Duodenal Rare Neuroendocrine Tumor: Clinicopathological Characteristics of Patients with Gangliocytic Paraganglioma

Abstract

Gangliocytic paraganglioma (GP) has been regarded as a rare benign tumor that commonly arises from the second part of the duodenum. As GP does not exhibit either prominent mitotic activity or Ki-67 immunoreactivity, it is often misdiagnosed as neuroendocrine tumor (NET) G1. However, the prognosis might be better in patients with GP than in those with NET G1. Therefore, it is important to differentiate GP from NET G1. Moreover, our previous study indicated that GP accounts for a substantial, constant percentage of duodenal NETs. In the present article, we describe up-to-date data on the clinicopathological characteristics of GP and on the immunohistochemical findings that can help differentiate GP from NET G1, as largely revealed in our new and larger literature survey and recent multi-institutional retrospective study. Furthermore, we would like to refer to differential diagnosis and clinical management of this tumor and provide intriguing information about the risk factors for lymph node metastasis on GP.

Progress in the treatment of esophageal neuroendocrine carcinoma

“…[1][2][3] Some types of NENs have a genetic predisposition and may be associated with genetic mutations, such as multiple endocrine neoplasia type 1 and 2 (MEN-1 and MEN-2), familial paraganglioma syndrome, von Hippel-Lindau syndrome, and Carney-Stratakis syndrome. 4 NENs are divided into two categories according to the clinical symptoms and levels of hormone secretion: functional and nonfunctional. 5 Over the past 15 years, the incidence of NENs has increased each year, although this may be due to the availability of improved diagnostic techniques; it further demonstrates that the incidence continues to rise.…”

Gangliocytic Paraganglioma: a Diagnostic Pitfall of Rare Neuroendocrine Tumor

“…Thus, we would like to emphasize the usefulness of immunohistochemical examinations using progesterone receptor and pancreatic polypeptide to differentiate them. Namely, typical epithelioid cell (a major component of GP)shows positive immunoreactivity with them, whereas tumor cell of NET G1 was negative for both immunohistochemistries [4,5]. We believe that these findings may be useful to differentiate GP from NET G1, including small specimen obtained from biopsy.…”

“…In addition, GP often shows positive immunoreactivity for somatostatin [3,4]. To differentiate GP from paraganglioma, pathologists should consider the primary tumor site and the existence of ganglion-like cell (paraganglioma lacks ganglion-like cells, and it is very rare in the duodenum) [4]. On the other hand, morphological differences between GP and somatostatinoma make differentiation easy, and immunoreactivity for progesterone receptor and pancreatic polypeptide which is discussed below may be useful to differentiate them [5].…”

“…In fact, there are similar morphological and immunohistochemical features between paraganglioma and GP, except for the existence of ganglion-like cell in GP [3]. In addition, GP often shows positive immunoreactivity for somatostatin [3,4]. To differentiate GP from paraganglioma, pathologists should consider the primary tumor site and the existence of ganglion-like cell (paraganglioma lacks ganglion-like cells, and it is very rare in the duodenum) [4].…”

The role of insulin-like growth factor binding protein-related protein 1 (IGFBP-rP1) in endometrial carcinoma

“…Previous investigations showed that IGFBP-rP1 also plays an important role in the neuroendocrine differentiation for certain type of tumor [26]. It has been largely accepted that tumor with neuroendocrine differentiation is one of the most important prognostic factors [27][28][29]. In the view of the neuroendocrine differentiation, investigations of IGFBP-rP1 in EC have greater significance.…”