Gastric glomus tumor: A rare etiology of upper gastrointestinal bleed

Manoj A Vyawahare; Bharati N Musthyala; Ramakant T Tayade

doi:10.4103/IJPM.IJPM_470_20

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CASE REPORT

Year : 2021 | Volume : 64 | Issue : 4 | Page : 795-798

Gastric glomus tumor: A rare etiology of upper gastrointestinal bleed

Manoj A Vyawahare¹, Bharati N Musthyala², Ramakant T Tayade³
¹ Department of Medical Gastroenterology, American Oncology Institute @ Nangia Specialty Hospital, MIDC Hingna, Nagpur, Maharashtra, India
² AmPath Central Reference Laboratory, Nallagandla, Serilingampally, Hyderabad, Telangana, India
³ Department of Surgical Oncology, American Oncology Institute @ Nangia Specialty Hospital, MIDC Hingna, Nagpur, Maharashtra, India

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Date of Submission	01-May-2020
Date of Decision	03-Jun-2020
Date of Acceptance	28-Oct-2020
Date of Web Publication	20-Oct-2021

Abstract

The glomus tumor of stomach is an unusual submucosal mesenchymal tumor of the gastrointestinal tract. We describe a 42-year-old female who presented with chronic anemia and an episode of painless hematemesis. A preoperative diagnosis of the probable gastric gastrointestinal stromal tumor was made. Post-surgical histopathological examination of the specimen demonstrated a glomus tumor of the stomach confirmed on immunohistochemistry. The present case highlights the importance of morphology and immunohistochemistry in differentiating the subepithelial tumors of the stomach and one must consider glomus tumor in differential diagnosis of these gastric lesions.

Keywords: Gastric glomus tumor, gastric subepithelial lesion, glomus tumor

How to cite this article:
Vyawahare MA, Musthyala BN, Tayade RT. Gastric glomus tumor: A rare etiology of upper gastrointestinal bleed. Indian J Pathol Microbiol 2021;64:795-8

How to cite this URL:
Vyawahare MA, Musthyala BN, Tayade RT. Gastric glomus tumor: A rare etiology of upper gastrointestinal bleed. Indian J Pathol Microbiol [serial online] 2021 [cited 2023 May 29];64:795-8. Available from: https://www.ijpmonline.org/text.asp?2021/64/4/795/328560

Introduction

Glomus tumor is an uncommon mesenchymal tumor that arises from the glomus apparatus, which is an arteriovenous shunt concerned in body temperature regulation. This tumor is seen most typically within the fingers and also the extremities but seldom seen in internal organs. Gastric glomus tumors (GGT) are extremely rare with an estimated frequency of approximately 1% of gastrointestinal soft tissue tumors.^[1] GGT are typically seen in submucosa and/or muscularis propria and frequently located in the antrum. The preoperative diagnosis of GGT is challenging due to a lack of characteristic clinical symptoms, endoscopic and imaging findings. Hence, the ultimate diagnosis of GGT typically rests on histopathological and immunohistochemistry (IHC) analysis of the resected specimen. Surgical resection with negative margins is the treatment of choice for GGT. These tumors are generally benign, however, seldom might show malignant behavior. In this report, we present a case of GGT in a 42-year-old female who was treated with exploratory laparotomy and wedge resection of tumor.

Case Presentation

A 42–year-old female presented to us with chief complaints of an episode of painless hematemesis – approximately 200 ml fresh to altered blood along with history of melena. She revealed the history of dyspnoea on exertion and generalized weakness since 3 months prior to presentation with hematemesis. She was evaluated at local medical center and found to have severe anemia. She had received three units packed red cell transfusion over that period.

