CASE REPORT
Available from: URL: http://www.jgld.rO/2015/l/19.html DOI: http://dx.doi.org/10.15403/jgld.2014.1121.abo
Gangliocytic Paraganglioma: a Rare Cause of Gastrointestinal Bleeding Alina B oeriu1, D aniela D obru 1, Rares G eorgescu2, Sim ona M ocan3, Cristian B oeriu 4
1) Department of
ABSTRACT
Gastroenterology; 2) Department of Surgery, University of Medicine and Pharmacy Tirgu Mures; 3) Department of Pathology, Tirgu Mures County Emergency Hospital; 4) Emergency Department,
Duodenal neuroendocrine tumors (NETs) are rare tumors, consisting of five different types of tumors. In many cases, they may be asymptomatic, leading to delay in diagnosis. Clinical symptoms are related to local tumor growth and mucosal ulceration. We report a 38-year old man with duodenal gangliocytic paraganglioma causing overt upper gastrointestinal bleeding and anemia. We describe specific clinical and histopathological features of the tumor, and review the diagnostic and therapeutic strategy. Gangliocytic paragangliomas are regarded as benign tumors. However, the disease recurrence and the malignant potential of the tumor have also been reported.
University of Medicine and Pharmacy, Targu Mures
Key words: gangliocytic paraganglioma - immunostaining - surgical excision - metastasis.
Romania
Address fo r correspondence:
INTRODUCTION
CASE REPORT
Dr. Boeriu Cristian Mures County Emergency Hospital, Str.Gheorghe Marinescu nr. 50 540136 Tirgu Mures, Romania [email protected]
Received: 07.12.2014 Accepted: 22.12.2014
G a stro in te stin a l n e u r o endocrine tumors (NETs), also called carcinoids, arise from cells of neuroendocrine systems, and were first described as a distinct class of malignant tumors with less aggressive behavior th a n a d e n o c a rc in o m a s of the gastrointestinal tract [1], Morphologically, carcinoids are identified by positive staining for neuroendocrine markers such as chromogranin A, synaptophysin, Leu7, and n e u ro n -sp e c ific enolase (NSE). Duodenal NETs are rare tumors, representing about 4% from all gastrointestinal carcinoids [2], Different types of neuroendocrine tumors have been described in the duodenum, c o n sistin g of g astrin o m as, somatostatinomas, n o n functional NETs, gangliocytic paragangliom as and poorly differentiated NETs [3].
A 38-year old man was referred to our department with melena. He had a previous history of mild anemia (hemoglobin 10.7 g/dl), intermittent hematochezia, and fatigue in the past year. A colonoscopy performed for the evaluation of anemia and rectal bleeding revealed hemorrhoids. At admission to our hospital, physical examination showed pallor, tachycardia and hypotension. Laboratory values revealed severe anemia and iron deficiency (hemoglobin 5.2 g/dl, hematocrit 18%, serum iron 20 pg/dl). After hemodynamic resuscitation, the patient underwent an esophagogastroduodenoscopy. On endoscopy, a submucosal ulcerated tum or with active bleeding was detected in the second part of the duodenum (Fig. 1). Biopsy specimens from the lesion showed normal duodenal mucosa. A CT scan of the abdomen was performed and no local lymph node involvement was identified. The patient underwent local surgical excision. The histopathological exam ination of the resected specimen evidenced a gangliocytic paraganglioma, 15 mm in size, located in the submucosa (Fig. 2), consisting of three components: predom inant spindle cells (Schwann cells) forming small fascicles, large epithelial (endocrine) cells in nests and pseudo-glandular structure and large ganglion cells scattered singly and in small clusters (Fig. 3). Immunostaining examinations showed positivity of spindle cells for S-100, while the endocrine cells were positive for chromogranin A (Fig. 4). J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
110
Boeriu et al
Fig. 2. Histopathology specimen: polypoid lesion covered by duodenal mucosa, represented by a welldelineated nodular lesion localized in the submucosa (arrow) (H&E, x2).
Fig. 1 A, B. Endoscopic views of the duodenal tumor.
