Surgical treatment of extramammary Paget's disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision

Article Abstract:

Paget's disease gets its name from a description in 1895 of an eczematous lesion of the areola (surrounding the nipple). It is now widely appreciated that Paget's disease may occur in many tissues. The most common extramammary location is the anogenital region, but Paget's disease may be seen in the armpits, chest, auditory canal, and on the knee. Extramammary Paget's disease, now recognized as a distinct clinical entity, is slow-growing and may remain localized for many years. However, the condition may eventually become an invasive and fatally metastatic cancer, thus Paget's disease should be excised when diagnosed. The cancerous cells almost always extend beyond the boundary of the clinically apparent lesion, and therefore the surgeon is generally obliged to cut out a generous hunk of tissue to ensure that the entire lesion is removed. An alternative to this radical excision is Mohs micrographic surgery. In this technique, small portions of tissue at the boundary are removed and quick-frozen in preparation for histological processing. While the operation is in progress, tangential sections for microscopy are cut from the tissue on the cryostat or a freezing microtome. The surgeon may then be informed whether the margin is seen to be free of cancerous cells, or not. The advantage of this method is that smaller amounts of tissue may be removed and important structure may be spared; indeed, the surgery can often be performed under local anesthesia as an outpatient procedure. Of course, it must be clear that use of the Mohs micrographic method should not compromise the safety of the patient. This technique was evaluated in the cases of five men and one woman with extramammary Paget's disease. Four patients are without evidence of disease at periods ranging from 23 to 42 months. A review of the literature suggested that the recurrence rate with conventional surgery is about one-third, and that other researchers have achieved recurrence rates from 17 to 28 percent with Moh's surgery. Accordingly, Moh's micrographic surgery is at least as successful as conventional surgery. Conventional surgery, with its broader swath of tissue destruction, is not necessary in most cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Care and treatment, Methods, Cold, Cryotherapy, Carcinoma, Ductal, Ductal carcinoma, Cryosurgery

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Oesophageal cancer in South Africa: a review of 1926 cases

Article Abstract:

Although cancer of the esophagus was rare among black South Africans five decades ago, its incidence has increased at such an alarming rate that today it is the most common cancer among black South African men, and second only to cancer of the cervix among black South African women. Esophageal cancer accounts for 45.8 percent of all cancer in the rural Transkei region. In urban areas, the incidence is greater, and is now 125 new cases per 100,000 men per year and 37 per 100,000 women per year. The problem is exacerbated by the belief, held by many physicians, that the disease is likely to be more advanced and, consequently, less treatable among blacks at the time of diagnosis. These healers often do not bother to attempt to heal the disease, but merely to palliate the symptoms. A review of 1,926 new cases of esophageal cancer showed that 2.8 percent of the patients were assessed as Stage I at diagnosis, 19.8 percent at Stage II, and 77.4 percent at Stage III. The 22.6 percent of the patients diagnosed at stages I and II are good candidates for curative therapy, and a full 79 percent are likely to benefit from therapy which is more aggressive than the palliative intubation usually given to blacks with Stage III disease. Seventy-eight percent of the patients had good or fair performance at the time of diagnosis, that is, they could care for themselves and perform normal daily activities. This finding contradicts the prevailing notion that the black patients are generally more debilitated at the time of diagnosis. It was determined, however, that diagnosis was delayed in some patients who sought the help of general practitioners or traditional healers prior to hospital admission. It is hoped that education of health workers and the public may reduce this delay, and that the results of this study will result in the allocation of more resources to treat esophageal cancer among South African blacks. In the long run, however, the greatest benefit can only come from screening programs to identify esophageal cancer before symptoms develop, at a time when therapy provides the greatest chance of cure. (Consumer Summary produced by Reliance Medical Information, Inc.)

South Africa, Demographic aspects, Cancer, Health, Health and race, Ethnic groups, Esophageal cancer

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Clinical course of essential thrombocythemia in 147 cases

Article Abstract:

The characteristics, treatment, and clinical course were assessed in 147 patients with essential thrombocythemia, an increase in the number of platelets (cells involved in blood clotting). Most patients were about 60 years old, and more women were affected than men. At diagnosis, 53 patients were without symptoms and 50 patients had symptoms mainly involving the nerves that control the muscles of the blood vessels. Thrombosis, blood clot formation, was detected in the large vessels of 27 patients and a predisposition to bleeding developed in 27 patients; seven patients had both bleeding and blood clot formation. A prolonged bleeding time and/or decreased aggregation of platelets were detected in 45 of 61 patients; bleeding occurred more often in patients with an abnormally high number of platelets. Only 4 of 87 karyotypes, photographic records of cell genetic material, were abnormal. Cytoreductive agents, or drugs that decrease the number of cells, were given to 129 patients at diagnosis or during follow-up; agents that prevent cell aggregation were given to 60 patients. Cytoreductive agents included radioactive phosphorus (22 patients), busulfan (35 patients), and hydroxyurea (72 patients). Hydroxyurea failed to control thrombocythemia in 12 patients, who required alternative treatment. During the follow-up period, blood clot formation was detected in 14 treated and in two untreated patients, whereas bleeding occurred in only one case. Almost 74 percent of the patients had survived at a seven-year follow-up, and only one case of thrombocythemia progressed to acute non-lymphocytic leukemia, a type of blood cancer. The findings show that the formation of blood clots is the major complication of essential thrombocythemia, which can be therapeutically controlled. The most effective treatment of thrombocythemia is cytoreductive therapy, particularly in patients with a low risk of blood clot formation. (Consumer Summary produced by Reliance Medical Information, Inc.)

Complications and side effects, Drug therapy, Blood platelet disorders, Platelet function disorders

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