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Review
. 2013 Dec 15;7(1):432-7.
eCollection 2014.

Pulmonary gangliocytic paraganglioma: a case report and review of the literature

Dongliang Lin  1 , Yanjiao Hu  1 , Xiaoming Xing  1 , Li Ding  2 , Hui Liu  1 , Yujun Li  1 , Fenggang Xiang  1
Affiliations
Review

Pulmonary gangliocytic paraganglioma: a case report and review of the literature

Dongliang Lin et al. Int J Clin Exp Pathol. .

Abstract

Gangliocytic paraganglioma (GP) is a rare histologic type of neuroendocrine tumors. We report a case of pulmonary GP in a 29-year-old male presenting with an asymptomatic endobronchial nodule. Grossly, the tumor showed a 4.0x3.8x3.5 cm well-defined nodule with yellowish cut surface. Microscopically, the tumor was composed of three distinct cellular types: epithelioid cells, ganglion-like cells and spindle cells. Meanwhile, transitional cells, having morphologic features between ganglion-like and epithelioid cells, were also presented. The epithelioid cells arranged in various morphologic architectures, including Zellballen, papillary, cystic and microcystic pattern. The epithelioid cells were positive for AE1/AE3, CAM 5.2, chromogranin A and synaptophysin. Ganglion-like cells showed immunoreactivity for chromogranin A and synaptophysin. A few ganglion-like cells were also positive for AE1/AE3 and/or CAM 5.2. The spindle cells were positive for S-100 protein and neurofilament. The transitional cells showed a similar immunohistochemical profile to the epithelioid cells. The authors believe stem cell theory is a reasonable explanation for the origin of GP. GP probably originate from some kind of mucosa associated stem cell which can differentiate into diverse cellular lineages.

Keywords: Gangliocytic paraganglioma; bronchial; histogenesis; histomorphology; immunohistochemistry.

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Figures

Figure 1
Figure 1
Chest CT scan showing an endobronchial mass almost completely obstructing the bronchial lumen of right lower lobe.
Figure 2
Figure 2
Histopathological features. The epithelioid cells arranging in various histological structures, including Zellballen (A), papillary (B), cystic (C), and microcystic pattern (D). The transitional cells, larger than epithelioid cells but smaller than ganglion-like cells, having morphologic features between ganglion-like and epithelioid cells (E).
Figure 3
Figure 3
Immunohistochemical staining of GP. The epithelioid cells were positive for AE1/AE3 (A), chromogranin A (B) and synaptophysin (C). A few CAM 5.2 positive ganglion-like cells were also identified (D). The spindle cells were positive for S-100 protein (E).
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