Fig. 3. Small clusters of epithelial cells (thin arrow) and isolated large ganglion cells localized in a fibrillary background (thick arrow) (H&E, x20).
The patien t was discharged with norm al levels of hemoglobin and hematocrit. One year following surgical resection, he showed no endoscopic or imaging evidence of tumor recurrence, and normal laboratory parameters.
DISCUSSION Gangliocytic paraganglioma was first described by Dahl et al. in 1957 [4], The tumor is usually detected in the sixth decade of life [5], Patient ages range from 15 years to 82 years, with male predominance [6], Gangliocytic paragangliomas account for 6% to 9% of duodenal NETs and represent the third most frequent histopathologic type after gastrinomas and som atostatinom as [3], They are considered benign hamartomatous tumors, derived from the ventral pancreatic primordium [2, 7], The duodenum and periam pullary region represent the most common sites of occurrence (90%) [6], but the tumor has also been described in other anatomic locations: esophagus, stomach, jejunum, appendix, pancreas, spinal cord, nasopharynx, and the lung [8- 15]. The diagnosis may be often delayed because of the small size of tumors, most of them being asymptomatic or showing nonspecific symptoms, such as vague abdominal discomfort or dyspepsia. In many cases, gangliocytic paraganglioma represents an incidental endoscopic finding, when a polypoid submucosal mass is detected mainly in the first or the second part of the duodenum. Due to the submucosal location of the tumor, the reported diagnostic rate by biopsy before surgical intervention is low J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
Fig. 4. Positive immunostaining of epithelial cells and ganglion cells for chromogranin A (x4).
(11.4%) [6]. Barium studies of the upper gastrointestinal tract reveal an intraluminal filling defect [5]. Clinical symptoms include intestinal obstruction, biliary obstruction, overt gastrointestinal bleeding and anemia, related to local tumor growth and mucosal ulceration [16]. In a literature survey performed by Okubo et al., gastrointestinal bleeding was reported to be the most common symptom, followed by abdominal pain and anemia [6]. Although in most cases the tumors are endocrinologically silent, rare situations have been described when the tumor synthesizes somatostatin leading to diarrhea and steatorrhea [17], Histologically, the tum or is composed of epithelioid endocrine cells, ganglion cells and spindled Schwann-like cells, intermingled with the smooth muscle and small pancreatic duct, especially in tumors located in the ampullary region, producing a very complex lesion [18]. The components are
Duodenal gangliocytic paraganglioma
identified by using specific im m unohistochem ical m arkers. Spindle cells stain positive for S-100 protein, neuron-specific enolase (NSE) and synaptophysin. G anglion cells are positive for synaptophysin, neuron-specific enolase, som atostatin and p ancreatic polypeptide (PP). E ndocrine cells show positive staining for neuron-specific enolase, synaptophysin, pancreatic polypeptide, som atostatin, chrom ogranin A, cytokeratins and serotonin [6], Radical surgical excision or endoscopic resection o f the tu m o r rep resen t available th e ra p eu tic alternatives [5, 19]. A previous evaluation by im aging studies is necessary for d e te rm in in g en d o sco p ic resectability. Local lym ph n od es m etastasis should be excluded before endoscopic treatm ent. E ndoscopic u ltraso n o g rap h y (EUS) evaluates th e dep th o f tu m o r invasion and local lym ph node m etastases [20], The tu m o r is m ainly located w ithin the subm ucosal layer, b u t in som e cases it m ay involve the m uscularis p ropria and even the m esentery [17]. C om puted tom ography (CT) and m agnetic resonance im aging can be used to evaluate m etastatic spread o f the tu m o r [2]. G a n g lio c y tic p a ra g a n g lio m a s are u su a lly b e n ig n , in contrast w ith gastrin-cell or som atostatin-cell tum ors arising in th e sam e areas. Occasionally, a large tu m o r (over 2 cm) m ay spread to local lym ph nodes, m ainly due to the endocrine com p o n ent o f the tum or. A tu m o r exceeding th e subm ucosal layer represents a risk factor for lym ph nodes m etastases [6]. The recurrence o f a tu m o r after therapeutic procedures has been reported [16,21,22]. Specific histologic features to predict m alig n an t p o te n tia l have n o t been yet defined. Therefore, p an creaticoduodenectom y w ith lym ph node dissection has been proposed as a better option in large tum ors w ith nuclear pleom orphism , m itotic activity, and infiltrative m argins [16]. Liver and lym ph nodes m etastases have been reported in a patien t presenting a sm all (1 cm in diam eter), non-infiltrative gangliocytic paragangliom a, w ith Ki-67 proliferation index less th an 1% [24]. C riteria for predicting the risk o f m etastasis are still u n d er evaluation [18]. Im m unohistochem ical evaluation using Ki-67 seem s to have a lim ited value as a prognostic indicator, as well as bcl-2 and p53 [6]. However, based on p u b lish ed data, patien ts w ith gangliocytic paragangliom as have a good prognosis, even in the presence o f lym ph nodes or liver m etastasis [6, 23], The difficulty in p re d ic tin g th e b ehavior o f the tu m o r w arrants an accurate evaluation by im aging studies, p rio r to therapeutic intervention, as well as a long-term follow-up. In o u r patient w ith gangliocytic paragangliom a confined to the subm ucosa neither tu m o r recurrence nor m etastasis have been detected over a one-year follow -up period.
