Kitai Kim

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.

The Uterine Corpus

During the reproductive years, the size and weight of a normal uterus vary according to parity. Nulliparous uteri weigh 40 to 100 g and measure about 8 cm in length, 5 cm in width at the fundus, and 2.5 cm in anteroposterior thickness. Muciparous uteri weigh up to 250 g and measure 12 cm × 5–7 cm × 3.5 cm.

Overexpression of HER2/neu oncogene in pancreatic cancer correlates with shortened survival

SummaryFor the purpose of determining the prognostic significance of HER2/neu oncogene in pancreatic and ampullary cancers, 21 pancreatic cancers of ductal origin and six cancers of the ampulla of Vater were studied immunohistochemically using the monoclonal antibody (MAb) CB11, specifically reactive with HER2/neu product. Staining of the epithelium of the normal duct and acini was negative or weakly positive. Moderately and strongly positive reactions indicated the overexpression of this gene, and were found in 10 of 21 (47.6%) pancreatic cancers of ductal origin and in 2 of 6 (33.3%) ampullary adenocarcinomas. Overexpression of HER2/neu was closely and inversely related to the survival of the patients with pancreatic cancer of ductal origin: 19.1±11.7 mo for those not overexpressing vs 7.3±3.8 mo for the overexpressors (p<0.01). Among the pancreatic cancer group, 11 patients underwent cancer resection. The average survival for the 7 with nonoverexpressing cancer was 21.4±14.3 mo vs 10.5±3.6 mo for those with the overexpressing tumor. Among those not undergoing resection, the average survival for the 4 with nonoverexpressing cancer was 15.0 ±3.8 mo as contrasted to 5.2±2.1 mo for the overexpressors (p<0.01). Although the number of patients is small, these findings duggest that the overexpression of HER2/neu gene product may be frequently found in pancreatic cancer of ductal origin and may be one of the useful prognostic biomarkers for this cancer.

Carcinoid Tumors of the Pancreas: Status Report Based on Two Cases and Review of the World's Literature

SummaryConclusion. The diagnosis of a pancreatic carcinoid should be based on the measurement of serotonin in serum or its demonstration in the tumor and/or by the measurement of its derivative (5-HIAA) in urine. Carcinoid of the pancreas is a rare but definite entity; usually having metastasized by the time of diagnosis. The term “serotonin-producing tumor of the pancreas” has been suggested as an alternative designation for “pancreatic carcinoid.”Background. The literature on carcinoid tumors of the pancreas is confusing because much of it preceded the development of the more specific immunological, chemical and staining techniques currently available.Methods. 43 case reports were collected from the world’s literature, based on a demonstrable pancreatic neuroendocrine tumor plus a positive finding of at least one of the following without another dominant hormone being demonstrated: elevation of 5-Hydroxytryptamine (5-HT) (serotonin) in the serum or detected in tumor tissue, and/or elevation of 5-Hydroxyindole acetic acid (5-HIAA) in the urine. In addition to these two hormone-specific assays, information was collected on the silver-staining properties of the tumor; properties which have traditionally been associated with carcinoid tumors. Positive silver staining in tumor cells (argyrophilic and/or argentaffin reaction) is strongly indicative of the carcinoid tumor but the findings are less specific than the hormone assays and immunohistologic stains.Results. In this review of 43 cases, including two current ones, the pancreatic carcinoid tumor has the following important features:1.It is a rare tumor that is usually diagnosed late when the tumor is large and has metastasized. Thirty-eight (88.4%) have been malignant. They are, therefore, associated with a high incidence of the “carcinoid syndrome.”2.To date, prognosis in therapy is poor, based on delayed diagnosis, a resultant low incidence of resectability, and an uncertain duration of survival after resection.3.Pancreatic carcinoid tumors remain difficult to differentiate from other endocrine tumors. The measurement of urinary 5-HIAA excretion or the demonstration of elevated serotonin level in the tumor or in serum is essential to its distinction. Silver staining of the tumor, although of historic importance, has been superceded by the hormone-specific studies.4.To distinguish it from other endocrine tumors of the pancreas, the terms “pancreatic serotoninoma” or “serotonin-producing tumor of the pancreas” have been suggested as possible alternatives.Its growth characteristics may be related more to its cell of origin than to its extent of hormone secretion. Not all of the tumors result in recognizable hyperserotoninemia.

