Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We presented a case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis. We have not found a hemangioblastoma with these associations, as far as we have seen in the literature. 36-year-old male was admitted with complaints of nausea, vomiting and ataxic gait in June, 1970. On admission, the examination revealed no evidence of increased intracranial pressure except for elevated CSF pressure by lumbar puncture and incoordination. The peripheral blood count disclosed slight erythrocythemia. Vertebral angiography revealed a vascular lesion of 2.0 cm in diameter situated almost in the midline of caudal cerebellum receiving its blood supply from the right posterior inferior cerebellar artery. In addition, a right vertebro-occipital anastomosis was visualized. Plain reoentgenograms showed persistent metopic suture and spina bifida occulta of C 5 - 6. After admission, installation of Ommaya reservoir and decompressive suboccipital craniectomy were performed, and a thyroid papillary adenocarcinoma was totally removed. After discharge, he had been well for two years until a month previously to the second admission, when he commenced to have again headache, nausea, and vomiting with ataxic gait. Vertebral angiography showed the tumor enlarged in size measuring 4.0 X 5.0 cm and the tumor stain was more irregular and less homogenous than 3 years before. Brain scan revealed an increased uptake in the midline of the posterior fossa. After readmission, in April, 1973, he gradually developed dysphagia, disturbance of articulation and inactivity of mentality and died from pneumonia in October, 1974. Autopsy revealed a vascular tumor originated from the medial portion of the right cerebellum and the tumor showed multiple cyst formation in the rostral part in contrast to the caudal solid mass. Histologically the tumor tissue was composed of capillaries supported by fine argyrophilic fibers, large clear interstitial cells containing lipid granules and hemosiderin pigment. Carcinoma of the right lobe of the thyroid was found with metastasis to the bone marrow, lungs and anterior cervical lymphnodes and lymphnodes at the left supraclavicular angle. Bone marrow showed marked erythropoiesis. The case reported here provides an evidence to suggest that there is more than a random relationship between hemangioblastoma, dysraphic state and thyroid carcinoma. The other association, the vertebrooccipital anastomosis may result from the enhanced demand of blood supply by hemangioblastoma but this speculation needs further examination.
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PMID:[A case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis (author's transl)]. 79 Feb 13

Hemangioblastomas of the medulla oblongata were successfully excised in two separate cases. The uncertainties of this procedure involve the value of preoperative radiation therapy, the relationship between the tumor and the medulla oblongata, and the surgical techniques themselves. Cardiovascular and respiratory disorders often complicate this type of surgery, and postoperative dysphagia is a frequent sequela.
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PMID:Successful total extirpation of hemangioblastoma originating in the medulla oblongata. 404 Feb 69

A 41-year-old woman with a 12-year history of von Hippel-Lindau disease presented with progressive quadriparesis and difficulty swallowing. MRI revealed a well-circumscribed, partially cystic cerebellar neoplasm, consistent with hemangioblastoma. The tumor was resected and the diagnosis of hemangioblastoma confirmed. Embedded within the hemangioblastoma was a small focus of metastatic renal cell carcinoma (RCC). RCC metastatic to a CNS hemangioblastoma is the second most common type of tumor-to-tumor metastasis, which may be due to a number of factors. Proper immunostaining panels are required to clearly identify these cases since both tumor may have similar histology.
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PMID:A 41-year-old woman with von Hippel-Lindau and a cerebellar lesion. 2043 72

Hemangioblastoma in the medulla oblongata is a relatively rare tumor. We present the case of a giant hemangioblastoma occurring in the dorsal medulla oblongata. A 33-year-old man with no neurological symptoms was diagnosed with a hemangioblastoma in the dorsal medulla oblongata, and opted for observation in the outpatient department. After 22 months of observation time, MRI scans showed rapid local tumor progression and obstructive hydrocephalus. At this point, he presented with mild dysphagia as a preoperative neurological deficit. Total surgical removal of the tumor was performed after temporary ventricle drainage and preoperative embolization of the feeding artery. Postoperatively, he became fully conscious but developed bulbar palsy followed by tracheostomy. During the 12 months of postoperative follow-up, severe dysphagia was still present.
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PMID:[Case of surgical treatment for giant hemangioblastoma in the dorsal medulla oblongata]. 2239 51