111
well as the recu rren ce after incom plete excision have been reported. D ue to the possible aggressive behaviour and the lack o f predictive m arkers for progression o f the tum or, close surveillance is advised. Conflicts of interest: Nothing to declare. Authors’ contributions: A.B. performed the endoscopic examinations and wrote the manuscript. R.G. performed the surgical resection and the literature search. S.M. performed the histopathological examination. C.B. and D.D. critically reviewed the manuscript. All authors approved the final manuscript.
REFERENCES 1. Kloppel G. O berndorfer and his successors: from carcinoid to neuroendocrine carcinoma. Endocr Pathol 2007; 18: 141-144. doi: 10.1007/s 12022-007-0021 -9 2. Oberg K. G astrointestinal C arcinoid Tumors (G astrointestinal Neuroendocrine Tumors) and the Carcinoid Syndrome. In: Sleisenger and Fordtran’s Gastrointestinal and Liver Diseases. 9thEdn, Philadelphia: Saunders, 2010, 475-490. 3. Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol 2005; 19: 675-697. doi: 10.1016/j.bpg.2005.05.009 4. Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas: brief review with report of a duodenal ganglioneuroma. Am 1 Pathol 1957; 33: 953-966. 5. Suk FM, Lin YH, Hsieh MC. Clinical Challenges and Images in GI. Duodenal gangliocytic paraganglioma. Gastroenterology 2008; 135: 361, 714. doi: 10.1053/j.gastro.2008.06.056 6. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011; 11: 187. doi: 10.1186/1471-2407-11-187 7. 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. doi: 10.1097/00000478-198501000-00007 8. W einrach DM, Wang KL, Blum MG, Yeldandi AV, Laskin WB. M ultifocal presentation of gangliocytic paragangliom a in the mediastinum and esophagus. Hum Pathol 2004; 35: 1288-1291. doi: 10.1016/j.humpath.2004.07.013 9. Burke AP, Helwig EB. Gangliocytic paraganglioma. Am J Clin Pathol 1989; 92: 1-9. 10. Aung W, Gallager HJ, Joyce WP, Hayes DB, Leader M. Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma. J Clin Pathol 1995; 48: 84-85. doi: 10.1136/jcp.48.1.84
CONCLUSION
11. van Eeden S, Offerhaus GJ, Peterse HL, Dingemans KP, Blaauwgeers HL. Gangliocytic paraganglioma of the appendix. Histopathology 2000;
Clinical presentation of patients with duodenal gangliocytic p a ra g a n g lio m a m ay be a sy m p to m a tic u n til th e m u c o sal u lc e ra tio n causes an ov ert g astro in te stin a l b leeding. T he tu m o r m ay be associated w ith signs o f obstruction (intestinal o r biliary), depen d in g on its size and the proxim ity to the am pulla o f Vater. Im aging studies help to m ake the therapeutic decision. The tu m o r can be cured by surgical or endoscopic tre a tm e n t, alth o u g h lym ph nodes and liver m etastasis, as
36: 47-49. doi: 10.1046/j,1365-2559.2000.00881.x 12. Tomic S, Warner T. Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases. Am J Gastroenterol 1996; 91: 607-608. 13. Vural M, Arslantas A, Isiksoy S, Adapinar B, Atasoy M, Soylemezoglu F. Gangliocytic paraganglioma of the cauda equina with significant calcification: first description in pediatric age. Zentralbl Neurochir 2008; 69: 47-50. doi: 10.1055/S-2007-985162 J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
Boeriu et al
112
14. Sinkre P, Lindberg G, Albores-Saavedra J. Nasopharyngeal gangliocytic paraganglioma. Arch Pathol Lab Med 2001; 125: 1098-1100. 15. Hironaka M, Fukayama M, Takayashiki N, Saito K, Sohara Y, Funata N.
