1,721,410 research outputs found
White sponge naevus with minimal clinical and histological changes: Report of three cases
No full textWhite sponge naevus (WSN) is a rare autosomal dominant disorder that predominantly affects non-cornified stratified squamous epithelia: oral mucosa, oesophagus, anogenital area. It has been shown to be related to keratin defects, because of mutations in the genes encoding mucosal-specific keratins K4 and K13. We illustrate three cases diagnosed as WSN, following the clinical and histological criteria, with unusual appearance. They presented with minimal clinical and histological changes that could be misleading in the diagnosis. The patients showed diffuse irregular plaques with a range of presentations from white to rose coloured mucosae involving the entire oral cavity. In one case the lesion was also present in the vaginal area. The histological findings included epithelial thickening, parakeratosis and extensive vacuolization of the suprabasal keratinocytes, confirming WSN diagnosis. Clinical presentation and histopathology of WSN are discussed in relation to the differential diagnosis of other oral leukokeratoses. © Blackwell Munksgaard 2006. All rights reserved
Medullary thyroid carcinoma: state of the art
Full text linkMedullary thyroid carcinoma (MTC) constitutes about 3-10% of all thyroid cancers. It arises from the parafollicular C cells that produce calcitonin (CT) and occurs as a sporadic form. or less commonly, as a hereditary form, as part of multiple endocrine neoplasia syndromes types 2A (MEN 2A) and 2B (MEN 2B). The hereditary forms are autosomal dominant traits associated with germline mutations of RET proto-oncogene. Progresses in genetics have permitted an improvement of management, screening and treatment. Surgery is the only successful treatment for MTC, as there is no effective adjuvant therapy for residual disease. A total thyroidectomy and vigilant management and surveillance of the neck are recommended. Interdisciplinary management including surgeons, endocrinologists, pathologists, radiotherapists, radiologists, and oncologists should be considered
Ringraziamento a FREGO MAURO in: IACOBONE M., BARZON L., PORZIONATO A., MASI G., MACCHI V., MARINO F, VIEL G., FAVIA G. Parafibromin expression, single-gland involvement, and limited parathyroidectomy in familial isolated hyperparathyroidism.
No full textCushing's Syndrome
No full textIn 1932 Harvey W. Cushing, a Boston neurosurgeon, defined a syndrome characterized by muscular weakness, obesity, abdominal striae, diabetes and arterial hypertension, which he called “pituitary basophilism”, implying that it was a specific pituitary disease. Today, all conditions resulting in chronic glucocorticoid excess are known as Cushing’s syndrome. Iatrogenic Cushing’s syndrome is due to increased glucocorticoid intake and should be distinguished from the primary form. Spontaneous Cushing’s syndrome may be of pituitary or ectopic origin (corticotropin-dependent) or of adrenal origin (corticotropin-independent)
Angiosarcoma of the head and neck with intra-oral presentation. A clinico-pathological study of four cases
No full textAngiosarcoma of the head and neck is a rare malignant tumour that most commonly involves the skin and subcutis, particularly of the scalp. It has been reported in intra-oral locations very rarely. This study reports on the clinico-pathological features of four such cases, in elderly patients and manifesting an aggressive course. All patients were admitted for rapidly enlarging masses involving the hard palate, alveolar crest and retromolar trigone and displaying bone destruction in three cases. Morphologically, three cases were composed by bundles of spindle cells admixed with variably sized abortive vascular channels which only showed slit-like spaces separating large clusters of epithelioid cells. In all instances, intra-cytoplasmic vacuoles containing intact or fragmented red blood cells were distinctly evident. All neoplasms showed consistent CD 31 immunoreactivity and variable degrees of CD 34 and Factor VIII related antigen positivity and were responsible for the death of the patients, for local progression or lung metastases, within 6 months from the original diagnosis. Due to their rarity, angiosarcomas with intra-oral presentation may be diagnosed with difficulty, especially on small incisional biopsies, and their differentiation from spindle cell carcinoma, malignant melanoma, anaplastic lymphoma, malignant myoepithelioma and other sarcomas that more commonly arise at this site should be based on accurate immunohistochemical characterisation. (C) 2002 Elsevier Science Ltd. All rights reserved
L'autotrapianto di paratiroidi ed il follow-up dei pazienti operati per iperparatiroidismo secondario
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