Chemotherapy, radiation therapy, targeted therapy, ablation, and embolization can be used for disease that has spread or come back. Bhanu prakash usmle, fmge and neet pg 24,364 views. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Glomus tympanicum tumors are small sized tumors originating in the middle ear. Diagnosi e terapia della sindrome paraganglioma springerlink. Adobe acrobat reader dcdownload kostenloser pdfviewer fur. A paraganglioma grows in the sympathetic or parasympathetic nerves. A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Paragangliomas are closely related to pheochromocytomas which are sometimes referred to as intraadrenal paragangliomas.
Paraganglioma, pheochromocytoma, sdhd, imprinting, parentoforigin. To our knowledge this is the first report of this kind of disease in the literature. Resembles paragangliomas elsewhere, with zellballen diffuse patterns of large polygonal cells in clusters separated by a delicate fibrovascular stroma. If you have problems viewing pdf files, download the latest version of adobe reader. Frequently, these tumors are associated with the vagus, tympanic plexus nerve, the carotid artery, or jugular bulb. Click on the link to view a sample search on this topic. All structured data from the file and property namespaces is available under the creative commons cc0 license. Ppt pheochromocytoma powerpoint presentation free to.
Donald current opinion in otolaryngology and head and neck surgery 12. Temporal bone paragangliomas tbps are benign tumors arising from neural crest cells located along the jugular bulbus and the. In other words, there are an adrenal tumor pheochromocytoma and extraadrenal tumor. The most frequent installer filenames for the program are. Pheochromocytoma and paraganglioma treatment is usually surgery and drug therapy. Iparagangliomi sono i tumori dei paragangli sia quelli di origine simpatica che parasimpatica. Pheochromocytomas are rare tumors that usually form in your adrenal glands. The facts you need to know pheochromocytoma is a part of the pheochromocytoma and paraganglioma group of syndromes. Here, we present an oroginal case of paraganglioma of the retroperitoneum with metastasis to the abdominal vertebra in a 42yearold female patient who was successfully treated by complete removal of the tumor and. According to the suggestions of neumann and coworkers we will use the term pheochromocytoma for all tumors in the thorax and the abdomen whereas the term head and neck paraganglioma hnp is only used for tumors in the head and neck region. Is essential to rule out the existence of paraganglioma in all retroperitoneal tumors, even in asymptomatic and hemodynamically stable patients. Download free adobe acrobat reader dc software for your windows, mac os and android devices to view, print, and comment on pdf documents. A 39yearold man was referred to the vascular surgeons with a rightsided cervical mass, palpitations, headaches and sweating. The paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively.
Paraganglioma and pheochromocytoma links to information. Normally, these hormones help the body deal with stress. Genetic testing is recommended for all patients with a paraganglioma and in selected patients with pheochromocytoma. The term pheochromocytoma is reserved for intraadrenal tumors. Files are available under licenses specified on their description page. Paraganglia definition of paraganglia by medical dictionary. Preoperative diagnosis helps to plan treatment and prevent complications. Introductionintroduction glomus tumors paragangliomas, chemodectomas glomus tumor. Theyll give your presentations a professional, memorable appearance the kind of sophisticated look that todays audiences expect. This article belongs to the special issue pheochromocytoma pheo and paraganglioma pgl. Paraganglioma dapcit current trends in the diagnosis nad management of head and neck paragangliomas chetan s. A paraganglioma is a type of tumour that arises from the peripheral nervous system part of the nervous system outside the brain and spinal cord, which is further divided into the sympathetic nervous system and the parasympathetic nervous system. Report of two cases article pdf available february 2016. Download harrisons principles of internal medicine 20th edition vol.
A glomus jugulare tumor grows in the temporal bone of the skull, in an area called the jugular foramen. We are one of the largest centers in the world to properly diagnose and treat patients with pheochromocytoma and paraganglioma. Pubmed is a searchable database of medical literature and lists journal articles that discuss paraganglioma and gastric stromal sarcoma. Extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. He has a history of metastatic paraganglioma initially diagnosed in september 2009 after symptoms of left chest and left groin pain and fatigue, followed by a finding on ct scan, ultrasound and mri of an 18x16x8. Pheochromocytomas and extraadrenal paragangliomas are rare tumors arising from neural crest tissue that develops into sympathetic and parasympathetic paraganglia throughout the body. Paraganglioma and pheochromocytoma are considered the same tumour. Backgroundcommonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically.
Mediastinal paraganglioma radiology reference article. Speakers are also expected to openly disclose intent to discuss any off label, experimental, or investigational use of drugs, devices, or equipment in their presentations. A tumor involving the paraganglia is known as a paraganglioma. Hereditary paragangliomapheochromocytoma is an inherited condition characterized by the growth of noncancerous benign tumors in structures called paraganglia. This patient was an extraordinary example of a rare tumor. A total of 24 retroperitoneal extraadrenal paraganglioma lesions in 24 patients were included in this study. Mediastinal paragangliomas are rare tumors derived from chromaffin cells neuroectodermal cells that can arise from two major clusters of sympathetic ganglion cells. Mr imaging features of benign retroperitoneal extra. The diagnosis and clinical significance of paragangliomas in. Paraganglioma is a neuroendocrine neoplasm, which is extremely rare in the vulva and. Winner of the standing ovation award for best powerpoint templates from presentations magazine. Paragangliomas arise from sympathetic or parasympathetic paraganglia and should now be defined by their site and type. Most pheochromocytomas start in the adrenal medulla, in the adrenal glands. M y l o n a s department of neurological surgery, walsgrave.
Terminology in science and clinical practice regarding tumors of the paraganglial system is divergent. The carotid paraganglioma was an incidental finding on the occasion of an mri angiography performed as part of explorations of tympanojugular paraganglioma in 1 case. The prognosis of pulmonary paraganglioma is typically good since most tumors are benign tumors. We retrieved a cohort of 86 patients diagnosed with a paraganglioma of the head and neck between 1945 and 1960 in the netherlands. Treatment of head and neck paragangliomas moffitt cancer center. Most, if not all, of the paraganglia secrete epinephrine or norepinephrine.
Unlike other types of cancer, there is no test that determines benign from malignant tumors. People with mutations in the gene inherit an increased risk of this condition, not the condition itself. The paraganglioma was discovered intraoperatively during a cervicotomy performed for a supposed chronic lymphadenopathy in 3 cases. Pheochromocytomas are tumors that produce too much of the hormones called catecholamines epinephrine and norepinephrine. Age and sex distribution pulmonary paraganglioma is a highly infrequent lung tumor that is usually observed in. Click export csv or ris to download the entire page or use the checkboxes to select a subset of records to download.
He had presented with abdominal discomfort 12 months earlier. Hereditary paragangliomapheochromocytoma genetics home. Pdfreader, pdfviewer kostenlos adobe acrobat reader dc. Dates of death were retrieved from the national bureau of genealogy. Normally, these nerves respond to changes in body temperature or blood pressure. Paragangliomasparagangliomas byby mohammed husseinmohammed hussein 2. For language access assistance, contact the ncats public information officer. Ffdg avidity of pheochromocytomas and paragangliomas. Paraganglia are groups of cells that are found near nerve cell bunches called ganglia. Histopathology and immunohistochemistry of adrenal. University of utah cme statement the university of utah school of medicine adheres to accme standards regarding industry support of continuing medical education. Retroperitoneal paraganglioma with metastasis to the. Ct of the neck with contrast demonstrated a large right carotid paraganglioma, extending superiorly from below. Pheochromocytoma is a part of pheochromocytoma and paraganglioma group of syndromes.
Paraganglioma is a malignant tumor that arises from the extra adrenal paraganglionic cells of the sympathetic or. The tumor is often slow growing and noncancerous benign. This area contains nerve fibers, called glomus bodies. Download fulltext pdf download fulltext pdf nonfunctional paraganglioma of the urinary bladder treated by transurethral resection. They are rare tumors, with an overall estimated incidence of 0 000. A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites including the head, neck, thorax and abdomen. Find evidencebased information on pheochromocytoma and paraganglioma treatment, research, and genetics. A focal area of early intense radiotracer uptake will be seen in the region of the paraganglioma, and is sensitive for detecting tumors greater than 1. A pheochromocytoma is a tumor arising in the adrenal gland medulla. Paraganglioma you and your hormones from the society for. Too much of either one can lead to serious conditions, such as high blood pressure and stroke. Paragangliomas 1 genetic and rare diseases information. The term paraganglioma is a generic one applied to tumors arising from paraganglia regardless of location 1, 2. Although, in some cases, the prognosis may be linked to the underlying men 2 disorder and its severity.
Some times paraganglioma are called extra adrenal pheochromocytoma meaning pheos found outside the adrenal glands. The jugular foramen is also where the jugular vein and several important nerves exit the skull. Pheochromocytoma and paragangliomahealth professional. Nonsyndromic paraganglioma can be inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to increase the risk of developing a paraganglioma or pheochromocytoma. The program lies within education tools, more precisely languages. The only exception is the paraganglioma of the adrenal. Treatment of head and neck paragangliomas kenneth hu, mark s.
Worlds best powerpoint templates crystalgraphics offers more powerpoint templates than anyone else in the world, with over 4 million to choose from. Paraganglioma and gastric stromal sarcoma genetic and. Paragangliomas are rare neuroendocrine tumors that arise from the extraadrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. However, the distinction between pheochromocytoma and paraganglioma is an important one because of implications for treatment, genetic testing, and risk stratification. The main difference is that pheochromocytoma are found on the adrenal glands, while paraganglioma can be found anywhere along the sympathetic and parasympathetic nerve chain. Octreotide scanning is useful for detecting the presence of multicentric or metastatic paragangliomas, and. Original download harrisons principles of internal.
Carotid body and carotid sinus anatomy, functions, clinical application medical animation duration. Scor global lifes public web site a 37yearold male applied for life insurance. We have a unique blend of academic and clinical medicine. Glomus jugulare tumor information mount sinai new york. Resection of a large carotid paraganglioma in carney. The peripheral nervous system is involved in controlling a variety of body functions including blood pressure, heart rate. They are rare chromaffin tumors that arise from neural crest cells. Investigations had revealed a gastrointestinal stromal tumour gist and left adrenalectomy. Our nih research protocol focuses on the genetics, diagnosis, localiz. Pheochromocytoma is tumor arising in the adrenal gland medulla, and paraganglioma arising in sympathetic or parasympathetic paraganglia. Nonsyndromic paraganglioma genetics home reference nih.
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