19. Trawick E, Salaria S, Yachimski P. Endoscopic resection of an ampullary gangliocytic paraganglioma. Gastrointest Endosc 2014; 79: 716-717. doi: 10.1016/j.gie.2013.11.036
Pulmonary gangliocytic paraganglioma: case report and comparative
20. Yoshikane H, Tsukamoto Y, Niwa Y, et al. Carcinoid tumors of the
immunohistochemical study of related neuroendocrine neoplasms. Am J
gastrointestinal tract: evaluation with endoscopic ultrasonography.
Surg Pathol 2001; 25:688-693. doi: 10.1097/00000478-200105000-00020
G astrointest E ndosc 1993; 39: 375-383. doi: 10.1016/S 0016-
16. Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg 2007; 11: 1351-1354. doi: 10.1007/sll605-007-0217-9 17. Moonim MT. Tumours of chromaffin cell origin: phaeochromocytoma and paraganglioma. Diagnostic Histopathology 2012; 18:234-244. doi: 10.1016/j.mpdhp.2012.03.003 18. Papathomas TG, de Krijger RR, Tischler AS. Paragangliomas: update
5107(93)70109-1 21. Dookhan DB, Miettinen M, Finkel G, Gibas Z. Recurrent duodenal gangliocytic paraganglioma with lymph node metastasis. Histopathology 1993; 22: 399-401. doi: 10.1111/j. 1365-2559.1993.tb00145.x 22. Sundarajan 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: el39-141.
on differential diagnostic considerations, composite tumors, and recent
23. Rowsell C, Coburn N, Chetty R. Gangliocytic paraganglioma: a rare
genetic developments. Semin Diagn Pathol 2013; 30: 207-223. doi:
case with metastases of all 3 elements to liver and lymph nodes. Ann
10.1053/j.semdp.2013.06.006
Diagn Pathol 2011; 15:467-471. doi: 10.1016/j.anndiagpath.2010.07.009
J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
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Available from: URL: http://www.jgld.rO/2015/l/19.html DOI: http://dx.doi.org/10.15403/jgld.2014.1121.abo
Gangliocytic Paraganglioma: a Rare Cause of Gastrointestinal Bleeding Alina B oeriu1, D aniela D obru 1, Rares G eorgescu2, Sim ona M ocan3, Cristian B oeriu 4
1) Department of
ABSTRACT
Gastroenterology; 2) Department of Surgery, University of Medicine and Pharmacy Tirgu Mures; 3) Department of Pathology, Tirgu Mures County Emergency Hospital; 4) Emergency Department,
Duodenal neuroendocrine tumors (NETs) are rare tumors, consisting of five different types of tumors. In many cases, they may be asymptomatic, leading to delay in diagnosis. Clinical symptoms are related to local tumor growth and mucosal ulceration. We report a 38-year old man with duodenal gangliocytic paraganglioma causing overt upper gastrointestinal bleeding and anemia. We describe specific clinical and histopathological features of the tumor, and review the diagnostic and therapeutic strategy. Gangliocytic paragangliomas are regarded as benign tumors. However, the disease recurrence and the malignant potential of the tumor have also been reported.
University of Medicine and Pharmacy, Targu Mures
Key words: gangliocytic paraganglioma - immunostaining - surgical excision - metastasis.