After resuscitation, she underwent upper gastrointestinal endoscopy that revealed a subepithelial lesion with central ulceration and adherent clot in antrum of stomach along the greater curvature [Figure 1]a. Biopsies were not obtained in view of stigmata of recent hemorrhage. She underwent CECT abdomen for further evaluation and that showed contrast-enhanced focal well-defined 2.5 cm × 2.5 cm mass lesion in gastric antrum. The lesion was seen to extend through the whole thickness of the wall with extramural involvement suggesting possibility of gastrointestinal stromal tumor (GIST) [Figure 1]b. In view of bleeding subepithelial gastric lesion, likely GIST, she underwent exploratory laparotomy with wedge resection of gastric tumor. The postoperative recovery was uneventful. On pathological examination, there was a 2.5 cm × 3 cm submucosal fleshy grey white tumor with central areas of hemorrhage [Figure 2]a. Microscopy showed ulcerated tumor comprising nested/sheet-like perivascular arrangement of round mostly monomorphic neoplastic cells with sharply punched out nuclei and moderate amount of eosinophilic cytoplasm. Occasional mitosis was noted [Figure 2]b and [Figure 2]c. The tumor was seen to infiltrate all the layers of the stomach with extension beyond muscle layer. Six small nodes identified showed reactive changes only. Morphology was suggestive of solid variant of glomus tumor.

Figure 1: (a) Endoscopic view showing subepithelial lesion with central ulceration with adherent clot in Gastric Antrum. (b) CECT abdomen showing contrast enhanced 2.5 cm × 2.5 cm mass lesion in gastric antrum (arrow)

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Figure 2: (a) Macroscopy showing ulcerated subepithelial tumor. (b and c) Histopathology showing subepithelial nested tumor, with perivascular arrangement of tumor cells with eosinophilic cytoplasm and punched out round nuclei. (d): Pericellular net-like positivity on reticulin stain

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Differential diagnosis included the more commonly reported tumors such as GIST, paraganglioma and neuroendocrine tumors. Pericellular net-like positivity noted with reticulin stain [Figure 2]d. IHC showed positivity for α-Smooth Muscle Actin (αSMA), h- caldesmon and synaptophysin. Ki 67 index was 5%-8%. Tumor cells were negative for CD117, chromogranin, S100 and PCK [Figure 3]. Diagnosis of solid variant of glomus tumor was confirmed after exhaustive panel of IHC studies. She was advised close follow-up due to high-risk factors for malignancy like larger tumor size, deep invasion and high mitotic index.

Figure 3: Immunohistochemistry showing positive staining of a SMA, Synaptophysin, Caldesmon, Ki67 and negative staining of CD117, S100, Chromogranin and Pan CK

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Discussion

The glomus apparatus/bodies are arteriovenous shunts composed of three vascular components: (1) an afferent artery separated from (2) an efferent venous system by (3) a branched and convoluted channel. The narrow convoluted channel consists of a single layer of endothelium encased in multiple layers of epithelioid glomus cells. A sheath of delicate connective tissue with many nerve fibers surrounds the area of anastomosis.^[2] The glomus apparatus concerned with body thermal regulation function is found at the stratum reticularis of the dermis, mainly within the subungual region, lateral areas of digit and palm of the hands, and on the feet ventral surface.^[3]

The glomus tumor of the stomach is an extremely rare submucosal mesenchymal tumor of the gastrointestinal tract where antrum is the most common location, particularly in submucosa and/or muscularis propria. There have been very few cases reported after the first case report of GGT by Key et al. in 1951.^[4] The evaluation of a large series of 32 cases of gastrointestinal glomus tumors (31 cases – Gastric; 1 case – Ileal localization) by Miettinen et al. revealed that GGT are approximately 1% of all gastrointestinal soft tissue tumors and they occur predominantly in female (female:male = 3:1) and usually occur in fifth or sixth decade of life.^[1] On the contrary, Lin J et al. noted equal sex distribution with mean age of 49.3 years in recently published series of 21 cases of GGT.^[5]

GGT may present with dyspepsia, epigastric pain, nausea, vomiting, hematemesis or melena. The overt or occult gastrointestinal bleed is because of ulceration of overlying mucosa of the subepithelial GGT. Few cases may be asymptomatic and GGT may be incidental findings on upper gastrointestinal endoscopy. In our case, middle-aged lady presented with anemia of 3 months duration and an episode of overt upper gastrointestinal bleed.

