1/177. Intracardiac leiomyomatosis: echocardiographic features. Intravenous leiomyomatosis is a histologically benign smooth-muscle tumor arising from either a uterine myoma or the walls of a uterine vessel with extension into veins. Echocardiographic features of two cases of intravenous leiomyomatosis with extensive spread into the right-sided cardiac chambers and pulmonary arteries are described. Both patients were middle-aged women, with prior history of hysterectomy 12 and 10 years earlier who presented with cardiac symptoms and signs. Distinctive echocardiographic features include 1) elongated mobile masses extending from the veins of the lower body, including inferior vena cava and azygos vein; 2) multiple venous attachments or metastases; and 3) filling of venous vessels and right-heart chambers. Intracardiac leiomyomatosis should be considered in a female patient presenting with an extensive mass in the right-sided cardiac chambers. ( info) |
2/177. Multiple pulmonary leiomyomatous hamartoma with secondary ossification. A 31-year-old woman presented with multiple pulmonary leiomyomatous hamartoma (MPLH) with secondary ossification. She had a past history of parosteal osteosarcoma. The pulmonary lesions were composed of spindle-shaped cells arranged in interlacing fascicles, among which glands or duct-like spaces were scattered. As some lesions contained bony tissues, it was unclear whether or not the pulmonary lesions were metastases of parosteal osteosarcoma. However, the majority of spindle-shaped cells were positive for alpha-smooth muscle actin, including cells proliferating around the bony tissues. Clonality analysis using a target of human androgen receptor (HUMARA) gene disclosed that the pulmonary nodules were polyclonal. These findings do not indicate that the lesions were metastatic. We would like to emphasize that MPLH can show osseous metaplasia. ( info) |
3/177. Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension. Intravenous leiomyomatosis with cardiac extension is a rare entity. The case of a 49-year-old patient is described: she was operated on for intracaval intra-atrial leiomyomatosis. After an incomplete procedure (the tumour appeared not totally resectable), the patient was treated for a period of three years with a GnRH-analogue, whereafter the patient was doing clinically well and the tumour, although it regained some growth, was in a stable situation. This new strategy seems of certain importance to the surgeon, as it carries an alternative to a high-risk reoperation. To our knowledge, this is the first description of such a combined therapeutical approach. ( info) |
4/177. Multiple leiomyoma of the renal capsule. Multiple renal capsular leiomyoma is an extremely rare tumour. We describe one such case which was managed by renal sparing surgery. ( info) |
5/177. Pulmonary leiomyomatosis in women after hysterectomy for uterine myoma. Benign metastasizing leiomyoma? INTRODUCTION: Leiomyomas, which usually occur multilocular in uterus, can develop even if rarely in other organs with smooth muscle cells. The tumour is considered benign; 2 case reports supports the hypothesis that uterus myoma could metastasize, and in the metastasis sites grow invasively. methods: 2 female patients 44 y. and 29 y. old were admitted to our clinic for MPL. Due to increasing tumor size respectively dypnea, they were operated on. Multiple nodules of the left lung in one case, and a mediastinal tumour in the other were resected; resected tumour was histologically examined. RESULTS: In both cases it was a matter of well-differentiated leiomyosarcoma. The mediastinal tumour has already invade the N. phrenicus. Postoperatively there were no complications. patients discharged in well-doing state, medical control one year later revealed no new growth. CONCLUSION: Multiple pulmonary leiomyomas are rare, they occur in sexually mature women in coincidence with uterus myoma. Even though many authors assume that MPL is a lung metastasis of benign tumours, the pathogenesis is still hypothetical. Supporting this thesis is the hormone dependence of both the uterine and the pulmonary tumours; against it, is that extrapulmonary locations are too rarely observed. The still open pathogenetical question has no therapeutical consequence. Whenever technically possible, a radical, parenchyma-saving surgical therapy should be the first choice. Otherwise hormon-ablation is a good alternative. ( info) |
