Secondary breast angiosarcoma: A multicentre retrospective survey by the national Italian association of Breast Surgeons (ANISC).

BACKGROUND: Breast angiosarcoma is a malignant mesenchymal neoplasm, which accounts for approximately 2% of all soft tissue sarcomas. Secondary breast angiosarcoma (SBA) may be related to chronic lymphedema after a mastectomy with lymph node dissection (Stewart Treves syndrome) and previous radiotherapy for complications from breast radiation treatment. It is a very rare condition; therefore, diagnosis and management are still a challenge. METHODS: The ANISC collected SBA data by means of a survey sent to all Italian breast centres in the ANISC. The clinicopathological characteristics and the management of this disease were analysed. RESULTS: Twenty-four centres participated in this survey in which 112 cases of SBA were analysed. The median age of the women with SBA was 68.9 years and it appeared approximately 90 months after the first irradiation for breast cancer. In 92% of cases, a mastectomy was performed without axillary dissection for those patients having a high grade of SBA (74.2%). The prognosis was worse in the high-grade cases (overall survival-OS: 36 months) as compared with the low-grade cases (OS: 48 months). After a follow-up of 5 years, 50.5% of the patients were still alive. Disease-free survival (DFS) was 35 months, and there were no differences between the groups of patients with either high- or low-grade histology. CONCLUSIONS: Secondary breast angiosarcoma is a very aggressive disease associated with a short survival outcome. The surgical approach still remains an important step in the course of treatment; furthermore, an accurate histological examination is helpful in establishing the prognosis of the patient. A mastectomy is mandatory. A longer OS was observed in patients with low-grade angiosarcoma as compared to high-grade angiosarcoma (C.I. 40-57 vs. 31-41 months).

Diet only and diet plus simvastatin in the treatment of heterozygous familial hypercholesterolemia in childhood.

This study was a 1-year clinical study on 16 (7 males and 9 females) pediatric patients with heterozygous familial hypercholesterolemia treated with hypocholesterolemic diet only, or with diet plus drug (simvastatin 10 mg/day). According to the study protocol, the children were submitted to a 3-month washout (free diet). Then they were given a diet (American Heart Association, step 2) for 6 months. After 6 months they were divided into two groups matched for sex, age and body mass index (BMI). Diet only was given to group A (n = 8); simvastatin (10 mg/daily) was given to group B, for 1 year. All patients were examined at baseline, and monitored for safety during the study by pediatricians. All patients were submitted to noninvasive cardiovascular examinations (exercise electrocardiogram, echocardiography). After 12 months of treatment with simvastatin, total cholesterol (TC) and low density lipoprotein cholesterol (LDLC) showed a statistically significant reduction (group B). The decrease of TC and LDLC in patients on diet only was 4% and 3% (all) and 17% and 4% (group A) after 6 and 12 months, respectively.

Disorders of the intestinal mesenteric lymphatic system.

Disorders of the intestinal lymphatic transport system are rare and typically associated with protein losing enteropathy (PLE). Hypoproteinemia caused by intestinal lymphangiectasia is often associated with lymphedema of the legs and occasionally with chyluria, chylometrorrhea and chylous ascites. This article examines the varied presentations of lymphangiectasia syndromes including its pathophysiology. Diagnosis is based on signs and symptoms, specific laboratory findings, and confirmed by contrast small bowel series, lymphography and best of all laparoscopy. We describe 12 patients with PLE secondary to primary intestinal lymphangiectasia (1980-1991). Treatment was non-operative (dietary) in 8 patients and surgical in 4 including segmental resections of the jejunum in two, lymphatic-mesenteric venous anastomosis in one, and peritoneal-venous (LeVeen) shunt in one with overall satisfactory results.

[Historical development of the surgical treatment of gross obesity]. / Evoluzione storica della terapia chirurgica della grande obesità.

