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             Gangliocytic Paraganglioma 3

                        

 

Duodenal gangliocytic paraganglioma: a radiological-pathological correlation.Ann Diagn Pathol. 2005 Jun;9(3): 143-7.

Duodenal gangliocytic paraganglioma is a rare tumor that characteristically occurs in the second portion of the duodenum and typically presents with gastrointestinal bleeding. Gangliocytic paragangliomas have a characteristic triphasic microscopic appearance with epithelioid cells, spindle cells, and ganglion cells, resulting in a complex histology with features of paraganglioma, carcinoid, and ganglioneuroma. Duodenal gangliocytic paragangliomas have an excellent prognosis after surgical resection but metastatic spread to regional lymph nodes and recurrence may rarely occur. We report a case of duodenal gangliocytic paraganglioma and discuss the radiological and pathological differential diagnosis of this rare entity.

Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case.Eur J Med Res. 2005 Mar 29;10(3):135-8.

The authors report a large duodenal somatostatinoma, a very rare tumor entity. A 8.5 cm globular mass in the area of the unicate process of the pancreas was detected in a 45 year old caucasian female by computerized tomography. The patient had only mild complaints. Initial treatment consisted of right pancreatectomy with preservation of the pylorus. Histological evaluation rendered a diagnosis of low-grade malignant neuroendocrine carcinoma with expression of somatostatin, respectively of somatostatinoma arising in the duodenum and infiltrating into the pancreas. 26 months after the initial surgery liver and lymph node metastases were detected and surgically removed. This case confirms that duodenal somatostatinomas are very difficult to diagnose preoperatively because of unspecific symptoms. Most duodenal somatostatinomas are found incidentally. Treatment of choice is radical surgical resection with a possible cure in early stages of the disease. Even a large tumor as ours is resectable with negative surgical margins. Management of recurrent or metastatic disease is also surgical. Additional chemotherapy and supportive care may be beneficial for the patient.

Duodenal Gangliocytic Paraganglioma

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Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature.World J Surg Oncol. 2005 Mar 1;3(1):15.

BACKGROUND: Gangliocytic paraganglioma are rare neoplasms that predominantly arise in periampulary region. Though considered benign the disease can spread to regional lymphatics. CASE PRESENTATION: A 49 year old woman presented with melena and was found to have a periampullary mass. Endoscopic evaluation and biopsy demonstrated a periampullary paraganglioma. The tumor was resected with pylorus-preserving pancreaticoduodenectomy and was found to represent a gangliocytic paraganglioma associated with nodal metastases. In a controversial decision, the patient was treated with adjuvant external beam radiation therapy. She is alive and well one year following resection. The authors have reviewed the current literature pertaining to this entity and have discussed the biologic behavior of the tumor as well as the rationale for treatment strategies employed. CONCLUSION: Paraganglioma is a rare tumor that typically resides in the gastrointestinal tract and demonstrates low malignant potential. Due to rarity of the disease there is no consensus on the adjuvant treatment even though nearly 5% of the lesions demonstrate the malignant potential.

Duodenal gangliocytic paraganglioma.An Sist Sanit Navar. 2005 Jan-Mar;28(1):109-13.

We present the case of an 85 year old male who was admitted to hospital with abdominal pain and jaundice. Different explorations were performed for this reason, with a mass observed in his 2nd duodenal portion. Histological study showed that it was a duodenal gangliocytic paraganglioma. The clinical characteristics of this infrequent tumour are described and we review the diagnosis and treatment.

 

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