Romania
Address fo r correspondence:
INTRODUCTION
CASE REPORT
Dr. Boeriu Cristian Mures County Emergency Hospital, Str.Gheorghe Marinescu nr. 50 540136 Tirgu Mures, Romania [email protected]
Received: 07.12.2014 Accepted: 22.12.2014
G a stro in te stin a l n e u r o endocrine tumors (NETs), also called carcinoids, arise from cells of neuroendocrine systems, and were first described as a distinct class of malignant tumors with less aggressive behavior th a n a d e n o c a rc in o m a s of the gastrointestinal tract [1], Morphologically, carcinoids are identified by positive staining for neuroendocrine markers such as chromogranin A, synaptophysin, Leu7, and n e u ro n -sp e c ific enolase (NSE). Duodenal NETs are rare tumors, representing about 4% from all gastrointestinal carcinoids [2], Different types of neuroendocrine tumors have been described in the duodenum, c o n sistin g of g astrin o m as, somatostatinomas, n o n functional NETs, gangliocytic paragangliom as and poorly differentiated NETs [3].
A 38-year old man was referred to our department with melena. He had a previous history of mild anemia (hemoglobin 10.7 g/dl), intermittent hematochezia, and fatigue in the past year. A colonoscopy performed for the evaluation of anemia and rectal bleeding revealed hemorrhoids. At admission to our hospital, physical examination showed pallor, tachycardia and hypotension. Laboratory values revealed severe anemia and iron deficiency (hemoglobin 5.2 g/dl, hematocrit 18%, serum iron 20 pg/dl). After hemodynamic resuscitation, the patient underwent an esophagogastroduodenoscopy. On endoscopy, a submucosal ulcerated tum or with active bleeding was detected in the second part of the duodenum (Fig. 1). Biopsy specimens from the lesion showed normal duodenal mucosa. A CT scan of the abdomen was performed and no local lymph node involvement was identified. The patient underwent local surgical excision. The histopathological exam ination of the resected specimen evidenced a gangliocytic paraganglioma, 15 mm in size, located in the submucosa (Fig. 2), consisting of three components: predom inant spindle cells (Schwann cells) forming small fascicles, large epithelial (endocrine) cells in nests and pseudo-glandular structure and large ganglion cells scattered singly and in small clusters (Fig. 3). Immunostaining examinations showed positivity of spindle cells for S-100, while the endocrine cells were positive for chromogranin A (Fig. 4). J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
110
Boeriu et al
Fig. 2. Histopathology specimen: polypoid lesion covered by duodenal mucosa, represented by a welldelineated nodular lesion localized in the submucosa (arrow) (H&E, x2).
Fig. 1 A, B. Endoscopic views of the duodenal tumor.
Fig. 3. Small clusters of epithelial cells (thin arrow) and isolated large ganglion cells localized in a fibrillary background (thick arrow) (H&E, x20).
The patien t was discharged with norm al levels of hemoglobin and hematocrit. One year following surgical resection, he showed no endoscopic or imaging evidence of tumor recurrence, and normal laboratory parameters.
DISCUSSION Gangliocytic paraganglioma was first described by Dahl et al. in 1957 [4], The tumor is usually detected in the sixth decade of life [5], Patient ages range from 15 years to 82 years, with male predominance [6], Gangliocytic paragangliomas account for 6% to 9% of duodenal NETs and represent the third most frequent histopathologic type after gastrinomas and som atostatinom as [3], They are considered benign hamartomatous tumors, derived from the ventral pancreatic primordium [2, 7], The duodenum and periam pullary region represent the most common sites of occurrence (90%) [6], but the tumor has also been described in other anatomic locations: esophagus, stomach, jejunum, appendix, pancreas, spinal cord, nasopharynx, and the lung [8- 15]. The diagnosis may be often delayed because of the small size of tumors, most of them being asymptomatic or showing nonspecific symptoms, such as vague abdominal discomfort or dyspepsia. In many cases, gangliocytic paraganglioma represents an incidental endoscopic finding, when a polypoid submucosal mass is detected mainly in the first or the second part of the duodenum. Due to the submucosal location of the tumor, the reported diagnostic rate by biopsy before surgical intervention is low J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
Fig. 4. Positive immunostaining of epithelial cells and ganglion cells for chromogranin A (x4).
