1/19. A case of familial angiolipomatosis with Lisch nodules.Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/19. The management of drooling problems in children with neurological dysfunction: a review and case report.Drooling in children with neurological dysfunction indicates neurogenic failure to coordinate the muscles of the tongue, soft palate, and face which act in the first stage of swallowing. This causes excessive pooling of saliva in the anterior part of the mouth and consequent overspill. Treatment for drooling includes behavioral, pharmacological, and surgical modalities. Correcting a malocclusion has also been reported to help eliminate drooling. This paper describes the treatment of a child with neurofibromatosis, psychomotor, developmental, and neurologic retardation, and difficulty with speech. The patient was referred to our clinic to correct his drooling. Simple orthodontic treatment succeeded in eliminating the drooling and improving his speech.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
3/19. Ruptured internal carotid aneurysm resulting from neurofibromatosis: treatment with intraluminal stent graft.PURPOSE: This report shows a method of treatment for life-threatening hemorrhage due to rupture of an aneurysm in the cervical internal carotid artery caused by neurofibromatosis. methods: Ten days after delivery of healthy twins, a 28-year-old woman with known neurofibromatosis had sudden massive swelling in the left neck. After initial tracheostomy, angiography confirmed rupture of the mid cervical internal carotid artery as well as contribution to the resultant pseudoaneurysm from external carotid branches. Treatment began with coil embolization of the external carotid branches. The internal carotid lesion, a defect approximately 1 cm in length, was then closed through use of two stent grafts, each made from Palmaz stents and 3-mm polytetrafluorethylene grafts predilated to 6 mm. The neck hematoma was then evacuated surgically. RESULTS: Completion angiography and computed tomographic scanning confirmed control of the hemorrhage. The patient survived neurologically intact with the exception of cranial nerve deficits caused by the hemorrhage. The tracheostomy tube was removed 3 weeks postoperatively. Follow-up computed tomographic scanning showed a gradual decrease in the size of the cervical soft tissue and no recurrent aneurysm. CONCLUSION: Neurofibromatosis is a rare cause of aneurysmal degeneration of blood vessels. Repair of a ruptured cervical internal carotid artery aneurysm, though feasible, is difficult with stent grafts; however, this is a better option than surgical intervention in inaccessible vessels.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
4/19. A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
5/19. Neurofibromatosis involving the urinary bladder.We present two interesting cases of a 24-year-old man and a 14-year-old boy, uncle and nephew, with lower urinary tract symptoms, cafe au lait patches and subcutaneous nodules. ultrasonography and computed tomography scans showed a large, irregular lobulated soft tissue mass between the bladder and sacrum. cystoscopy, laparotomy and biopsies revealed neurofibromatosis involving the urinary bladder. No enlargement of the tumor or upper urinary tract obstruction has occurred during the long-term follow up. We recommend meticulous follow up of patients with giant intrapelvic neurofibromatosis.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
6/19. Malignant fibrous histocytoma of bone in neurofibromatosis--a case report.Multiorgan neoplasms are encountered frequently in patients with neurofibromatosis, which is a genetic disorder. Though skeletal abnormalities are common in these patients, the occurrence of primary bone sarcomas is very rare. We hereby report a case of post-radiation malignant fibrous-histiocytoma of bone developing in an elderly patient with neurofibromatosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/19. Neurofibromatosis: a diagnostic mimicker on CT in a known case of malignancy.A known case of early carcinoma cervix was found to have mediastinal widening on chest radiograph and hypoechoic oval retroperitoneal lesions on USG abdomen. CECT chest and abdomen showed these to be non enhancing lymph node like round to oval discrete mass lesions in mediastinum, abdomen and pelvis. With no other suggestion of carcinoma spread, local or distant and uncommon incidence of extensive lymphadenopathy in a early carcinoma cervix, biopsy from one of the representative lesion was performed which revealed it to be benign neurofibroma. Differentiation of these strategically located benign nerve sheath tumors from lymphadenopathy can sometimes be challenging on CT scan and in a known case of malignancy or with history of surgery for malignant neoplasm it may cause concern for disease spread or local tumor recurrence. Associated imaging and clinical features can sometimes be helpful in reaching the correct diagnosis.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/19. CT diagnosis of macrodystrophia lipomatosa. A case report.Radiographs and CT scans of a 45-year-old male with progressive enlargement of his right upper limb and shoulder are presented. Extensive soft-tissue hypertrophy with linear radiolucent bands (fat) limited to the lateral aspect of the limb were seen. exostoses-like bony overgrowth were also seen along interphalangeal joints. At CT, hypertrophic adipose tissue intermingling with muscle fibers was demonstrated, a diagnostic finding distinguishing the lesion from plexiform neurofibrolipomatosis, Klippel-Trenaunay syndrome and other angiomatous lesions.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
9/19. Segmental neurofibromatosis [NF type - v].Segmental neurofibromatosis is a rare variant of neurofibromatosis in which skin lesions are confined to a circumscribed body segment. A case of a 39-year-old man with this condition is presented, who was having multiple soft skin tumours over a localized area of back with no associated cafe au lait spots, axillary freckles or lish nodules. histology confirmed the diagnosis of neurofibroma.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
10/19. Sclerosing segmental neurofibromatosis.Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait macules and/or neurofibromas limited to a single body segment. The neurofibromas in segmental neurofibromatosis are usually soft, non-tender nodules as in other types of neurofibromatosis. Histopathologically, they are usually non-encapsulated, loosely textured dermal tumors. We report a case of sclerosing segmental neurofibromatosis, in which the patient presented with several grouped, erythematous to brownish, firm tender nodules on the left side of the posterior neck. Histopathologically, the stroma was mostly very fibrotic, especially around hair follicles, in addition to the usual features of neurofibroma. The atypical clinical feature, hardness, and tenderness of the lesions may be associated with the fibrosis.- - - - - - - - - - ranking = 0.16554075333837keywords = soft (Clic here for more details about this article) |
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