1/10. Sinonasal teratocarcinosarcoma ("mixed olfactory neuroblastoma-craniopharyngioma") presenting with syndrome of inappropriate secretion of antidiuretic hormone.Sinonasal teratocarcinosarcoma (SNTC) is a rare, aggressive, histologically heterogeneous neoplasm of the paranasal sinuses and nasopharnyx of adults that is composed of variably benign or malignant neuroepithelial, epithelial, and mesenchymal elements. Occasional cases show intracranial extension and may be operated on by neurosurgeons and encountered by neuropathologists who may not be familiar with the entity. STNCs have not previously been associated with functional hypersecretory status. We report a 59-year-old male who presented with headache and syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and was subsequently found to have a bulky tumor of the frontal and ethmoid sinuses with focal dural invasion. The tumor was predominantly composed of olfactory neuroblastoma areas (90% of tumor) admixed with unusually well-developed craniopharyngioma-like mature squamous epithelium and ghost cells ( 10% of tumor). Scattered neuroblastoma tumor cells showed strong immunoreactivity with antibodies to arginine vasopressin, supporting ectopic hormone secretion by the tumor. While the coexistence of neuroectodermal and oral ectodermal-like differentiation in SNTCs is characteristic, in our case it was developed to an extreme functional and morphologic degree and was unassociated with other mesenchymal or epithelial elements often found in these complex tumors. SNTCs with limited differentiation have prompted controversy in classification.- - - - - - - - - - ranking = 1keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, vasopressin, secretion, hormone (Clic here for more details about this article) |
2/10. Olfactory neuroepithelioma arising from the olfactory placode.The patient was a 54-year-old man, who had lost his sense of smell 6 years previously and had started to become forgetful about 6 months prior to presenting at hospital. MRI admission showed a large multicystic tumor with Gd-DTPA enhancement extending from the anterior cranial fossa through the sphenoid sinus and into the nasal cavity. Histopathological examination revealed extensive proliferation of small round cells that were divided by connective tissue septae. The tumor cells occasionally formed tubular structures, although no basement membranes were present. On immunostaining, round tumor cells were positive for neuron-specific enolase, synaptophysin, and chromogranin a, while cells forming tubules were positive for AE 1 and CAM 5.2. Almost all of the tumor cells were positive for Ber-EP4, and some of the epithelioid cells surrounding the tubular structures were also positive for luteinizing hormone-releasing hormone (LH-RH). Electron microscopy demonstrated sporadic intercellular junctions, many microtubules in the tumor cell processes, and clear- and dense-cored vesicles in the cytoplasm. Based on the results, this case appears to be the first documented neuroepithelioma with Ber-EP4- and LH-RH-positive cells arising from the olfactory placode.- - - - - - - - - - ranking = 0.0002793131582211keywords = hormone (Clic here for more details about this article) |
3/10. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.We report a 56-year-old man in whom an olfactory neuroblastoma with epithelial and endocrine differentiation transformed into a mature ganglioneuroma after chemoradiotherapy. The tumor arising from the sphenoidal and maxillary sinuses showed rapid growth into the frontal lobe and metastasis to the cervical lymph nodes. The patient showed signs of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A radical craniofacial resection of the primary tumor was performed after 16 Gy of local irradiation and systemic chemotherapy. Three months after the operation, the patient died of mediastinal metastasis. The biopsy before chemoradiotherapy showed a neuroblastoma with Homer-Wright rosettes, fibrillary matrix, Flexner-Wintersteiner rosettes and antidiuretic hormone production. After chemoradiotherapy, the histology changed to that of a ganglioneuroma consisting of large ganglion cells and schwann cells without immature neuroblastoma components. Although transformation to ganglioneuroma in an adrenal neuroblastoma is common, an olfactory neuroblastoma showing ganglioneuronal maturation after chemoradiotherapy has not been reported. The pluripotent progenitor cells of the olfactory neurons may be the origin and their existence explains why various neoplasms with neuronal and epithelial differentiation arise from the olfactory mucosa.- - - - - - - - - - ranking = 0.2671534488339keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
4/10. Olfactory neuroblastoma. A report of 3 cases.Olfactory neuroblastoma is a rare malignant tumour, usually diagnosed at advanced stages. We studied 3 patients who were treated at our Institute between 1991 and 1999. One patient presented with a stage A and 2 with a stage B tumour. One patient presented with coma due to inappropriate secretion of antidiuretic hormone associated with a stage B tumour. All 3 patients were treated with complete surgical resection via a lateral rhinotomy approach and postoperative radiotherapy. There was no involvement of the cribriform plate. One patient developed a metachronous regional metastasis and was treated with neck dissection and radiotherapy. All 3 patients are free from recurrence with a follow-up period of 9 years, 18 months and 1 year, respectively. Combination therapy is the cornerstone of treatment for olfactory neuroblastoma. Complete surgical resection is the most important prognostic factor and can be accomplished via lateral rhinotomy for early stage tumours.- - - - - - - - - - ranking = 0.19909407838713keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
