1/60. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study.We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type iv, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract.- - - - - - - - - - ranking = 1keywords = lower (Clic here for more details about this article) |
2/60. myxoma of the humerus: an exceptional site of origin.myxoma of bone, outside of the jaws, is exceptional. We present such a tumor in the humerus and discuss the criteria for the diagnosis of myxoma.- - - - - - - - - - ranking = 4.8486072637735keywords = jaw (Clic here for more details about this article) |
3/60. Clinical characteristics of a familial inherited myxomatous valvular dystrophy mapped to Xq28.OBJECTIVES: The purpose of this study was to describe the phenotypic characteristics of an inherited myxomatous valvular dystrophy mapped to Xq28. BACKGROUND: Myxomatous valve dystrophies are a frequent cause of valvular diseases, the most common being idiopathic mitral valve prolapse. They form a group of heterogeneous diseases difficult to subclassify. The first mapping of the gene for a myxoid valvular dystrophy to Xq28 allowed investigation of the phenotype of affected members in a large family and characterization of the disease. methods: Among the 318 members in the pedigree, 89 agreed to participate in this study. Phenotypic characteristics were investigated using clinical examination, transthoracic echocardiography and biological analysis (F.VIII activity). Genetic status was based on haplotype analysis. RESULTS: Among 46 males, 9 were hemizygous to the mutant allele and had an obvious mitral and/or aortic myxomatous valve defect, and 4 had undergone valvular surgery. All had typical mitral valve prolapse associated in six cases with moderate to severe aortic regurgitation. The valve defect cosegregated with mild hemophilia a (F.VIII activity = 0.32 /- 0.05). The 37 remaining males had normal valves and normal F.VIII activity. Heterozygous women were identified on the basis of their haplotypes. Among the 17 women heterozygous to the mutant allele, moderate mitral regurgitation was present in 8, associated with mild mitral valve prolapse in 1 and aortic regurgitation in 3, whereas 2 women had isolated mild aortic regurgitant murmur. In heterozygotes, the penetrance value was 0.60 but increased with age. CONCLUSION: X-linked myxomatous valvular disease is characterized by mitral valve dystrophy frequently associated with degeneration of the aortic valves affecting males and, to a lower severity, females. The first localization of a gene for myxomatous valvular diseases is the first step for the subclassification of these diseases.- - - - - - - - - - ranking = 0.5keywords = lower (Clic here for more details about this article) |
4/60. Aggressive angiomyxoma: irradiation for recurrent disease.BACKGROUND: Aggressive angiomyxoma (AAM) is a tumor of the soft tissues predominately occurring in the genital and pelvic area with a strong propensity to local recurrences. The entity was first described in 1983. The tumor is regarded as low-grade sarcoma by some authors; its cause and pathogenesis are presently unknown. PATIENT AND METHOD: This is a case report on a 27-year-old man who underwent 4 surgical procedures of the left lower extremity because of a recurrent soft tissue neoplasm, initially (August 1993) diagnosed as a myxolipoma. The patient suffered from recurrences in February 1995, September 1996 and February 1998. The diagnosis was revised at the time of the latest recurrence. A palliative resection with macroscopic residuals left was performed in February 1998, followed by a radiation therapy with 56 Gy total dose and a concomitant administration of the radiosensitizer razoxane per os. The single radiation doses were 200 cGy 5 times a week. RESULTS: The small residuals of the tumor obviously regressed although an objective response could not be shown because the lesion was not clearly measurable. A follow-up 2 years after the radiation treatment revealed no recurrence. The time of the local control achieved as yet is already longer than any former time to regrowth between the surgical procedures. This is, to our knowledge, the first description of a therapeutic irradiation of a recurrent aggressive angiomyxoma. CONCLUSION: radiation therapy combined with the sensitizer razoxane is able to control a recurrent AAM for an unknown time. It remains open whether a radiation treatment alone would have had a similar effect.- - - - - - - - - - ranking = 0.5keywords = lower (Clic here for more details about this article) |
5/60. myxoma of the mandibular condyle. A case report and review of the literature.myxoma of the mandibular condyle is extremely rare and may cause signs and symptoms like those seen in patients with temporomandibular joint dysfunction (TMD). The histogenesis, histologic profile, and management of myxoma of the jaws remain controversial. We report a case of myxoma involving the mandibular condyle appearing as TMD, and we review the literature to highlight current controversies surrounding this lesion.- - - - - - - - - - ranking = 4.8486072637735keywords = jaw (Clic here for more details about this article) |
