A gangliocytic patially glandular paraganglioma with lymph node metastasis
A gangliocytic patially glandular paraganglioma with lymph node metastasis
Huijuan Shi 0
Ju Han 0
Ni Liu 0
Ziyin Ye 0
Zhixun Li 0
Zhi Li 0
Tingsheng Peng firstname.lastname@example.org 0
0 Department of Pathology, the First Affiliated Hospital of Sun Yat-sen University , 58, Zhongshan Road II, Guangzhou 510080 , P. R. China
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.
Gangliocytic paraganglioma; Glandular component; Lymph node metastasis; Duodenum
Letter to the editor
Gangliocytic paraganglioma (GP) is an infrequent
neuroendocrine tumor, usually being found in the second
portion of the duodenum. The histological diagnosis requires
the identification of three elements as epithelioid cells,
spindle-shaped cells and ganglion-like cells [
tumor cells arrange in solid and trabecular pattern,
mainly comprising spindle cells, mixed with nests of
epithelioid cells and large cells with gangliocytic
differentiation. Few cases were reported to contain distinct
epithelial component forming glandular structure.
Generally, this tumor is regarded as a benign tumor, but a
few cases with lymph node metastasis have been
reported before (Table 1). In addition to the rarity of the
tumor, the present case suggests the malignant potency
of this tumor. Herein we reported a rare case of
gangliocytic paraganglioma with lymph node metastasis, comprising
distinct glandular component in primary tumor and the
metastatic lymph nodes.
The patient present here, a 47-year-old man with an
unremarkable previous medical history, had a 4-month
history of left lower quadrant abdominal pain before
admission. He had experienced weight loss of approximately
5 kg during the previous 4 months and had been
previously treated with H2 blockers and proton pump
inhibitors without significant relief. CT scan of the abdomen
showed a neoplasm with 6.6 × 4.0 × 3.5 cm at the papilla
of Vater in duodenum (Figure 1a). Peripancreatic lymph
nodes swelled to the largest diameter as 3.0 cm. No
dilatation of the biliary or pancreatic duct was observed. A
gastrointestinal endoscopy detected a 4.0 × 2.5 cm,
polypoid, ulcerated ampullary tumor in duodenum. Endoscopic
ultrasonography suggested that the tumor involved in the
whole duodenal mucous layer and the lymph node around
enlarged. Pancreatcoduodenectomy accompanied by
peripancreatic lymph node dissection were performed.
Intraoperative biopsy of the enlarged lymph nodes showed
regional atypical glandular component in the lymph nodes,
leading to the misimpression as a metastatic
neuroendocrine carcinoma firstly.
Gross examination revealed the surgical specimen
comprising a portion of duodenum with ampulla, the
gallbladder, and head of the pancreas. A 4.0 × 4.0 ×
2.3 cm polypoid tumor was found at the papilla of Vater
(Figure 1b). A total of twenty lymph nodes were also
removed respectively, including 7 peripancreatic lymph
nodes with greatest diameter as 3.0 cm, 5 suprapyloric
and 8 subpyloric lymph nodes. Microscopically, the
tumor were localized in submucosal layer, invading a
part of the muscularis propria (Figure 2a), being mainly
consisted of spindle cells, with some nests of epithelial
cells (Figure 2b-c), and scattered big ganglion-like cells
(Figure 2d). The spindle cells formed slender fascicles,
with elongated and plump nucleus, and attenuated
eosinophilic cytoplasm. The epithelial cells arranged in the nests
and trabeculae, with round to oval-shaped nucleus and
pale eosinophilic cytoplasm. The ganglion-like cells were
rarely seen, with round nucleus, large conspicuous
nucleolus, and abundant eosinophilic cytoplasm (Figure 2d).
Specially, small amounts of distinct atypical glandular
components were presented (Figure 3a-b). The tumor
also invaded a portion of pancreatic tissue. Eight of the
twelve lymph nodes were involved in metastatic tumor
(Figure 4a-b). The atypical metastatic glandular
components in the lymph nodes had caused the misimpression
as metastatic neuroendocrine carcinoma on the initial
frozen slides. Immunohistochemically, the neoplastic
epithelial cells were positive for the epithelial and
neuroendocrine marker as CK, Neuron-specific enolase
(NSE), Chromogranin A (CgA), Synaptophysin (Syn),
CD56. The spindle tumor cells arrouding the epithelial
nests were positive for S-100, partly for NSE, CgA, Syn,
CD56 and CD34, but negative for CK. The ganglion
cells were characteristic positive for S-100. Ki-67
labeling index estimated less than 1%. CD117, Actin and
Desmin were negative in all of the three components
(Figure 5a-h). Based on all these clinicopathological
features, we finally made a diagnosis of gangliocytic
paraganglioma with glandular component and lymph nodes
metastasis. To date, approximately two years routine
follow-up after the surgery is established, and the
patient remains well and no recurrence has been recognized.
Because of the metastasis, the malignant potential of this
tumor could not be excluded. A long time following up is
needed to know exactly the prognosis.
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. [
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 [
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
] (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
] 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.
The authors declare that they have no competing interests.
HS drafted the manuscript and performed the literature review. JH conducted
the pathological examination and literature review. NL conducted the
immunohistochemical staning. ZY participated in the final diagnosis. ZXL carried
out the pathological examination. ZL participated in the immunohistochemical
analysis. TP gave and reviewed the final histopathological diagnosis, and revised
and gave final approval of the version to be published. The final manuscript
was read and approved by all authors.
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