Abstract
Gastroenteropancreatic neuroendocrine tumors are the most incident neuroendocrine tumors. In the new WHO classification (2010) the embryological derivation of each neoplastic entity is one of the most important parameters. Gangliocytic Paraganglioma is a tumor originating in the hindgut, a rare neoplasm, generally affecting the second portion of the duodenum, the majority of which are benign.Cases of gangliocytic paraganglioma with local metastasis or local recurrence have also been reported.We describe a GP in a 48-year-old caucasian male with an unusual site (4th portion of duodenum) and an interesting immunohistochemical and molecular pattern. In particular, we examined the expression of some neuroendocrine markers and a marker of neuronal differentiation, NeuroD1, whose expression can help to better understand the nature of this neoplasia.Virtual slidesThe virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3720959161096807
Highlights
Recent studies have highlighted the presence of paraganglioma in other sites, in the retroperitoneum [2], with a case that metastasizes to the vertebra [3]
In this report we present a case of Gangliocytic paraganglioma on which some neuroendocrine markers (Syn, CgA, CD56, NSE) and NeuroD1 expression have been tested by IHC
The majority of the reported cases of duodenal Gangliocytic paraganglioma (GP) has been of benign nature, even if some cases in literature showed the presence of regional lymph nodes metastasis [5]
Summary
Gangliocytic paraganglioma (GP) is a rare gastrointestinal tumor of uncertain histogenesis, with immunohistochemical features of neuroendocrine type. It is composed of three cellular types: epithelioid, ganglion and spindle cells, whose identification is supported by immunohistochemical (IHC) detection of neuronal and neuroendocrine markers (synaptophysin, chromogranin A, CD56, S-100, NSE and Somatostatin) [6]. In this report we present a case of Gangliocytic paraganglioma on which some neuroendocrine markers (Syn, CgA, CD56, NSE) and NeuroD1 expression have been tested by IHC. Case presentation GP shows evidence of neuronal differentiation, supported by cell positivity for several neuroendocrine (ND) markers. In this case, a 48-year-old caucasian man was referred to our Institution after recurrent upper GI bleeding with melena. QRT PCR analysis showed NeuroD1 over-expression compared to other cancer samples used as controls (Figure 4)
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