1/191. Juvenile hyaline fibromatosis: clinical heterogeneity in three patients.BACKGROUND: Systemic hyalinoses are genetic generalized fibromatoses characterized by an accumulation of hyalin in the dermis. Two distinctive syndromes are recognized in the literature: infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF). ISH and JHF are sometimes difficult to separate since they show significant overlap. OBSERVATIONS: We report on 3 children from two unrelated families suffering from JHF. The first child is severely handicapped by joint contracture, massive hyperplasia of the gingivae, diffuse skin papules and subcutaneous nodules occupying the scalp, face, perianal area, palms, soles and chest. At the same age, the second child only shows pearly skin papules on the face, groin and perianal area and gingival hyperplasia without joint stiffness or any other subjective complaint. The third patient, a brother of the second child, developed mild skin abnormalities by the end of the first year. The occurrence in siblings and consanguinity in the second family suggests autosomal recessive inheritance. Histological skin examination in the 3 cases showed hyaline deposition in the dermis and abnormal ultrastructure of fibroblasts. Biochemical findings showed mucopolysaccharide abnormalities in both families. CONCLUSION: Our patients do not only illustrate the different expressions of JHF but also show some overlap with ISH, suggesting a common cause for both disorders. Genetic studies will finally answer this question.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
2/191. Fibromyxoma of the rib: report of a case.We present herein the case of a 36-year-old man with fibromyxoma of the rib. At the age of 33 years, an abnormal shadow of the chest was found on a radiographic examination, subsequent to which the mass grew slowly into the intrathorax, and he was referred to our hospital. An x-ray film and computed tomography scan on admission showed an extrapleural mass, 7 x 5 cm in diameter, in the left sixth rib with no evidence of destruction, cortical expansion, or rupture. The patient underwent peripheral osteotomy of the sixth rib, which was resected with a 3-cm margin around the mass. Microscopically, a diagnosis of fibromyxoma was confirmed. To the best of our knowledge, this is the first reported case of fibromyxoma originating in a rib.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
3/191. Solitary fibrous tumor of the spinal cord. Report of a case with scrape cytology.BACKGROUND: Solitary fibrous tumor is a rare spindle cell tumor and has been forced at a variety of sites. To the best of our knowledge, only two cases of solitary fibrous tumor arising in the spinal cord have been reported; no cytologic findings were documented. CASE: A 62-year-old male presented with a spinal cord tumor. A scrape smear of the resected tumor revealed naked, spindle-shaped nuclei. Some nuclei were twisted or had long spindles. In the background, abundant, thin and thick collagen fibers were present. Immunohistochemically, the spindle cells were positive for CD34 and negative for S-100 protein and alpha-smooth muscle actin. Histologic diagnosis of the tumor was benign solitary fibrous tumor. CONCLUSION: Our case indicates that solitary fibrous tumor can occur in the spinal cord and should be differentiated from other benign spindle cell tumors, such as meningioma and schwannoma. The key cytologic features of solitary fibrous tumor may be the presence of abundant thin and thick collagen fibers in scrape specimens.- - - - - - - - - - ranking = 0.19117207537543keywords = back (Clic here for more details about this article) |
4/191. Desmoplastic fibroblastoma (collagenous fibroma).Desmoplastic fibroblastoma is a vary rare subcutaneous proliferation. We describe a case of desmoplastic fibroblastoma in a 24-year-old Korean woman who presented with a 2.5 cm solitary and firm nodule on her back which had been present for 3 months. Histologic studies showed a well demarcated subcutaneous tumor composed of stellate or spindle shaped cells embedded in hypovascular fibrous or fibromyxoid stroma. No mitotic figures, calcification, or necrosis were observed. The stellate or spindle shaped cells were positive for vimentin. The stroma stained positively with alcian blue and Masson trichrome. S-100 protein, actin, desmin, and elastic fiber stains were all negative in the stellate or spindle shaped cells. There has been no recurrence or metastasis of the tumor over an 18-month follow-up.- - - - - - - - - - ranking = 0.19117207537543keywords = back (Clic here for more details about this article) |
5/191. Congenital annular multiple fibrofolliculomas occurring with deformity of the ear and ventricular septal defect.We describe a 5-year-old girl who had multiple fibrofolliculomas with an unusual annular configuration, present since birth, localized to the mid-back. She had no family history of similar skin lesions. Examination showed a depigmented patch on her left buttock and other congenital anomalies, i.e. deformity of the auricle of the ear and ventricular septal defect. There has been no previous report of congenital multiple fibrofolliculomas occurring with congenital malformations such as deformity of the auricle of the ear and ventricular septal defect. The congenital occurrence and unusual configuration of the lesions in our patient may suggest a naevoid origin for these tumours.- - - - - - - - - - ranking = 0.19117207537543keywords = back (Clic here for more details about this article) |
6/191. Desmoid tumor of the chest wall following chest surgery: report of a case.Desmoid tumors of the chest wall following chest surgery are a rare occurrence. A case of this disease is reported herein together with a review of the literature. A 74-year-old man, who had previously undergone a right lower lobectomy for squamous cell carcinoma of the lung, was referred to our hospital with an abnormal shadow on his chest X-ray. The tumor, located in the right lateral chest wall, was successfully resected by an aggressive, wide extirpation, and a final diagnosis of a desmoid tumor originating in the chest wall was made. When following up patients after surgery for lung cancer, the possibility of desmoid tumors developing in the incised chest wall should therefore be kept in mind.- - - - - - - - - - ranking = 14keywords = chest (Clic here for more details about this article) |
7/191. A case of spinal cord compression syndrome by a fibrotic mass presenting in a patient with an intrathecal pain management pump system.A 45-year-old woman presented with increasing low back pain, progressive anesthesia in her lower extremities and difficulty ambulating. She had a history of chronic low back pain problems for which, 26 months earlier, she had an intrathecal infusion pump permanently placed for pain and spasm control. Urgent magnetic resonance imaging (MRI) of the lumbar spine revealed a mass at the site of the tip of the intrathecal catheter with high grade spinal cord compression at the level of L-1. At surgical laminectomy the compressing lesion was found to be a reactive tissue fibroma. As more patients receive these devices the physician should consider cord compression syndrome in patients presenting with symptoms of increasing low back pain, anesthesia and progressive proprioceptive loss.- - - - - - - - - - ranking = 0.57351622612628keywords = back (Clic here for more details about this article) |
8/191. Odontogenic fibromyxoma arising from the periodontal ligament.A case is reported of an intraosseous odontogenic fibromyxoma which presented as an epulis and appeared to arise from the periodontal ligament of an upper lateral incisor. True neoplasms rarely present in this fashion, and the literature reveals no report of an intraosseous myxomatous neoplasm exhibiting such clinical features. Evidence supporting the periodontal ligament origin of the lesion is presented and discussed.- - - - - - - - - - ranking = 0.1826259808006keywords = upper (Clic here for more details about this article) |
9/191. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.- - - - - - - - - - ranking = 2.8648490293093keywords = discomfort (Clic here for more details about this article) |
10/191. Elastofibroma of the neck.Elastofibromas are benign lesions of the chest wall. We describe the first reported case of elastofibroma in the neck. Imaging features as well as location of the lesion were atypical. On computed tomography and magnetic resonance imaging the lesion contained a marked preponderance of fat, because the lesion arose within fat.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
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