5/10. Ectopic Cushing's syndrome caused by an esthesioneuroblastoma.OBJECTIVE: To report a case of florid Cushing's hormone (ACTH) secretion related to the presence of an esthesioneuroblastoma (ENB). methods: We present clinical, laboratory, and pathologic findings in a 36-year-old Caucasian man presenting with Cushing's syndrome. Results of computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET) scanning, and somatostatin receptor scintigraphy are presented, along with tumor pathology findings. RESULTS: After initial biochemical studies suggestive of ectopic Cushing's syndrome, CT of the chest and abdomen revealed multiple cavitated pulmonary lesions, an ischiorectal mass, and bilateral adrenal hyperplasia. MRI of the pituitary gland revealed normal findings. Both PET scanning with [18 F]-flurodeoxyglucose (FDG) and somatostatin receptor scintigraphy with 111 indium-penetetreotide (Octreoscan) revealed strong tracer uptake in the ethmoid region. CT and MRI of the sinuses and brain subsequently localized a 5-cm mass in the ethmoid sinuses with intracranial extension. On biopsy, pathology results were consistent with a diagnosis of ENB, and immunohistochemical analysis revealed that tumor cells were strongly positive for ACTH, synaptophysin, and S-100, providing definitive diagnosis of ACTH-producing ENB. Hypercortisolemia was initially controlled by metyrapone, then by external beam radiation therapy (RT). CONCLUSION: This case illustrates the usefulness of nuclear imaging in the diagnosis of ENB, and the importance of prompt control of hypercortisolemia in Cushing's syndrome.- - - - - - - - - - ranking = 0.001243903128901keywords = secretion, hormone (Clic here for more details about this article) |
6/10. Expression of somatostatin receptors in arginine vasopressin hormone-secreting olfactory neuroblastoma--report of two cases.OBJECTIVE: arginine vasopressin hormone-secreting olfactory neuroblastomas are extremely rare, with fewer than twenty cases reported in the literature. Two of these cases, both initially presenting with the syndrome of inappropriate antidiuretic hormone, are presented. The second tumour was successfully identified using somatostatin receptor (octreotide) radiographic scintography. METHOD: The pathological specimens from both cases were examined immunohistochemically for somatostatin receptors. RESULTS: Samples from both cases demonstrated positivity for somatostatin receptors. CONCLUSIONS: This report demonstrates the potential use of somatastatin analogues in the investigation, follow-up and treatment of patients with olfactory neuroblastoma.- - - - - - - - - - ranking = 0.085186178019496keywords = antidiuretic hormone, antidiuretic, vasopressin, hormone (Clic here for more details about this article) |
7/10. Esthesioneuroblastoma recurrence presenting as a syndrome of inappropriate antidiuretic hormone secretion.BACKGROUND: Esthesioneuroblastoma is an uncommon intranasal tumor. These neuroendocrine neoplasms are rarely associated with excess hormone syndromes, and only nine cases of inappropriate antidiuretic hormone syndrome (SIADH) secondary to an esthesioneuroblastoma have been described. In all these cases, electrolyte abnormalities were seen when the tumor initially developed. We report a unique case of esthesioneuroblastoma recurrence manifesting as SIADH as the solely presenting feature. methods AND RESULTS: A 34-year-old woman was referred to us for evaluation for hyponatremia. She had undergone resection of an esthesioneuroblastoma at age 18 with radiotherapy. The patient had undergone annual CT of the nasal area and was considered to be disease free. The study of the hyponatremia was consistent with a SIADH, and MRI revealed an intranasal mass. The resection of the tumor reversed the hyponatremia, and pathologic analysis revealed a recurrence of esthesioneuroblastoma. CONCLUSIONS: Biochemical analysis should be performed in the follow-up of patients with esthesioneuroblastoma. In our case, the biochemical abnormality led to the diagnosis of the recurrence.- - - - - - - - - - ranking = 0.34485349501211keywords = antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
8/10. Olfactory neuroblastoma: report of a case associated with inappropriate antidiuretic hormone secretion.Esthesioneuroblastoma or olfactory neuroblastoma, is a rare tumour. It was first described by Berger et al. in 1924. Since then, approximately 250 cases have been reported. These neuroendocrine neoplasms are rarely associated with hormone excess syndromes. We report a case of olfactory neuroblastoma initially manifested with a syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 27-year-old man. A literature review is briefly presented.- - - - - - - - - - ranking = 0.47588820295248keywords = inappropriate secretion, antidiuretic hormone, antidiuretic, secretion, hormone (Clic here for more details about this article) |
9/10. Olfactory neuroblastoma invading the oral cavity in a patient with inappropriate antidiuretic hormone secretion.Olfactory neuroblastoma is an uncommon intranasal neoplasm that has not been previously documented to invade the oral cavity. The tumor's variable clinical manifestations and microscopic features may create a diagnostic dilemma for the clinician. The neoplasm has been identified as a direct cause of ectopic arginine vasopressin production leading to inappropriate antidiuretic hormone secretion. An unusual case of olfactory neuroblastoma invading the oral cavity through the maxillary sinus in a patient with pathologic antidiuretic hormone secretion is reported.- - - - - - - - - - ranking = 0.41826740691479keywords = antidiuretic hormone, antidiuretic, vasopressin, secretion, hormone (Clic here for more details about this article) |
10/10. Cushing's syndrome secondary to olfactory neuroblastoma.A case of olfactory neuroblastoma in a 36-year-old woman who presented with florid Cushing's syndrome is reported. A nasal polyp, which proved to be an olfactory neuroblastoma, was resected. The procedure was followed by complete remission from the endocrinologic abnormalities. Postoperatively, the patient was well for 5 years until recurrence of both Cushing's syndrome and the nasal polyp was noted. Following combined transnasal-transcranial resection of the tumor, which extended into the anterior cranial fossa, the patient again experienced complete remission of Cushing's syndrome. immunohistochemistry showed the tumor to be positive for neuron-specific enolase, synaptophysin, chromogranin, adrenocorticotropic hormone, beta-endorphin, and S-100 protein. Electron microscopy revealed neuritic processes containing microtubules and neurosecretory granules. This is the first reported case of Cushing's syndrome secondary to olfactory neuroblastoma.- - - - - - - - - - ranking = 0.00013965657911055keywords = hormone (Clic here for more details about this article) |
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