1/4. costello syndrome: a cancer predisposing syndrome?costello syndrome is a specific MCA/MR syndrome mainly characterized by dysmorphic facial features, peculiar biphasic growth pattern, motor and mental retardation, ectodermal anomalies involving skin and nails, and age dependent development of nasal and perianal papillomata. heart malformations and/or hypertrophic cardiomyopathy are frequently observed. We report a 4-year-old girl with costello syndrome who developed an intrathoracic ganglioneuroblastoma. In previous reports two patients with ectodermal tumours have been described, a ganglioneuroblastoma of the adrenal gland and an epithelioma. This third report suggests that neural crest neoplasia may be a significant risk factor for children with costello syndrome.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/4. Aggressive basal cell carcinoma with invasion of the parotid gland, facial nerve, and temporal bone.Aggressive variants of basal cell carcinoma (BCC), such as infiltrating, morpheaform, and basosquamous types, are associated with invasion of underlying tissues and are often difficult to treat.1 BCCs located in embryonic fusion planes, such as the periauricular region, are thought to exhibit deep extension and, subsequently, high recurrence rates, although this theory has been challenged and remains controversial.2-4 Despite the known features of aggressive BCC, parotid gland invasion and temporal bone and facial nerve involvement are rarely reported occurrences. We describe two patients with morpheaform BCC in the periauricular region demonstrating direct invasion of the parotid gland and concomitant facial nerve involvement. These patients require complex surgical management, as highlighted in this report.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
3/4. Malignant transformation of neurofibromas at multiple sites in a case of neurofibromatosis.A patient with classical neurofibromatosis is reported in whom malignant transformation of neurofibromas at multiple sites occurred, leading to a fatal outcome. One of these malignant tumours developed within the thyroid gland.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/4. Amplification of N-myc gene and increase of urinary VMA and HVA in patients with neuroblastic tumors.Neuroblastic tumor cases in our departments were evaluated in terms of the stage of the tumor, N-myc amplification, urinary vanillylamndelic acid (VMA)/homovanillic acid (HVA) and survival rate. Two asymptomatic cases, diagnosed when under a year old by mass screening, had no amplifications of N-myc but showed more than one value of urinary VMA/HVA ratio. The patients are now doing well eight years after complete excision of the neuroblastoma which had originated in the sympathetic ganglion. On the other hand, two other symptomatic cases, operated on at the ages of 3 and 5 years, showed remarkable amplifications with less than one value of urinary VMA/HVA, and died from the tumor soon after partial resection of the neuroblastoma and ganglioneuroblastoma which had originated in the adrenal gland and the sympathetic ganglion, respectively. The present monograph reports our cases and discusses prognostic factors.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |