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2010, Volume 26, Number 1, Page(s) 074-077
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DOI: 10.5146/tjpath.2010.00999 |
Glomus Tumor of the Stomach: Case Report |
Olcay AK NALBANT1, Peyker TEMİZ2, Safiye VURAL1, M. CELALETTİN KELEŞ3 |
1Departments of Pathology, M.H. Manisa State Hospital, MANİSA, TURKEY
2Celal Bayar University, Faculty of Medicine, MANİSA, TURKEY
3Celal Bayar University, General Surgery, M.H. Manisa State Hospital, MANİSA, TURKEY |
Keywords: Glomus tumor, Stomach, Immunohistochemistry, Differential diagnosis |
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Glomus tumors are benign, solitary neoplasms originating from
modified smooth muscle cells of the glomus body. These tumors are
rare in the gastrointestinal system. We present a glomus tumor that
arose in the antrum of a 43-year-old female. During laparoscopic
cholecystectomy for cholelithiasis, an antral submucosal tumor
was found incidentally and total excision with wedge resection was
performed. Histopathological and immunohistochemical features of
this tumor were similar to those of a glomus tumor in any localization.
In this report, we discuss the differential diagnosis of gastric glomus
tumor. |
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Glomus tumors originate from modified smooth muscle
cells of the glomus body, a neuroarterial receptor that
regulates blood flow within arteries 1. Glomus tumors
are most commonly found in the skin and subcutaneous
tissue, but can also occur in the viscera 2-4. In the gut,
they are most commonly found in the stomach 2 Gastric
glomus tumors represent a rare entity 5,6 and a majority
of gastric glomus tumors tend to occur in the antrum 5,7.
These tumors are generally benign, but rare cases resulting
in metastasis and death have been reported 2,7. |
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Abstract
Introduction
Case Presentation
Disscussion
References
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A 43-year-old female presented with episodes of right
upper quadrant pain. During laparoscopic cholecystectomy
for cholelithiasis, an antral submucosal tumor was found
incidentally and total excision with wedge resection was
performed. The tumor was 2 cm in greatest diameter and
the cut surface of the tumor was grey-pink and solid with a
nodular appearance. The mucosa overlying the tumor was intact. Histologically, the tumor was well-circumscribed
and highly vascular, and tumor nests were separated by
fascicles of smooth muscle (Figure 1A,B). The tumor
cells were uniform, small, round to oval central nuclei
containing stippled chromatin (Figure 1C). Nucleoli were
not prominent. The cytoplasms were eosinophilic or clear.
One mitosis was found in 50 HPF (high-power fields).
Immunohistochemically, the tumor cells stained diffusely
positive for smooth muscle actin (SMA)(Neomarkers,
CA, USA) (Figure 2A) and vimentin (Neomarkers, CA,
USA) (Figure 2B), focal positive for CD34 (Neomarkers,
CA, USA) (Figure 2C), and focal and weak positive for
synaptophysin (Dako, Denmark) (Figure 2D). All other
markers, including cytokeratins (AE1/AE3, Neomarkers,
CA, USA), CD117 (Dako, Denmark), chromogranin A
(Neomarkers, CA, USA), S-100 (Dako, Denmark), and
desmin (Dako, Denmark) were negative.
Click Here to Zoom |
Figure 1: Histological features of gastric glomus tumor. (A) The
antral mucosa overlying the tumor on the left (thin arrows) and
tumor nests separated by smooth muscle fascicle on the right
(thick arrow) (H&E, x40); (B) Tumor nests, vascular component
and smooth muscle (thick arrow) (H&E, x200); and (C) Uniform
tumor cells, with small, round to oval nucleus and eosinophilic or
clear cytoplasms (H&E, x400). |
Click Here to Zoom |
Figure 2: Immunohistochemical features of gastric glomus tumor. (A) Diffuse SMA positivity (x100); (B) diffuse vimentin positivity
(x100); (C) focal CD34 positivity (x100); and (D) focal and weak synaptophysin positivity (x200). |
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Abstract
Introduction
Case Presentation
Disscussion
References
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Glomus tumor is derived from the specialized cells of the
glomus apparatus 2. Vascular tumors of the gastrointestinal tract are extremely rare accounting for <2% of benign
tumors, but the largest portion of such tumors are glomus
tumors 1. Gastric glomus tumors represent a rare disease,
and they are much less common than gastrointestinal
stromal tumors (GIST ) 5. Gastric glomus tumors often
cause gastrointestinal bleeding, which can be acute and
even life-threatening, or chronic with anemia; ulcer-like
symptoms are also common 5,7. Our patient had no
significant medical problems associated with the gastric
lesion. The tumor was found incidentally when the patient
underwent laparoscopic cholecystectomy.
