1/13. Subglottic leiomyoma: report of a case.Subglottic leiomyoma is a rare disease. We encountered such a tumor in a 7-year-old boy who was transferred to our hospital with respiratory distress and hoarseness of 2 week's duration. Stridor was noted and flexible fiberoscopy revealed a huge mass over the subglottis. The tumor was removed endoscopically. Pathologic examination disclosed a leiomyoma. The patient recovered well and no recurrence was noted during 17 months of follow-up. Although subglottic leiomyoma is rare, it should be included in the differential diagnosis of a subglottic tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/13. Localized retroperitoneal lymphangioleiomyomatosis mimicking malignancy. A case report and review of the literature.lymphangioleiomyomatosis (lymphangiomyomatosis [LAM]), a rare disease of unknown etiology that is seen only in women usually in the reproductive period, generally presents with features of pulmonary involvement. Extrapulmonary involvement, such as angiomyolipomas and retroperitoneal adenopathy, can occur in up to 75% of cases. It is very rare, however, for patients to present with features of extrapulmonary LAM. We present an unusual, localized case of LAM presenting with neurologic symptoms related to a retroperitoneal mass in a 51-year-old woman. magnetic resonance imaging showed that the mass involved retroperitoneal lymph nodes, and a clinical diagnosis of atypical sarcoma (possibly from a uterine primary) was made. The mass was resected, and a total abdominal hysterectomy was performed. On pathologic examination, the mass showed classic histologic features of LAM with spread along lymphatic channels in the lymph nodes. Intralymphatic projections simulated lymphatic metastasis; however, the cytologic features were benign. Immunostains revealed the tumor to be positive for smooth muscle actin and desmin, but negative for HMB-45. The uterus was unremarkable, except for a subserosal leiomyoma. Although intratumoral variability for HMB-45 has recently been described, to the best of our knowledge, this is the first documented case of HMB-45-negative, histologically classic LAM. Because of the presence of several atypical features in this case, such as age, location, compressive neurologic presentation, radiologic impression of atypical sarcoma, and HMB-45 negativity, we feel that this case may represent a distinct, as yet uncharacterized variant of LAM.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/13. A case of multiple leiomyomatous lesions of the lung: an analysis of flow cytometry and hormone receptors.A 36 year old woman was admitted to our department because of a chest X-ray which showed multiple developing shadows. She underwent bilateral exploratory thoracotomies and a total 5 tumors were resected and pathologically diagnosed as benign metastasizing leiomyoma, the largest of which was positive for the progesterone receptor and negative for the estrogen receptor. A histogram of this tumor using a flow cytometer showed a diploid pattern and 4.6 percent of the s phase which was not more than that of a leiomyoma of the uterus from another patient. Two months later, she underwent a hysterectomy and bilateral salpingo-oophorectomy for treatment of the positive progesterone receptor in the pulmonary lesions. The resected uterine myoma and normal myometrium showed positive estrogen and progesterone receptors. For the subsequent 28 months she has been free of any further symptoms. Benign metastasizing leiomyoma of the uterus is a rare disease and very interesting because of its histological benignity and hormonal dependency. However, according to the literature, it is often confused in entity due to the fact that normal lung tissue also possesses hormone receptors. Considering our data on hormone receptors, it is rational to think that multiple leiomyomatous lesions in the lung should only be diagnosed as benign metastasizing leiomyomas when they possess positive estrogen and progesterone receptors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/13. Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency.Bronchial leiomyoma is a rare disease in children. Recently, the association of leiomyoma and hiv infection was reported. We describe a boy with a cellular immunodeficiency, who had endobronchial leiomyoma. The tumor cells were positive for Epstein-Barr virus-encoded rna-1 (EBER-1) and Epstein-Barr virus-determined nuclear antigen-2, suggesting a role of Epstein-Barr virus in the pathogenesis of leiomyoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/13. Major malformations in a case of infantile myofibromatosis.A case of infantile myofibromatosis associated with oesophageal atresia, annular pancreas, additional sacral vertebra and hypoplatic right kidney in a male neonate is reported. The possibility of associated malformations in this rare disease is outlined.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/13. Leiomyomatosis peritonealis disseminata.A 49 year old woman presented with leiomyomatosis peritonealis disseminata (LPD) which is a benign and very rare disease entity. This patient is particularly interesting because she presented with many atypical features. She is the second Oriental case reported, and the follow-up period was long. The disease was symptomatic and progressive despite bilateral salpingo-oophorectomy, hysterectomy and repeated excision of all the recurrent nodules. She had documented recurrences of LPD in three episodes at 4 years apart. There was no obvious systemic hormonal abnormality including pregnancy or oestrinizing granulosa tumour, and she had never taken any contraceptive drugs. The importance of differentiation of this condition from generalized metastatic intra-abdominal malignancy is discussed as LPD has a very good prognosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/13. Use of LHRH analogue to obtain reversible castration in a patient with benign metastasizing leiomyoma.The use of superactive LHRH analogue to provide a reversible form of castration is discussed in the context of the management of a patient with benign metastasizing leiomyoma. The aetiology and prognosis of this rare disease are briefly considered.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/13. Intracaval and intracardiac leiomyomatosis of uterine origin.Intracaval leiomyomatosis of uterine origin is a rare disease. Extension to the right heart is exceptional. Based on the review of 11 cases reported in the literature and the case presented herein, which was treated successfully, the diagnostic and therapeutic problems are discussed. diagnosis should be suggested when a female patient operated on previously for myofibroma of the uterus by hysterectomy, presents with a picture of cardiac myxoma. diagnosis can be confirmed by iliocavogram and computerized tomography of the abdomen. Excision calls for a cardiac procedure under extracorporeal circulation and caval exploration which may be performed either simultaneously or as a two stage procedure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/13. Leiomyoma of the rectum.Rectal leiomyoma is a rare disease. In japan, only 77 cases were reported before 1984. A study was carried out by compiling information from clinical cases together with two recent cases of rectal leiomyoma. The patients were mainly in their 50s to 60s with a mean age of 53.6 years. The sex ratio of males to females, was 1.5:1. The most common main complaints were anal bleeding and melena and subsequently cylindrical shaped stool or constipation, attributable to rectal stenosis. The tumors were located at 8 cm or less from the anal verge in 87% of the case. Surgery is considered the best method of treatment. Five patients noted a recurrence and three underwent extirpation as their initial operation. Although it is frequently difficult to establish whether leiomyoma is benign or malignant, small lesions (less than 2 cm in diameter) are best treated by local excision.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/13. Leiomyomatous lung lesions: a proposed classification.A case of multiple pulmonary leiomyomas is presented. In a review of the literature, a confusing array of rare diseases all pertaining to multiple leiomyomas was found. These were reduced to three entities: leiomyomatosis in women, metastatic leiomyoma in men and children, and multiple pulmonary fibroleiomyomatous hamartoma occurring in anyone. The leiomyomatous diseases in women are related to uterine leiomyomas and they are hormone-sensitive. This is of great prognostic importance. The classification, definitions, and discussion should prove helpful in understanding these rare multiple diseases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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