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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 775-777
Gangliocytic paraganglioma: A rare presentation as intestinal intussusception

1 Department of Pathology, Military Hospital, Jalandhar, India
2 Department of surgery, Military Hospital, Jalandhar, India

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Date of Web Publication27-Oct-2010


Gangliocytic paragangliomas are rare benign neoplastic lesions of neuroendocrine origin occurring primarily in peri-ampullary region. Commonly occurring as small submucosal lesions, presentation as a large polyp with acute ileo-ileal intussusception in a 62-year-old woman is rare and is described in this case report.

Keywords: Gangliocytic paraganglioma, ileo-ileal intussusception, myofibroblastic tumor, neuroendocrine

How to cite this article:
Sirohi D, Sengupta P, Kumar H, Rao P P. Gangliocytic paraganglioma: A rare presentation as intestinal intussusception. Indian J Pathol Microbiol 2010;53:775-7

How to cite this URL:
Sirohi D, Sengupta P, Kumar H, Rao P P. Gangliocytic paraganglioma: A rare presentation as intestinal intussusception. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jul 25];53:775-7. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/775/72085

   Introduction Top

Gangliocytic paragangliomas are rare non-secretory tumors with neuronal and neuroendocrine features originating from dispersed neuroendocrine system containing groups of neurosecretory cells scattered throughout the body. Gangliocytic paragangliomas are rare neoplasms that are characterized by the presence of three different cells; epithelioid cells with endocrine growth pattern, resembling paraganglioma or carcinoid tumor cells; spindle cells reminiscent of Schwann cells and ganglion or ganglion-like cells. Gangliocytic paragangliomas have been reported in the duodenum, jejunum, pylorus of stomach and cauda equina region of spinal cord. We report a case of gangliocytic paraganglioma presenting as acute ileo-ileal intussusception in a 62-year-old woman.

   Case Report Top

A 62-year-old woman presented to the hospital emergency with acute intestinal obstruction of 4-h duration. An emergency laparotomy showed ileo-ileal intussusception and resection of segment of the ileum was carried out with end-to-end anastomosis.

The specimen received was of ileo-ileal resection, 10.5 cm in length. Serosa was congested near the base of intussusception segment. Cut surface showed a pedunculated polyp at the base of intussusception measuring 4.5 2.5 2.0 cm [Figure 1]a. The cut surface of the polyp was whitish, firm and homogeneous with congestion at the base [Figure 1]b. Adjacent segment of intestine was unremarkable.
Figure 1 :(a) Resected segment of ileum cut open, showing a polyp at the base of intussusceptions, (b) Firm, whitish cut surface of the polyp

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Microscopic examination of the polyp showed a soft tissue lesion comprising of proliferating network of vessels separated by plump cells in an edematous stroma [Figure 2]a. The cells had inconspicuous cytoplasm with round to oval vesicular nuclei and conspicuous nucleoli Admixed were a few clusters of benign ganglion cells [Figure 2]b. A moderate polymorphous inflammatory infiltrate comprising of polymorphonuclear leukocytes, lymphocytes, plasma cells and mast cells, which was more conspicuous at the periphery of the lesion was noted. There was no necrosis or hemorrhage. Adjacent intestinal segment showed normal histology.
Figure 2 :(a) Polyp displaying proliferating network of vessels separated by plump cells with bland morphology (H and E; ×400), (b) Sections of the polyp showing clusters of benign ganglion cells (H and E; ×400), (c) Cells showing a diffuse cytoplasmic positivity for NSE (IHC for NSE; ×400), (d) Cells show a diffuse cytoplasmic positivity for chromogranin (IHC for chromogranin; ×400)

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A differential diagnosis of inflammatory myofibroblastic tumor and gangliocytic paraganglioma was considered. Immunohistochemistry (IHC) was negative for desmin, pan CK and CD117. The predominant cells between the vessels were positive for NSE [Figure 2]c and chromogranin [Figure 2]d. The vessels were highlighted by CD31. Scattered sustentacular cells were positive for S-100. CK8 and 18 were not tested for because of non-availability. Based on the IHC and histomorphology, a diagnosis of gangliocytic paraganglioma (GP) was made. The other morphological differentials include carcinoid and ganglioneuroma that can be excluded with adequate sampling of the lesion and use of specific keratins.

