Paragangliomas are rare neuroendocrine tumors where hypoxiainducible factor plays a critical role in tumorigenesis. A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Pdf familial malignant retroperitoneal paraganglioma. In 60% of cases, they secrete hormones and are classified as pheochromocytomas. The pdf file you selected should load here if your web browser has a pdf reader plugin installed for example, a recent version of adobe acrobat reader. Secretory paraspinal paraganglioma of thoracolumar spine. Pdf extraadrenal paragangliomas eap are rare endocrine.
When presenting within the abdominal cavity, they may arise as. Paragangliomas are extraadrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and. Pheochromocytoma and paraganglioma pheopgl and cyanotic congenital heart disease cchd are both rare diseases. Extraadrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing softtissue mass at ct, multiple areas of signal void interspersed with. The combination of necrosis with degree of tumour enhancement during the arterial phase increased the probability that a retroperitoneal mass would represent extraadrenal paraganglioma versus. Diagnosis and surgical treatment of retroperitoneal. Malignant abdominal paraganglioma with lymph node metastasis. Paragangliomas are characterized by secretions of excessive catecholamines, including epinephrine. Barfield r, hill da, hoffer fa, tekautz t, spunt sl 2002 retroperitoneal paraganglioma.
A pheochromocytoma adrenalineproducing tumor that grows outside of the adrenal glands is known as extraadrenal paragangliomas. Retroperitoneal paraganglioma is a rare tumor that develops from chromaffin cells that. It has been suggested that patients with congenital heart disease, in particular, may. We describe an inoperable case of a large retroperitoneal paraganglioma. A rare case of retroperitoneal paragangliomacase report. A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites including the head, neck, thorax and abdomen. But it can invade nearby parts of the body, become cancerous. Retroperitoneal paraganglioma with metastasis to the.
Survival of patients undergoing resection of retroperitoneal paraganglioma, stratified by metastasis. Pdf retroperitoneal extraadrenal paraganglioma researchgate. We report a case of nonfunctional retroperitoneal paraganglioma. Paragangliomas are very rare tumors derived from neuroendocrine cells of autonomic nervous system. Alternatively, you can also download the pdf file directly to your computer, from where it can be opened using a pdf reader. Retroperitoneal paraganglioma in a patient with fontan. Aditya date 1, bharat khadav 2, mackson nongma ithem 3. Nonfunctional pelvic retroperitoneal paraganglioma mimicking. We report a case of non functional retroperitoneal paraganglioma. Wed like to understand how you use our websites in order to improve them. Local recurrence of retroperitoneal paraganglioma treated laparoscopically ryuta watanabe, md, akira ozawa, md, tokuhiro iseda, md department of urology, matsuyama shimin hospital, ehime, japan all. Successful management of unsuspected retroperitoneal paraganglioma via the use of combined epidural and general anesthesia.
Extraadrenal paragangliomas account for only 10 to 15% of all paragangliomas and may present. Of 253 resections, 22 9% were retroperitoneal and were selected for further study. Retroperitoneal laparoscopic management of paraganglioma. Retroperitoneal paragangliomas are uncommon and we present two cases of familial malignant retroperitoneal paraganglioma. The preoperative diagnostic accuracy rate of retroperitoneal paragangliomas may be improved by focusing on the predilection sites and ct. The number of silent extraadrenal paraganglioma of retroperitoneum was 18 % of all 67 retroperitoneal paraganglioma resected by operation during this period of time in pumch.
An 81yearold woman was admitted with progressive abdominal fullness. Extraadrenal paragangliomas eap account for only 1015% of all paragangliomas of which 85%are located in the retroperitoneum. They are rare tumors, with an overall estimated incidence of 0. Survival of patients undergoing resection of retroperitoneal paraganglioma stratified by a size, b functional status, c margin status, and d nodal. Pheochromocytomas are catecholamine secreting tumors of the adrenal medulla chromaffin cells, however, when present extraadrenally they are called paragangliomas. A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components. Diagnosis and surgical treatment of retroperitoneal paraganglioma. Seventynine patients, 67 women and 12 men, were identified, 17 22%. Local recurrence of retroperitoneal paraganglioma treated. Paraganglioma is a rare tumor originates from paraganglial tissue which is composed of extraadrenal chromaffin cells. An unusual retroperitoneal mass non functioning retroperitoneal paraganglioma.
Hepatic metastatic paraganglioma 12 years after retroperitoneal. A single institute experience the harvard community has made this article openly available. It has been estimated that 10% of paragangliomas arise outside the adrenal glands 2. Retroperitoneal paraganglioma incidentally detected by. Retroperitoneal composite pheochromocytomaganglioneuroma. Successful treatment for retroperitoneal cavernous. Retroperitoneal paraganglioma represents between 21. Paragangliomas are rare neoplasms arising from cells of the primitive neural crest. Pdf retroperitoneal paragangliomas are rare neoplasms usually seen as histological surprises and can be confused with more common retroperitoneal. Nonfunctional retroperitoneal paraganglioma presenting as acute upper gastrointestinal hemorrhage. Pdf malignant retroperitoneal paraganglioma treated with.
Download fulltext pdf multiple retroperitoneal paragangliomas article pdf available in indian journal of surgery 751 june 20 with 24 reads. Mayo clinic records, the world literature, and the authors files were searched for patients with all or 2 of the 3 tumors. Retroperitoneal paraganglioma presenting as a chest pain. The tumor is often slow growing and noncancerous benign. Two cases of retroperitoneal functioning paraganglioma. Primary retroperitoneal paraganglioma simulating a.
Large retroperitoneal paraganglioma concurrent with. Retroperitoneal paraganglioma manifesting as paralytic. Diagnosis of functional paraganglioma by ultrasonographer. Estimation of 24 hour urinary catecholamine 23 weeks after surgery gives idea about biochemical cure and also recurrence or metastasis in later postoperative period. A resected case of liver metastases from extraadrenal. Successful management of unsuspected retroperitoneal. This tumor can affect people of any age but most often shows up between the ages of 30 and 50. K pandurengan, r natarajan, r marudavanan, t sowmya, k raja chidambaram. We reported a 30yearold patient with a right adrenal gland nodule and a. The most likely diagnosis is of a paraganglioma, with the main differential being a.
Paraganglioma of the gallbladder jama surgery jama network. These tumours are often difficult to diagnose and treat. Some features of this site may not work without it. We report a 49yearold male who underwent lymphadenectomy for a retroperitoneal tumor with a largest dimension of 6. Pdf retroperitoneal paraganglioma raymond barfield. Primary retroperitoneal paraganglioma simulating a pancreatic. Paragangliomas are extraadrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen.
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