Necrotising fasciitis involving the periorbita is a devastating infection. Potential outcomes range from severe disfigurement, loss of the eye and even to death. Early recognition is critical, although its initially non-distinctive appearance frequently delays diagnosis and treatment. Herein, the authors have performed a systematic review of previously published cases including clinical features, diagnoses and differential diagnoses, pathological characteristics and management. Periorbital necrotising fasciitis is seen mainly in adults with a female predominance (54%); about one-half (47%) of the patients were previously healthy. The infection can follow local blunt trauma (17%), penetrating injuries (22%) and face surgery (11%), but in about one-third of cases (28%) no cause was identified. Non-specific erythema and localised painful swelling of the eyelids characterise the earliest manifestation of the disease, followed by formation of blisters and necrosis of the periorbital skin and subcutaneous tissues. The causative organism in periorbital infection was mainly β-haemolytic Streptococcus alone (50%), occasionally in combination with Staphylococcus aureus (18%). The overall mortality rate was 14.42%. The main risk factor for mortality was the type of causative organism, since all reported cases of death were caused by β-haemolytic Streptococcus alone or associated with other organisms. Unlike necrotising fasciitis affecting other body sites, there was not a strong correlation with age >50 years or the presence of associated chronic illness. Management of periorbital necrotising fasciitis is then based on early distinction of symptoms and signs and aggressive multidisciplinary treatment. Thus, the delay between initial debridement and initiating parenteral broad-spectrum antibiotic therapy should be considered the most critical factor influencing morbidity and mortality.
Apoptosis is important for both tissue development and differentiation; its deregulation may contribute to tumourigenesis. In order to clarify the role of Bcl-2, an apoptosis-inhibiting protein, in pancreatic morphogenesis and tumour progression, its immunohistochemical expression was evaluated in 12 samples of fetal pancreas, in 10 samples of adult pancreas with ductal hyperplastic lesions, in 120 cases of primary pancreatic ductal adenocarcinoma, and in 43 synchronous metastatic lymph nodes. To evaluate the role of apoptosis in pancreatic cancer, p53 expression was also studied in tumour samples. Bcl-2 cytoplasmic acinar and ductal immunostaining was found in all fetal and adult tissue samples; ductal hyperplastic lesions were constantly negative. Thirty out of 120 (25%) tumours and 3 out of 43 (7%) lymph nodes expressed Bcl-2, whereas 67 out of 120 (56%) expressed nuclear p53. Well-differentiated tumours (G1) were more frequently Bcl-2-positive (p=0.002); furthermore, there was an inverse correlation between Bcl-2 and p53 expression in primary tumours (p=0.02). Neither Bcl-2 nor p53 influenced patients' prognosis, which was instead affected by N (p=0.02) and M (p<0.0001) status and stage of the disease (p=0.002). It is concluded that Bcl-2 regulates pancreatic morphogenesis and tissue homeostasis from early fetal to adult life and can be considered a phenotypic marker of normal exocrine pancreas. On the other hand, the lack of expression in preneoplastic lesions and the low positivity found in primary tumours and lymph node metastases suggest that Bcl-2 does not play a centralrole in pancreatic tumourigenesis and cancer progression.
These three new cases and the review of 43 cases reported in the literature call attention to the clinical features of carcinomas arising in the context of rhinophyma, raising further concerns about the possible association between these two entities. The need for histologic examination of all surgically removed tissue in patients with rhinophyma is highlighted. Several macroscopic changes, including ulceration, drainage, and a rapid growth pattern, should alarm the physician and should be considered as suspicious of a malignant degeneration. Unexpected clinical modifications of a preexisting long-lasting silent rhinophyma could indicate the possibility of hidden malignancy rather than a rhinophyma itself. Although evidence of an association between the two entities remains inconclusive, half of the malignancies reported in our review were incidental findings associated with rhinophyma. Thus, since rhinophyma should not be considered solely a cosmetic problem, we recommend that all specimens be reviewed by a pathologist and if malignancy is diagnosed, re-excision with clear margins should be achieved when necessary with periodic follow-up.
SBC is a rare type of BC, characterized by triple-negative features with an unexpectedly good prognosis. More data are needed to fully understand the behavior of this cancer and genomic profiling could be helpful in improving its diagnosis and management.
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