Reference is made to the experimental preliminary remark and the first, clinical observations underlying the surgical management of severe obesity, together with the techniques employed, starting with the jejunal-colic by-pass of Payne et al. This, however, led to serious liver damage and electrolyte imbalance, whereupon Payne et al. proposed end-to-side and Scott end-to-end jejunal-ileal by-pass. The end-to-end variety has been used with excellent results in 33 cases. Mason's gastric and gastroplastic techniques is till now unfrequently used. An account is also given of experimental ileo-cholecystostomy and biliopancreatic by-pass. In agreement with most other workers, a preference is expressed for the end-to-end jejunal-ileal by-pass as the most suitable technique available at present.

[Short-term tolerability of 2 types of termino-terminal jejuno-ileal bypass]. / Tollerabilità a breve termine di due tipi di by-pass digiuno-ileale termino-terminale.

The short-term tolerability of two types of end-to-end jejuno-ileal bypasses with different distal ileal loop lengths was evaluated. A comparison was made for 20 days of the behaviour of the main parameters influenced by the malabsorption syndrome thus induced. It was found that the operation involving a greater loss of small intestine was burdened with a greater number of discharges per day, an increase in total water loss, and an elevatem operative risk. This was compensated by more rapid and more evident loss of weight. Evaluation of the real meaning of the inversion of the A:G ratio observed in the short term with this type of operation was postponed to allow a longer follow-up period to run.

[Technical aspects of experimental reimplantation of limbs in the dog]. / Aspetti technici del reimpianto sperimentale di arti nel cane

Reimplantation of traumatically amputated limbs or their part is now carried out with increasing success due to the perfection of surgical techniques, especially in the case of microsurgery. Experimental perfection of such techniques is clearly the first step towards the clinical employement of reimplantation. Amputation and reimplantation of the hind leg in five dogs is reported. The first objective was the establishment of the reimplantation technique, whose salient features included: perfusion with dextran 40 and Trasylol, Kunster's centromedullary nail for osteosynthesis, microneurorrhaphy to bring together individual nerve funiculi, and vascular microsurgery based on previous experimental work. The immediate results show that the steps of the technique have now been established. Confirmation of their long-term validity, especially as far as functional recovery is now awaited.

[Treatment of gastroduodenal ulcers perforated into the free peritoneum. Apropos of 122 cases]. / Sul trattamento delle ulcere gastro-duodenali perforate in peritoneo libero. A proposito di 122 casi.

The treatment used in 122 patients suffering from perforation of gastroduodenal ulcer in free peritoneum is presented. 77 were subjected to straightforward suture of the perforation. Hemigastrectomy was carried out in 43 and in 2 superselective vagotomy associated with removal of the duodenal ulcer and duodenoplasty. Mortality in the series was 11.04% after suture but allowance must be made here for the serious condition of the patients who underwent this form of operation. Of the patients treated with straightforward perforation suture, 36.5% were reoperated for the reappearance of ulcerous symptomatology, while the remaining 63.7% were cured. On the basis of this experience, it is considered that superselective vagotomy associated or otherwise with pyloroplasty, and subtotal gastrectomy are indicated in patients in good general condition and in whom ulcer perforation symptomatology goes back less than 12 hours.

[LDL-apheresis: current status]. / La LDL-Aferesi: stato attuale.

PURPOSE: State of the art of LDL-apheresis and treatment of severe familiar hypercholesterolemia. PATIENTS AND METHODS: Clinical experience of treatment with LDL-apheresis of pediatric and adult patients with familial hypercholesterolemia using the following techniques: D.A.L.I., H.E.L.P., D.S.C. RESULTS: The outcome of treatment with LDL-apheresis in young patients, using the most recently introduced techniques, is reported. We have submitted to LDL-apheresis 11 pediatric patients. The youngest is aged 3.5. LDL-apheresis is able at improving the metabolic impairment and halting the natural evolution of atherosclerotic disease and atherosclerotic complications. CONCLUSIONS: At present, LDL-apheresis is the most effective and safe therapeutic approach to the treatment of homozygous, heterozygous and double heterozygous familial hypercholesterolemia.

Effect of L-carnitine on plasma lipoprotein fatty acids pattern in patients with primary hyperlipoproteinemia.

