Gangliocytic paraganglioma of duodenum

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1 J M e d l l i e d S c i ; 3 ( 1 ) : w w w. j m a s. i n P r i n t I S S N : O n l i n e I S S N : X Journal of M e d i cal & llied Sciences ase report of duodenum S.S.S. Quadri 1, Shyamala Srujana 1, N. Sreemani Kumari 1, P. Jijiya ai 1, K. Jayasree 2 1 epartment of Pathology & 2 epartment of General Surgery, Gandhi Medical ollege and Hospital, Musheerabad, Secunderabad , ndhra Pradesh, India. rticle history: bstract Received 10 October 2012 ccepted 07 February 2013 Early online 25 February 2013 Print 28 February 2013 orresponding author S.S.S. Quadri epartment of Pathology, Gandhi Medical ollege and Hospital, Musheerabad, Secunderabad , ndhra Pradesh, India. Phone: quadri05@hotmail.com n elderly person, aged 60 years, presented with pain abdomen localized to the epigastric region, of one year duration. There was a past history of a single episode of passing black-colored tarry stools and hematemesis. Ultrasonography (USG) of abdomen reported a suspected duodenal / periampullary growth. iopsy performed was inconclusive. ontrast enhanced computerized tomography (ET) scan abdomen suggested suspected submucosal leiomyoma of duodenum with intrahepatic biliary distension. Whipple s procedure was performed which recovered a growth measuring 6x5x5cms arising in periampullary region of second part of duodenum. Pancreatic duct and common bile duct () were dilated. The histopathological findings were suggestive of neuroendocrine tumor of duodenum. The diagnosis of gangliocytic paraganglioma was confirmed by immunohistochemical stains. Key words:, duodenum, Whipple s procedure 2013 eccan ollege of Medical Sciences. ll rights reserved. G angliocytic paraganglioma (GP) is a rare tumor, occurring exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, having rare malignant potential with tendency to metastasize to regional lymph nodes. ase report n elderly person, aged 60 years, presented with complaint of pain abdomen of 1 year duration, localized to epigastric region. Past history of a single episode of passing black-colored tarry stools, along with history of hematemesis 5 months back was noted. Patient gave history of weight loss and loss of appetite. He was a chronic alcoholic. Family history was not significant. His physical examination was non-contributory. Ultrasonography (USG) of abdomen reported a duodenal / periampullary growth. n endoscopic biopsy performed on the growth was inconclusive. Subsequently, contrast enhanced computerized tomography (ET) scan abdomen revealed a mass measuring about 6cms in the second portion of the duodenum suggestive of submucosal leiomyoma of duodenum with intrahepatic biliary distension. Surgery was indicated based on these findings indicating high possibility of malignancy. Whipple s procedure was performed and a growth measuring 6x5x5cms was found in the ampulla of Vater. No evidence of any distant metastasis or lymph node enlargement was noted. Macroscopic and microscopic findings Gross: Whipple s specimen consisting of gall bladder along with partial distal gastrectomy, 29

2 Fig 1.Gross: Proximal resected end with pylorus (upper red arrow), duodenum (blue arrow), distal end of duodenum (green arrow), gall bladder (lower left red arrow) Fig 2.Gross: Tumor (red arrow), proximal resected end, pylorus (black arrow), distal resected end, duodenum (green arrow), gall bladder (blue arrow) :40X View-normal duodenum with brunner s glands.. :10X View-Tumour in nests &trabeculae. duodenum and pancreas was received. Gall bladder measured 6x4cms, distended with bile, stomach 3x2 cms, pancreas 4x3cms, tumor 6x5 cms and bile duct 8cms in length (Fig 1 & 2). :4X View-normal duodenum & pancreas. :40X View-Tumour in zell-ballen like pattern. Fig 3. H & E stain showing normal duodenum, pancreas and tumor. Sections revealed a submucosal tumor, having a triphasic pattern composed of epithelioidneuroendocrine cells, spindle cells with schwannian differentiation and scattered ganglion cells. The epithelioid cells were arranged in solid nests, ribbons, trabeculae and pseudoglandular patterns. Individual cells had moderate to abundant eosinophilic to basophilic cytoplasm with ovoid nuclei. Spindle cells having elongated nuclei, formed slender fascicles wrapping around and sweeping in between the nests of epithelioid cells. J Med llied Sci 2013; 3(1) 30

