acinar cells and ductal cells can be cytologically atypical, but benign acinar cells maintain a cohesive lobulated arrangement and benign ductal cells are present in uniform, flat, honeycomb sheets. Single atypical cells usually are rare or not seen. In contrast, aspirates of well-differentiated pancreatic carcinoma contain cells with larger nuclei that are at least two times the size of normal ductal cells and demonstrate nuclear molding and variability in nuclear size defined by cells having nuclei at least three times the size of other atypical ductal cells within the same honeycomb group. Other cytologic features that can be seen in aspirates of pancreatic carcinoma include cells demonstrating nuclear grooves, pseudoinclusions, and macronucleoli. The most helpful feature for a carcinoma diagnosis is the presence of more than occasional individual, intact, atypical epithelial cells. The definitive separation is even more challenging when cells from both acute pancreatitis and pancreatic carcinoma are present in the same aspirate. The pathologist needs to raise the diagnostic threshold for adenocarcinoma when neutrophils are present in the smears, especially when seen infiltrating ductal groups, and withhold a definitive diagnosis of carcinoma when single atypical epithelial cells are not present.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Invasive pancreatic ductal (pancreatobiliary, ordinary ductal, tubular, scirrhous) adenocarcinoma (DA) is by far the most common tumor of the pancreas and thus is sometimes considered synonymous with “pancreas cancer.” In addition to its classical pattern (characterized by well-defined tubules lined by cuboidal cells), DA sometimes exhibits other morphologic patterns: in some cases, instead of medium size tubules, DA may form large ductal units (microcystic, large duct-type adenocarcinoma) mimicking intraductal neoplasia. Sometimes, the glands form a cribriform/vacuolated pattern in which the vacuoles are large and the nuclei compressed at the periphery of the cell, imparting to the cell an adipocyte-like appearance. DA also may grow in nests, or as individual cells forming indian files, simulating mammary lobular carcinoma. Poorly differentiated forms of DA (pleomorphic, anaplastic) may grow in sheets and may display a hepatoid pattern.The case presented here is an example of a relatively more common and morphologically distinctive variant of DA, the foamy gland pattern, which is deceptively benign appearing and prone to be misdiagnosed as a benign process. In fact, this patient had undergone surgery before this pattern was fully recognized, and the frozen section of the tumor was reported as negative. In this variant, infiltrative glands are well formed and lined by columnar cells that have abundant, pale cytoplasm. The nuclei often are well polarized to the base but wrinkled. The most characteristic features that help distinguish foamy gland patterns from benign mucinous ducts are (1) foamy/microvesicular, pale cytoplasm in which the vesicles are small, fine and evenly sized; and (2) a chromophilic condensation in the apical edge of the cytoplasm that forms a thin, well-delineated band reminiscent of a brush border (brush border-like zone or BLZ). Although the BLZ is strongly positive with mucin markers, the microvesicular component of the cytoplasm is negative (in contrast with the benign mucinous ducts, which often are PAS+). In addition, p53 often is overexpressed in the nuclei of these malignant glands.Recognition of these morphologic variants of DA is important to avoid misdiagnosis, especially in small specimens, and also is important for the differential diagnosis of metastatic tumors.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Intraductal papillary-mucinous tumor of the pancreas is a rare indolent tumor that most frequently presents in older men after a somewhat protracted course that clinically resembles chronic pancreatitis. The treatment of choice is surgical resection, after which patients generally do quite well. The authors present a case of intraductal papillary-mucinous adenocarcinoma with cytologic, gross, and histologic findings and a review of the literature.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Most solid pancreatic masses are ordinary ductal (pancreatobiliary, tubular) adenocarcinomas (DA), so “pancreas cancer” often is considered synonymous with DA. There exist other types of invasive carcinoma in the pancreas, although most have the same ominous prognosis as DA. However, recent studies have shown that colloid (pure mucinous, mucinous noncystic, muconodular) carcinomas have distinct clinical and pathologic characteristics. These tumors are characterized by invasive stromal mucin pools with scant malignant cells. They most often were classified as ordinary DA or sometimes misdiagnosed as signet-ring cell carcinoma or mucinous cystadenocarcinoma. Their clinical presentation is similar to that of DA, but more commonly the chief complaint is abdominal pain, rather than back pain. Frequently, they achieve large sizes (mean, 6.0 cm) by the time of diagnosis. Signet-ring cells often are present, but these cells invariably are confined to the stromal mucin without individual cell infiltration into the stroma. In more than half of the cases, they are associated with a low-grade, noninvasive tumor, namely intraductal papillary mucinous neoplasm. Rarely they arise from a mucinous cystic neoplasm. Just like their counterparts in the breast, colloid carcinomas may follow a protracted clinical course that is significantly better than that of DA, even in the cases with perineurial invasion or lymph node metastasis. Preliminary evidence suggests they also may have a different molecular pathogenesis. Thus, colloid carcinoma ought to be recognized as a distinct tumor in the pancreas. When encountered, the diagnosis should be conveyed clearly, and nonspecific terms such as “mucinous carcinoma” should be avoided (or followed by a more specific designation) because most pancreatic tumors of ductal origin have some degree of mucin formation.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Acinar cell carcinoma is a rare primary tumor of the pancreas that exhibits evidence of acinar differentiation in the form of enzyme production, in addition to having characteristic histologic features. A case is presented that demonstrates the uncommon lipase hypersecretion syndrome, characterized by subcutaneous fat necrosis caused by lipase secreted by the tumor cells. The presence in this case of the syndrome in the absence of hepatic metastases is unusual and perhaps is explained by the large size of the primary tumor (17 cm). The tumor exhibited the typical hypercellular pattern, with a solid and acinar architecture. An unusual degree of nuclear pleomorphism was focally noted. Enzyme production by the tumor cells was demonstrated immunohistochemically with stains for trypsin, chymotrypsin, and lipase, and electron microscopic analysis revealed zymogen granules. The patient lived 18 months after surgical resection, which is a typical course for acinar cell carcinoma, an aggressive tumor with a slightly better prognosis than ductal adenocarcinoma of the pancreas.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

Abdominal CT scan revealed a small cystic mass in the tail of the pancreas of a 63-year-old woman who was experiencing weight loss. A fine needle aspiration biopsy was diagnosed as an islet cell neoplasm. At resection, a 2.5-cm cavitary mass without central necrosis was excised and confirmed histologically as a purely cystic neuroendocrine tumor. Cystic islet cell neoplasms are among the rarest entities in the differential diagnosis of cystic mass lesions of the pancreas.

ISSN:1082-9784    

出版商:OVID    

年代:2001

数据来源: OVID