1/73. Malignant peripheral nerve sheath tumour in the maxilla associated with von Recklinghausen's disease.Malignant transformation of neurofibromatosis is one of the most serious complications of von Recklinghausen's disease (VRD). The most common associated malignancy is the malignant peripheral nerve sheath tumour (MPNST). Few cases of MPNST associated with VRD in the maxillary region have been reported. This report describes a rare case of MPNST in the maxilla and the aggressive nature of MPNST associated with VRD.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/73. Embryonal sarcoma of adult and pediatric kidneys: report of a case with localized submucosal invasion of the renal pelvis and long-term survival.A case of cystic embryonal sarcoma of the kidney (CESK) with a rapidly fatal outcome was recently reported.1 Here, we report another case of a 12-year-old boy with a localized but ill-defined submucosal lesion of CESK in the right renal pelvis. The tumor consisted principally of small mesenchymal cells with oval to spindle nuclei and scanty cytoplasm, infiltrating in dense arrangements. Two growth patterns were distinguished in the tumor cells: (i) a diffuse infiltrating pattern without an epithelial component; and (ii) a foliated pattern with an epithelial lining over the surface. Foci of the diffuse pattern predominated over those that were lobular, infiltrating superficial layers of renal sinuses and along pyramids, in both of which remarkable intravenous invasion was evident. Foci of the foliated pattern invaded deeper portions of a few sinuses and frequently penetrated into their veins, producing together with their epithelial lining a characteristic foliated structure. Lining epithelial cells around lobular foci often appeared hob-nailed or eosinophilic in the cytoplasm. Despite the remarkable intravenous encroachment, the patient has remained well without a recurrence for more than 26 years after a simple nephrectomy. The present case report expands our understanding of the biological nature of CESK.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/73. Corticotroph pituitary carcinoma: case report and literature review.Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-year-old female first presented with an invasive and clinically non-functioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing's disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke's hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/73. Marginal-zone B-cell lymphoma, extranodal-malt-type: report of three cases.A high proportion of extranodal lymphoid infiltrates are diffuse small lymphocytic proliferations [1, 2] and therefore pose a particularly challenging diagnostic problem [1-4]. Their benign or malignant nature cannot be determined using clinical and radiologic criteria. The application of traditional morphologic criteria (i.e., cytologic maturity and polymorphism favor benignancy, while cytologic atypia and monomorphism favor malignancy) [5, 6], has probably improved diagnostic accuracy. However, these criteria generally have not been helpful in evaluating the large number of lymphoid infiltrates composed of monomorphic collections of small cytologically-mature appearing lymphoid cells [1-4] and, therefore, have not always accurately predicted clinical behavior. Extranodal lymphoid proliferations are also of considerable biologic interest since their pathogenesis and natural history have not been fully elucidated. Localized non-Hodgkin's lymphoma, extranodal-MALT-type. was diagnosed in our Department in three cases: Two arose in the orbit and the third one in the tonsil. The tumors had a typical histologic appearance. The microscopical features and immunohistochemical profile are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/73. Solitary fibrous tumor of the orbit with extraorbital extension: case report.OBJECTIVE AND IMPORTANCE: solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin that typically arise in the pleura. Only 24 cases of SFTs in the orbit have been reported, all located within the orbit and generally with a benign course. We report the first case of an orbital SFT with extraorbital extension and short-term regrowth. CLINICAL PRESENTATION: A 54-year-old man presented with proptosis and double vision that had persisted for 7 months. The tumor extended from the right extraconal inferolateral orbit to the extradural middle cranial fossa and cavernous sinus, via the superior orbital fissure, on magnetic resonance imaging scans. Positron emission tomography with [(18)F]fluorodeoxyglucose demonstrated faint uptake in the orbital portion. INTERVENTION: Resection of the tumor was performed twice, because of short-term regrowth of the residual tumor in the orbit. The histological diagnosis was a SFT. The MIB-1 labeling index was 7% and the mitotic count was 5 mitotic figures/10 high-power fields at the time of the second operation. These findings indicate the malignant nature of the tumor. CONCLUSION: The natural history of SFTs of the orbit remains unclear, and the importance of careful and continued follow-up monitoring of the tumor should be emphasized.