   Discussion Top

Gangliocytic paraganglioma; a rare tumor of controversial histogenesis is almost exclusively seen in the periampullary region of the second portion of the duodenum. [1] Similar tumors have however also been reported in a variety of other intestinal and extra-intestinal locations. The tumor typically occurs in adults during the third through the ninth decades of life (median reported age, 53 years) and has a slight male predominance. [1] Clinically they are usually small, solitary submucosal lesions 1 to 3 cm in size with a nodular or polypoid configuration. Rare reports of multiple GPs in the duodenum and head of pancreas and multifocal presentations in mediastinum and esophagus are present in literature. [2]

GPss are benign lesions with good prognosis. Metastasis to regional lymph nodes and bone are rare and this does not substantially affect the patient's otherwise excellent outcome. [3] The retroperitoneal and regional nodes should be evaluated for any metastasis since radical surgery for malignant duodenal gangliocytic paraganglioma with lymph node metastasis confers an excellent prognosis in such cases. [4]

Intussusception in children tends to be idiopathic in most cases in contrast to adults who have an underlying etiology. The non-idiopathic causes include polyps, lipomas, Meckel's diverticulum, intestinal duplication, Henoch-Schφnlein purpura, lymphomas, hypertrophied Peyer's patches secondary to infection, adenovirus infection, foreign bodies, parasitic infestations, celiac disease and cystic fibrosis. Previous abdominal surgery can predispose to the formation of adhesions and subsequent intussusceptions. Benign neoplasms underlie most cases of small intestinal intussusceptions, whereas malignant neoplasms are the underlying etiology in most large bowel intussusceptions.

The term GP was first coined by Kepes and Zacharias [5] in 1971 for lesions with paraganglion-like and ganglioneuromatous appearance. The histogenesis of gangliocytic paragangliomas is controversial. Histologically, they are composed of epithelioid, neuronal and peripheral nerve sheath elements in varying proportions. [6] Some authors consider these tumors as true neoplasms of neuroendocrine origin. [7] Because of the proximity of most gangliocytic paragangliomas to pancreatic tissue and the occasional expression of pancreatic enzymes, others believe that they are hamartomas developing in misplaced embryonic pancreatic tissue. [6]

Initially thought to arise from normal cells around the periampullary region of the small bowel, the occurrence of multifocal GPs in sites far from the periampullary region and pancreas along with previous reports of extra-ampullary GPs favor origin of the tumor from a more omnipresent progenitor cell. [2],[8] David M et al.[2] propose a migratory neural crest stem cell with potential for divergent (neuroendocrine and neuroectodermal) differentiation as a potential cell of origin. Cases of GP have been reported in the setting of type 1 neurofibromatosis. [1]

The immunoexpression of keratin in epithelioid cells in 50 to 70% of GPs supports a neuroendocrine differentiation and distinguishes them from paragangliomas, which typically do not express keratin. The use of more specific keratins such as keratin 8 (CAM 5.2) and keratin 18, which are more commonly expressed in neuroendocrine tumors, the true rate of keratin expression in the epithelioid cell component of GP may be much higher than that in earlier published results, because most of those studies used broad-spectrum keratin cocktails. [9]

Furthermore, the authors contend that the GP is a neoplastic process based on its multiple sites of origin, its potential for recurrence and metastatic spread and the morphological and immunohistochemical evidence of multilineage differentiation.

   Conclusion Top

In conclusion, we report a case of GP presenting as intestinal intussusception developing at the base of an intestinal polyp, which morphologically resembled an inflammatory myofibroblastic tumor, but the immunopositivity for NSE, chromogranin and S-100 favored a diagnosis of gangliocytic paraganglioma.

   References Top

1.Albores-Saavedra J, Henson DE, Klimstra D. Endocrine tumors and related neoplasms of the ampullary region, in tumors of the Gallbladder, Extrahepatic Bile Ducts and Ampulla of Vater. Atlas of Tumour Pathology. 3 rd series, Washington, DC: Armed Forces Institute of Pathology; 2000. p. 317-40.  Back to cited text no. 1
2.Weinrach DM, Wang KL, Blum MG, Yeldandi AV, Laskin WB. Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus. Hum Pathol 2004;35:1288-91.  Back to cited text no. 2
3.Sundararajan V, Robinson-Smith TM, Lowy AM. Duodenal gangliocytic paraganglioma with lymph node metastasis: A case report and review of the literature. Arch Pathol Lab Med 2003;127:139-41.  Back to cited text no. 3
4.Hashimoto S, Kawasaki S, Matsuzawa K, Harada H, Makuuchi M. Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis. Am J Gastroentrol 1992;87:1216-18.  Back to cited text no. 4
5.Kepes JJ, Zacharias DL. Gangliocytic paragangliomas of the duodenum: A report of two cases with light and electron microscopic examination. Cancer 1971;27:61-7.  Back to cited text no. 5
6.Perrone T, Sibley RK, Rosai J. Duodenal gangliocytic paraganglioma: An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. Am J Surg Pathol 1985;9:31-41.  Back to cited text no. 6
7.Collina G, Maiorana A, Trentini GP. Duodenal gangliocytic paraganglioma: Case report with immunohistochemical study on the expression of keratin polypeptides. Histopathology 1991;19:476-8.  Back to cited text no. 7
8.Konok GP, Sanchez-Cassis G. Gangliocytic paraganglioma of the duodenum. Can J Surg 1979;22:173-5.  Back to cited text no. 8
9.Chu PG, Weiss LM. Keratin expression in human tissues and neoplasms. Histopathology 2002;40:403-39.  Back to cited text no. 9

Correspondence Address:
Deepika Sirohi
Military Hospital, Jalandhar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72085

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