Data from 23 cases of lymphangiosarcoma associated with chronic lymphedema seen at the Mayo Clinic and 163 cases from the world literature are reviewed. The available information in 162 cases of postmastectomy lymphedematous lymphangiosarcoma revealed that the lesion occurred at an average age of 63.9 years (range 44 to 84 years) and an average of 10 years 3 months after mastectomy. Fifty per cent of the 129 patients eligible for follow‐up were dead within 19 months after treatment, and only 11 patients have survived 5 years or more. Amputation gave a slightly better prognosis than did radiation therapy. Of 24 patients (two from the Mayo Clinic) with lymphangiosarcoma and non‐postmastectomy lymphedema, 12 (50%) were dead within 34 months after treatment. Only two of the patients were long‐term survivors (5 and 15 years), and both were treated by amputation. Early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.
Three patients with polyarteritis nodosa associated with periosteal new bone formation in the lower extremities are described. Seven cases with many similar features have been collected from the literature. Pain, swelling, and skin changes in the lower extremity with periosteal reaction seem to characterize one of the syndromes found in polyarteritis nodosa.Diffuse periosteal new bone formation is an uncommon feature of polyarteritis nodosa, but it does occur in association with a specific set of symptoms and findings.The unusual roentgenographic findings in case 1 prompted us to review the roentgenograms of other patients with polyarteritis nodosa. From 1968 to 1972, one hundred fifty-eight patients with a diagnosis of polyarteritis nodosa have been seen at the Mayo Clinic. This diagnosis was proposed on the basis of clinical findings and confirmed by histologic examination of biopsy specimens. Approximately half of these patients had had roentgenograms made of one or more of their extremities. A review of these roentgenograms disclosed two additional cases associated with periosteal new bone formation. REPORT OF CASESCase 1. A 46-year-old male teacher came to the Mayo Clinic in November 1971 because of pain and ulceration of the skin on the dorsum of the right foot. He had had recurrent ulcers on the right, and occasionally the left, ankle and foot since 1963, with exacerbations usually in the fall of each year. Investigation at another institution in March 1967 revealed electromyographic evidence of a peripheral neuropathy involving both lower extremities. Skin biopsy a t that time was reported to be negative. In December 1970 he was again extensively investigated at the same institution. T h e skin biopsy at that time revealed acute and subacute inflammation in the superficial dermis. T h e eosinophil count and the serum glutamic-oxalacetic transaminase (SGOT) value were increased.When the patient was first examined at the Mayo Clinic he complained of aching pain of such severity that he was unable to perform his teaching duties. There was mild swelling of the lower part of the right leg and the ankle, with a blotchy bluish discoloration of the skin. There were numerous circular brown macules and a large ulcer. L'aricose veins were not prominent and arterial pulsations were normal.Relevant laboratory data were: hemoglobin, 10.4 g/ 100 ml; erythrocyte count, 4,43O,OOO/cu mm; leukocyte count, 8,20O/cu mm with 11.5% eosinophils; peripheral blood smear, consistent with blood-loss anemia; erythrocyte sedimentation rate, 73 mm in 1 hour (Westergren); SGOT, 15 IU/liter; sulfobromophthalein (BSP) retention, 23% in 1 hour; serum protein electrophoresis, albumin 3.43 g/ 100m1, al-globulin 0.22 g/100 ml, =,-globulin 0.73
too small. It will never hurt him." What are the results of such advice? Invasion of the eye, unnecessary dis¬ figurement, suffering and pain, more extensive surgery, radiation therapy, inconvenience, expense, and even death.
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