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1/5. Cowden disease.

    Cowden disease represents an unusual, but unique syndrome which can be recognized most consistently by the development of characteristic verrucous, keratotic, papular, and nodular lesions about facial orifices, on the oral mucosa, and over the dorsal surfaces of the forearms and hands. The cause of these diverse hyperplastic changes is not known. Recognition of these lesions as signs of more extensive disease should alert the physician to examine the thyroid gland, breasts, female reproductive tract, GI tract, and skeleton for evidence of the associated changes we have enumerated. Because of the tendency for lesions of the thyroid, breast, and intestines to undergo malignant change, these patients require close observation and evaluation.
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2/5. carcinoembryonic antigen: clinical and historical aspects.

    To further define and determine the usefulness of CEA, 1100 CEA determinations have been made over the past two years at The ohio State University hospitals on patients with a variety of malignant and nonmalignant conditions. Correlation of CEA titers with history and clinical course has yielded interesting results not only in cancers of entodermally derived tissues, for which CEA has become an established adjunct in management, but also in certain other neoplasms and inflammatory states. The current total of 225 preoperative CEA determinations in colorectal carcinomas shows an 81% incidence of elevation, with postoperative titers remaining elevated in patients having only palliative surgery but falling to the negative zone after curative procedures. An excellent correlation exists between CEA levels and grade of tumor (more poorly differentiated tumors showing lower titers). Left-side colon lesions show significantly higher titers than right-side lesions. CEA values have been shown to be elevated in 90% of pancreatic carcinomas studied, in 60% of metastatic breast cancers, and in 35% of other tumors (ovary, head and neck, bladder, kidney, and prostate cancers). CEA levels in 35 ulcerative colitis patients show elevation during exacerbations (51%). During remissions titers fall toward normal, although in 31% still remaining greater than 2.5 ng/ml. In the six colectomies performed, CEA levels all fell into the negative zone postoperatively. Forty percent of adenomatous polyps showed elevated CEA titers (range 2.5-10.0) that dropped following polypectomy to the negative zone. Preoperative and postoperative CEA determinations are important in assessing the effectiveness of surgery. Serial CEA determinations are important in the follow-up period and in evaluation of the other modes of therapy (e.g., chemotherapy). These determinations of tumor antigenicity give the physician added prognostic insight into the behavior of the tumor growth. Rectal examination with guaiac determinations, sigmoidoscopy, cytology, barium enema, and a good clinical evaluation remain the primary tools for detecting colorectal disease. However, in the high-risk patient suspicious of developing cancer, CEA determinations as well as colonoscopy are now being used increasingly and provide additional highly valuable tools in the physician's armamentarium.
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3/5. Multifocal lymphangioendotheliomatosis with thrombocytopenia: a rare cause of gastrointestinal bleeding in the newborn period.

    Severe gastrointestinal bleeding in the newborn period is a serious but uncommon phenomenon that has a broad differential diagnosis. In the following case report we describe a rare phenomenon in which a newborn presents with severe hematemesis, hematochezia, and thrombocytopenia that are resistant to repeated platelet and packed red blood cell transfusions. Previous cases have been reported, but none of the patients described presented within the first 8 days of life. The early age of presentation and refractory nature of this disease entity to multiple therapies make it a diagnostic and therapeutic dilemma for all physicians involved in the care of newborns.
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4/5. Malignant tumors associated with carcinoid tumors of the gastrointestinal tract.

    Twenty-three associated malignant neoplasms mostly adenocarcinomas were recognized in 96 patients with gastrointestinal carcinoid tumors, between 1968 and 1977 at the University of Rochester Medical Center. Associated malignancies were present in 17 patients, affected various organs and led to an early death even though the carcinoid tumors generally remained localized. There was a 36% incidence of a second malignant neoplasm in patients with ileal carcinoid, but no second tumor in patients with carcinoid of the rectum. The presence of a carcinoid tumor should alert the physician to search for a second neoplasm.
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5/5. neuroendocrine tumors: common presentations of uncommon diseases.

    In recent decades, isolation of the peptides secreted by gastrointestinal neuroendocrine tumors has greatly advanced our understanding of the pathophysiology of the syndromes resulting from over-production of these biologically active compounds. Early detection of these lesions permits localization and surgical extirpation prior to the development of mestastatic disease. In 1995, early detection of gastrointestinal neuroendocrine tumors hinges on an appropriate index of suspicion on the part of the primary care physician. The prompt recognition of the typical clinical constellations of symptoms associated with these tumors is essential to their timely diagnosis and management.
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