This article illustrates the anatomic distribution of the healthy paraganglion system and the imaging and pathologic features of various extraadrenal paragangliomas occurring in the head and neck, chest, and abdomen. The paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively. Our case highlights the importance of including the extraadrenal paraganglioma in the differential diagnosis of retroperitoneal tumours. But when it occurs below the diaphragm, in the organ of zuckerkandl or retroperitoneum, it is also called extraadrenal pheochromocytoma. Laboratory investigations showed an increase in liver enzyme levels. A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Operative findings the patient underwent left lateral sectionectomy for the liver metastases from the paraganglioma. It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the vagus nerve and hypoglossal nerve. A neuroendocrine paraganglioma was identified by histopathologic evaluation of the mass. Jun he, xingjun wang contributed equally to this work.
It is fascinating and challenging to medical fields because. This case is unusual in that the neoplasm showed extensive calcification, osseous metaplasia, and marrow elements. Retroperitoneal paraganglioma with metastasis to the abdominal. Is the most common of the head and neck paragangliomas. Rearrange individual pages or entire files in the desired order. Retroperitoneal and nonfunctioning forms are very rare. Mr imaging features of benign retroperitoneal extraadrenal. Retroperitoneal paraganglioma with metastasis to the. They are often asymptomatic and can reach a substantial size. An unusual presentation of mesenteric paraganglioma. Pheochromocytoma pcc and paraganglioma pgl are rare neuroendocrine neoplasms. Extraadrenal retroperitoneal paraganglioma in a dog.
Extraadrenal pheochromocytoma is uncommon, and extraadrenal composite pheochromocytoma is extremely rare. A paraganglioma is considered cancerous malignant if it spreads to other parts of the body metastasizes. All authors have read and approved the final manuscript. Pdf retroperitoneal extraadrenal paraganglioma researchgate. 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 authors. The immunohistochemical profile of investigated paraganglionic tumors.
These tumours are often difficult to diagnose and treat. The pdf file you selected should load here if your web. Paraganglioma is a rare neuroendocrine neoplasm which tends to develop in the extraadrenal glands. Retroperitoneal, paraganglioma, surgery introduction paragangliomas are rare tumors arising from extraadrenal chromaffine tissues, that is the paraganglia, which are widely distributed near or within the autonomic nervous system in a variety of retroperitoneal sites and in the sympathetic ganglia of various viscera. Pdf on oct 17, 2018, sanae ghammad and others published malignant retroperitoneal paraganglioma treated with radiotherapy. An 81yearold woman was admitted with progressive abdominal fullness. An unusual localization of retroperitoneal paraganglioma.
Local recurrence of retroperitoneal paraganglioma treated. This tumor can affect people of any age but most often shows up between the ages of 30 and 50. Box,j ohnsoncity,tn,u sa w estravineroad,suitesandkingsport,tn,u sa. To determine whether mri feature analysis can differentiate benign retroperitoneal extraadrenal paragangliomas and schwannomas. Some features of this site may not work without it. 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. In 60% of cases, they secrete hormones and are classified as pheochromocytomas.
We report a case of a 42 year old female presenting with abdominal pain who had a retroperitoneal tumour situated at the aortic bifurcation. Is essential to rule out the existence of paraganglioma in all retroperitoneal tumors, even in asymptomatic and hemodynamically stable patients. This is the third report of this kind in the english. Pheochromocytoma arising from outside the adrenal glands is commonly called paraganglioma. We report a case of a retroperitoneal paragangliomaof an unusual localization that. Retroperitoneal paraganglioma in a patient with fontan. We report a case of extraadrenal retroperitoneal paraganglioma in a 24yearold female. Large retroperitoneal paraganglioma concurrent with. Pathological examination confired the diagnosis of retroperitoneal paraganglioma, with no lymph node metastasis.
Retroperitoneal extraadrenal paragangliomas account for % of retroperitoneal tumors, originate in the paraganglionic system and are the. Subcutaneous ureteral bypass for treatment of bilateral. This case report describes a patient who initially underwent work up for a suspected pancreatic head mass which was discovered to be a retroperitoneal paraganglioma by frozen section. Estimation of 24 hour urinary catecholamine 23 weeks after surgery gives idea about biochemical cure and also recurrence or metastasis in later postoperative period.
Case report retroperitoneal paraganglioma presenting as a. A rare serious case of retroperitoneal paraganglioma misdiagnosed as duodenal gastrointestinal stromal tumor. On ct images, a retroperitoneal paraganglioma appears as a hypervascular mass. This case reminds us that neuroendocrine tumor should be included in the differential diagnosis of a retroperitoneal mass although composite paraganglioma ganglioneuroma in the retroperitoneum is.
Conclusion retroperitoneal extraadrenal paraganglioma eap are relatively rare neuroendocrine tumors. There was an upper paramedian, left sided, large, palpable mass on the physical examination. Case report retroperitoneal paraganglioma presenting as a chest pain. Pdf malignant retroperitoneal paraganglioma treated with. A resected case of liver metastases from extraadrenal. The retroperitoneal paragangliomas have a slight predilection.
The pathological examination concluded that it was compatible with a paraganglioma, 6cm long, with complete excision, no microscopic sign of malignancy, and no endovascular tumor extension. Several radiologic imaging and nuclear imaging techniques are currently available for evaluating extraadrenal paragangliomas. Primary retroperitoneal paraganglioma simulating a. Chemotherapy, radiation therapy, targeted therapy, ablation, and embolization can be used for disease that has spread or come back. Case report an 11yearold female child with hypoplastic left heart. Retroperitoneal laparoscopic management of paraganglioma. Successful management of unsuspected retroperitoneal. A 47yearold man with incidentaloma and a histologically proven benign retroperitoneal paraganglioma at the right prevertebral region among the inferior vena cava. A rare case of retroperitoneal paragangliomacase report. Surgical excision by left costal approach was performed in figure 3.
Preoperative diagnosis helps to plan treatment and prevent complications. Primary retroperitoneal paraganglioma simulating a pancreatic. Paragangliomas are rare neoplasms arising from cells of the primitive neural crest. The tumor is often slow growing and noncancerous benign. A laparotomy using an upper abdominal median incision plus high transverse incision was performed under general anesthesia.
Excision of a retroperitoneal paraganglioma as a incidental finding in a 60yr old female under general anaesthesia dr. A 60yearold man with hypertension, positive vma24 h and a histologically proven benign retroperitoneal paraganglioma at the right prevertebral region between the inferior vena cava and aorta. Pdf extraadrenal paragangliomas eap are rare endocrine tumors arising from embryonic neural crest cells. Mesentery paraganglioma seems to occur more often in women with a mean age of 53yearold table 1. To the best of our search, there are only two case reports of multiple retroperitoneal paragangliomas with no known familial association.
Retroperitoneal paragangliomas are uncommon tumors causing considerable difficulty in both diagnosis and treatment. To our knowledge this is the first report of this kind of disease in the literature. Retroperitoneal paraganglioma manifesting as paralytic. For this reason, these patients, like the one in this case study, should be oriented to genetic evaluation. The patient was followed up for 5years with no distant metastasis or anemia, then she was lost to followup until liver metastasis was detected. Extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. Retroperitoneal paraganglioma presenting as a chest pain. Paraganglioma in the bladder is extremely rare and accounts for 0. Merge pdf files combine pdfs in the order you want with the easiest pdf merger available. The diagnosis of nonfunctional retroperitoneal paraganglioma and its surgical management can be difficult. Retroperitoneal composite pheochromocytomaganglioneuroma. A 47yearold man with incidentaloma and a histologically proven benign retroperitoneal paraganglioma at the right prevertebral region among the inferior vena cava, pars horizontalis duodeni and aorta. Retroperitoneal paraganglioma resected through the posterior. Pheochromocytoma and paraganglioma treatment is usually surgery and drug therapy.
Paraganglioma of the kidney seldom occurs and is rarely malignant. At lap tion of a malignant nonfunctional paraganglioma of the arotomy, the tumor was found to be well circumscribed retroperitoneum in a patient with familial head and neck and located in the retroperitoneal space with no evidence paragangliomas. We believe chronic hypoxia related to single ventricle palliation to be a trigger for the development of paraganglioma in the abovesaid patient. Pdf mr imaging features of benign retroperitoneal extra. Paragangliomas are extraadrenal chromaffin tumours that develop at the expense of neuroectodermal cells of the autonomous nervous system. Successful management of unsuspected retroperitoneal paraganglioma via the use of combined epidural and general anesthesia. In a series of 22 cases of extraadrenal retroperitoneal paraganglioma from memorial sloankettering cancer center mskcc, the 5year survival for tumors not resected was 19% compared with 75% after complete resection. Paragangliomas of the retroperitoneum are a rare group of tumours with malignant potential that cause considerable difficulty both in diagnosis and treatment. To change the order of your pdfs, drag and drop the files as you want. Hypertension and sympathetic hyperactivity was the most common clinical presentation. For confirmation of diagnosis, we used chromogranin fig. Cancerous forms represent 20%50% of cases and are characterized by the occurrence of ganglionic or distant metastases in 30% of cases. The adhesion from the previous surgery was separated. A second surgery was performed to place an sub device for the left kidney and remove the retroperitoneal mass.
The initial management of paralytic ileus caused by a paraganglioma involves providing the patient with adequate alphablockers and hydration. A case report paragbrahmbhatt, 1 pranavpatel, 1 atifsaleem, 1 rathinarayan, 2 andmarkyoung 1 departmentofinternalmedicine,easttennesseestateuniversity,p. Paraganglioma is a rarely type of neuroendocrine neoplasm with the ability of neuropeptide and catecholamine secretion. A rare serious case of retroperitoneal paraganglioma. The transverse and longitudinal diameters of the tumor are 3. Its complete surgical removal is often challenging as these tumors are located near multiple vital blood vessels. Mr imaging features of benign retroperitoneal extra. Retroperitoneal paraganglioma a rare cause of arterial. A case report parag brahmbhatt, 1 pranav patel, 1 atif saleem, 1 rathi narayan, 2 and mark young 1 1 department of internal medicine, east tennessee state university, p. Paragangliomas are extraadrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. Cells from a cancerous malignant paraganglioma can migrate to lymph nodes, bone, liver, lungs or elsewhere. A rare case of retroperitoneal paragangliomacase report and. Pdf extraadrenal retroperitoneal paraganglioma with.
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