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      Duodenal gangliocytic paraganglioma: A very rare cause for upper gastrointestinal bleeding: Case report with review of literature

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          Highlights

          • Gangliocytic paraganglioma is an exceedingly rare tumors that is mainly arises from the second part of the duodenum.

          • The tumor was first described by Dahl et al. in 1957.

          • The tumor has a characteristic 3 distinct types of cells: spindle cells, epithelial cells, and ganglion cells.

          • The tumor must be differentiated from other tumors.

          • Although it is a benign tumor but some cases have lymphatic or distant organ metastases.

          Abstract

          Introduction

          Gangliocytic paraganglioma is a rare tumors mainly arises from the second part of the duodenum. The tumor is generally benign, but some cases are associated with lymph node or distant metastasis. It must be differentiated from other tumors. The main treatment is good local surgical resection. Most patients have good prognosis after an appropriate resection.

          Case presentation

          A 47-year-old male patient presented with recurrent attacks of epigastric pain and melena. The general examination revealed pallor with no jaundice. The abdomen was soft with no tenderness. There were no stigmata of chronic liver diseases. The hemoglobin level was 8 gm/dl with normal liver and renal functions. The patients received compatible blood. Endoscopy showed a large pedunculated polyp with ulceration in the second part of the duodenum just distal to ampulla of Vater. During surgery, the anterior wall of duodenum was opened, and a 4 cm pedunculated polyp was excised which was arising from the posterior wall of the duodenum just distal to the ampulla of Vater. The frozen section examination revealed a benign lesion. The final histopathology result was consistent with the diagnosis of gangliocytic paraganglioma. The patient had smooth post-operative recovery and no postoperative complications.

          Conclusions

          Gangliocytic paraganglioma is a rare tumor which mostly affects the second part of the duodenum. The tumor is considered benign but the possibility of the malignancy with lymphatic and distant metastases must be excluded before any surgery. The management plan must be addressed carefully and continuous follow up is recommended.

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          Most cited references10

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          Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

          We describe a case of duodenal gangliocytic paraganglioma showing lymph node metastasis. A 61-year-old Japanese man underwent pylorus preserving pancreaticoduodenectomy to remove a tumor at the papilla of Vater. The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish. Neither necrosis nor hemorrhage was present. Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells. Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56. In contrast, spindle-shaped cells showed positive reactivity for S-100 protein, but not for synaptophysin, somatostatin or CD56. Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression. In addition to the rarity of the tumor, we are describing here the present case suggests the malignant potency of the tumor despite lack of acceptable prognostic indicators for neuroendocrine tumor.
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            Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature

            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.
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              A case of gangliocytic paraganglioma in the ampulla of Vater

              Background Duodenal gangliocytic paraganglioma is an extremely rare tumor and few cases have been reported to date. Case presentation The authors report a case of gangliocytic paraganglioma verified by post-op pathology after pancreaticoduodenectomy for a tumor in the ampulla of Vater. The 56-year-old male patient concerned visited our emergency room with melena that started one week prior to hospitalization. The patient was diagnosed to have a tumor in the ampulla of Vater with bleeding on its surface. However post-op, he was diagnosed as having gangliocytic paraganglioma by immunohistochemistry. Conclusion This tumor has precise clinical implications, and if continuous follow up is conducted after careful diagnosis and surgical treatment, invasive major operations, such as, radical pancreaticoduodenectomy can be avoided.
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                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                23 September 2020
                2020
                23 September 2020
                : 75
                : 408-412
                Affiliations
                [a ]Department of Surgery, College of Medicine, University of Duhok, Kurdistan Region, Iraq
                [b ]Department of Pathology, University of Duhok, Kurdistan Region, Iraq
                Author notes
                [* ]Corresponding author at: Department of Surgery, College of Medicine, University of Duhok, Nakhoshkhana Road, 8 AM-1014, Duhok, Kurdistan Region, Iraq. ayad.mohammed@ 123456uod.ac
                Article
                S2210-2612(20)30802-6
                10.1016/j.ijscr.2020.09.129
                7527677
                33002850
                c5d73b08-29b0-45ae-a625-65d987a11d25
                © 2020 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                : 24 August 2020
                : 19 September 2020
                : 19 September 2020
                Categories
                Case Report

                gangliocytic paraganglioma,gastrointestinal bleeding,spindle cells,epithelial cells,ganglion cells

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