(11.4%) [6]. Barium studies of the upper gastrointestinal tract reveal an intraluminal filling defect [5]. Clinical symptoms include intestinal obstruction, biliary obstruction, overt gastrointestinal bleeding and anemia, related to local tumor growth and mucosal ulceration [16]. In a literature survey performed by Okubo et al., gastrointestinal bleeding was reported to be the most common symptom, followed by abdominal pain and anemia [6]. Although in most cases the tumors are endocrinologically silent, rare situations have been described when the tumor synthesizes somatostatin leading to diarrhea and steatorrhea [17], Histologically, the tum or is composed of epithelioid endocrine cells, ganglion cells and spindled Schwann-like cells, intermingled with the smooth muscle and small pancreatic duct, especially in tumors located in the ampullary region, producing a very complex lesion [18]. The components are
Duodenal gangliocytic paraganglioma
identified by using specific im m unohistochem ical m arkers. Spindle cells stain positive for S-100 protein, neuron-specific enolase (NSE) and synaptophysin. G anglion cells are positive for synaptophysin, neuron-specific enolase, som atostatin and p ancreatic polypeptide (PP). E ndocrine cells show positive staining for neuron-specific enolase, synaptophysin, pancreatic polypeptide, som atostatin, chrom ogranin A, cytokeratins and serotonin [6], Radical surgical excision or endoscopic resection o f the tu m o r rep resen t available th e ra p eu tic alternatives [5, 19]. A previous evaluation by im aging studies is necessary for d e te rm in in g en d o sco p ic resectability. Local lym ph n od es m etastasis should be excluded before endoscopic treatm ent. E ndoscopic u ltraso n o g rap h y (EUS) evaluates th e dep th o f tu m o r invasion and local lym ph node m etastases [20], The tu m o r is m ainly located w ithin the subm ucosal layer, b u t in som e cases it m ay involve the m uscularis p ropria and even the m esentery [17]. C om puted tom ography (CT) and m agnetic resonance im aging can be used to evaluate m etastatic spread o f the tu m o r [2]. G a n g lio c y tic p a ra g a n g lio m a s are u su a lly b e n ig n , in contrast w ith gastrin-cell or som atostatin-cell tum ors arising in th e sam e areas. Occasionally, a large tu m o r (over 2 cm) m ay spread to local lym ph nodes, m ainly due to the endocrine com p o n ent o f the tum or. A tu m o r exceeding th e subm ucosal layer represents a risk factor for lym ph nodes m etastases [6]. The recurrence o f a tu m o r after therapeutic procedures has been reported [16,21,22]. Specific histologic features to predict m alig n an t p o te n tia l have n o t been yet defined. Therefore, p an creaticoduodenectom y w ith lym ph node dissection has been proposed as a better option in large tum ors w ith nuclear pleom orphism , m itotic activity, and infiltrative m argins [16]. Liver and lym ph nodes m etastases have been reported in a patien t presenting a sm all (1 cm in diam eter), non-infiltrative gangliocytic paragangliom a, w ith Ki-67 proliferation index less th an 1% [24]. C riteria for predicting the risk o f m etastasis are still u n d er evaluation [18]. Im m unohistochem ical evaluation using Ki-67 seem s to have a lim ited value as a prognostic indicator, as well as bcl-2 and p53 [6]. However, based on p u b lish ed data, patien ts w ith gangliocytic paragangliom as have a good prognosis, even in the presence o f lym ph nodes or liver m etastasis [6, 23], The difficulty in p re d ic tin g th e b ehavior o f the tu m o r w arrants an accurate evaluation by im aging studies, p rio r to therapeutic intervention, as well as a long-term follow-up. In o u r patient w ith gangliocytic paragangliom a confined to the subm ucosa neither tu m o r recurrence nor m etastasis have been detected over a one-year follow -up period.