In the gut, glomus tumors are most commonly found in the
stomach2, and a great majority of gastric glomus tumors
occurs in the antrum5,7, as seen in our patient. Miettinen
et al.7 found a significant female predominance in their
study and our patient was also female.
Although massive6 and multiple8 gastric glomus
tumors have been reported, glomus tumors are usually 2 to
3 cm small,1,7, and solitary9 lesions. The tumor in the
current case was 2 cm in greatest diameter.
Glomus tumors are positive for SMA2,5,7-14 and vimentin2,7,8,10-14 and negative for keratin1,2,5,7,9,11,12,14,
desmin2,7-12,14, S-1001,2,5,7,9,12,14, chromogranin
A1,7,8,10-12, and CD1172,5,7,11-13. In this case,
tumor cells were diffusely positive for SMA and vimentin,
but negative for keratin, desmin, S-100, chromogranin A,
and CD117.
Immunostains with antibodies to synaptophysin1,11,12
and CD345,9,11 are usually negative; however, focal
synaptophysin7 and/or CD347,13 positivity have been
reported as seen in our tumor.
Gastric glomus tumors must be differentiated from
epithelioid GIST , carcinoid tumor, paraganglioma and
epithelioid leiomyoma.
Dilated veins or capillaries seen in epithelioid GIST s are
not similarly prominent or typically seen in most glomus
tumors. Epithelioid GIST s are positive for CD117 and very
often (70%) for CD34 and are more commonly negative
than positive for SMA7. This tumor was CD117 negative,
focal CD34 positive and diffuse SMA positive.
Histologically, carcinoid tumor is composed of uniform
round cells, similar to glomus tumor. Carcinoid cells are
positive for keratins, chromogranin A and synaptophysin7. In the presented case, the former two antigens were
never expressed and the latter was rarely expressed by the
glomus tumor cells.
Some variants of paraganglioma can closely mimic solid
variant of the glomus tumor. However, paragangliomas are
strongly positive for chromogranin A and synaptophysin.
Paragangliomas also typically have S-100 protein positive
sustentacular cells that are absent in glomus tumor7. In
this case, chromogranin A and S-100 were negative and
synaptophysin was focal positive.
Some authors suggest that epithelioid leiomyoma and
glomus tumor are indistinguishable. In any event, a smooth
muscle tumor is usually positive for desmin, whereas it is
often negative in glomus tumor11, similar to our case.
Most gastric glomus tumors are benign2,4,5,7, but
rare cases have resulted in metastasis and death2.
Occasionally, glomus tumors display unusual features such
as large size, deep location, infiltrative growth, mitotic
activity, nuclear pleomorphism and necrosis13. Folpe et al.13 published a proposal for classifying glomus tumor.
Malignant glomus tumor is applied to tumors with a deep
localization and a size >2 cm in diameter, atypical mitotic
figures or a moderate to high nuclear grade, and >5 mitoses
per 50 HPF. Symplastic glomus tumor indicates tumors with
high nuclear grade in the absence of any other malignant
features. Glomus tumor of uncertain malignant potential
is applied to those that lack criteria for malignant glomus
tumor or symplastic glomus tumor but have high mitotic
activity and one of the following: superficial location, large
size or deep localization. Glomangiomatosis refers to tumors
with histological features of diffuse angiomatosis and excess
glomus cells.
The gastric site was classified as a deep site as one factor
denoting potential malignancy13. However, gastric
glomus tumors should not be equated with tumors situated in deep peripheral soft tissues, and they should be considered
a separate site-related category7.
The tumor in our case was 2 cm in greatest diameter without
atypia and with low mitotic activity (1 mitotic figure/50
HPF). The patient is well with no recurrence over a 2.5 year
follow-up since surgery. Nevertheless, long-term follow-up
of this patient is necessary.
Approximately 130 cases of gastric glomus tumor have been
reported in the literature5. This case is valuable due to its
rarity. Determining of differential diagnosis and prognosis
of these tumors in detail will shed further light on this
entity. |
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Abstract
Introduction
Case Presentation
Discussion
References
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Abstract
Introduction
Case Presentation
Discussion
References
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