1/182. Composite ganglioglioma and dysembryoplastic neuroepithelial tumor.Both ganglioglioma and dysembryoplastic neuroepithelial tumors are well-recognized glial-neuronal neoplasms associated with chronic epilepsy and cortical dysplasia (neuronal migration abnormalities). The exact relationship between these 2 glial-neuronal tumors continues to be debated. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36-year-old woman in the left temporal lobe region. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. A focal area of cortical dysplasia is also identified. The MIB-1 labeling indexes in both components were low (<1% of tumor cell nuclei). The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/182. The inv(11)(p15q22) chromosome translocation of therapy-related myelodysplasia with NUP98-DDX10 and DDX10-NUP98 fusion transcripts.Chromosomal abnormalities involving the 11p15 or 11q22-23 bands have been reported in several types of human neoplasms including hematopoietic malignancies. The abnormalities are observed in therapy-related malignancies and less frequently in de novo myeloid malignancies. Abnormality of the MLL gene located on chromosome 11q23 has been well known in therapy-related myeloid malignancies, but it has been reported only recently that the inv(11)(p15q22) in de novo or therapy-related myeloid malignancies results in the fusion of NUP98 on chromosome 11p15 and DDX10 on chromosome 11q22. NUP98 is a nucleoporin that composes the nuclear pore complex and is the target gene in leukemia with the t(7;11)(p15;p15). The DDX10 gene encodes a putative adenosine triphosphate-dependent DEAD box rna helicase. Here we present another patient with acute myelocytic leukemia (M4) transformed from chronic myelomonocytic leukemia with the inv(11) chromosome who had been treated with etoposide for a germ cell tumor. By reverse transcription polymerase chain reaction (RT-PCR) of the rna from the leukemic cells of the patient, DDX10-NUP98 and NUP98-DDX10 fusion transcripts were detected. Our case confirms that the inv(11) is a rare chromosomal translocation that is associated with therapy-related or de novo myeloid malignancy and involves NUP98 and DDX10 but not MLL. RT-PCR of the fusion transcripts might be applied to the detection of a small number of leukemic cells in the bone marrow or blood of patients in remission or in the cells harvested for autologous transplantation.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/182. Angiosarcoma of the testis.A primary angiosarcoma of the testis in a 74-year-old patient was a highly anaplastic epthelioid angiosarcoma, which was positive for endothelial markers immunohistochemically. The tumour was unrelated to testicular germ cell neoplasm; the patient had received no previous radiation or chemotherapy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/182. Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases.Five patients with dysembryoplastic neuroepithelial tumour (DNT) showing extensive secondary haemorrhage, a finding not previously associated with these neoplasms, are described. The clinical presentations, neuroimaging findings, and histopathological features of these patients are reviewed. One patient, a previously asymptomatic 12 year old girl, presented with an acute intracerebral haemorrhage into a DNT. A further four young adults with histories of intractable partial and generalised seizures dating from childhood showed significant chronic haemorrhages within DNT, the MRI appearances in one patient giving a false impression of a cavernoma. Histopathology disclosed vascular abnormalities within these tumours which, together with other factors discussed, may have predisposed these tumours to haemorrhage.- - - - - - - - - - ranking = 1.0004193797946keywords = neoplasm, cerebral (Clic here for more details about this article) |
5/182. A frame shift mutation in the dna-binding domain of the androgen receptor gene associated with complete androgen insensitivity, persistent mullerian structures, and germ cell tumors in dysgenetic gonads.OBJECTIVE: To describe the molecular, cytogenetic, immunohistochemical, and endocrinologic characteristics of a young 46,XY female with persistent mullerian structures and germ cell tumors in dysgenetic gonads. DESIGN: Descriptive case study. SETTING: Mackay Memorial Hospital and National Yang-Ming University, Taipei, taiwan. PATIENT(S): A 22-year-old 46,XY female with persistent mullerian structures, a low level of serum testosterone, and no apparent adnexal masses. INTERVENTION(S): Laparoscopic removal of the dysgenetic gonads. MAIN OUTCOME MEASURE(S): Detection of an androgen receptor gene mutation by a semiautomated dna sequencer, of the chromosomal complement by cytogenetic examination, of placental alkaline phosphatase activity by immunohistochemical analysis, and of neoplasms in dysgenetic gonads by histologic studies. RESULT(S): A unilateral gonadoblastoma and a contralateral gonadoblastoma associated with a dysgerminoma were found in the excised gonads. The tumors had a 46,XY complement. Placental alkaline phosphatase was present in the tumor cells. A frameshift mutation in the dna-binding domain of the androgen receptor gene was detected in the patient's blood and the tumor tissues. A five-nucleotide "AGGAA" deletion at codons 608 and 609 of the androgen receptor gene resulted in a missing arginine and lysine as well as a frameshift that introduced a stop codon 12 amino acid downstream from the mutation. CONCLUSION(S): Molecular genetic analysis of the androgen receptor gene aids in the rapid diagnosis of complete androgen insensitivity irrespective of atypical clinical phenotypes and endocrinologic parameters.