6/177. Low-grade endometrial stromal sarcoma with intracardiac extension. Evolution of extensive smooth muscle differentiation and usefulness of immunohistochemistry for its recognition and distinction from intravenous leiomyomatosis. This case, a rare example of low-grade endometrial stroma sarcoma with extensive smooth muscle differentiation which extended to the inferior vena cava and cardiac chambers closely resembling intravenous leiomyomatosis grossly and microscopically, illustrates the importance of extensive sectioning and the usefulness of immunohistochemistry. Although spindle cell components arranged in interlacing bundles consistent with smooth muscle differentiation were recognizable in the primary tumor (on retrospective review), extensive smooth muscle differentiation in the recurrent tumors masked prototypical morphologic features of stromal sarcoma and only small neoplastic stromal components were preserved in limited areas, leading to initial failure to distinguish the lesion from intravenous leiomyomatosis. The immunophenotyping disclosed two distinct cell populations in the tumor: i.e. vimentin-positive and smooth muscle marker negative stromal cells, and vimentin-negative spindle-shaped desmin-positive smooth muscle cells. Our observation suggests that the predominance of a smooth muscle component in such a tumor can be misleading and does not always warrant a diagnosis of intravenous leiomyomatosis, nor does it predict a benign clinical course. This case also provides an insight into the relationship of the endometrial stroma and myometrium, and their cell of origin and the histogenesis of endometrial stromal sarcoma. ( info) |
7/177. Fine-needle aspiration cytology of esophageal leiomyomatosis. Leiomyomata are the most common benign neoplasms of the esophagus, but they are still very rare in comparison to malignant tumors of this organ. We report on the aspiration cytology findings of a case of esophageal leiomyomatosis in a 19-yr-old man. Diagn. Cytopathol. 1999;21:197-199. ( info) |
8/177. leiomyomatosis peritonealis disseminata: does malignant transformation occur? A literature review. leiomyomatosis peritonealis disseminata (LPD) is a rare smooth muscle tumor. In the literature more than 100 cases have been described. LPD is characterized by multiple small nodules on the peritoneal surface, mimicking a malignant process with metastases, but generally demonstrates benign histologic features. Exposure to estrogen seems to play an etiologic role. Many patients have uterine leiomyomas as well. The diagnosis of LPD is easily made on biopsy. Reduction of estrogen exposure is generally sufficient to cause regression of LPD. Surgical castration or gonadotrophin releasing hormone agonists seem good alternatives in the case of progression or recurrence of LPD. In six patients a malignant leiomyosarcoma has been described shortly after the diagnosis of LPD was made. Five of these patients did not have uterine leiomyomas or exposure to exogenous or increased endogenous estrogen. The relationship with pregnancy in the sixth patient may be coincidental. Whether malignant transformation of LPD occurs remains uncertain. Characteristics of these patients differ from those of LPD patients and may indicate a high malignant potential, necessitating a different approach. ( info) |
9/177. recurrence of uterine intravenous leiomyomatosis with intracardiac extension. Diagnostic considerations and surgical removal. A 28-year-old woman (gravida 2, para 2) was admitted 20 months after a hysterectomy because of fibromyoma. The hysterectomy specimen had shown intravenous leiomyomatosis. The patient presented with unspecific abdominal symptoms, serologic signs of hepatic and renal failure and clinical right-sided heart failure. Progression despite treatment with a gonadotropin-releasing hormone analogue promoted transferral to the present centre. Abdominal ultrasonography, phlebography and transoesophageal echocardiography showed a left pelvic mass and a seemingly free-floating tumour extending from the left main iliac vein via the inferior caval vein to the right ventricle. During a combined cardiac and distal caval approach using extracorporeal circulation, a 45 cm massive leiomyoma was removed successfully. Seven weeks later the left pelvic tumour was removed radically together with left oophorectomy. At control 12 months later the patient was well and without any remaining symptoms. ( info) |
10/177. 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. ( info) |