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1/308. Hodgkin's disease during pregnancy: diagnostic and therapeutic management.

    OBJECTIVES: To evaluate the possibility that women affected by Hodgkin's disease (HD) during their second or third trimester of pregnancy can safely carry their pregnancy to term. methods: From 1986 to 1997, 6 women came to our Center during the second trimester of pregnancy and were diagnosed as having HD. Three of these 6 patients were treated with chemotherapy before delivery and 3 of them were kept under observation and started treatment after delivery. RESULTS: All 6 women gave birth to a healthy female. CONCLUSIONS: The pregnancy does not worsen the course of the illness and does not compromise long-term clinical remission and recovery.
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2/308. myocardial infarction and death after caesarean section in a woman with protein s deficiency and undiagnosed phaeochromocytoma.

    We describe the case of a 36-year-old woman, with a previous history of recurrent abortion due to protein s deficiency, undergoing an elective Caesarean section at 39-weeks gestation. During pregnancy no signs of hypertension or cardiovascular disease were reported, but at the end of the surgical procedure, the patient developed acute hypertension, leading to myocardial infarction, severe heart failure and death. The autopsy revealed a 2-cm undiagnosed phaeochromocytoma in the right adrenal gland. Clinical diagnostic features of phaeochromocytoma during pregnancy as well as the main therapeutic approaches suggested in the literature are discussed.
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3/308. Management of cancer in pregnancy: a case of Ewing's sarcoma of the pelvis in the third trimester.

    Ewing's sarcoma of the pelvic bones was diagnosed in a 21-year childbearing woman, raising major medical and ethical problems. The diagnostic and therapeutic approaches during the sixth month of gestation were tailored in order to cure the patient and avoid unnecessary toxicity to the fetus. Ancillary tests included ultrasound and MRI studies of the pelvis. ifosfamide and adriamycin, premedicated by granisetron, were administered during gestation, and were found to be safe. cesarean section was the preferred way of delivery since the tumor involved the pelvic bones. The outcome was a disease-free patient and a small healthy baby who is now two years of age.
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4/308. Endometrial carcinoma associated with pregnancy: A report of three cases and review of the literature.

    Endometrial carcinoma associated with pregnancy is uncommon. In case 1, a 40-year-old gravida 2, para 2, was diagnosed with focal well-differentiated papillary adenocarcinoma 4 months postpartum. In case 2, a 35-year-old gravida 1, para 0, was diagnosed with a well-differentiated papillary adenocarcinoma of the endometrium after a D&C for an incomplete abortion at 7 weeks gestation. In case 3, a 32-year-old gravida 2, para 1, was diagnosed with a moderately differentiated adenocarcinoma with squamous metaplasia 4 months postpartum. All are without evidence of disease more than 2 years after therapy. A literature review shows 24 previous cases of pregnancy associated with endometrial cancer. These cases demonstrate the importance of endometrial sampling for abnormal postpartum bleeding despite the protective effects of pregnancy.
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5/308. The evolution of linitis plastica as a consequence of advanced gastric carcinoma in pregnancy.

    A 40-year-old multigravid woman was examined on multiple occasions during pregnancy because of persistent gastrointestinal symptoms. A metastatic, unresectable gastric carcinoma that had evolved to linitis plastica was diagnosed at 26 weeks' gestation. The patient was delivered of a viable infant at 27 weeks' gestation, and she died of disease 1.5 months after the diagnosis was made.
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6/308. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

    Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990. The aim of the study, based on a review of the literature, also was to provide a comprehensive view of this uncommon disease. The clinical features were in keeping with previous studies, but we found that exertional dyspnea and pneumothorax were the most common features, and chylous involvement was less frequent. LAM was diagnosed after menopause in about 10% of cases. The onset of LAM occurred during pregnancy in 20% of cases, and a clear exacerbation of LAM was observed in 14% of cases during pregnancy. Pulmonary LAM was diagnosed on lung histopathology in 83% of cases, but renal angiomyolipoma, observed in 32% of our patients, may be a useful diagnostic criterion when associated with typical multiple cysts on chest CT scan or with chylous effusion. Chest CT scan was more informative than chest X-ray (normal in 9% of cases), and may be indicated in spontaneous pneumothorax or renal angiomyolipoma in women of childbearing age. About 40% of the patients had a normal initial spirometry, while an obstructive ventilatory defect (44%), a restrictive ventilatory defect (23%), was observed in other patients. Initial diffusing capacity for carbon monoxide was frequently decreased (82%). Hormonal therapy was administered in 57 patients, but a clear > or = 15% improvement of FEV1 was observed in only 4 evaluable patients, treated with tamoxifen and progestogens (n = 2), progestogen (n = 1), and oophorectomy (n = 1). Probably 1 of the most urgent needs for clinical research in LAM is to test the currently available hormonal treatments in the context of international multicenter prospective controlled studies. pleurodesis was performed in 40 patients. lung transplantation was performed in 13 patients, 7.8 /- 5.2 years after onset of LAM, in whom the mean FEV1 was 0.57 /- 0.15 L. After a follow-up of 2.3 /- 2.2 years, 9 patients were alive. Mean follow-up from onset of disease to either death or closing date was 8.2 /- 6.3 years. overall survival was better than usually reported in LAM, and Kaplan-Meier plot showed survival probabilities of 91% after 5 years, 79% after 10 years, and 71% after 15 years of disease duration.
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7/308. Primary cardiac sarcoma in pregnancy: a case report and review of the literature.