3 :10X View-Tumour in nests & pseudoglandular patterns. :10X View-Tumor with spindle cells in fascicles. The ganglion cells were seen either in small clusters or individually scattered. Individual ganglion cells had abundant eosinophilic cytoplasm with round eccentric nucleus and prominent nucleolus. Some cells showed intracytoplasmic brown-colored Nissl s substance. Mitotic count was one-tenth per HPF. Pleomorphism and necrosis was absent. Pancreas, gall bladder, common bile duct were not invaded by the tumor. No lymph node metastasis was detected (Fig 3 & 4). provisional diagnosis of neuroendocrine tumor of periampullary region (second part of duodenum) was considered. Subsequently, immunohistochemistry (IH) was done (Table 1), which showed positivity for chromogranin, synaptophysin, NSE and S-100 whereas cytokeratin and E were negative (Fig 5 & 6). ased on the above IH features a diagnosis of gangliocytic paraganglioma of periampullary /second part of duodenum was confirmed. iscussion (GP) is a rare tumor, occurring exclusively in the second portion of the duodenum 1,2. ahl et al in , first described Fig 4. H & E stain showing components of tumor cells :40X View-Tumor in pseudoglandular pattern. :40X View-Tumor-spindle cells with elongated nuclei. the lesion and it was further characterized as a benign non-chromaffin paraganglioma by Taylor and Helwig in The term gangliocytic paraganglioma was coined by Keeps and Zacharias in1971, recognizing the features in common with both paraganglioma and ganglioneuroma 5. Gangliocytic paraganglioma is usually seen in the periampullary region of duodenum, though rare cases have been reported in jejunum, pylorus, esophagus 6, pancreas 7 and appendix 8. Table 1: Immunohistochemistry report IH marker hromogranin + S NSE + Ki 67 E - ytokeratin - Result 1% positivity J Med llied Sci 2013; 3(1) 31

4 :10X,:40X View-IH:hromogranin Positive. :10X;:40X-IH:ytokeratin Negative. Fig 5. Immunohistochemical stain showing chromogranin positivity &:10X & &:40X Views- IH:S 100-Positivity in Spindle & Sustentacular cells inbetween & around epitheloid cells Respectively. Fig 6.Immunohistochemical stain showing S100 positivity J Med llied Sci 2013; 3(1) 32

5 Recently, 3 cases of pulmonary GP have been reported 9,10.The age at presentation ranges from years, with slight male predominance. linically, GPs arising in the gastrointestinal tract present with bleeding, abdominal pain or obstruction; though some cases were incidental findings at autopsy 2. bdominal pain is the most common presenting symptom. GP has three characteristic histologic components: epithelioid, ganglion and spindle cell. The proportion of the three cell types varies in each tumor, but each component shows characteristic immunohistochemical staining, similar to those observed in our case 11.They may also show positivity for pancreatic polypeptide 12. Theories on the origin of GPs vary widely, and yet have not been able to explain the combination of endocrine, ganglion and spindle cells observed in a single tumor. The tumor components are of different embryologic origins; the first being of endodermal origin and the others originating from neural crest tissue. Initially, it was suggested that these tumors were of ectodermal origin, from pluripotent stem cells derived from the neural crest, which were found in Lieberkühn's glands or the celiac ganglion during fetal development 3 but in view of the occurrence of GPs in different sites in the duodenum and its variable histology, it has been proposed that they originated from endodermal pluripotent progenitor stem cell that has the potential for divergent differentiation 13. Some authors proposed GPs were hamartomas of endodermal (epithelial cells) and neuroectodermal (ganglion and spindle cells) origin 11. Most authors considered them to be variants of gastrointestinal tract paragangliomas 14. Paragangliomas may differentiate to other neuroectodermal elements, including neurons and schwann cells 15. Most GPs are benign and are amenable to local resection. However, instances of recurrence, tumor metastasizing to regional lymph node involvement and distant metastases have been reported. This tumor metastasis show all the three cell components 16. In a single case of regional lymph node metastasis, tumor consisted of only epithelioid cell component 17. Some GP may recur with lymph node metatsasis 18. onclusion may be misdiagnosed in view of non-specific clinical symptoms like pain abdomen for peptic ulcer disease and radiologically for periampullary adenocarcinomas or gastrointestinal stromal tumor, as observed in our case. J Med llied Sci 2013; 3(1) lso, endoscopic biopsy may be inconclusive in view of submucosal location of tumor as noted in our study. This tumor though rare should be considered as a differential diagnosis in neoplasms of periampullary region and it has a good prognosis as compared with other tumors. Since GP may recur or metastasize, pancreaticoduodenectomy with lymph node dissection may be indicated for large lesions with infiltrative margin, or lesions with pleomorphism and mitoses. cknowledgment: None onflict of interest: None References 1. urke P and Helwig E.. m J lin Pathol 1989;92: Scheithauer W, Nora FE, Lehago J, Wick MR, rawford G, Weiland LH, arney J. uodenal gangliocytic paraganglioma: linicopathologic and immunocytochemical study of 11 cases. m J lin Pathol 1986; 86: ahl EV, Waugh JM, ahlin. Gastrointestinal ganglioneuromas: brief review with report of a duodenal ganglioneuroma. m J Pathol1957; 33: Taylor H and Helwig E. enign nonchromaffin paragangliomas of the duodenum. Virchows rch Pathol nat Physiol Klin Med1962;335: Kepes JJ and Zacharias L. s of the duodenum. report of two cases with light and electron microscopic examination. ancer 1971;27: Weinrach M, Wang KL, lum MG, Yelandi V, Laskin W. Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus. Hum Pathol 2004;35: Henry, Ghalel-Mechaoui H, ottero N, Pradier T, Moindrot H. of the pancreas with bone metastasis. nn hir 2003;128: Van Eeden S, Offerhaus GJ, Peterse HL, ingemans KP, laauwgeers HL. Gangliocytic paraganglioma of the appendix. Histopathology 1993;63: Hironaka M, Fukayam M, Takayashiki N, Saito K, Sohara Y, FunataN. Pulmonary gangliocytic paraganglioma: ase report and comparative 33

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