title: Literature survey on epidemiology and pathology of gangliocytic paraganglioma.
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.
authors: Department of Surgical Pathology, Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-Ku, Tokyo, 143-8541, Japan.
authors: Yoichiro
authors: Y
authors: Okubo
authors: Megumi
authors: M
authors: Wakayama
authors: Tetsuo
authors: T
authors: Nemoto
authors: Kanako
authors: K
authors: Kitahara
authors: Haruo
authors: H
authors: Nakayama
authors: Kazutoshi
authors: K
authors: Shibuya
authors: Tomoyuki
authors: T
authors: Yokose
authors: Manabu
authors: M
authors: Yamada
authors: Kayoko
authors: K
authors: Shimodaira
authors: Daisuke
authors: D
authors: Sasai
authors: Takao
authors: T
authors: Ishiwatari
authors: Masaru
authors: M
authors: Tsuchiya
authors: Nobuyuki
authors: N
authors: Hiruta
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