A gangliocytic patially glandular paraganglioma with lymph node metastasis
© Shi et al.; licensee BioMed Central Ltd. 2014
Received: 8 January 2014
Accepted: 4 March 2014
Published: 20 March 2014
Gangliocytic paraganglioma (GP) is an infrequent neuroendocrine tumor usually with three elements as epithelioid cells, spindle-shaped cells and ganglion-like cells, which is generally regarded as a benign tumor. Only a few cases with lymph node metastasis have been reported. Herein, we reported a 47-year-old man of GP with distinct glandular component embedded in the spindle tumor cells in the primary tumor and the metastatic lymph nodes. The immunohistochemical profile was helpful to give the final diagnosis as gangliocytic paraganglioma. Here, we added one more GP case with regional lymph nodes metastasis. And particularly, there were small amount of distinct glandular component both in the primary tumor and the metastatic lymph nodes, which indicated that adenocarcinoma might coexist with GP. And GP should also be distinguished from carcinoid tumor, paraganglioma, ganglioneuroma, or GIST.
KeywordsGangliocytic paraganglioma Glandular component Lymph node metastasis Duodenum
Letter to the editor
Gangliocytic paraganglioma cases with lymph node metastasis
Chief clinical presentation
Büchler et al.
Inai et al.
Hashimoto et al.
Dookhan et al.
Sundararajan et al.
Bucher et al.
Wong et al.
Witkiewicz et al.
Mann et al.
Abdominal pain, vomiting, weight loss
Okubo et al.
Epigastralgia, tarry stool
Saito et al.
Gastrointestinal bleeding, anemia
Uchida et al.
Ogata et al.
Gastrointestinal bleeding, anemia
Barret et al.
GP is an infrequent neuroendocrine tumor usually appearing in the second part region of duodenum. The most common clinical manifestation is gastrointestinal bleeding (45.1%) due to mucosal erosion or ulceration, followed by abdominal pain (42.8%) and anemia (14.5%). GP has been known well after it was firstly reported by Dahl et al. and named entity by Kepes et al.[1, 2]. Confirmation of three identical components comprising epithelial cells, spindle cells, and ganglion cells was essential for the diagnosis. GP should be distinguished from carcinoid tumor, ganglioneuroma, pigmented paraganglioma, and spindle cell tumors as GIST[4–6]. Immunohistochemical examination was also an important diagnostic clue to identify the three cellular components of GP.
In this case the epithelial component in the metastatic lymph nodes led to the thought as metastatic neuroendocrine carcinoma in frozen slides. With more than ten tissue blocks section, three components as spindle cells, epithelial cells, and ganglion cells through light on the diagnosis as gangliocytic paraganglioma. Immunohistochemical staining had confirmed the diagnosis. In this case, MIB-1 was estimated as less than 1%, suggesting the low proliferative rate of this tumor, which might not reflect the prognostic value in GP.
Although GP is generally considered as a benign periampullary lesion, however, it is very unwise to assume that this tumor must be a benign entity. Metastasis to regional lymph nodes by this tumor and/or local recurrence has been reported several times in the literature[7–20] (Table 1). Here, we added a GP case with lymph node metastasis to that list. Although there is still no distinct evidence that the lymph node metastasis indicating malignant prognosis, lymphovascular invasion may be a major factor in the malignant potential of GP. In hence, it was important to image the examination to investigate the possibility of lymph node metastasis before an operation.
Anders had reported a GP case with an advanced duodenal adenocarcinoma coexisted. In our case we also found small amount of distinct glandular components besides three typical tumor cells of GP. Hence, it could not be excluded for the potential that adenocarcinoma coexist with GP at the same location. Although the patient remains well and no recurrence after nearly two years routine follow-up, a long time follow-up is needed to know whether there is a malignant capacity of this case.
Herein we presented a rarely gangliocytic patially glandular paraganglioma with lymph node metastasis. In addition to the rarity of the tumor, we wish to emphasize the pleomorphic morphologic features mimicking adenocarcinoma and the malignant potency of gangliocytic paraganglioma with lymph nodes metastasis.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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