PURPOSE: To investigate the effect of L-carnitine (L-C) administration on plasma lipoprotein fatty acids pattern in patients with primary hyperlipoprotinemia. PATIENTS AND METHODS: The effect of L-C on plasma lipoprotein fatty acids pattern was investigated in 24 male and female hyperlipoproteinemic patients, aged 51.3 +/- 7.8 years (lipoprotein phenotypes: IIb and IV, WHO, 1970). After hypolipidemic diet (P, 22%; C, 48%; L, 30%; S, 10%; M, 10%; PV, 10%, cholesterol < 300 mg/d) lasting 30 days, L-C was given at a daily dosage of 1 g t.i.d. for 90 days. RESULTS: Plasma total polyunsaturated to saturated fatty acids ratio (8/5) showed a statistically significant increase after L-C (baseline: 0.74 +/- 0.2, vs 120 days: 0.84 +/- 0.2; p < or = 0.03). A statistically significant decrease of HDLs 16:0 (baseline: 28.1 +/- 4.2 vs 120 days: 26.4 +/- 3.7; p < or = 0.002), HDL3s 16:0 (baseline: 28.6 +/- 4.5 vs 120 days: 26.9 +/- 3.6; p < or = 0.001) and VLDLs 14:0 (baseline: 2.0 +/- 0.8 vs 120 days: 1.6 +/- 0.6; p < or = 0.02), specular to a statistically significant increase of VLDL's 18:2 (baseline: 21.9 +/- 5.1 vs 120 days: 24.3 +/- 4.9; p < or = 0.01), was also observed. Plasma levels of total cholesterol and LDL-cholesterol were reduced after one month. Plasma apolipoprotein AI and B levels were significantly increased after 30 and 60 days of treatment with L-C. CONCLUSIONS: A less atherogenic plasma lipoprotein fatty acids profile was observed after 120 days of combined treatment with diet and L-C.

Pattern of red blood cells and platelets cholesterol and fatty acids in homozygous familial hypercholesterolemic patients treated with LDL-apheresis.

Two homozygous familial hypercholesterolemic patients were treated with dextran-sulfate cellulose LDL-apheresis (DSC-LDL/A). We evaluated qualitatively and quantitatively, red cell and platelets membrane cholesterol and fatty acids, before and after LDL-apheresis. Fatty acids and cholesterol of red blood cells and platelets were determined by gas-chromatographic technique. We failed to observe any quantitative or qualitative modification, as far as the youngest patient (MD) is concerned. Only in the oldest patient (SM), docosaesanoic acid (22:6) values, were significantly reduced by LDL-A on quantitative basis. In the same patient, also mirystic acid (14:0) values, were significantly decreased as determined by qualitative method. The above mentioned fatty acids were significantly changed in platelets on treatment with LDL-apheresis performed on weekly basis.

Effect of oral and transdermal hormone replacement therapy on lipid profile and Lp(a) level in menopausal women with hypercholesterolemia.

OBJECTIVE: The aim of this randomized clinical study was to evaluate the hormonal replacement therapy (HRT) effect on plasma lipoproteins and Lp(a) profile in 42 menopausal women with primary hypercholesterolemia (total cholesterol > 240 mg/dL). SETTING: University clinic. PATIENTS AND METHODS: 42 hypercholesterolemic menopausal women were randomly assigned to the following groups; (1) transdermal estradiol, 50 micrograms + medroxyprogesterone 10 mg/day for days; (2) conjugated equine estrogens, 0.625 mg/day + medroxyprogesterone acetate 10 mg/day for 12 days; (3) no treatment. At baseline and after 3 and 6 months two blood samples were collected with a 24-hour interval in order to reduce intraindividual and laboratory variability. Serum total cholesterol, HDL cholesterol, triglycerides, LDL cholesterol, and Lp(a) were determined. RESULTS: Total cholesterol and LDL cholesterol significantly decreased after 6 months in both treated groups in comparison to untreated women; HDL cholesterol and triglycerides showed only minimal changes. HRT at the dosage utilized in the study did not seem influence the Lp(a) concentrations after 3 and 6 months. CONCLUSIONS: Both transdermal and oral estrogens at medium dosage have a favorable influence on total cholesterol and LDL-cholesterol level of hypercholesterolemic menopausal women, but Lp(a) remains resistant to manipulation.