6/60. magnetic resonance imaging of myxoma in the mandible: a case report.The findings of conventional radiography, computed tomography, and magnetic resonance imaging (MRI) are reported for an odontogenic myxoma arising in the mandible of a 48-year-old Japanese man. The MRI characteristics of an intraosseous myxoma are described for only the third time. MRI showed a well-defined and smooth-walled mass lesion with high-signal intensity on T2-weighted images and low-intermediate-signal intensity on TI-weighted images. Soft tissue myxomas have been reported to show low-signal intensity on TI-weighted images and high-signal intensity on T2-weighted images. However, the first MRI report of an intraosseous myxoma showed a higher-signal intensity on TI-weighted images and lower-signal intensity on T2-weighted images. The MRI findings in this study thus correspond to those of soft tissue myxomas and do not agree with observations of the first MRI report of the intraosseous myxoma. Further studies are needed to clarify the reasons for this difference.- - - - - - - - - - ranking = 203.59253808864keywords = mandible, lower (Clic here for more details about this article) |
7/60. myxoma of the jaws. Report of three cases.Odontogenic myxoma is a locally aggressive, uncommon benign tumour which arises from mesenchymal tissues normally present in developing teeth. The most frequent locations of odontogenic myxoma are the posterior regions of the mandible, as well as the condylar region. Since odontogenic myxomas are not associated with any specific clinical or radiological sign, a histopathological examination of the specimen is required for confirmation of the primary diagnosis. We report three cases of myxoma diagnosed during the last 18 years. Two of them were located in atypical regions of the mandible and one was located in the maxilla. Presence of a slow-growing swelling associated with expansion of the bone plates raised suspicion of a tumour in two cases, while in the third patient the myxoma was an incidental finding during radiological examination. Due to the unspecific nature of these lesions, in every case a histopathological examination of the surgical specimen was required for diagnostic confirmation. In one of the three reported cases, we shall underline the need to follow a correct diagnostic work-up of all radiolucent lesions of the jaws, in order to avoid contraindicated therapeutic procedures.- - - - - - - - - - ranking = 105.48005155432keywords = mandible, jaw (Clic here for more details about this article) |
8/60. Right atrial myxoma complicated with pulmonary embolism.A 25-year-old woman was admitted to our hospital with chest pain and dyspnea, and was diagnosed as having a right atrial myxoma complicated with pulmonary embolism. An emergency operation was performed with cardiopulmonary bypass. A papillary pedunculated tumor was found having a narrow-based attachment to the free atrial wall. After the tumor was carefully removed together with the atrial wall around the attachment, pulmonary embolectomy was performed. Several fragments of the tumor were removed, and sufficient back-flow from the pulmonary artery was established. The postoperative course was uneventful. However, a non-perfused area was observed in the left lower lung on pulmonary hemodynamic scintigraphy at 3 months after the operation. Long-term observation is required due to the high risk for metastasis and recurrence, and further surgical treatment remains the most appropriate treatment option. A second operation may be needed to prevent progression in complications.- - - - - - - - - - ranking = 0.5keywords = lower (Clic here for more details about this article) |
9/60. Perineal excision of a large angiomyxoma in a young woman following magnetic resonance and angiographic imaging.BACKGROUND: Aggressive angiomyxomas are rare, arise from connective tissue of the perineum or the lower pelvis, and affect predominantly young women. CASE: We describe an unusual case of aggressive angiomyxoma in which the perineal approach was possible owing to MRI scanning and selective angiography indications. CONCLUSION: In cases of large aggressive angiomyxomas these diagnostic procedures should make it possible to decide which operative route might be best for the patient.- - - - - - - - - - ranking = 0.5keywords = lower (Clic here for more details about this article) |
10/60. Intramuscular myxoma: a rare neck mass in a child.Intramuscular myxoma (IM) is a true mesenchymal benign tumour which is rare in both adults and children. In children, it most commonly affects the heart, mandible or maxilla. We report a 5-year-old child with IM presenting as a posterolateral neck mass and review the literature. IM, although very rare, should be included in the differential diagnosis of neck masses in the paediatric age group.- - - - - - - - - - ranking = 40.618507617729keywords = mandible (Clic here for more details about this article) |
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