3 DISCUSSION
GTs
arising from the small intestine are extremely rare. So far, only 8
cases have been described in the English
literatures.3-5 Among them, two cases located at the
duodenum, one case at jejunum, and five cases at the ileum. The
clinicopathologic features of small intestinal GTs are summarized in
Table 1. The case reported in our study is a 30-year-old female.
Together with this case, the sex distribution is basically balanced
(5 males and 4 females). Small intestinal GTs usually
present non-specific symptoms such as abdominal pain and intestinal
bleeding (melena or hematochezia). Larger tumors can cause intestinal
stenosis and obstruction.
Due
to the deep location and non-specific symptoms, small intestinal tumors
including GTs are usually hard to differentiate and diagnose.
Abdominal contrast-enhanced CT may be useful to distinguish GTs from
other small intestinal tumors. According to previous description (mainly
on gastric GTs), hemangioma-like globular enhancement with central
fill-in and persistent homogeneous enhancement were both visualized in
GTs.11 As for the reported case, the tumor on
contrast-enhanced CT showed homogeneous high attenuation in the
arterial, venous, and delayed phases. GTs in gastric usually display
a submucosal pattern of growth. In the case reported by Knackstedt C et
al., the GT located in duodenal bulb was polyp-like.7Shelton JH et al. reported a GT of the ampulla, in which a protruding
mass was seen.8 However, until now, there is no
enteroscopic image presented in the jejunum or ileum GTs. In this paper,
for the first time, we acquired the endoscopic imaging of small
intestinal GT by balloon-assisted enteroscopy. Intriguingly,
the enteroscopic imaging showed a fibrous capsule-covered mass, without
any epithelial coverage, and on the surface of the tumor, no definite
hemorrhage, erosions or ulcers were observed. Only some minor erosions
on the area around the tumor edge were seen, which may cause the
persistent melena.
Most
GTs depend on postoperative pathological diagnosis. Under the
microscope, the tumor consists of a large number of smooth muscle
bundles and dilated capillaries. Immunohistochemically, tumor cells were
stained positive for SMA and collagen type Ⅳ. Negative CD117 and DOG-1
expressions
help
in excluding the diagnosis of gastrointestinal stromal tumors, while
negative CgA and Syn
expressions
help in excluding neuroendocrine neoplasms.12 Criteria
for malignant GTs proposed by Folpe et al. include tumors with a deep
location and a size of more than 2 cm, or atypical mitotic figures, or
moderate to high nuclear grade and ≥5 mitotic figures/50
HPF.13 According to the criteria, the present case was
considered as low
possibility
of malignancy. And 6 months follow-up showed
no
indication of recurrence or metastasis after a partial enterectomy,
which supporting the benign diagnosis.
All
the small intestinal GTs recorded in the literature have received
surgical resection, but no patients received regional lymphadenectomy.
After surgery, also no patients received adjuvant radiotherapy or
chemotherapy. However, 2 patients developed metastases in the 8 reported
cases. Therefore, standardized systemic treatment needs to be further
studied. For gastric GTs, endoscopic or laparoscopic resection is
recommended. Also due to the fact of
extremely
rare cases of
malignant
GTs in gastric, no standardized neoadjuvant or adjuvant treatment was
suggested. Radiotherapy or chemotherapy has been utilized for the
treatment of malignant GTs of head and neck. However, in the reported
cases, tumor progression was not reliably altered.14
In
conclusion, we have reported a case of GT of small intestine in a
30-year-old female patient. The final diagnosis is made by histological
and immunohistochemical examination.
Due to the exceedingly rare occurrence, there is no standardized
management pathway for these patients. As there is a potential for
malignancy, early diagnosis and treatment are important for a good
prognosis.