Our results indicate that sacs from patients with inguinal hernias and especially from male inguinal hernias have fully differentiated SMCs. On the other hand SMCs in sacs obtained from boys with hydrocele are in an intermediate state of differentiation-dedifferentiation. This phenotypic modulation may represent attempted apoptosis of SMCs, since sacs more sensitive to apoptosis appeared to have more dedifferentiated SMCs. It also probably depicts the differing influence of sympathetic and parasympathetic tonuses during the descent of the testis and the obliteration of PV.
Melanotic neuroectodermal tumor of infancy should be included in the differential diagnosis of skull lesions in infants. Radical surgery must be considered as the treatment of choice and close follow-up for at least 2 years is necessary.
We retrospectively studied 18 patients with cystic fibrosis, who underwent surgery for gallbladder disease from 1975 to 1990. A long delay between the onset of symptoms and the diagnosis was noted (mean 8.7 months). This delay was attributed to masking of the symptoms of biliary disease by the malabsorption and pulmonary symptoms seen in this patient population. We do not recommend routine intraoperative cholangiography in patients with cystic fibrosis and gallbladder disease. Cystic fibrosis is a disease with progressive pulmonary deterioration. Cholecystectomy can be performed in these patients with relative safety if careful preoperative and postoperative care is provided. We recommend early operative intervention in the patient with gallbladder disease and cystic fibrosis.
Ewing sarcoma (ES), described as a diffuse endothelioma of the bone, is divided into two categories: osseous and extraosseous, which mainly affects adolescents. Extraosseous Ewing Sarcomas (EES) are rare tumors originating from soft tissues. Their clinical presentation depends mainly on the primary location of the tumor and are highly chemosensitive and radiosensitive. The purpose of this study was to describe the clinical characteristics and outcomes of 3 children with EES and uncommon presentation treated in our Unit. The diagnosis of EES was confirmed by biopsy and cytogenetic analysis with fluorescence in situ hybridization (FISH). Surgical excision was planned as primary treatment, followed by adjuvant chemotherapy according to EURO-E.W.I.N.G protocol. To date, all patients are alive, 1, 3 and 4 years after completion of treatment, with no signs of recurrence or metastasis.
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