Refractory orthostatic hypotension (OH) has been described following surgery for posterior fossa tumors. We present the case of a patient with refractory OH following attempted surgical resection. We also reviewed the available literature to describe pathophysiologic mechanisms for this rare entity. A 58-year-old female was found to have a hemangioblastoma at the cervicomedullary junction following workup for dysphagia and coordination difficulties. She underwent successful suboccipital craniotomy and gross total resection. However, the patient's symptoms returned several years later and a magnetic resonance imaging (MRI) showed tumor recurrence. A surgical resection was attempted but could not be performed due to significant scarring. Following discharge, she returned to our care with severe syncopal episodes, refractory OH, and an inability to ambulate. Aggressive medical therapy resulted in a gradual improvement in her ability to ambulate and a reduction in her orthostatic episodes. Unfortunately she died due to sepsis from aspiration pneumonia several months later. A survey of the literature yielded a total of 10 reports (14 patients) with refractory OH as a result of tumors in the cervicomedullary region. Five of fourteen patients died from complications related to OH and brainstem compression while the remainder had some improvement and were discharged. Refractory OH can rarely be a presenting sign of a tumor in the cervicomedullary junction or can manifest following surgical resection of tumors in this region. Recognition of OH and the institution of medical therapy (sodium and fluid replacement) and pharmacotherapy may curb the significant morbidity associated with this condition.
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PMID:Refractory Orthostatic Hypotension Caused by a Recurrent Hemangioblastoma: Case Report and Review of the Literature. 2715 70

This video demonstrates the microsurgical resection of brainstem hemangioblastoma. The patient is a 32-yr-old woman with Von Hippel Lindau syndrome who presented with quadriparesis and inability to swallow. Magnetic resonance imaging (MRI) of the neuroaxis revealed a brainstem cystic lesion with contrast-enhancing tumor nodule right along the posterior aspect of the lower part of medulla oblongata. The surgery was performed in the prone position with suboccipital craniectomy and partial C1 posterior arch removal. The aim of the surgery was to remove the tumor nodule.1-12The tumor was separated from the right dorsal nerve roots, and then progressively dissected with coagulation of arterial feeders and draining vein and division of the pia circumferentially. Postoperative MRI revealed complete resection. The patient completely recovered from her quadriparesis and difficulty swallowing.
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PMID:Microsurgical Resection of Medulla Oblongata Hemangioblastoma: 2-Dimensional Operative Video. 2963 2

Patients with von Hippel Lindau (VHL) disease develop multiple central nervous system hemangioblastomas (HB). The surgical resection of VHL-HBs is the standard of clinical care when these HBs become symptomatic due to tumor growth, edema, or cyst formation. VHL-HBs frequently present at the obex of the brainstem, making this a challenging surgical problem. Here, we present a case of a large symptomatic brainstem VHL-HB that was resected using a dorsal midline approach and midline myelotomy. This 35-year-old man with VHL disease and multiple prior resections of cerebellar hemangioblastomas presented with progressive bilateral upper extremity weakness, ataxia, and dysphagia. The accompanying two-dimensional (2D) video demonstrates the key techniques for resection of this brainstem hemangioblastoma, including recognizing the pial presentation, sharp pial/subpial dissection, and en bloc removal. Subsequently, we discuss circumferential dissection, identification, and division of feeding vessels. We also demonstrate key maneuvers to recognize and divide the main arterial pedicle while preserving the anterior spinal artery. The patient tolerated the resection without complication and the patient was discharged home after 6 days at his baseline neurologic function. This work was supported by the Intramural Research Program of the National Institutes of Health. The link to the video can be found at: https://youtu.be/mb2VqcLpxDQ .
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PMID:Resection of von Hippel Lindau Related Brainstem Hemangioblastoma. 3175 57