The lack of characteristic clinical symptoms, endoscopic and imaging findings of GGT make preoperative diagnosis difficult. Upper gastrointestinal endoscopy usually reveals subepithelial lesion, mostly in antrum and occasionally with ulceration of overlying mucosa. Endoscopic biopsies may fail to provide representative samples due to subepithelial nature of lesion. Endoscopic ultrasound (EUS) is another imaging modality that reveals heterogenous, hypoechoic mass, usually originating from third or fourth layer of gastric wall. EUS-guided biopsy may help to obtain preoperative diagnosis. On CECT abdomen, these tumors manifest as well-circumscribed submucosal masses showing strong enhancement on arterial phase and persistent enhancement on portal venous phase, which reflects their hypervascular nature. However, endoscopic and imaging techniques fail to differentiate glomus tumors from other stromal or mesenchymal lesions. Hence, the final diagnosis of GGT is based on postoperative histopathological and IHC studies. Laparoscopic or laparotomy wedge resection with negative margins should be the treatment of choice of GGT. Endoscopy in our case showed gastric subepithelial lesion in antrum with overlying mucosal ulceration and adherent clot that was suggestive of recent hemorrhage. We could not biopsy it due to risk of active hemorrhage with likelihood of dislodging adherent clot. After CECT abdomen showing gastric hypervascular mass suggesting GIST, she underwent wedge resection of tumor.

Microscopic examination of H- and E-stained slides demonstrated classical histological features of glomus tumor similar to those described in soft tissue location. Tsuneyoshi M and Enjoji M divided glomus tumor into three subtypes by light microscopy examination according to the relative proportions of glomus cells, blood vessels and smooth muscle components: (1) Solid glomus tumor (75%): composed of nests of glomus cells surrounding capillary-sized vessels. (2) Glomangioma [20%]: composed of cavernous hemangioma-like vascular structures surrounded by small clusters of glomus cells. (3) Glomangiomyoma [5%]: same as that of a solid tumor or hemangioma, but with prominent vascular and smooth muscle components.^[6] Pericellular net-like positivity with reticulin stain has been described in these tumors.

GGT must be differentiated from more commonly reported tumors such as GIST, neuroendocrine tumor or paraganglioma and IHC plays an important role in differentiating these tumors [Table 1].

Table 1: Differential diagnosis of gastric subepithelial lesions with microscopic and immunohistochemistry characteristics

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Glomus tumors are characteristically and diffusely immunoreactive for αSMA, Muscle Specific Actin (MSA), calponin and h-caldesmon.^[5],[7] Synaptophysin positivity is an occasional finding in GGT suggestive of presence of neuroendocrine granules in the glomus cells. Synaptophysin has been shown to be positive in 20% of cases of GGT, which may possibly result in these tumors being misdiagnosed as carcinoid tumors.^[8] IHC in our case demonstrated positivity for αSMA, h-caldesmon and synaptophysin and negative staining for CD117, chromogranin, S100 and PCK. Ki 67 was 5%-8%. The classical morphology, reticulin stain and typical IHC profile confirmed the impression of solid variant of glomus tumor. Although rare, one must consider glomus tumor in the differential diagnosis for subepithelial tumors of the stomach.

GGTs are generally benign, however, seldom might show malignant behavior. Till date, a very few cases of metastatic GGT have been described.^[9] Similar to criteria for peripheral soft tissue glomus tumors as proposed by Folpe A et al., GGT with deep location and size >2 cm or with atypical mitotic figures or with mitotic activity >5 mitoses/50 HPF may be considered as malignant GGT and shall be closely followed up.^[10] Presently, there are no guidelines for management and follow up of GGT due to paucity of reported cases. Our case has been advised close follow-up due to the high-risk features like size, location and slightly higher proliferative index.

Conclusion

The glomus tumor of stomach is an extremely rare, usually solitary submucosal mesenchymal tumor of the gastrointestinal tract. One must consider glomus tumor in differential diagnosis of gastric subepithelial tumors. GGTs are generally benign, however, seldom might show malignant behavior. In the absence of characteristic clinical symptoms, endoscopic and imaging findings of GGT, preoperative diagnosis is challenging. Hence, the final diagnosis of GGT is based on postoperative histopathological examination and IHC analysis. This emphasizes the importance of accurate morphological assessment supported by a panel of IHC studies in establishing the diagnosis of this unusual distinctive subepithelial tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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