A gangliocytic patially glandular paraganglioma with lymph node metastasis
© Shi et al.; licensee BioMed Central Ltd. 2014
Received: 8 January 2014
Accepted: 4 March 2014
Published: 20 March 2014
Gangliocytic paraganglioma (GP) is an infrequent neuroendocrine tumor usually with three elements as epithelioid cells, spindle-shaped cells and ganglion-like cells, which is generally regarded as a benign tumor. Only a few cases with lymph node metastasis have been reported. Herein, we reported a 47-year-old man of GP with distinct glandular component embedded in the spindle tumor cells in the primary tumor and the metastatic lymph nodes. The immunohistochemical profile was helpful to give the final diagnosis as gangliocytic paraganglioma. Here, we added one more GP case with regional lymph nodes metastasis. And particularly, there were small amount of distinct glandular component both in the primary tumor and the metastatic lymph nodes, which indicated that adenocarcinoma might coexist with GP. And GP should also be distinguished from carcinoid tumor, paraganglioma, ganglioneuroma, or GIST.
Letter to the editor
Gangliocytic paraganglioma cases with lymph node metastasis
Chief clinical presentation
Büchler et al.
Inai et al.
Hashimoto et al.
Dookhan et al.
Sundararajan et al.
Bucher et al.
Wong et al.
Witkiewicz et al.
Mann et al.
Abdominal pain, vomiting, weight loss
Okubo et al.
Epigastralgia, tarry stool
Saito et al.
Gastrointestinal bleeding, anemia
Uchida et al.
Ogata et al.
Gastrointestinal bleeding, anemia
Barret et al.
GP is an infrequent neuroendocrine tumor usually appearing in the second part region of duodenum. The most common clinical manifestation is gastrointestinal bleeding (45.1%) due to mucosal erosion or ulceration, followed by abdominal pain (42.8%) and anemia (14.5%). GP has been known well after it was firstly reported by Dahl et al. and named entity by Kepes et al.[1, 2]. Confirmation of three identical components comprising epithelial cells, spindle cells, and ganglion cells was essential for the diagnosis. GP should be distinguished from carcinoid tumor, ganglioneuroma, pigmented paraganglioma, and spindle cell tumors as GIST[4–6]. Immunohistochemical examination was also an important diagnostic clue to identify the three cellular components of GP.
In this case the epithelial component in the metastatic lymph nodes led to the thought as metastatic neuroendocrine carcinoma in frozen slides. With more than ten tissue blocks section, three components as spindle cells, epithelial cells, and ganglion cells through light on the diagnosis as gangliocytic paraganglioma. Immunohistochemical staining had confirmed the diagnosis. In this case, MIB-1 was estimated as less than 1%, suggesting the low proliferative rate of this tumor, which might not reflect the prognostic value in GP.
Although GP is generally considered as a benign periampullary lesion, however, it is very unwise to assume that this tumor must be a benign entity. Metastasis to regional lymph nodes by this tumor and/or local recurrence has been reported several times in the literature[7–20] (Table 1). Here, we added a GP case with lymph node metastasis to that list. Although there is still no distinct evidence that the lymph node metastasis indicating malignant prognosis, lymphovascular invasion may be a major factor in the malignant potential of GP. In hence, it was important to image the examination to investigate the possibility of lymph node metastasis before an operation.
Anders had reported a GP case with an advanced duodenal adenocarcinoma coexisted. In our case we also found small amount of distinct glandular components besides three typical tumor cells of GP. Hence, it could not be excluded for the potential that adenocarcinoma coexist with GP at the same location. Although the patient remains well and no recurrence after nearly two years routine follow-up, a long time follow-up is needed to know whether there is a malignant capacity of this case.
