1/6. Aggressive congenital fibromatosis of the small intestine in the newborn. Report of a case and review of the literature.A case of so-called congenital fibro(leio)myosarcoma of the small intestine in a 18-day-old female baby, treated only with surgical resection, was studied by immunohistochemistry and electron microscopy in order to investigate the proliferating cell type. The tumour cells showed positivity only for vimentin and CD 34 and were negative for smooth muscle actin, desmin, alpha-sarcomeric actin, factor VIIIR: Ag and S-100 protein. Ultrastructural findings showed oval nuclei with prominent nucleoli, rare intracytoplasmic mitochondria and well developed rough endoplasmic reticulum. According to histoimmunological and electron microscopy findings the proliferating cells were likely to be of fibroblastic origin. A 7-year follow-up showed a favourable clinical evolution thus confirming that surgical resection can be a sufficient therapeutic approach. The morphological findings and clinical behaviour suggest that more appropriate terminology for this tumour would be "aggressive congenital fibromatosis" which better highlights its local progressive invasion without metastases.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/6. Aggressive fibromatosis of the head and neck (desmoid tumours).Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/6. Unusual jaw lesions in the paediatric and adolescent patient: a management challenge.While major maxillofacial pathology in the young patient is relatively uncommon, non-malignant conditions may pose a significant treatment dilemma due to their aggressive or unpredictable behaviour. Several such diseases managed by the Oral and Maxillofacial Surgery Unit at the Royal Children's Hospital of Melbourne have been selected for review. Illustrative case reports to highlight the principles of management are presented.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/6. Aggressive psammomatoid ossifying fibroma of the inferior turbinate and lateral nasal wall.OBJECTIVE: Fibroosseous lesions are rare entities of the nose and paranasal sinuses, the terminology and classification of which are still confusing. Psammomatoid or aggressive (juvenile) ossifying fibroma is a benign messenchymal tumor usually met in the young age (5-15 years), in the sinonasal tract, bearing distinctive histomorphologic features and a tendency towards locally aggressive behaviour. METHODOLOGY: We report here a rare case of an aggressive psammomatoid ossifying fibroma of the inferior turbinate and the lateral nasal wall, with obstruction of the nasolacrimal duct, in a 68-year-old woman. RESULTS: diagnosis was based on physical examination, CT scan imaging and histopathological examination. Treatment consisted of endoscopic intranasal resection of the tumor accompanied by removal of the lateral nasal wall. CONCLUSIONS: Fibro-osseous tumors of the nose and paranasal sinuses require aggressive surgical approach in order to avoid recurrence. Complete surgical excision may not always be possible mainly due to the tumor's extent and location.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/6. Interferon-induced remission of rapidly growing aggressive fibromatosis in the temporal fossa.Aggressive fibromatosis is the name for uncommon soft-tissue neoplasms arising within musculoaponeurotic tissue. They show benign histologic features but have an aggressive local behaviour and frequently recur after surgery or radiation. A 48-year-old black woman presented with recurrent aggressive fibromatosis after primary radiotherapy in the left temporal fossa involving the base of the skull. The patient received interferon alpha2a subcutaneously for 6 months. A slow but steady reduction of the tumour was observed, and pre-existing symptoms disappeared.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/6. A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.Desmoid tumours, also known as aggressive fibromatoses, are rare lesions having intermediate biological behaviour between benign fibrous lesions and fibrosarcomas. Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in isolated form. We report a barium study, ultrasound, computed tomography and magnetic resonance imaging findings of a case in a nine-year-old boy with intermittent nausea and vomiting. Although intraabdominal desmoids are usually detected as a solitary lesion in sporadic cases, the case presented here had two mesenteric lesions in the left upper quadrant. intestinal obstruction and invasion of colon wall had occurred.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |