1/304. Simultaneous involvement of the jejunum and the colon by type-1 neurofibromatosis.Type-1 neurofibromatosis (NF-1) or Von Recklinghausen disease is an autosomal dominant hereditary condition that may affect the gastrointestinal tract in 25% of cases and which takes three main forms: ganglioneuromatosis, stromal tumors, and tumors in the duodenum and periampullar region. Not infrequently, these patients present with gastrointestinal bleeding. We present the case of a 48-year-old patient diagnosed as having NF-1, with relapsing episodes of gastrointestinal hemorrhage, in which we discovered the simultaneous presence of a stromal tumor in the jejunum together with polypoid and diffuse ganglioneuromatosis in the colon.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/304. Ganglioneuromatous polyposis of the colon associated with adenocarcinoma and primary hyperparathyroidism.A rare case of ganglioneuromatous polyposis of the colon found in association with mucinous adenocarcinoma and primary hyperparathyroidism caused by a parathyroid adenoma in a 77-year-old woman is described. We discuss the clinical implications of this finding and review the literature.- - - - - - - - - - ranking = 0.5keywords = ganglion (Clic here for more details about this article) |
3/304. Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2b: implications for treatment.Three infants, who presented with intestinal obstruction due to diffuse transmural intestinal ganglioneuromatosis, are described. mutation analysis of exon 16 of the RET proto-oncogene revealed germline M918T and thus, a molecular diagnosis of multiple endocrine neoplasia type 2b (MEN 2B). Two infants developed medullary carcinoma of the thyroid. The third had a prophylactic thyroidectomy despite no obvious thyroid masses and normal calcitonin concentrations, but microscopic multifocal medullary carcinoma was found on histological examination. Early recognition of intestinal ganglioneuromatosis with germline RET M918T mutation in pseudo-Hirschsprung's disease is an indication for prophylactic thyroidectomy.- - - - - - - - - - ranking = 3keywords = ganglion (Clic here for more details about this article) |
4/304. Adnexal-centered giant congenital melanocyte nevus with extensive ganglioneuromatous component and trisomy 7.Adequate interpretation of clinical and histopathologic features of giant congenital melanocytic nevus (GCMN) in newborns is a continued challenge. A GCMN with three large nodules and three polypoid exophytic tumors presented in the dorsum of a female full-term newborn, the borders exhibiting a spotted grouped pattern. Microscopic examination revealed a peculiar adnexal-centered (eccrine sweat gland ducts, acrosiringia, and hair infundibula) compound nevus expressing pagetoid intraepidermal spreading of epithelioid melanocytes. The nodules represented an extensive ganglioneuromatous component. The neurons and their neuropil were positive for neuron-specific enolase, S-100, synaptophysin, tyrosine hydroxilase, and PGP 9.5. In addition to these components, a poorly differentiated, fusiform, low-mitotic rate population of cells undergoing epithelioid differentiation (and probably neuronal differentiation) with nodular arrangement was also present in the polypoid tumors and deeper parts of the nevus, in part intermixed with the neurons. These cells were vimentin positive but S-100 negative. FISH studies revealed these cells to express three signals for the centromeric probe for chromosome 7 whereas the neuronal component showed just two. Adnexal-centered arrangement of melanocytes has not been emphasized in GCMN. Ganglioneuromatous differentiation has been rarely reported in this condition. trisomy 7 in GCMN has been reported only once previously.- - - - - - - - - - ranking = 2.5keywords = ganglion (Clic here for more details about this article) |
5/304. Retroperitoneal ganglioneuroma: report of a case diagnosed by fine-needle aspiration cytology, with review of the literature.A case of ganglioneuroma presenting as a retroperitoneal mass in a 5-yr-old girl was diagnosed by preoperative fine-needle aspiration cytology. The cytologic smears predominantly showed clusters of schwann cells, with scattered mature ganglion cells. The cytologic diagnosis of ganglioneuroma was subsequently confirmed on histopathologic examination. To date, very few reports on the cytologic features of this tumor exist. The importance of considering a confident diagnosis by cytology, and of the uncommon age group that may be affected, is stressed. Diagn. Cytopathol. 1999;21:194-196.- - - - - - - - - - ranking = 3.5keywords = ganglion (Clic here for more details about this article) |
6/304. rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma.rhabdomyosarcoma is a common childhood malignancy that may occasionally occur as a component of a mixed mesenchymal tumor, e.g., a triton tumor or malignant ectomesenchymoma. A case is reported of a 13-year-old boy who had resection of a paratesticular embryonal rhabdomyosarcoma with subsequent radiation and chemotherapy. Two years later, a retroperitoneal metastasis was resected. histology showed a mixture of rhabdomyoblasts, ganglion cells, and a third population of cells with combined features of these two distinct cell types. Electron microscopy confirmed the presence of rhabdomyoblasts with characteristic bundles of myofilaments and Z-band material, and ganglion cells with prominent nuclei and nucleoli, rough endoplasmic reticulum, dense core granules, filaments, and tubules. Notably, the third cell population showed features of both rhabdomyoblasts and ganglion cells. immunohistochemistry confirmed the mixed population of rhabdomyoblasts (positive for vimentin, desmin, negative for S-100, NSE), ganglion cells (positive for S-100 and NSE, negative for vimentin and desmin), and the third population expressing all test antigens. The features of this metastatic lesion are those of a malignant ectomesenchymoma with combined rhabdomyosarcoma and ganglioneuroma components. It is postulated that this lesion results from biphenotypic expression of tumor cells that previously expressed only rhabdomyoblastic differentiation. The role of prior chemotherapy and radiotherapy in this particular case is unclear.- - - - - - - - - - ranking = 2.5keywords = ganglion (Clic here for more details about this article) |
7/304. Endocrinologically active retroperitoneal ganglioneuroma with positive iodine-131-metaiodobenzylguanidine scintigraphy.BACKGROUND: A case of retroperitoneal ganglioneuroma incidentally found by ultrasonography in a 56-year old woman is presented. methods/RESULTS: Computed tomography revealed a solid round tumor 6 cm in diameter on the upper pole of the right kidney. iodine-131-metaiodobenzylguanidine weakly accumulated in the tumor 24 and 48 h after the injection. Her serum catecholamines were within normal limits apart from slightly elevated norepinephrine at one of the two examinations. During the operation her serum epinephrine and dopamine levels were elevated to about 50 and 800 times higher than pre-operative values, respectively. CONCLUSION: This case is a rare ganglioneuroma in an adult patient that is endocrinologically active.- - - - - - - - - - ranking = 3keywords = ganglion (Clic here for more details about this article) |
8/304. Trigeminal ganglioneuroma.We present the case of an 8-year-old girl with a ganglioneuroma in the left cerebellopontine angle region. The tumor originated from the sensory root of the trigeminal nerve. Histopathologically, it was composed of neoplastic ganglion cells and schwann cells, leading us to the diagnosis of ganglioneuroma. Intracranial ganglioneuroma is very rare. To our knowledge, this is the first report of a trigeminal ganglioneuroma. The nature and origin of this tumor are discussed and the literature reviewed.- - - - - - - - - - ranking = 4.5keywords = ganglion (Clic here for more details about this article) |
9/304. Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.- - - - - - - - - - ranking = 3.5keywords = ganglion (Clic here for more details about this article) |
10/304. Ganglion-cell tumor of the filum terminale: immunohistochemical characterization.A case of an unusual spinal neuronal tumor is described in a 36-year-old woman presenting with a buttock pain. The spinal tumor was fully characterized by neuroradiological means, and in particular MRI was of significant value in delineating the extension of the tumor within the spinal canal and its exophitic growth pattern. Pathologically, a well circumscribed tumor originating from the intradural filum terminale characteristically comprised both large and small cells, resembling mature and immature neuronal cells, respectively. In addition, two neuronal markers, i.e., chromogranin a (CGA) and neuron-specific enolase (NSE), and other markers such as glial fibrilary acidic protein (GFAP), S-100 protein, HNK-1, tyrosine hydroxylase and beta 2-microgloblin were investigated immunohistochemically. We found that both neuronal cells expressed immunoreactivity for CGA and NSE, and small neuronal cells showed more intense CGA immunoreactivity, indicating an earlier stage of neuronal differentiation. Weakly positive immunoreactivity for HNK-1 was also demonstrated in small neuronal cells, consistent with evidence of maturation along a neuronal differentiation. From these findings a pathological diagnosis of ganglioneuroma was made. This unique group of ganglion-cell spinal tumors is reviewed in the literature and differential diagnosis and immunohistochemical features are discussed.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
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