111
well as the recu rren ce after incom plete excision have been reported. D ue to the possible aggressive behaviour and the lack o f predictive m arkers for progression o f the tum or, close surveillance is advised. Conflicts of interest: Nothing to declare. Authors’ contributions: A.B. performed the endoscopic examinations and wrote the manuscript. R.G. performed the surgical resection and the literature search. S.M. performed the histopathological examination. C.B. and D.D. critically reviewed the manuscript. All authors approved the final manuscript.
REFERENCES 1. Kloppel G. O berndorfer and his successors: from carcinoid to neuroendocrine carcinoma. Endocr Pathol 2007; 18: 141-144. doi: 10.1007/s 12022-007-0021 -9 2. Oberg K. G astrointestinal C arcinoid Tumors (G astrointestinal Neuroendocrine Tumors) and the Carcinoid Syndrome. In: Sleisenger and Fordtran’s Gastrointestinal and Liver Diseases. 9thEdn, Philadelphia: Saunders, 2010, 475-490. 3. Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol 2005; 19: 675-697. doi: 10.1016/j.bpg.2005.05.009 4. Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas: brief review with report of a duodenal ganglioneuroma. Am 1 Pathol 1957; 33: 953-966. 5. Suk FM, Lin YH, Hsieh MC. Clinical Challenges and Images in GI. Duodenal gangliocytic paraganglioma. Gastroenterology 2008; 135: 361, 714. doi: 10.1053/j.gastro.2008.06.056 6. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011; 11: 187. doi: 10.1186/1471-2407-11-187 7. 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. doi: 10.1097/00000478-198501000-00007 8. W einrach DM, Wang KL, Blum MG, Yeldandi AV, Laskin WB. M ultifocal presentation of gangliocytic paragangliom a in the mediastinum and esophagus. Hum Pathol 2004; 35: 1288-1291. doi: 10.1016/j.humpath.2004.07.013 9. Burke AP, Helwig EB. Gangliocytic paraganglioma. Am J Clin Pathol 1989; 92: 1-9. 10. Aung W, Gallager HJ, Joyce WP, Hayes DB, Leader M. Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma. J Clin Pathol 1995; 48: 84-85. doi: 10.1136/jcp.48.1.84
CONCLUSION
11. van Eeden S, Offerhaus GJ, Peterse HL, Dingemans KP, Blaauwgeers HL. Gangliocytic paraganglioma of the appendix. Histopathology 2000;
Clinical presentation of patients with duodenal gangliocytic p a ra g a n g lio m a m ay be a sy m p to m a tic u n til th e m u c o sal u lc e ra tio n causes an ov ert g astro in te stin a l b leeding. T he tu m o r m ay be associated w ith signs o f obstruction (intestinal o r biliary), depen d in g on its size and the proxim ity to the am pulla o f Vater. Im aging studies help to m ake the therapeutic decision. The tu m o r can be cured by surgical or endoscopic tre a tm e n t, alth o u g h lym ph nodes and liver m etastasis, as
36: 47-49. doi: 10.1046/j,1365-2559.2000.00881.x 12. Tomic S, Warner T. Pancreatic somatostatin-secreting gangliocytic paraganglioma with lymph node metastases. Am J Gastroenterol 1996; 91: 607-608. 13. Vural M, Arslantas A, Isiksoy S, Adapinar B, Atasoy M, Soylemezoglu F. Gangliocytic paraganglioma of the cauda equina with significant calcification: first description in pediatric age. Zentralbl Neurochir 2008; 69: 47-50. doi: 10.1055/S-2007-985162 J Gastrointestin Liver Dis, March 2015 Vol. 24 No 1: 109-112
Boeriu et al
112
14. Sinkre P, Lindberg G, Albores-Saavedra J. Nasopharyngeal gangliocytic paraganglioma. Arch Pathol Lab Med 2001; 125: 1098-1100. 15. Hironaka M, Fukayama M, Takayashiki N, Saito K, Sohara Y, Funata N.
19. Trawick E, Salaria S, Yachimski P. Endoscopic resection of an ampullary gangliocytic paraganglioma. Gastrointest Endosc 2014; 79: 716-717. doi: 10.1016/j.gie.2013.11.036
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