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Open Access Research article

Literature survey on epidemiology and pathology of gangliocytic paraganglioma

Yoichiro Okubo1, Megumi Wakayama1, Tetsuo Nemoto1, Kanako Kitahara1, Haruo Nakayama2, Kazutoshi Shibuya1*, Tomoyuki Yokose3, Manabu Yamada1, Kayoko Shimodaira1, Daisuke Sasai1, Takao Ishiwatari1, Masaru Tsuchiya4 and Nobuyuki Hiruta1

Author Affiliations

1 Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan

2 Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6, Ohashi, Meguro, Tokyo, 153-8515, Japan

3 Department of Pathology, Kanagawa Cancer Center, 1-1-2, Nakao, Asahi-Ku, Yokohama-city, Kanagawa, 245-0815, Japan

4 Division of General and Gastroenterological Surgery, Department of Surgery (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan

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BMC Cancer 2011, 11:187  doi:10.1186/1471-2407-11-187

Published: 20 May 2011

Abstract

Background

Although gangliocytic paraganglioma (GP) has generally been regarded as a neuroendocrine tumor, its origin remains unclear. We therefore aimed to investigate the details of this disease by carefully analyzing and extracting common features of the disease as presented in selected publications.

Methods

We searched for English and Japanese cases of GP using the PubMed and IgakuChuoZasshi databases on August 2010. We then extracted and sampled raw data from the selected publications and performed appropriate statistical analyses. Additionally, we evaluated the expression of hormone receptors based on our previously reported case.

Results

192 patients with GP were retrieved from the databases. Patient ages ranged from 15 y to 84 y (mean: 52.3 y). The gender ratio was 114:76 (male to female, 2 not reported). Maximum diameter of the tumors ranged from 5.5 mm to 100 mm (mean: 25.0 mm). The duodenum (90.1%, 173/192) was found to be the most common site of the disease. In 173 patients with duodenal GP, gastrointestinal bleeding (45.1%, 78/173) was found to be the most common symptom of the disease, followed by abdominal pain (42.8%, 74/173), and anemia (14.5%, 25/173). Rate of lymph node metastasis was 6.9% (12/173). Our statistical analysis indicated that significant differences were found for gender between GP within the submucosal layer and exceeding the submucosal layer. Furthermore, our immunohistochemical evaluation showed that both epithelioid and pancreatic islet cells showed positive reactivity for progesterone receptors.

Conclusions

Our literature survey revealed that there were many more cases of GP exceeding the submucosal layer than were expected. Meanwhile, our statistical analyses and immunohistochemical evaluation supported the following two hypotheses. First, vertical growth of GP might be affected by progesterone exposure. Second, the origin of GP might be pancreatic islet cells. However, it is strongly suspected that our data have been affected by publication bias and to confirm these hypotheses, further investigation is required.