1/12. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/12. Recurrent leiomyomatosis peritonealis disseminata exacerbated by in vitro fertilization.leiomyomatosis peritonealis disseminata is a rare disease that is most often discovered during pregnancy or in patients with a history of oral contraceptive use. We report the first case of a patient with the disease who conceived by in vitro fertilization and the complications that occurred.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/12. Bilateral pheochromocytoma during pregnancy.BACKGROUND: pheochromocytoma is a rare disease of the chromaffin cells that secrete catecholamines. It may occur during pregnancy. Bilateral pheochromocytoma in pregnancy is even rarer. CASE: A 26-year-old woman, gravida 2, para 0-0-1-0, 18 weeks' pregnancy, was initially seen with elevated blood pressure (170/100 mmHg) and mild headache. The cause of hypertension was conventionally investigated and bilateral pheochromocytoma was finally searched for and found. Bilateral adrenalectomy was undertaken at 23 weeks' gestation and cesarean section was performed at 31 weeks' gestation due to intrauterine growth retardation (IUGR) and compromised fetal well-being. The maternal outcome was uneventful and the baby was physiologically complicated only by neonatal jaundice. CONCLUSION: pheochromocytoma should be searched for in the conventionally differential diagnosis in hypertension during pregnancy, especially in the young. early diagnosis and proper management with medical treatment followed by surgical removal of the tumor usually result in good maternal and fetal outcomes.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/12. Generalised Burkitt's lymphoma involving both breasts--a case report.BACKGROUND: Burkitt's lymphoma is a disease of children age ranging 8-10 years. lymphoma involving the breast is an unusual clinical entity, which is rarely distinguished preoperatively from other more common forms of breast cancer. The Management differs from the more typical adenocarcinoma of the breast in that the emphasis is on systemic therapy. CASE: We report a 27-year-old pregnant Nigerian civil servant at 28 weeks gestation, who presented with multiple organ swellings including both breasts. She was diagnosed histologically as primary breast Burkitt's lymphoma. She was treated with systemic chemotherapy after spontaneous abortion. She had a recurrence of the lesion in the right breast a month later that was excised. Presently there is no evidence of any mass. CONCLUSION: Compared with breast carcinoma, primary breast lymphoma is a rare disease but should be considered in the differential diagnosis of breast masses.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/12. Primitive neuroectodermal tumor (PNET) of the brain diagnosed during pregnancy.BACKGROUND: brain tumors in pregnancy are extremely rare events. A case of a patient with primitive neuroectodermal tumor (PNET) of the brain diagnosed during the second half of pregnancy is reported. CASE REPORT: The first case of PNET of the brain diagnosed in a 26-year-old woman, gravida 1 para 0, in her 20th week of pregnancy is presented. The patient presented with headache, nausea and blurred vision. magnetic resonance imaging revealed a large ill-defined tumoral mass in the left frontal cortex. Gross subtotal resection was achieved via a left frontal craniotomy. Pathological evaluation revealed histology and immuno-histochemistry consistent with the diagnosis of PNET. Adjuvant radiation therapy to the neurocranium was administered. Additionally, chemotherapy with Temodal 5 mg (Temozolomid) was initiated at 30 weeks of gestation. The patient delivered her baby via cesarean section at 34 weeks of gestation. After delivery, external beam radiation to the craniospinal axis completed the therapy. The patient is currently being followed-up at the Department of Oncology University of Vienna Medical School, without any evidence of disease, 3 months after diagnosis. CONCLUSION: This case is the first reported description of a PNET in pregnancy. A brief discussion of this rare disease and its management in pregnancy is presented. The treatment options seem to be reduced in pregnant women and mainly depend on the patient's condition as well as the gestational age at presentation. In a multidisciplinary approach, an optimal therapy schedule should be assessed depending on these two parameters.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/12. Renal cell carcinoma in pregnancy.A rare case of renal cell carcinoma occurring during pregnancy is described. The modalities used to make the diagnosis and the antepartum management of the patient are discussed. The case reported herein demonstrates the major diagnostic challenge of the young pregnant patient with a rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/12. Hairy cell leukemia in pregnancy: a case report.Hairy cell leukemia (leukemic reticuloendotheliosis) is a rare disease. No previously reported cases in pregnancy could be found with the use of available computer-assisted medical literature search programs. The pregnancy was complicated by progressive thrombocytopenia, leukopenia, and anemia that did not improve with delivery. The deterioration was eventually abated by postpartum splenectomy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/12. Intradural lipoma presenting in pregnancy.Progressive peripheral neuropathy is an unusual condition in pregnancy requiring specialist opinion to identify the possible underlying pathology. The case presented is that of a patient with cervical intradural lipoma and worsening neurological symptoms, treated surgically at the time of caesarean section. This rare disease and its management are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/12. pheochromocytoma in pregnancy: case report and review of the literature.pheochromocytoma is a rare disease that may occur during pregnancy. Only a few hundred cases have been published in the literature. Manifestations include hypertension with various clinical presentations, possibly resembling those of pregnancy-induced hypertension, or pre-eclamptic toxemia. Differentiation of these conditions is not always feasible, thus creating a serious risk, because fetal and maternal morbidity and mortality are far higher with pheochromocytoma. Biochemical measurements of catecholamines and their metabolites are apparently a convenient way to establish diagnosis during pregnancy, inasmuch as interpretation of radiological evaluation is complicated by the gravid uterus, and might even be potentially dangerous due to the use of ionizing radiation. More sophisticated methods for evaluation are not always practical during pregnancy. Medical treatment aims at controlling symptoms, mandating the use of alpha- and beta-receptors blockade medication. Surgical intervention is the only possible curative method available, but the critical issue is probably to identify the exact timing during the course of pregnancy for such intervention, or the ability to control symptoms until delivery. Although malignant transformation of pheochromocytoma have been reported, it is extremely uncommon. The overall prognosis is mainly affected by early diagnosis, and multidisciplinarian management.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/12. pheochromocytoma in pregnancy: a review of the Japanese literature.We review the 35 cases with pheochromocytoma associated with pregnancy including our own 3 cases in the Japanese literature from 1958 to 1993. In a total of 35 cases, the overall maternal mortality rate was 11% and the fetal loss 22%. Recently, after 1981, the maternal mortality rate and the fetal loss were significantly reduced to one patient (6%). Although antepartum diagnosis of pheochromocytoma was made in only 32% of the patients, maternal mortality was reduced to zero and fetal loss to 18%. An initial diagnosis of toxemia of pregnancy was made in 17 patients (49%) and of pheochromocytoma in 10 patients (29%). A diagnosis of pheochromocytoma was made before delivery in 11 patients (31%), after delivery in 22 patients (63%), at autopsy in one patient (3%), and unknown in one patient (3%). The symptoms mostly suggestive of pheochromocytoma were paroxysmal hypertension in 27 (77%) of the 35 patients. The clinical symptoms generally appeared in the third trimester in 23 patients (66%). pheochromocytoma is a rare disease, but a high index of clinical suspicion must be kept in mind and pheochromocytoma must be listed in the differential diagnosis of hypertension associated with pregnancy. Recently, abdominal ultrasound study and MR imaging can be used to localize the tumor during the antepartum period. Once the diagnosis is confirmed, alpha-blockade is essential and beta-blockade may be required. In the first and second trimesters, tumor resection has a good fetal outcome; in later pregnancy, delivery by elective caesarean section followed by tumor resection is recommended.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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