    Primary cardiac sarcoma (PCS) is a rare disease with a poor prognosis, because of diagnostic delay, therapeutic difficulties, and high metastatic potential. Surgery is the standard treatment. A case of PCS in pregnancy is reported, with a review of published surgical series of PCSs, focusing on the role of surgery and adjuvant therapy. Prompt surgery improved cardiac function and patients' outcome in comparison with untreated cases. The role of adjuvant treatment was analyzed only in a few series, mainly without distinction between postoperative chemotherapy and radiotherapy; adjuvant therapy improved survival in the larger series of resected PCSs. Only three other cases of PCS in pregnancy were reported. In the present case, resection was performed with no major complication for the mother and the infant. Even if the patient's survival was short, cardiac surgery allowed prolonging of pregnancy until an acceptable possibility of fetal survival was reached. Although resection is not curative in most cases, surgery remains the treatment of choice for PCS and has a definite palliative significance. The role of postoperative chemotherapy and radiotherapy is difficult to ascertain; however, adjuvant chemotherapy seems advisable in high-grade tumors.
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8/308. Gastric adenocarcinoma presenting with persistent, mild gastrointestinal symptoms in pregnancy. A case report.

    BACKGROUND: Mild gastrointestinal symptoms are common during pregnancy but can also be the only symptoms in stomach cancer until the late stage. Clinicians' reluctance to pursue diagnostic studies appears to be a major contributing factor to delayed diagnosis and poor outcome. We report a case of maternal death to alert clinicians to this rare possibility. CASE: A 36-year-old woman had persistent, mild epigastric discomfort, nausea, vomiting and frequent episodes of dark stools since the second trimester of pregnancy. These were attributed to peptic ulcer and an iron supplement given, without investigation. gastroscopy was performed only at 32 weeks of gestation, when the patient had heavy hematemesis. biopsy confirmed the diagnosis of poorly differentiated adenocarcinoma of the stomach. cesarean section was performed after steroid therapy. Advanced stomach cancer with stomach perforation was found. Curative surgery was not possible. The patient died four weeks after delivery. CONCLUSION: stomach cancer is a rare complication of pregnancy. Delay in diagnosis is commonly due to clinicians' reluctance to request diagnostic studies and the nonspecific symptoms of the disease. Early recognition and diagnosis are the only possibilities for a better outcome. Clinicians must be alert to this possibility and include this in the differential diagnosis of minor gastrointestinal discomfort during pregnancy.
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9/308. Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature.

    Intravenous leiomyomatosis is an uncommon clinical entity characterized by the growth of a benign, smooth muscle tumour within the venous system. Intracardiac extension of this tumour is rare, and approximately 35 cases have been reported in the literature. The second case of massive intracardiac extension of intravenous leiomyomatosis is reported in a pregnant patient diagnosed after the unusual presentation of seizure activity. Tumours were successfully removed from the right internal iliac vein, inferior vena cava, and right atrium and ventricle in a single-stage operation using cardiopulmonary bypass and circulatory arrest. Concomitant total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Nine months after diagnosis, the patient was evaluated for recurrent disease. A review of the literature from 1994 to 1998 is presented.
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10/308. Arteriovenous shunting in a giant renal angiomyolipoma. A rare condition.

    We report a case of a 33-year-old woman with tuberous sclerosis and bilateral angiomyolipomas. She suffered from acute left flank pain due to retroperitoneal haemorrhage. During renal arteriography an arteriovenous shunting was found in the left tumour. angiomyolipoma is a rare cause of angiographically demonstrable arteriovenous shunting.
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