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/182. Diagnostic and therapeutic approach to sialoblastoma: report of a case.The natural history of a rare parotid tumor, the sialoblastoma (embryoma) is reported. It is a blastematous neoplasm said to recapitulate the epithelial differentiation of a gland at various stages of development. The tumor grew in a period of 5 years (from shortly after birth until it was excised) to a firm asymptomatic mass measuring 5 cm in greatest diameter. With a diagnosis of adenoid cystic carcinoma (a common misdiagnosis), the patient was referred to the reporting institution, where the diagnosis was revised. Five months later, completion parotidectomy for presumed recurrence failed to demonstrate residual tumor. One year later, the patient appears to be free of disease. The authors propose that sialoblastomas should be regarded neither as benign nor malignant, but as one single disease with local infiltrative potential. Based on this concept, sialoblastomas can be treated with early conservative surgery alone, provided that free margins are obtained.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/182. radiation-induced brain calcification: paradoxical high signal intensity in T1-weighted MR images.BACKGROUND: Irradiation to the central nervous system (CNS) in childhood is known to induce cerebral calcification after a latent period. Calcification has been generally found to show nil or a reduction in signal intensity in magnetic resonance (MR) images. However, we have studied three patients with radiation-induced brain calcification, who manifested increased signal intensity on T1-weighted MR images. METHOD: Three girls had each been diagnosed as having a suprasellar germ cell tumour and were treated with conventional fractionated radiotherapy in their childhood. In one case, chemotherapy was given prior to the CNS irradiation. FINDINGS: All three patients survived their disease, and a follow-up CT scan revealed calcification in the brain, which has shown an increased signal intensity in the T1-weighted images of MR. INTERPRETATION: Cerebral calcification may be presented as a high signal intensity in the T1-weighted MR images. This may be explained by a surface-relaxation effect by the calcium salt particle, precipitated in the brain due to radiation-induced mineralising microangiopathy.- - - - - - - - - - ranking = 0.022541220554255keywords = brain, cerebral (Clic here for more details about this article) |
8/182. Ovarian germ cell neoplasm in pregnancy.Ovarian germ cell neoplasm in pregnancy, with variable outcomes after debulking surgery is described.- - - - - - - - - - ranking = 5keywords = neoplasm (Clic here for more details about this article) |
9/182. Transverse sinus-tentorium splitting approach for pineal region tumors--case report.A 15-year-old boy with a large pineal region mass was admitted to our institute. The tentative diagnosis was mixed germ cell tumor. Tumor resection was carried out via a transverse sinus-tentorium splitting approach. The tumor tissue was completely resected, and no operative complication other than transient vertical gaze paresis was noted. The histological diagnosis was mixed germ cell tumor composed of mature and immature teratoma, germinoma, and embryonal carcinoma. After three courses of chemotherapy, the patient underwent external irradiation. He remained asymptomatic with no signs of recurrence 42 months after the surgery. The combination of the infratentorial supracerebellar approach and the occipital transtentorial approach provides excellent views and work space above and below the tentorial notch. Transverse sinus section is not mandatory for this approach, but sectioning of the unilateral transverse sinus and the tentorium along the rectal sinus allows retraction of the falx and the underlying brain to the opposite side. Thus, a much wider horizontal and vertical projection is obtained. This approach enables safer and more extensive tumor removal for large pineal region tumors.- - - - - - - - - - ranking = 0.0031602629656684keywords = brain (Clic here for more details about this article) |
10/182. Undifferentiated embryonal sarcoma with unusual features arising within mesenchymal hamartoma of the liver: report of a case and review of the literature.Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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