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/73. Extramammary Paget's disease found by abnormal vulvar brush sampling.INTRODUCTION: Doctors are usually reluctant to perform a vulvar biopsy on a patient with non-specific chronic vulvitis--especially because of the rarity of vulvar malignancy in young women--until the lesion is suspected of being malignant. Therefore, most cases of extramammary Paget's disease (EMPD) were originally misdiagnosed as chronic and recurrent vulvar lesions. Late diagnosis of invasive lesions occurring in elderly females have resulted in cases of death. CASE: A 37-year-old patient showed an extended lesion on the vulva and perineum. In addition, abnormal cells were found from a vulvar scrape smear, and a following punch biopsy was used to diagnose and determine the extension of the disease. CONCLUSION: diagnosis and demarcation of EMPD remain difficult due to the multifocal lesions and subtle nature of the disease. Brush sampling taken from suspicious areas can be a guide for multiple biopsies to demarcate the lesion before major surgery. A brush biopsy is presented as a first-step method to detect vulvar malignancy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/73. chordoma of the thoracic spine--case report.A 44-year-old woman presented with a thoracic chordoma with intrathoracic extension manifesting as complaints of lower extremity weakness, hypesthesia below the levels of T5-6, and sphincter incontinence. Almost total resection combined with anterior interbody fusion and stabilization was possible through a left transpleural transthoracic approach. She suffered recurrence after 2 years and was considered inoperable. biopsy revealed a malignant chordoma with no sarcomatous differentiation. chordoma is an uncommon malignant bone tumor originating from remnants of the embryonal notochord, occurring mostly along the axial skeleton, at the extremity of the vertebral spine, and is least common in the thoracic region. Differential diagnosis is problematic and biopsy is helpful particularly if considered inoperable. Thoracic chordomas of the malignant type manifest as cord or root compression. Classical malignant chordomas must be distinguished from chondroid, benign, or other types of chordomas, since the biological behavior and clinical features are distinct. However, the differential diagnosis cannot be based on histological examination, but long-term follow up is required. Most patients have extradural and intraspinal tissue extension at the time of diagnosis, which makes complete resection impossible. Aggressive surgery without violation of surgical borders is the best choice in the treatment of thoracic chordoma. Thoracic chordoma is a recurring neoplasm and is prone to dissemination and sarcomatous differentiation despite its slow-growing nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/73. Proliferating (LMP) mucinous tumors of the ovaries with microinvasion: morphologic assessment of 13 cases.From a consecutive series of 124 proliferating (low malignant potential) mucinous ovarian tumors, 13 FIGO stage Ia tumors exhibited 1 or more microfoci of stromal invasion. The proliferating mucinous tumors with microinvasion were of intestinal (7/88), endocervical-like (4/26), or mixed epithelial differentiation (2/10) and were more likely to show high-grade (10/42) than low-grade (3/82) epithelial proliferative changes. Morphologically, foci of microinvasion were characterized by single or small groups of epithelial cells in the tumor stroma. These cells were accompanied by a sparse lymphocytic infiltrate and stromal desmoplasia in their immediate vicinity and by a greater degree of nuclear anaplasia in the invading cells and in the lining cells of the nearby glands or cysts than was present elsewhere in the tumors. Focal inflammatory and degenerative changes, commonly seen in ovarian mucinous tumors, were discriminated from microinvasive carcinoma by the nature of the inflammatory response, the presence of stromal mucin deposits, and the absence of nuclear anaplasia. Follow-up was available in 10 of 13 cases (mean 7.5 years) and did not identify any adverse clinical consequences for the patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/73. Clear cell sarcoma of the kidney with invasion of the inferior vena cava.OBJECTIVE: We present a case of clear cell sarcoma of the kidney (CCSK) in 53-year-old white man who was treated with surgery. This case represents the oldest patient with CCSK published in the English literature. methods: Right radical nephrectomy with lymphadenectomy and thrombectomy was performed. RESULTS: Histological findings indicated a CCSK. Tumor cells showed positive vimentin staining. CONCLUSION: CCSK is considered a rare and highly malignant renal tumor. The malignant nature may relate not only to the biological features of these tumor cells, but also to the high resistance against radiation and chemotherapy. The treatment of CCSK has been a subject of controversy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/73. Spinal intramedullary teratoma with exophytic components: report of two cases and review of the literature.True intramedullary teratoma is an extremely rare tumor, with only seven cases reported in the literature. The authors present two personal cases of spinal intramedullary cystic teratoma of the conus medullaris with exophytic growth and some unusual aspects. Their cases are unusual not only because they were diagnosed by MRI but also because the monitored microsurgical removal of the tumor was apparently total, with excellent results, in clinical and neuroradiologic terms. We recommend early radical surgery whenever possible, when the patient's neurological status is still good. To prevent traumatizing adjacent spinal cord for cases in which the teratoma tightly adheres to the functional neurological tissue of the spinal cord with no cleavage plane, we do not recommend an aggressive surgical attempt. Because of the mostly benign nature of this disease, the symptomatic recurrence of such incompletely resected mature teratomas is slow and may eventually require a second surgical procedure. The relevant literature is also reviewed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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