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1/16. Case report: two cases of biliary papillomatosis with unusual associations.

    Papillomatosis arising from the biliary tree is a well recognized but rare entity. We encountered two patients with this condition. However, one of them had associated hepatocellular carcinoma and cirrhosis and the other had concomitant recurrent pyogenic cholangitis. To our knowledge, these associations have not been reported before. We, therefore, present these clinical problems and highlight the added difficulty in the management of these patients.
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2/16. Panendoscopy and synchronous second primary tumors in head and neck cancer patients.

    endoscopy techniques are used to diagnose and to determine the extent and exact location of malignancies in the head and neck region, bronchial tree and esophagus. Panendoscopy is used to find the primary tumor in the case of metastatic disease from unknown primaries or to detect a simultaneous second primary tumor at the time of diagnosis of a malignancy in the upper aerodigestive tract (UADT). The value of panendoscopy has been debated lately because of the relatively small proportion of malignant findings and because of the lack of convincing data concerning its effect on survival rates. However, despite the relatively low proportion of positive findings, their significance is often crucial for the individual patient. The significant number of late metachronous, second primaries, especially in the lungs, also emphasizes the importance of follow-up endoscopies. This study consists of 203 consecutive patients with squamous cell cancer (SCC) of the upper aerodigestive tract who underwent panendoscopy in Turku University Central Hospital as part of the initial diagnostic workup from 1992-1999. Eight patients with synchronous second primaries were found to represent a prevalence of 3.9%, and in addition, 19 patients with metachronous tumors were diagnosed. In the case reports we illustrate the importance of some of these findings.
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3/16. Unilateral multiple primary tumours in an atomic-bomb survivor.

    We report here a case of an atomic-bomb survivor with sequential, unilateral, multiple-organ primary tumours after exposure to direct external radiation. This 67-year-old woman was 8 years old when she was exposed to radiation from the atomic bomb. At the time of the explosion, she was in an open area, but hiding behind a tree, which shielded her left side. Therefore, the right side of her body was exposed to radiation directly and primarily. Since then, she has been diagnosed sequentially with breast cancer, ovarian tumour, thyroid tumour, head skin cancer and lung cancer. In each case, the tumour was on the right side of her body at the ages of 31, 38, 54, 58 and 64 years old, respectively. This case study indicates that the risk of multiple primary tumours should be considered in older atomic-bomb survivors.
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4/16. Resection of a granular cell tumor at the hepatic confluence. A precarious location for a benign tumor.

    Granular cell tumors are lesions that account for less than 10 per cent of benign biliary tumors and only 1 per cent occur in the biliary tract. Since the first description, 45 other cases have been described to which we add a case. In reviewing the literature, a striking predominance in black females is evident. The most frequent location of these tumors in the biliary tree appears to be the common bile duct (50%), followed by the cystic duct (37%), the hepatic ducts (15%), and the gallbladder (4%). Most patients have been treated with resection followed by restoration of biliary continuity using biliary enteric diversion. In the case presented, the tumor was located at the hepatic confluence with extension into the hepatic substance requiring segmental duct cholangiojejunostomy after resection. This resulted in fulminant sepsis and death despite adequate preoperative biliary decompression and the lack of signs or symptoms of infected bile. Alternative forms of management in the treatment of this benign condition at this location are discussed.
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5/16. Granular cell myoblastoma of the bronchus. Case report and literature review.

    A case of multiple granular cell myoblastomas of the tracheobronchial tree is presented. The patient is well 32 months following endobronchial removal. Only 46 cases of solitary and two cases of multiple lesions of the tracheobronchial tree have been described in the literature. These lesions occur with equal frequency in men and women; at a median age of 38 years; more frequently on the right than on the left; and most commonly with cough as the presenting symptom. Most patients have been treated with major surgical resections, but conservative endobronchial removal may be effective therapy.
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6/16. Multicentric endobronchial granular cell myoblastoma.

