Screening of the G-6-PD deficiency in a sample of the Abruzzi population (author's transl)

The modified Beutler test has afterwards been employed for a screening research on 393 living in the Abruzzi subjects: none of them appeared to be affected by the G-6-PD defect.     

Analysis and long-term survival in sternectomy with plastic reconstruction for primary and secondary neoplasms of the sternum

From January 1980 to December 1999, 88 patients underwent surgical resection for tumours involving the sternum. Thirty were males, aged 16 to 76 years, and 58 females, aged 23 to 78 years (mean ages: 48 and 53 years, respectively). There were 30 primary malignant tumours, 28 local recurrences or distant metastases from breast cancer, 16 other tumours, and 14 radionecroses. Total sternectomy was performed in 8 cases, subtotal (> 50%) in 32, and partial (< 50%) in 48. Concurrent en-bloc resection of the anterior ribs was performed in 61 patients, and of the clavicle in 13. Resection was extended to the lung in 22 patients, to the pericardium in 17, to both in 2, to the diaphragm and pericardium in 4. Bone and soft tissue defects were repaired with prosthetic material associated with a muscular or myocutaneous flap in 55 patients, with prosthetic material alone in 13 cases, with a muscular or myocutaneous flap in 5 cases, and with other techniques in the remaining patients. The resection was considered to be macroscopically radical in 78 patients and palliative in 10 cases. There was one perioperative mortality and significant morbidity was limited to 13 cases. Among the patients treated with a radical intent, 48 were alive and disease-free at the end of follow-up. The 10-year survival rate was 85% in primary tumours. For breast cancer relapses, 10-year survival was the same as 5-year survival (41.8%). In our experience, an en-bloc sternal resection for a primary or secondary tumour, followed by plastic repair using prosthetic material and/or a myocutaneous flap, is a safe, effective treatment. This intervention permits the execution of extensive radical resections for sternal neoplasms, as well as enabling the patient to obtain a better quality of life. Long-term survival after radical sternectomy also depends on the histological type of the tumour.     

Surgical treatment of gastric metastases from cutaneous melanoma: experience of the National Cancer Institute of Milan.

AIM: To evaluate the role of a surgical approach in patients affected with gastric metastases from cutaneous melanoma. METHODS: A retrospective review of our local melanoma database of 2100 patients identified 31 cases with gastric metastatic deposits. Nine of them were considered candidates for surgical resection. RESULTS: Median overall survival of the 9 patients who underwent surgery was 14.2 months. Six (67%) underwent a local radical resection of disease, and 3 (33%) had a simple exploratory laparotomy. The median survival was 21.6 months (range, 4-32 months) for the subset receiving radical surgery and 3.6 months (range, 2-6 months) for the patients who had no resection. Median follow-up was 14.2 months. No specific correlation of serologic LDH levels and final outcome, as documented elsewhere, was observed. A marked decreased or substantial remission of symptoms with an improvement in quality of life was observed in all radically resected patients. CONCLUSIONS: Patients with gastric metastases from melanoma may benefit from surgery if all macroscopic disease can be removed. In addition, gastric resection in patients with symptomatic melanoma spread to the stomach provides important symptomatic palliation.     

Giant pilomatricoma

A 52-year-old man was examined for an ulcerated, rapidly growing reddish nodule. It was 5.5 cm high with an 11 x 6-cm base and located on the left clavicle. The lesion had been present for approximately 7 years, and the patient complained occasional burning and pain. Clinical differential diagnoses included cutaneous lymphoma, sarcoma, squamous cell carcinoma, and cutaneous metastasis. Histopathologic examination revealed a well-circumscribed tumor involving the whole dermis and the subcutis and composed of partially confluent aggregates of matrical cells admixed with eosinophilic cornified material containing shadow cells. In addition, multinucleated giant cells, areas of calcification and metaplastic ossification, edema, and hemorrhage were also observed. On the basis of histopathologic features, the diagnosis of pilomatricoma was made. Our report highlights an unusual clinical appearance of pilomatricoma that made us consider a variety of primary or secondary cutaneous neoplasms in its differential diagnosis.     

Successful outcome of haemodialysis-induced pseudoporphyria after short-term oral N-acetylcysteine and switch to high-flux technique dialysis

Pseudoporphyria is a blistering disease with skin fragility and shallow scarring that clinically and histopathologically closely resembles porphyria cutanea tarda. The two conditions can be distinguished by porphyrin levels that typically are elevated in porphyria cutanea tarda, but not or only slightly in pseudoporphyria. Pseudoporphyria can be induced by various medications (e.g. non-steroidal anti-inflammatory drugs, antibiotics, diuretics, retinoids), intense UV(A) exposure, or haemodialysis. Treatment of haemodialysis-associated pseudoporphyria is not yet standardized. We report here a 65-year-old male patient with chronic renal failure due to Waldenstr?m's macroglobulinaemia who was treated with conventional 3 times/week haemodialysis. He developed blistering skin changes on both hands, which were diagnosed as pseudoporphyria based on clinical, histopathological, and laboratory findings, and could be successfully managed with initial oral N-acetylcysteine and a switch from low-flux to high-flux membrane haemodialysis. The beneficial effect of the high-flux membrane technique in haemodialysis-associated pseudoporphyria has not been previously reported.