The radiological spectrum of orbital pathologies that involve the lacrimal gland and the lacrimal fossa.
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CT and MRI are utilized to differentiate between different types of masses and to determine the extent of lesions involving the lacrimal gland and the fossa. Although many diseases that affect the lacrimal gland and fossa are specifically diagnosed by imaging, it is frequently very difficult to differentiate each specific disease on the basis of image characteristics alone due to intrinsic similarities. In lacrimal gland epithelial tumors, benign pleomorphic adenomas are seen most commonly with a well defined benign appearance, and a malignant adenoid cystic carcinoma is seen with a typical invasive malignant appearance. However, a malignant myoepithelial carcinoma is seen with a benign looking appearance. Lymphomatous lesions of the lacrimal gland include a broad spectrum ranging from reactive hyperplasia to malignant lymphoma. These lesions can be very difficult to differentiate both radiologically and pathologically. Generally, lymphomas tend to occur in older patients. The developmental cystic lesions found in the lacrimal fossa such as dermoid and epidermoid cysts can be diagnosed when the cyst involves the superior temporal quadrant of the orbit and manifests as a non-enhancing cystic mass and, in case of a lipoma, it is diagnosed as a total fatty mass. However, masses of granulocytic sarcoma and xanthogranuloma, as well as vascular masses, such as a hemangiopericytoma, are difficult to diagnose correctly on the basis of preoperative imaging findings alone. A careful clinical evaluation and moreover, a pathologic verification, are needed. In this pictorial review, the various imaging spectrums of pathologic masses involving the lacrimal gland and fossa are presented, along with appropriate anatomy and pathology reviews. (+info)
MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors.
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Malignant peripheral nerve sheath tumors (MPNST) are sarcomas with poor prognosis and limited treatment options. Factors contributing to tumor progression are largely unknown. We therefore examined MPNST from 22 neurofibromatosis type 1 (NF1) patients, 14 non-NF1 patients, and 14 neurofibroma patients for matrix metalloproteinase 13 (MMP-13) expression. Because wild-type and mutant p53 were shown to differentially regulate MMP-13 expression, TP53 status and protein levels were also determined. MMP-13 expression was detected in 58% of MPNST and was significantly associated with recurrent MPNST (P = .019). p53 was observed in 78% of MPNST and was found to be strongly associated with MMP-13 expression (P = .005). In contrast, 14 neurofibromas lacked MMP-13 and p53 expressions. TP53 mutations were found in only 11% of MPNST and were associated with high tumor grades (P = .029). No significant association between mutant TP53 and MMP-13 was observed, indicating that other factors drive MMP-13 expression in MPNST. The presence of metastasis was linked to p53Pro(72) polymorphism (P = .041) and shorter survival. In summary, our data suggest that MMP-13 expression in nerve sheath tumors is coupled with malignant progression. Therefore, MMP-13 may serve as a marker for progression and as a therapeutic target. (+info)
Sacral window for the surgery of L5 neurofibroma: a technical note.
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OBJECTIVE: To describe a way to increase the exposure for L5-S1 neurofibroma extending to presacral area. SUMMARY OF BACKGROUND DATA: Since the neurofibromas of L5-S1 level generally extend to the extraforaminal and presacral area, their resection is difficult compared to other lumbar levels. METHODS: A 46-year-old female presented with severe pain in her left leg. There was a mild motor power loss in her left ankle and toe at plantar and dorsiflexion, and a mild hypoesthesia in the posterior of the left foot and its base. Lumbar computed tomography and magnetic resonance imaging showed a mass in the left intervertebral foramen extending to the extraforaminal presacral area at the level of L5-S1. The tumor was removed through a surgical bone window in the base of the sacrum and resection of L5 transverse process on the left side. RESULT: The tumor was removed totally. Following surgery, the patient's pain had totally disappeared and there was no alteration in her neurological status. CONCLUSION: L5-S1 neurofibroma was totally resected through a surgical window in the base of the sacrum. (+info)
Neurogenic tumor of the urinary bladder (a case report).
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Neurogenic tumors of urinary bladder are rare. A ganglionure fibroma arising from the trigons of urinary bladder in two year old boy presenting with hematuria, is described here. (+info)
Differentiation between schwannomas and neurofibromas in the extremities and superficial body: the role of high-resolution and color Doppler ultrasonography.
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OBJECTIVE: The purpose of this study was to verify whether high-resolution and color Doppler ultrasonography could distinguish schwannomas from neurofibromas. METHODS: Seventy-six tumors in 71 patients were included in this study. There were 46 patients (50 tumors) with schwannomas and 25 patients (26 tumors) with nondiffuse neurofibromas in the extremities or superficial parts of the body. Ultrasonographic examinations were performed in all patients. The tumor size, shape, location, internal echogenicity, posterior acoustic phenomenon (eg, shadowing or enhancement), capsule, relationship with the adjacent nerve, and presence of cystic components were documented. Color Doppler studies (75 lesions) and spectral Doppler studies (44 lesions) were performed. All data were analyzed with statistical software to check the significance. RESULTS: Both tumors presented as well-defined hypoechoic nodules on ultrasonography. They were oval or lobulated, showed posterior acoustic enhancement, and were hypervascular on color Doppler ultrasonography. There was no significant difference with respect to ultrasonographic morphologic characteristics. The rates of centric, eccentric, and undetermined associations with nerves were 40%, 20%, and 40% in the schwannomas and 46.2%, 0%, and 53.8% in the neurofibromas, respectively, showing a significant difference (P = .032). There was no significant difference in the chance of cystic changes between the schwannomas (30%) and neurofibromas (23%). CONCLUSIONS: No ultrasonographic finding (gray scale, color Doppler, or spectral analysis) allows differentiation between neurofibromas and schwannomas of the extremities. A nerve eccentrically entering a mass was seen only in schwannomas, and that may allow differentiation between neurofibromas and schwannomas. (+info)
Influence of hormones and hormone metabolites on the growth of Schwann cells derived from embryonic stem cells and on tumor cell lines expressing variable levels of neurofibromin.
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High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization.
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