Small Intestinal Glomus Tumor as an Uncommon Cause of Gastrointestinal Hemorrhage: A Case Report and Review of the Literature

Yan Tan^1,2, Lan Liu^1,2, Guifang Yang³, Qiu Zhao^1,2* and Yafei Zhang^1,2*
*Correspondence: Qiu Zhao, Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan,P.R, China, Email: Yafei Zhang, Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan,P.R, China, Email:

Keywords

Gastrointestinal hemorrhage • Benign • Glomus tumor • Small intestine

Introduction

Glomus tumors (GTs) within the gastrointestinal tract have been reported to usually occur in the stomach, especially at the gastric antrum. The vast majority of gastric GTs follow a benign clinical course, without histologic or clinical evidence of malignancy [1,2]. GTs rarely involve in the small intestine, with only 8 cases reported in English literature, among which 4 cases present malignant features [3-10]. Here, we report the ninth case of small intestinal GT in a female adult. Additionally, we reviewed all the reported cases to summarize its clinicopathologic features, diagnosis and treatment.

Case Report

A 30-year-old female referred to our hospital with chief complains of melena and fatigue for 3 weeks. There were no significant positive signs other than anemic appearance (hemoglobin 6.7 g/dL). Routine endoscopy (gastroscopy and colonoscopy) and abdominal computed tomographic (CT) in fundamental hospital, revealed no significant findings. After admission, capsule endoscopy was immediately applied and indicated a possible mucosal eminence in the jejunum (Figure 1A). Oral balloon-assisted enteroscopy was subsequently applied because the images captured by capsule endoscopy were limited. A 2.0 × 2.0 cm tumor without epithelial lining was discovered after insertion through pylorus about 250 cm (Figure 1B). Then abdominal enhanced CT was performed to further confirm the nature of the tumor. As shown in Figure 2, a solid tumor was observed in the upper-middle abdomen, which displayed homogeneous enhancement in the arterial phase and continuous enhancement in the delayed phase. No apparent invasion to the adjacent organs and lymphatic metastasis were observed from the CT scan.

The patient was then transferred to the Department of Gastrointestinal Surgery and underwent a partial enterectomy. A 3.5 cm long segment of jejunum was removed by surgical operation. Gross pathology showed a 2.0 × 1.3 × 1.3 cm gray-red polypoid protuberance in the surface of small intestinal mucosa (Figure 3A). HE stain illustrated that the tumor was well limited by a thin fibrous capsule, interspersed with congestive capillaries of various sizes, and admixed with smooth muscle bundles (Figures 3B and 3C). Muscularis propria and serosa didn’t show any damage. Immunohistochemical study showed that the tumor cells were strongly positive for SMA and collagen type IV (Figures 3D and 3E), and negative for CD31, CD34, CD117, Desmin, DOG-1, CgA, Syn, S100, EMA, STAT6, β-catenin, Caldesmon and Calponin. Ki-67 proliferation index was less than 1% (Figure 3F) and mitotic activity was about 1/50 HPF. The lesion was finally diagnosed as benign GT.

After surgery, the hemoglobin of this female patient recovered to a normal level soon and no further melena was observed. Due to half of small intestinal GT presenting malignant features, abdominal and pelvic CT reexaminations were performed every 6 months. No malignant transformation or recurrence was detected during a follow-up period of 12 months.

Discussion

GTs arising from the small intestine are extremely rare. So far, only 8 cases have been described in English literatures [3-10]. Among them, two cases located at duodenum, one case at jejunum, and five cases at ileum. The clinicopathologic features of small intestinal GTs are summarized in Table 1. Similar to other small intestinal tumors, gastrointestinal hemorrhage is the most common symptom of small inestinal GTs. Also, half of the reported 8 cases presented abdominal pain. The mean age of onset was 55.1 ± 17.9 years (range 29-82 years). Together with the present case, no sex trend was revealed (5 males and 4 females).

Due to the deep location and non-specific symptoms, small intestinal tumors are usually difficult to differentiate and diagnose. Abdominal enhanced CT or CT enterography is usually used to evaluate small intestinal lesions on imaging diagnosis. In the present case, a mass with homogeneous enhancement in the arterial phase and continuous enhancement in the delayed phase was found in the upper middle abdomen, which is similar to previous descriptions in gastric GTs [11]. GTs in stomach usually display a submucosal pattern of growth. In the case reported by Knackstedt C, et al. [7], the GT located in the duodenal bulb was polyp-like [7]. Shelton JH, et al. [8] reported a GT of the ampulla, in which a protruding mass was seen [8]. Although 6 cases had been reported, there is still no enteroscopic image presented for GTs located in jejunum or ileum. In this study, for the first time, we acquired the endoscopic imaging of small intestinal GT by balloon-assisted enteroscopy. The most significant feature of the small intestinal GT observed in enteroscopy is that the tumor is covered by a thin fibrous capsule, without epithelial lining.

Most GTs depends on the 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 may 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 AL, et al. [13] 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 12 months follow-up showed no indication of recurrence or metastasis after a partial enterectomy, which supports the benign diagnosis.

All the small intestinal GTs recorded in the literature have received surgical resection, but no patients received regional lymphadenectomy. Also, no patients received adjuvant radiotherapy or chemotherapy after surgery. However, 2 patients developed metastases and one died soon after the metastasis [3,4]. Therefore, standardized systemic treatment needs to be further evaluated. For gastric GTs, endoscopic or laparoscopic resection is recommended. Also due to the fact of extremely rare cases of malignant GTs in the stomach, 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].

Conclusion

In conclusion, we have reported a case of small intestinal GT in a 30-yearold female. 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. Since a potential for malignancy, early diagnosis and treatment are important for a better prognosis.

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