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Leiomyomatosis peritonealis disseminata is a rare condition characterized by the widespread formation of smooth muscle tumors within the abdominal cavity. Of 42 cases reported in the literature, only one case progressed to the development of malignant (cancerous) tumors. All patients were female, and the average age was 37 years. The cause of this disease is not known, but may be related to a hormonal factor, because in more than half the cases the women were either pregnant or using oral contraceptives. A case is described of a 25-year-old woman who developed pain in the right lower abdomen. Gynecologic examination and ultrasonography, the use of sound waves to visualize internal structures, revealed the presence of tumors in the uterus and other abdominal organs. The patient underwent laparoscopy, the examination of the abdomen using a tube-like optical device called a laparoscope. Several tumors consisting of smooth muscle cells were detected throughout the abdominal area, and two large nonmalignant tumors were removed. One year after the laparoscopy, a tumor was detected by physical examination in the right lower abdomen near the vagina and rectum. A second laparoscopy was performed and several tumors ranging in size from 0.5 to 10 centimeters were detected throughout the abdomen, including the uterus, ovaries, and liver. A diagnosis of leiomyosarcoma was made. The patient refused chemotherapy, or treatment with anticancer agents, and the tumors increased in size. The patient's condition deteriorated and she died 22 months after the initial diagnosis of leiomyomatosis peritonealis disseminata. This is only the second reported case of leiomyomatosis peritonealis disseminata progressing to malignancy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Malignant soft-tissue tumors of the abdominal wall consist of desmoid tumors and nondesmoid soft-tissue sarcomas. These neoplasms tend to invade adjacent musculoaponeurotic and bony structures. Transperitoneal organ invasion can also occur with devastating sequelae, especially after an incomplete excision. Extirpation with the full-thickness of the abdominal wall is required except for small, superficial lessions. A wide-margin resection, including any adherent viscus, segments of adjacent ribs, and iliac or pubic crest, offers the best assurance of local control. Adjunctive radiotherapy can be given when the margin of resection is unavoidably limited, but an effective radiation does can be difficult to administer, due to the sensitivity of underlying intestines. Metastases develop frequently with high-grade sarcomas; adjuvant chemotherapy may be tried in these patients, but its value remains unproven., The surgeon's main goal in treating cancer of the soft tissue of the abdominal wall is to control the tumor locally so that it does not recur and does not cross through the abdominal wall and invade vital internal abdominal organs. Malignant soft tissue tumors include sarcoma (malignant tumor beginning in fibrous, fatty, muscular, vascular, or nerve tissue usually first noticed as painless swelling) and desmoid tumors. Desmoid tumors are firm, circumscribed rubbery masses that infiltrate the fibrous sheaths and muscle fibers of the abdominal muscle. Sometimes called aggressive fibromatosis, they are infiltrative and prone to recur; many pathologists consider them to be low-grade malignant tumors. A review of surgical technique and the results of treatment of 55 patients treated for desmoid tumors or nondesmoid sarcoma is presented. Patients were treated over a 32-year period; there were 20 desmoid tumors and 35 nondesmoid sarcomas. Almost all of the patients with desmoid tumors were women of childbearing age; four of the men and two of the women were over the age of 50. The sarcomas did not show a particular preference for sex or age. The most common complaint was presence of a painless mass, although pain was noted in about half of the sarcoma patients. Except for small superficial masses, removal of the full depth of the abdominal wall with wide margins provides the best chance of local control of the tumor. If adequate tumor-free margin cannot be obtained, postoperative radiotherapy can be given. Local tumor control was achieved in 45 patients (86 percent). Twenty-four patients died of their disease; 23 had sarcoma and only 1 had desmoid tumor. Death was almost always due to metastasis (spread) to the lung. Metastasis often developed in patients with high-grade sarcomas. (Consumer Summary produced by Reliance Medical Information, Inc.)