Adenocarcinoma of duodenum and ampulla of Vater: Clinicopathology study and expression of p53, c-neu, TGF-α, CEA, and EMA

Oncogenes, tumor suppressor genes, and growth factors are being explored as to their role in the initiation and progression of most neoplasms, but little information exists on the expression of oncoproteins or growth factors in adenocarcinoma of the duodenum or ampulla of Vater. This report covers expressions of p53, c‐neu, TGF‐α, CEA, and EMA in duodenal adenocarcinoma and ampullary adenocarcinoma, as well as correlations between expressions and tumor stage, histological grade and patient survival. The expression of p53, c‐neu, TGF‐α, CEA, and EMA has been studied in 15 duodenal adenocarcinomas and in eight ampullary adenocarcinomas by avidin‐biotin‐peroxidase complex indirect immunoperoxidase technique. The positive reaction for p53, c‐neu, TGF‐α, CEA, and EMA in duodenal adenocarcinoma was 20%, 60%, 60%, 73%, and 100%, respectively, and in ampullary adenocarcinoma, 13%, 100%, 50%, 63%, and 100%. Among the duodenal tumors, C‐neu and p53 expression was noted more frequently in groups with high histological grades. Patients with c‐neu positive duodenal adenocarcinoma had a shorter survival than the patients with c‐neu negative duodenal adenocarcinoma (P < 0.01). C‐neu product may serve as an unfavorable prognostic indicator in duodenal adenocarcinoma. No statistically significant correlation was found between the expressions of CEA, EMA, p53, and TGF‐α and patient survival, tumor stage, or histological grade in either duodenal or ampullary adenocarcinomas.

The changing trends of uterine cancer and cytology. A study of morbidity and mortality trends over a twenty year period

Data on squamous carcinoma of the cervix from a 20 year study period (1955 to 1974) in metropolitan Toledo revealed a 66% reduction of the average annual age‐adjusted incidence rate and a 61% reduction in death rate of cervical squamous carcinoma when the first time period (1955 to 1958) was compared with the last time period (1971 to 1974). The decrease for both morbidity and mortality rates was more pronounced in women age 50 years and younger. The age‐adjusted death rate during this study period revealed 15.5/100,000 for black women and 8.7/100,000 for white women. The reduction in death rate of 83% in black women is more prominent than 54.5% in white women. The decrease in both morbidity and mortality for cervical squamous carcinoma has a close relation to cytologic screening activity. The factors of age and race, probably related to socioeconomic status, are two known determinants of risk for cervical squamous carcinoma. Data for endometrial carcinoma during this study period revealed 15.5/100,000 women in the average age‐adjusted incidence and an increase of 13.8% in average yearly rates when the first time period (1955 to 1958) were compared with the last time period (1971 to 1974). The peak age was 60 to 64 years old in the first time period and shifted to 70 to 74 years old in the last time period. The trend in metropolitan Toledo is comparable to that of Louisville, Kentucky. The mass cytologic screening program which contributed to a remarkable reduction in morbidity and mortality for the cervical squamous carcinoma, did not have any beneficial effect on endometrial carcinoma. Cancer 42:2439–2449, 1978.

Primary myelolipoma of mediastinum

Myelolipoma is a benign nonepithelial neoplasm composed of mature marrow elements and fat cells. This rare neoplasm is usually discovered by pathologists incidentally at autopsy, and, with rare exceptions, usually occurs in the adrenal glands. One of these exceptional anatomic sites is the mediastinum, and only three cases of primary mediastinal myelolipomas have been documented in the English-language literature (1-3). We are presenting a primary myelolipoma involving the posterior mediastinum that was discovered on routine chest radiographs and successfully resected. Computed tomography (CT) scan findings of this rare mediastinal tumor are also presented. This is the first case report in the English language of primary myelolipoma in the mediastinum that was detected clinically and resected successfully.

Neoplasms of the Skin

The integument may give rise to a wide variety of neoplasms, partly because of the plethora of specialized structures that compose the organ. A complete exposition of these is far beyond the scope of this chapter, which will be devoted to the most common tumors, or to neoplasms that serve as examples of categories of these lesions.

Extradural tumor causing spinal cord compression in Klippel-Trenaunay-weber syndrome

BACKGROUND Myelopathy in Klippel-Trenaunay-Weber syndrome is uncommon but has been reported secondary to spinal vascular malformations. REPORT A patient with Klippel-Trenaunay-Weber syndrome who presented with spinal cord compression from a spinal extradural mass lesion (angiomyolipoma) is described. DISCUSSION This association has not been reported previously but is consistent with the segmental vascular abnormalities observed in Klippel-Trenaunay-Weber syndrome.

Pulmonary vein tumor thrombosis and left atrial extension in lung carcinoma

A case of lung carcinoma extending into left atrium through a pulmonary vein and mimicking left atrial myxoma is presented. The localized enlargement of a pulmonary vein is seen as a possible CT sign of pulmonary vein tumor thrombosis. Computed tomography (CT) and echocardiography are complementary in the correct diagnosis of this condition.