Problems in diagnosis and management of desmoid tumors

Article Abstract:

Desmoid tumors are fibrous growths that arise in areas where muscles insert onto bone. The tumors rarely metastasize (spread) but they often recur and can invade surrounding tissues. For these reasons, they are considered a low-grade malignancy. Treatment usually consists of surgical removal; the role of radiation and chemotherapeutic agents is less well-defined. A retrospective analysis of the medical records of all patients who received a diagnosis of desmoid tumor at one hospital over a 10-year period was conducted. Twenty-nine patients were identified and there was a total of 32 primary tumors; one patient had four tumors. Eight had a history of trauma at the site of the desmoid tumor. Approximately two-thirds of the tumors were located outside the abdomen. Thirty-one tumors were completely removed. The total recurrence rate was 60 percent, with an average recurrence time of 21 months. The recurrence rate for children, at 88 percent, was higher than for adults, at 38 percent. Histological reports regarding the margins of the areas excised were available for only 14 primary tumors. Radiotherapy, in addition to surgery, was used to treat 11 tumors. Desmoid tumors are considered to be less important and not as threatening as they may appear, partly because they are rare and are not true cancers. A problem in evaluating treatment approaches is that, since these tumors have not been considered cancerous, data describing the margins of the resected areas have not been collected. It is important to determine whether tumors close to the resected margin are more likely to recur. Incompletely removed lesions should be treated with radiation. The desmoid tumor should be included in tumor registries to facilitate the gathering of information about this tumor type. (Consumer Summary produced by Reliance Medical Information, Inc.)

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Efficient management of adrenal tumors

Article Abstract:

There is often a delay in diagnosing adrenal tumors, tumors occurring in the hormone-producing adrenal gland located on the upper surface of the kidney. Patients may not seek medical care, and if they do physicians may not identify their symptoms as those of adrenal tumor. Adrenal tumors can produce three different hormonally-caused syndromes: hypertension (blood pressure elevation); virilizing (development of male secondary sex characteristics in a woman); and Cushing's syndrome (involving gain of body fat, fatigue, lack of menstrual periods or impotence, fluid retention, excess hair growth, and diabetes). Diagnostic evaluation for adrenal tumor includes many tests, often performed with the patient in the hospital. A review was undertaken to evaluate the accuracy and specificity of these diagnostic tests and to recommend the most efficient approach to diagnosis. Medical records of 121 patients who underwent surgery for adrenal tumors over a 20-year period were reviewed. The average time from onset of symptoms to diagnosis was 48.3 months. Patients who had hypertension as their main symptom were seen at an average of 57.7 months after symptom onset. This was significantly longer than for patients with Cushing's syndrome, who were seen at an average of 22.8 months after onset of symptoms. Computed tomography was the most sensitive diagnostic test. MIBG scanning (a scintigraphic technique using radioactive material) was useful in diagnosis of pheochromocytoma (one type of adrenal tumor), and angiography was helpful for patients with large tumors. Patients typically were in the hospital for tests for a prolonged period before surgery; greater use of outpatient facilities is recommended in order to reduce the preoperative hospital stay. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Statistics, Medical case management, Endocrine gland tumors

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Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors

Article Abstract:

Desmoid tumors are slow-growing sarcomas that may invade local tissue but do not metastasize (spread to distant parts of the body). Desmoid tumors may occur sporadically or may be associated with Gardner's syndrome, an inherited disease of the colon in which about 55 percent of patients develop desmoid tumors. Small tumors may be excised but large tumors, such as those within the abdomen, neck, thigh, pelvis, and shoulder, often cannot be removed. Results are presented of 17 years experience in treating patients with inoperable desmoid tumors. Ten patients were treated with testolactone, a drug that interferes with estrogen. Estrogen appears to nurture desmoid tumor growth, and its withdrawal causes resolution in about half the cases. Four tumors showed major regression, or more than a 50 percent reduction in tumor volume. Another eight patients received treatment with nonsteroidal anti-inflammatory medications (indomethacin, sulindac, warfarin and vitamin K1). This regimen resulted in one major regression, one partial regression, and three patients had tumor growth stopped for up to eight years. Another seven patients received both anti-inflammatory drugs and testolactone or tamoxifen (an anti-estrogen drug), resulting in five major regressions and one partial regression. These results suggest that nearly complete control of desmoid tumor growth in advanced disease can be achieved by a combination of two methods of treatment (testolactone or tamoxifen and nonsteroidal anti-inflammatory drugs). When anti-estrogen agents were not effective by themselves, the tumor usually responded to later treatment with nonsteroidal anti-inflammatory drugs. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Nonsteroidal anti-inflammatory drugs, Nonsteroidal anti-inflammatory agents, Growth, Antineoplastic agents, Estrogen, Tumors, Chemotherapy, Drug therapy, Combination, Combination drug therapy, Tamoxifen, Sarcoma, Estrogen antagonists, Gardner's syndrome

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