Literature survey on epidemiology and pathology of gangliocytic paraganglioma

Abstract

BackgroundAlthough 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.MethodsWe 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.Results192 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.ConclusionsOur 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.

A Rare Cause of Melaena-Duodenal Gangliocytic Paraganglioma

“…Furthermore, the same authors hypothesise that tumour progression may be related to sex hormone activity. This is supported by findings of increased progesterone receptors in epithelioid components of primary and metastatic foci [5].…”

“…Gangliocytic paraganglioma (GP) is a rare benign neuroendocrine tumour, majority being duodenal [4], in a mean age group of 52 to 54 years, with no significant gender difference [5].…”

“…The most common presenting symptom is gastrointestinal bleeding [5]; the other being abdominal pain. Biliary and duodenal obstruction is rare [6].…”

“…Despite the benign nature of GP, it has a 5% [9] to 6.9% [5] potential for lymph node metastases. Tumour size does not correlate with LN metastases [10,11].…”

Bowel obstruction secondary to yeyunal intussusception due to gangliocytic paraganglioma

“…The most common symptom is gastrointestinal bleeding, followed by abdominal pain and anemia (1)(2)(3)(4).…”