Herein we presented a rarely gangliocytic patially glandular paraganglioma with lymph node metastasis. In addition to the rarity of the tumor, we wish to emphasize the pleomorphic morphologic features mimicking adenocarcinoma and the malignant potency of gangliocytic paraganglioma with lymph nodes metastasis.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Dahl EV, Waugh JM, Dahlin DC: Gastrointestinal ganglioneuromas; brief review with report of a duodenal ganglioneuroma. Am J Pathol. 1957, 33: 953-965.PubMedPubMed CentralGoogle Scholar
- Kepes JJ, Zacharias DL: Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination. Cancer. 1971, 27: 61-67. 10.1002/1097-0142(197101)27:1<61::AID-CNCR2820270111>3.0.CO;2-I.PubMedView ArticleGoogle Scholar
- Nuño-Guzmána CM, Arróniz-Jáureguia J, Alvarez-Lópezb F, Corona JL, Cerda-Camacho F, Rostro R, Gutiérrez-Manjarrez JI: Obstructing gangliocytic paraganglioma in the third portion of the duodenum. Gastroenterol. 2012, 6: 489-495.Google Scholar
- van Eeden S, Offerhaus GJ, Peterse HL, Dingemans KP, Blaauwgeers HL: Gangliocytic paraganglioma of the appendix. Histopathology. 2000, 36: 47-49. 10.1046/j.1365-2559.2000.00881.x.PubMedView ArticleGoogle Scholar
- Jinchen H, Jitao W, Cai L, Jiang L, Lang Z, Qu G, Liu H, Yao W, Yu G: Retroperitoneal composite pheochromocytoma-ganglioneuroma: a case report and review of literature. Diagn Pathol. 2013, 8: 63-67. 10.1186/1746-1596-8-63.View ArticleGoogle Scholar
- Zhao L, Luo J, Zhang H, Da J: Pigmented paraganglioma of the kidney: a case report. Diagn Pathol. 2012, 7: 77-81. 10.1186/1746-1596-7-77.PubMedPubMed CentralView ArticleGoogle Scholar
- Büchler M, Malfertheiner P, Baczako K, Krautzberger W, Beger HG: A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction–malignant paraganglioma?. Digestion. 1985, 31: 54-59. 10.1159/000199178.PubMedView ArticleGoogle Scholar
- Inai K, Kobuke T, Yonehara S, Tokuoka S: Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy. Cancer. 1989, 63: 2540-2545. 10.1002/1097-0142(19890615)63:12<2540::AID-CNCR2820631231>3.0.CO;2-H.PubMedView ArticleGoogle Scholar
- Hashimoto S, Kawasaki S, Matsuzawa K, Harada H, Makuuchi M: Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis. Am J Gastroenterol. 1992, 87: 1216-1218.PubMedGoogle Scholar
- Dookhan DB, Miettinen M, Finkel G, Gibas Z: Recurrent duodenal gangliocytic paraganglioma with lymph node metastases. Histopathology. 1993, 22: 399-401. 10.1111/j.1365-2559.1993.tb00145.x.PubMedView ArticleGoogle Scholar
- 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: e139-e141.PubMedGoogle Scholar
- Bucher P, Mathe Z, Bühler L, Chilcott M, Gervaz P, Egger JF, Morel P: Paraganglioma of the ampulla of Vater: a potentially malignant neoplasm. Scand J Gastroenterol. 2004, 39: 291-295. 10.1080/00365520310007503.PubMedView ArticleGoogle Scholar
- Wong A, Miller AR, Metter J, Thomas CR: Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature. World J Surg Oncol. 2005, 3: 15-10.1186/1477-7819-3-15.PubMedPubMed CentralView ArticleGoogle Scholar
- Witkiewicz A, Galler A, Yeo CJ, Gross SD: Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg. 2007, 11: 1351-1354. 10.1007/s11605-007-0217-9.PubMedView ArticleGoogle Scholar
- Mann CM, Bramhall SR, Buckels JA, Taniere P: An unusual case of duodenal obstruction-gangliocytic paraganglioma. J Hepatobiliary Pancreat Surg. 2009, 16: 562-565. 10.1007/s00534-009-0092-8.PubMedView ArticleGoogle Scholar
- Okubo Y, Yokose T, Tuchiya M, Mituda A, Wakayama M, Hasegawa C, Sasai D, Nemoto T, Shibuya K: Duodenal gangliocytic paraganglioma showing lymph node metastasis: a rare case report. Diagn Pathol. 2010, 5: 27-10.1186/1746-1596-5-27.PubMedPubMed CentralView ArticleGoogle Scholar
- Saito J, Hirata N, Furuzono M, Nakaji S, Inase M, Nagano H, Iwata M, Tochitani S, Fukatsu K, Fujii H, Ishii E, Kataoka J, Mikata R, Masuya Y, Ito H, Ohmori J, Wakasugi S, Ebara M, Hoshi K: A case of duodenal gangliocytic paraganglioma with lymph node metastasis. Nihon Shokakibyo Gakkai Zasshi. 2010, 107: 639-648.PubMedGoogle Scholar
- Uchida D, Ogawa T, Ueki T, Kominami Y, Numata N, Matsusita H, Morimoto Y, Nakarai A, Ota S, Nanba S, Takada S, Iwado S, Kurome M, Ohe H, Okamoto R, Uematsu S, Nakagawa M, Ishida K, Araki Y, Mizuno M: A case of gangliocytic paraganglioma with lymphoid metastasis. Nihon Shokakibyo Gakkai Zasshi. 2010, 107: 1456-1465.PubMedGoogle Scholar
- Ogata S, Horio T, Sugiura Y, Aiko S, Aida S: Duodenal gangliocytic paraganglioma with regional lymph node metastasis and a glandular component. Pathol Int. 2011, 61: 104-107. 10.1111/j.1440-1827.2010.02620.x.PubMedView ArticleGoogle Scholar
- Barret M, Rahmi G, Duong van Huyen JP, Landi B, Cellier C, Berger A: Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report. Eur J Gastroenterol Hepatol. 2012, 24: 90-94. 10.1097/MEG.0b013e32834dfdfa.PubMedView ArticleGoogle Scholar
- Anders KH, Glasgow BJ, Lewin KJ: Gangliocytic paraganglioma associated with duodenal adenocarcinoma. Case report with immunohistochemical evaluation. Arch Pathol Lab Med. 1987, 111: 49-52.PubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.