The Turkish Journal of Gastroenterology
2007, Volume 18, No 3, Page(s) 188-191
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|Granular cell tumor of esophagus removed with
endoscopic submucosal dissection
|Sadettin HÜLAGÜ, Ömer ŞENTÜRK, Cem AYGÜN, Yeflim GÜRBÜZ, Orhan KOCAMAN,
Altay ÇELEBİ, Tolga KONDUK
|Department of Gastroenterology, Kocaeli University, Faculty of Medicine, Kocaeli
|Keywords: Esophagus, granular cell tumor, endoscopic
Granular cell tumors of the esophagus are rare neoplasms and
their diagnosis is mainly based on histopathologic examination
of endoscopic biopsies. With the development of endoscopy and
iodine staining, there has been a marked increase in local treatment
alternatives for early esophageal neoplasms. In this case
report, we describe a granular cell tumor of the esophagus in an
adult patient and briefly discuss the literature concerning clinicopathologic
aspects and management of these uncommon tumors.
In this patient, we describe the removal of the tumor by
endoscopic submucosal dissection technique.
Granular cell tumors (GCT), first described by Abrikossoff
in 1926, occur throughout the body, most
commonly in the tongue and skin, but also in the
breast, respiratory tract, biliary tree, nervous system
and gastrointestinal (GI) tract (1,2). Tumors
involving the GI tract account for 8% of all GCT
(1). Among the GCTs involving the GI tract, only
2% are found in the esophagus (2,3). The first case
of esophageal GCT was also described by Abrikossoff
in 1931 (4) and, since that time, over 200
cases have been reported in the literature (5). In
this case report, we aimed to describe a GCT of the
esophagus in an adult patient and its removal by
endoscopic submucosal dissection technique. We
also briefly discuss the literature concerning clinicopathologic
aspects and management of these uncommon
A 51-year-old Caucasian male had a 5-year history
of progressively worsening epigastric discomfort.
Additionally, he had a 6-month history of intermittent
dysphagia with epigastric pain radiating
to the retro-sternum. He was treated with proton
pump inhibitors; however, ongoing dysphagia and
pain led to his referral to the gastroenterology outpatient
An upper GI endoscopic examination was performed
which revealed a yellow submucosal tumor
with central depression, located 37 cm distal from
the incisor teeth (Figure 1). Microscopical examination
of biopsy specimens from the lesion demonstrated
a neoplasia consisting of nests and
cords of elongated and polygonal cells containing
small dark nuclei and abundant granular eosinophilic
cytoplasm. Overlying epithelium had pseudoepitheliomatous hyperplasia. Based on these histopathological
findings, the lesion was diagnosed
as a GCT (Figure 2). Computed tomography scan
of his chest and abdomen revealed no additional
pathology except the solid mass in the distal esophagus.
Esophageal endosonography revealed a 10
x 14 mm thick hypoechoic mass in the esophageal
wall. The mass was located in the submucosa, and
the muscularis propria was intact. There were no
periesophageal or celiac lymph nodes. The patient
subsequently underwent an endoscopic submucosal
dissection (ESD) using sodium hyaluronate, a
small-caliber-tip transparent hood and a needle
knife according to the method developed by Yamamoto
(6). ESD procedure was carried out by a single-
channel upper GI endoscope (Fujinon Co, Japan)
and a high frequency generator with an automatically
controlled system (Endocut mode; Erbotom,
ERBE Elektromedizin GmbH, Germany). A
transparent cap was fitted on the tip of the endoscope
to obtain a constant view and to create a tension
on the connective tissue for dissection. Lugol
chromoendoscopy was done to mark the margins
of lesion. With the use of an electrosurgical needle
knife, dots were placed about 5 mm outside of the
margins at 2 mm intervals. Following an initial injection
of 2 ml saline, a submucosal fluid cushion
was created using a solution prepared with hyaluronic
acid, saline, indigo carmine and epinephrine
mixture. About 2 ml of the solution was injected
into the submucosal layer at a time and repeated
until the mucosa was lifted. After lifting the lesion,
the mucosa was gently cut with the electrosurgical
needle knife, using the Endocut mode. En
bloc resection with tumor-free lateral/basal margins
was accomplished. To control minor bleedings,
hemostatic forceps were used in soft coagulation
mode. The patient was discharged after
one week without any complication. This method
enabled submucosal incision under direct visualization
and thus allowed precise determination of
both the lateral and vertical margins to be resected
(Figures 3, 4). Microscopically, the surgical
margin was free of tumor cells. Follow-up endoscopy
6 weeks after removal of tumor showed scar
tissue from prior mucosal dissection; no masses or
nodules were detected (Figure 5).