    Granular cell myoblastoma (GCM) is a rare benign neoplasm involving the tracheobronchial tree. It is believed to arise from the Schwann cell. Four cases of tracheobronchial GCM, all of which were multicentric, are presented and a conservative therapeutic approach is suggested.
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7/16. Squamous carcinoma of the lung in a nonirradiated, nonsmoking patient with juvenile laryngotracheal papillomatosis.

    Juvenile laryngotracheal papillomatosis (JLTP)--a disease characterized by the growth of multiple, recurrent, koilocytolic papillomas of the upper respiratory tree--occurs in 1,500 to 2,000 infants and children in the united states every year. In rare instances, the papillomas, which are thought to be caused by a human papillomavirus (HPV), may extend into the distal bronchi and lungs. They are often excised surgically or by laser resection, but may regress spontaneously. patients who receive radiation or cytotoxic drugs for this condition, or subsequently become smokers, may be predisposed to the development of bronchopulmonary carcinoma. Only six cases of bronchopulmonary carcinoma arising in persons with a history of JLTP but no history of these predisposing factors have been reported. Herein, we report the occurrence of squamous carcinoma of the left lung in a 28-year-old woman with JLTP since the age of 2 1/2 years. The carcinoma as well as randomly chosen papillomas excised over a period of years demonstrated positive nuclear staining for papillomavirus structural antigen. This is consistent with the current belief that human papillomavirus may be an important factor in the development of squamous carcinomas in various anatomic locations.
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8/16. radionuclide angiography and surgery for familial bilateral chemodectomas.

    carotid body tumours, or chemodectomas, can occur on both sides of the neck (5%), and this bilaterality increases sixfold (30%) where a familial pattern is evident. The known pathological behaviour of chemodectomas makes early recognition and management desirable, especially when an inherited predisposition can be anticipated. Difficulties in clinical diagnosis and the hazards of blind exploration have imposed a reliance on contrast angiography, which is a potentially dangerous procedure. The family tree of a patient with bilateral chemodectomas was traced and 28 individuals were studied using radionuclide angiography (99mTechnetium gluconate), a quick, safe technique with a high yield. Bilateral chemodectomas were observed in five siblings, establishing conclusively the existence of a dominant hereditary trait. Excision of newly discovered tumours was accomplished without complication. The techniques which permitted such a favourable surgical outcome are discussed, emphasising the view that these tumours are best dealt with by vascular surgeons.
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9/16. Congenital stenosis of the lower esophagus associated with leiomyoma and leiomyosarcoma of the gastrointestinal tract.

    The purpose of this paper is to report our analysis of four generations of a family with congenital strictures of the lower esophagus associated with leiomyoma of the gastrointestinal tract. Two members of the family died of sarcomatous degeneration of the leiomyoma and one is still alive 2 years after resection of the malignancy. Three family members had surgical repair of the congenital stricture. Surgical repair was unnecessary in one, for whom repeated dilatations alleviated the symptoms. Since there were no direct female descendants in this family, it is unknown whether this hereditary disease is carried by a sex-linked dominant gene. There are no reports in the literature of a familial tendency to congenital strictures of the esophagus, nor are there any reports of strictures in association with gastrointestinal leiomyoma and the later development of sarcomatous degeneration. The literature is reviewed, syndromes of leiomyoma of the esophagus and gastrointestinal tract are detailed, the particulars of the family tree and the patients are described, and the study is summarized.
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10/16. Multiple granular cel tumors of the gallbladder and biliary tree. Report of a case.

    Granular cell tumors are benign lesions that usually appear in the skin, oral activity, and mucous membrane. Occasionally, they will appear in visceral organs, and cause signs and symptoms similar to those of other organ-related diseases. We studied a patient with granular cell tumors of the common bile duct whose clinical appearance mimicked that of cholecystitis with cholelithiasis. Recognition of granular cell tumors on frozen section will allow surgical resection with probable cure.
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