1/8. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. 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) |
3/8. leiomyomatosis of mesenteric lymph nodes associated with duodenal adenocarcinoma.leiomyomatosis of lymph nodes is an extremely rare disease. Only a few cases have previously been reported in pelvic lymph nodes. They were related to a benign uterine leiomyoma, a metastasizing uterine leiomyoma, an endometrial adenocarcinoma, and an ovarian endometrioid carcinoma. We report on a case of leiomyomatosis of the mesenteric lymph nodes associated with a duodenal adenocarcinoma with no history of uterine leiomyoma or any gynecological malignancy. The patient, a 56-year-old woman, was found to have an adenocarcinoma of the duodenum. All mesenteric lymph nodes removed showed leiomyomatosis, which was verified by immunohistochemical study showing positive immunostaining for smooth muscle actin, desmin, and vimentin, but negative staining for HMB-45. It is necessary to make a differential diagnosis from other examples of spindle cell proliferation involving lymph nodes such as a hemorrhagic spindle cell tumor with amianthoid fibers (palisade myofibroblastoma), angiomyolipoma, lymphangiomyomatosis, inflammatory pseudotumor, and Kaposi's sarcoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Recurrent leiomyomatosis peritonealis disseminata after hysterectomy and bilateral salpingo-oophorectomy during combined hormone replacement therapy.leiomyomatosis peritonealis disseminata is a rare disease characterised by the occurrence of multiple smooth muscle cells building up tumour nodules within the peritoneal cavity. Approximately 50 cases of this disease have been reported in the world literature. To our knowledge, this is the first case reporting the recurrence of leiomyomatosis peritonealis disseminata during combined hormone replacement therapy after hysterectomy and bilateral salpingo-oophorectomy and six laparotomies because of recurrence of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Familial clustering of leiomyomatosis peritonealis disseminata: an unknown genetic syndrome?BACKGROUND: leiomyomatosis peritonealis disseminata (LPD) is defined as the occurrence of multiple tumorous intraabdominal lesions, which are myomatous nodules. LPD is a rare disease with only about 100 cases reported. The usual course of LPD is benign with the majority of the patients being premenopausal females. Only two cases involving men have been reported, no syndrome or familial occurrence of LPD has been described. CASE PRESENTATION: We describe a Caucasian-American family in which six members (three men) are diagnosed with leiomyomatosis peritonealis disseminata (LPD) and three deceased family members most likely had LPD (based on the autopsy reports). Furthermore we describe the association of LPD with Raynaud's syndrome and prurigo nodularis. CONCLUSION: Familial clustering of leiomyomatosis peritonealis disseminata (LPD) has not been reported so far. The etiology of LPD is unknown and no mode of inheritance is known. We discuss possible modes of inheritance in the presented case, taking into account the possibility of a genetic syndrome. Given the similarity to other genetic syndromes with leiomyomatosis and skin alterations, we describe possible similar genetic pathomechanisms.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. leiomyomatosis peritonealis disseminata in association with Currarino syndrome?BACKGROUND: leiomyomatosis peritonealis disseminata (LPD) is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity. No reports of multiple congenital malformations associated with LPD have been found in the English literature. CASE PRESENTATION: A 27 year-old patient referred to our gynaecology unit for pelvic pain, amenorrhoea, stress incontinence, chronic constipation and recurrent intestinal and urinary infections. Multiple congenital malformations had previously been diagnosed. Most of these had required surgical treatment in her early life: anorectal malformation with rectovestibular fistula, ectopic right ureteral orifice, megadolichoureter and hemisacrum. An ultrasound scan and computed tomography performed in our department showed an irregular, polylobate, complex 20 cm mass originating from the right pelvis that reached the right hypochondrium and the epigastrium. The patient underwent laparotomy. The three largest abdominal-pelvic masses and multiple independent nodules within the peritoneum were progressively removed. The histological diagnosis was of LPD. CONCLUSION: The case we report is distinctive in that a rare acquired disease, LPD, coexists with multiple congenital malformations recalling a particular subgroup of caudal regression syndrome: the Currarino syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Pulmonary benign metastasising leiomyoma: response to treatment with goserelin.Benign metastasising leiomyoma is a rare disease occurring predominantly in women of childbearing age and is hormonally influenced. The response of the disease to the luteinising hormone releasing hormone analogue goserelin is reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. leiomyomatosis peritonealis disseminata associated with endometriosis: a case report and literature review.leiomyomatosis peritonealis disseminata (LPD) is a rare disease characterised by the presence of multiple smooth muscle tumour nodules throughout the peritoneal cavity. Approximately 50 cases of LPD have been reported to date in the world literature. There is a very high association with excess exogenous and endogenous female gonadal steroids, specifically oestrogen and progesterone. LPD has been described almost only in premenopausal women and is mostly asymptomatic. Four cases of malignant transformation of this disease have been reported. We report a case of a patient with prolonged exposure to oral contraceptives, operated because of hypermenorrhea and 'myoma in statu nascendi'. A metastasing myomatous tumour was suspected during the operation. LPD was diagnosed by intraoperative histological examination. Abdominal hysterectomy was performed. Five months later a second laparotomy had to be done because of an ovarian endometriosis tumour of about 10 cm size. A bilateral salpingo-oophorectomy and omentectomy were performed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |