A rare serious case of retroperitoneal paraganglioma misdiagnosed as duodenal gastrointestinal stromal tumor. Spectrum of retroperitoneal and genitourinary paraganglioma. Metastatic retroperitoneal paraganglioma clinics in oncology. Aggressive surgery is mandatory to obtain disease free survival. Fiveyear and 10year disease free survival rates were 19% and 19% for tumors not resected and 75% and 45% for those completely resected.
Retroperitoneal paragangliomas are mostly benign with good prognosis. For this reason, these patients, like the one in this case study, should be oriented to genetic evaluation. We report a case of a 42 year old female presenting with abdominal pain who had a retroperitoneal tumour situated at. The paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively. Paragangliomas are rare neoplasms arising from cells of the primitive neural crest.
The recurrence rate for patients with a median followup of 2 years was 14%, eliminating patients with followup times less than 1. The tumor is often slow growing and noncancerous benign. Patient is currently symptom free a er month followup and is closely followed by physicians. A resected case of liver metastases from extraadrenal. Case report an 11yearold female child with hypoplastic left heart. Pheochromocytoma is a rare tumor that forms in the adrenal medulla the center of the adrenal gland.
We report a 49yearold male who underwent lymphadenectomy for a retroperitoneal tumor with a largest dimension of 6. Primary retroperitoneal paraganglioma simulating a. Retroperitoneal paraganglioma is a rare tumor that develops from chromaffin cells that. Nov 12, 2015 retroperitoneal nonfunctioning paraganglioma. A case report paragbrahmbhatt, 1 pranavpatel, 1 atifsaleem, 1 rathinarayan, 2 andmarkyoung 1 departmentofinternalmedicine,easttennesseestateuniversity,p. An unusual presentation of mesenteric paraganglioma. Retroperitoneal sarcoma surgical management of soft tissue. A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components. 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. Physical examination revealed upper abdominal tenderness without a.
There was an upper paramedian, left sided, large, palpable mass on the physical examination. Survival of patients undergoing resection of retroperitoneal paraganglioma stratified by a size, b functional status, c margin status, and d nodal status. Extraadrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing softtissue mass at ct, multiple areas of signal void interspersed with hyperintense foci saltandpepper appearance within tumor mass at mri, and an intense tumor blush with enlarged feeding arteries at angiography. Most paragangliomas are either asymptomatic or present as a painless mass. After the surgery, he had survived without any recurrence of the tumor. Certain inherited disorders and changes in certain genes increase the risk of pheochromocytoma or. In the original complaint, the patient requested medical examination on experiencing sudden nausea accompanied by upper left abdominal pain.
A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Pdf retroperitoneal paraganglioma raymond barfield. We report a case of a 42 year old female presenting with abdominal pain who had a retroperitoneal tumour situated at the aortic bifurcation. Large retroperitoneal paraganglioma concurrent with. Is essential to rule out the existence of paraganglioma in all retroperitoneal tumors, even in asymptomatic and hemodynamically stable patients. The retroperitoneal paragangliomas have a slight predilection. Pdf extraadrenal paragangliomas eap are rare endocrine tumors arising from embryonic neural crest cells. Laboratory investigations showed an increase in liver enzyme levels. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or. It is fascinating and challenging to medical fields because.
Retroperitoneal paraganglioma with metastasis to the. Retroperitoneal paraganglioma a rare cause of arterial. Methods in a sixyear period, 49 patients with primary retroperitoneal paragangliomas pg underwent retroperitoneal laparoscopic surgery in a. Mar 28, 20 the patient was followed up for four years and remained disease free. Surgical outcome of laparoscopic surgery, including. Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue. We present an unusual case of retroperitoneal composite paraganglioma ganglioneuroma discovered on computed tomography in a 63yearold female patient. Retroperitoneal paraganglioma in a patient with fontan. To our knowledge this is the first report of this kind of disease in the literature.
Primary retroperitoneal paraganglioma simulating a pancreatic mass. Malignant abdominal paraganglioma with lymph node metastasis. Apr 27, 2014 sangster g, do d, previgliano c, li b, lafrance d, heldmann m. Composite paragangliomaganglioneuroma in the retroperitoneum. The patient was followed up for four years and remained diseasefree. In 60% of cases, they secrete hormones and are classified as pheochromocytomas. 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. These results suggested a pheochromocytoma or paraganglioma.
Since retroperitoneal paragangliomas are rare, the behavior and treatment outcomes of this type of tumor remain unclear. Chemotherapy, radiation therapy, targeted therapy, ablation, and embolization can be used for disease that has spread or come back. Bifocal retroperitoneal paraganglioma in a young patient. A difficult tumour to diagnose and treat emma gannan, a, b. Histopathology unexpectedly revealed seminoma with an entrapped nerve ganglion. Authors report 5 cases of retroperitoneal paraganglioma, operated in the department of urology of hospital, between 20 and 2017. Ct and mr imaging findings of pancreatic paragangliomas.
Jul 16, 20 the paraganglioma is a rare tumor associated with high morbidity and mortality when the diagnosis is made intraoperatively. We believe chronic hypoxia related to single ventricle palliation to be a trigger for the development of paraganglioma in the abovesaid patient. Pheochromocytoma and paraganglioma treatment is usually surgery and drug therapy. This tumor can affect people of any age but most often shows up between the ages of 30 and 50. An 81yearold woman was admitted with progressive abdominal fullness. Massive extraadrenal retroperitoneal paraganglioma. Primary retroperitoneal neoplasms are a rare but diverse group of benign and malignant tumors that arise within the retroperitoneal space but outside the major organs in this space. Routine hematological examination and biochemical tests were within normal limits.