Granular cell tumors are uncommon tumors in the
GI tract and are very rare in the esophagus. Most
esophageal GCTs are found incidentally during
endoscopy, upper GI contrast studies, or at autopsy.
Although patients usually have no symptoms,
those with tumors larger than 1 cm may report dysphagia (5). Nausea, vomiting, and retrosternal
or epigastric pain occur less frequently
The endoscopic appearance of a GCT is characteristic.
The tumor usually appears as a yellow,
firm, polypoid submucosal mass. Differential diagnosis
should include esophageal cyst, epithelial lesions
such as glycogenic acanthosis, inflammatory
polyp, and squamous papilloma, and other submucosal
tumors, such as leiomyoma, lipoma, and hamartoma.
Many kinds of cells have been postulated as an
origin of the tumor including myoblasts, histiocytes,
perineural fibroblasts, Schwann cells, and undifferentiated
mesenchymal cells (8). Theories regarding
the non-neoplastic nature of the lesion
and its association with inflammatory as well as
degenerative processes have also been considered.
More strongly, the evidence of a close association
of tumor cells with peripheral nerves, and the presence
of S-100 protein, neuron-specific enolase,
and myelin proteins on immunohistochemical stains
support a Schwann cell derivation (9). Histologically,
these tumors consist of polygonal and fusiform
cells disposed in compact "nests" (10). Cells
have small dark nuclei and abundant, fine, granular
eosinophilic acid-Schiff–positive, diastase-resistant
cytoplasm (11). GCTs of the skin, larynx,
and esophagus are known to induce pseudoepitheliomatous
hyperplasia in the malpighian epithelium.
This feature may simulate a primary squamous
cell carcinoma (2, 12).
Although the natural history of the tumor is unclear,
most esophageal GCTs have a benign clinical
course. The infiltrative pattern of growth and the
presence of metastases are important features in
differentiating between malignant and benign tumors
because histologically they may appear very
similar. Malignant lesions are usually larger than
4 cm, display rapid recent growth, tend to recur locally
after resection, and may have such subtle
histologic features as nuclear pleomorphism, increased
nuclear size, tumor cell necrosis, large nucleoli,
mitotic figures (2 or more/10 HPF), and tumor
cell spindling (13).
Treatment of esophageal tumors has recently included
saline-assisted endoscopic mucosal resection
(EMR), which is considered a safe, successful
procedure, and complications are uncommon (14).
In a series of 650 esophageal mucosal cancers removed
with EMR, Makuuchi (15) reported a complication rate of 4.8% (perforation 0.7%, bleeding
3.1%, stricture 1.6%). ESD for esophageal pathology
has been recently established and the affected
mucosa is incised and removed using a variety of
endoscopic electrosurgical knives. Using ESD, a
wider range of the mucosa can be resected in one
piece more reliably using an endoscope. In our
case, after submucosal injection of sodium hyaluronate
to maintain sufficient thickening of the
submucosal tissue, dissection of the mucosa and
submucosa with a needle knife was performed by
the method developed by Yamamoto (6). We
preferred ESD for its more accurate resections
than conventional EMR and sodium hyaluronate
solution for it ability to maintain submucosal elevation
for a longer time. Additional esophagectomy
with lymph node dissection was not required
since no submucosal invasion or vessel permeation
was seen in the endoscopically resected specimen.
In summary, granular cell tumors of the esophagus
are rare neoplasms. Endoscopic biopsies are
the mainstay of diagnosis. Endoscopic and endosonographic
evaluation of the lesion defines the location
and extension of the tumor and its suitability
for endoscopic treatment. Endoscopic submucosal
dissection is a safe and accurate procedure for selected
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