A rare serious case of retroperitoneal paraganglioma. A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites including the head, neck, thorax and abdomen. Objectives to explore the feasibility and safety of retroperitoneal laparoscopic resection of paraganglioma rlpg in a large study population. All patients were admitted and evaluated by an endocrinologist and a urologist. Mr imaging features of benign retroperitoneal extraadrenal. Subscribe to this free journal for more curated articles on this topic. The most likely diagnosis is of a paraganglioma, with the main differential being a gastrointestinal stromal tumor do not typically enhance so vividly. Case report a 57yearold man presented to the endocrinology clinic with paroxysms of exacerbated hypertension, tachy. Primary retroperitoneal paraganglioma simulating a pancreatic. Arthoua tpf, platel jp, meusnier f, pourriere m, thouard h 1997 paragangliome retroperitoneal secretant. Estimation of 24 hour urinary catecholamine 23 weeks after surgery gives idea about biochemical cure and also recurrence or metastasis in later postoperative period. Clinical presentation is not always specific and paraganglioma may be discovered fortuitously.
After 6 cycles, urinary catecholamines had decreased to 43. A male patient, ages 41 years, was admitted to our hospital due to a pancreatic spaceoccupying lesion that had been present for more than 10 days. Approximately 50% of patients with recurrent disease. Phenoxybenzamine and propranolol are useful drugs for medical management of functional retroperitoneal paraganglioma. For confirmation of diagnosis, we used chromogranin fig. Pheochromocytomas and paragangliomas occur in 2 to 8 people per million and retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or adrenals. Retroperitoneal laparoscopic management of paraganglioma. Extraadrenal retroperitoneal paraganglioma in a dog. He had a history of hypertension and hyperthyroidism. The patient began cvd chemotherapy in july 1992, and after 2 cycles an mri scan showed a decrease in the tumor size. Extraadrenal retroperitoneal paraganglioma in a dog marcia. Case report retroperitoneal paraganglioma presenting as a. Mr imaging features of benign retroperitoneal extra. Rare case of temporally spontaneous regression of retroperitoneal.
Penelope van veenendaal, b adam scarlett, c and michael ng a a department of general surgery, ballarat base hospital, drummond st north, ballarat, vic 3350, australia. These tumours are often difficult to diagnose and treat. However, in retroperitoneal paraganglioma, the 5 and 10year disease free survival rates were 75% and 45% even after successful resection, indicating that more than half of these patients will experience a relapse if followed long enough after resection. Our case demonstrated the need to consider paraganglioma of the retroperitoneum in the differential diagnosis of retroperitoneal mass, metastatic tumors to the vertebra, and the importance of radical surgery for a successful management of the disease.
Unlike other types of cancer, there is no test that determines benign from malignant tumors. Mar 14, 2020 a paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. Paragangliomas occur most commonly in head and neck region and much less frequently, they are found in the retroperitoneum. Pheochromocytoma and paraganglioma treatment pdqpatient. Extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. Mesentery paraganglioma seems to occur more often in women with a mean age of 53yearold table 1.
Pdf on oct 17, 2018, sanae ghammad and others published malignant retroperitoneal. Experience at a north indian tertiary care center article pdf available in central european journal of urology 684. Composite paraganglioma ganglioneuroma of the retroperitoneum is very rare. Although computed tomography and magnetic resonance imaging can demonstrate important characteristics of these tumors, diagnosis is often challenging for radiologists. Joana santos2, rosa cardoso3, sofia carvalho3, sonia vilaca 4, manuela vasconcelos. Pdf malignant retroperitoneal paraganglioma treated with. Treatment of malignant pheochromocytomaparaganglioma with.
Our case demonstrated the need to consider paraganglioma of the. Preoperative diagnosis helps to plan treatment and prevent complications. Case report retroperitoneal paraganglioma presenting as a chest pain. We report a retroperitoneal paraganglioma that had spontaneously regressed, and recurrence 5 years later after ct scan diagnosis. Retroperitoneal paraganglioma, diagnosis and management. Once metastases occurred, the 5year survival rate was 36%. While all contain neurosecretory granules, only in % of cases is secretion of hormones such as catecholamines abundant enough to be clinically significant. Background extraadrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. Diagnosis and surgical treatment of retroperitoneal.
But it can invade nearby parts of the body, become cancerous malignant and spread. Abdominal paragangliomas are mostly retroperitoneal in location, accounting for 85% of all extra adrenal paragangliomas. Nonfunctional retroperitoneal paraganglioma presenting as acute upper gastrointestinal hemorrhage. The concentrations of the free plasmatic metanephrines. Cyanotic congenital heart disease chd has also been suggested as a risk factor for paraganglioma pheochromocytoma. Box,j ohnsoncity,tn,u sa w estravineroad,suitesandkingsport,tn,u sa.
Barfield r, hill da, hoffer fa, tekautz t, spunt sl 2002 retroperitoneal paraganglioma. The immunohistochemical profile of investigated paraganglionic tumors. Cancerous forms represent 20%50% of cases and are characterized by the occurrence of ganglionic or distant metastases in 30% of cases. We present an unusual case of retroperitoneal composite paraganglioma ganglioneuroma discovered on computed tomography in a 63yearold female. Conclusion retroperitoneal extraadrenal paraganglioma eap are relatively rare neuroendocrine tumors. Retroperitoneal paraganglioma presenting as a chest pain. Paragangliomas are extraadrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. Multiple tumors, tumors suspected of being malignant preoperatively, and tumors operated on using a retroperitoneal approach were excluded from the study. Histopathological examination of the resected specimen showed that the tumor was an extraadrenal paraganglioma. Retroperitoneal paraganglioma with metastasis to the abdominal. Although recurrence of mesenteric paraganglioma has not been reported, longterm followup.