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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 11  |  Issue : 4  |  Page : 264-267

Gastric glomangioma masquerading gastrointestinal stromal tumour - A case report with review of literature


1 Department of Pathology, Thanjavur Medical College and Hospital, Thanjavur, Tamil Nadu, India
2 Department of Surgical Gastroenterology, Thanjavur Medical College and Hospital, Thanjavur, Tamil Nadu, India

Date of Submission06-Jan-2022
Date of Decision30-Jan-2022
Date of Acceptance31-Jan-2022
Date of Web Publication04-Oct-2022

Correspondence Address:
Krishnan Govindaraman Padmanaban
Associate Professor, Department of Pathology, Thanjavur Medical College and Hospital, Thanjavur 613 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcsr.jcsr_5_22

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  Abstract 


Glomus tumours (GT) are mesenchymal tumours composed of modified smooth muscle cells of the glomus body. It commonly occurs in skin and soft tissue of the distal extremities. Gastric GT is a rare neoplasm of gastrointestinal tract that frequently mimics other mesenchymal neoplasms clinically and radiologically. We report a case of glomangioma of gastric antrum in a 55-year-old female, mimicking gastrointestinal stromal tumour (GIST) endoscopically. Radiographically, tumour was intramural, 6 cm in size, and initial biopsy was inconclusive. Laparoscopic-assisted partial gastrectomy was done. Histopathological examination showed uniform round cells arranged around the blood vessels, a characteristic of glomangioma. Tumour cells were strongly immunoreactive for smooth muscle actin and h-Caldesmon and negative for desmin, cytokeratin, CD34, CD117 and chromogranin, which helped rule out other differential diagnoses. Recognition of this rare entity is important as most of them are benign and surgical resection is curative.

Keywords: Gastric mesenchymal tumour, gastrointestinal stromal tumour, glomangioma, glomus tumour


How to cite this article:
Padmanaban KG, Aravindan U. Gastric glomangioma masquerading gastrointestinal stromal tumour - A case report with review of literature. J Clin Sci Res 2022;11:264-7

How to cite this URL:
Padmanaban KG, Aravindan U. Gastric glomangioma masquerading gastrointestinal stromal tumour - A case report with review of literature. J Clin Sci Res [serial online] 2022 [cited 2022 Dec 5];11:264-7. Available from: https://www.jcsr.co.in/text.asp?2022/11/4/264/347040




  Introduction Top


Glomus tumour (GT) is a mesenchymal tumour composed of modified smooth muscle cells, representing a neoplastic counterpart of the perivascular glomus bodies, which regulates arteriolar blood flow.[1],[2],[3] GT can occur almost anywhere in the body but commonly in the skin and soft tissue of distal extremities, particularly in the sub-ungual region where glomus bodies are abundant.[2],[3],[4],[5] Glomangioma is a type of GT characterised by cavernous haemangioma-like vascular spaces surrounded by clusters of glomus cells.[2],[6]

Gastrointestinal tract (GIT) involvement is uncommon, and when present, the stomach is almost exclusively affected.[5] Pre-operative diagnosis of a gastric GT can be challenging given its rarity and overlapping features with other mesenchymal lesions.[5],[7],[8] Here, we present a rare case of gastric glomangioma mimicking gastrointestinal stromal tumour (GIST) clinically with review of literature.


  Case Report Top


A 55-year-old female had epigastralgia for about 3 months. Endoscopic evaluation revealed a large sub-mucosal lesion with small surface ulceration in the antral region of the stomach [Figure 1]a. Computed tomography (CT) showed a fairly well-defined soft tissue density lesion in the muscularis layer measuring 6 cm × 4.5 cm with multiple dense calcific foci in the antral region with no invasive features or regional lymph node enlargement [Figure 1]b and [Figure 1]c, suggesting the possibility of GIST. As initial biopsy was inconclusive, considering the size of tumour and risk of bleeding, a laparoscopy-assisted partial gastrectomy was done.
Figure 1: Endoscopic photograph showing nodular mass in gastric antrum (a). Computed tomography of the abdomen (axial section) showing gastric soft tissue mass (arrow) (b); coronal section showing gastric mass with calcification (arrow) (c). Gross specimen photograph showing gastric glomangioma with calcification (d)

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Grossly, a nodular mass was seen in the muscularis layer along lesser curvature in gastric antrum. The mass was solid, grey-white, rubbery firm, with focal gritty, myxoid and haemorrhagic areas [Figure 1]d. Microscopically, a neoplasm composed of monotonous population of cells with uniform round nuclei and pale eosinophilic to clear cytoplasm having distinct cell borders was seen around medium-to-large-sized vessels in the muscularis propria [Figure 2]a, [Figure 2]b, [Figure 2]c and [Figure 2]d. Background stroma was vascular, myxoid, hyalinised with calcification (Figure 2b). Ki-67 proliferative index was 2%. Resected margins were free of tumour.
Figure 2: Photomicrograph showing glomus cells surrounding blood vessels (Haematoxylin and eosin, ×40) (a); glomangioma in muscularis layer with calcification (H and E, ×100) (b); monotonous tumour cells surrounding blood vessels (Haematoxylin and eosin, ×100) (c); glomus cells with clear cytoplasm (Haematoxylin and eosin, ×400) (d); glomus cells with diffuse strong immunoreactivity for smooth muscle actin (IHC, ×100) (e); and glomus cells exhibiting immunoreactivity for h-Caldesmon (IHC, ×100) (f)
IHC = Immunohistochemistrty


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Immunohistochemistry (IHC) panel was carried to confirm the diagnosis of glomangioma and to rule out other differential diagnosis. The tumour cells were strongly and diffusely positive for smooth muscle actin (SMA) with characteristic accentuation of cell membrane [Figure 2]e and h-caldesmon (Figure 2f) and focally positive for synaptophysin. Tumour cells were negative for pan-cytokeratin (CK), desmin, CD34, CD117 and chromogranin ruling out epithelial tumours, GIST, leiomyoma, paraganglioma and neuroendocrine tumours (NET). Based on the above clinicopathological characteristics, a final diagnosis of glomangioma was made.


  Discussion Top


Glomus tumours (GTs) are rare usually benign mesenchymal lesions of perivascular smooth muscle differentiation.[5],[6] Rare cases with malignant transformation has been described.[5],[8],[9],[10],[11] GT arises from the neuromyoarterial canals (Sucquet–Hoyer canals or glomus bodies), which are normally responsible for thermoregulation, via arteriovenous shunting of the blood.[5],[9] GT predominantly occurs in the skin and subcutaneous tissue of distal extremities where glomus bodies are abundant.[5] GT can occur in deep soft tissue and internal organs such as the nerves, bone, penis, bladder, mediastinum, GIT, liver and cervix.[2],[6] GIT involvement is exceedingly rare,[5],[6] with stomach being the most common site, rarely involving small and large bowel.[5],[9] The median age at presentation was 54 years (range 18–90 years), with a female predominance.[3],[5],[9] Patients can present with epigastralgia, gastrointestinal bleed, or can be asymptomatic.[2],[6],[9]

Endoscopic images show a solid, sub-mucosal tumour with or without ulceration[5],[6],[10] and do not differentiate it from other important diagnosis, e.g., GIST and neuroendocrine neoplasia.[10] On CT scan, GT often appears as circumscribed sub-mucosal masses with homogeneous density and occasional flecks of calcifications. On contrast CT, a sustained enhancement in venous phase is seen, a feature absent in GIST.[3],[5],[7] On endoscopic ultrasonogram (EUS), GT is usually seen in the fourth EUS layer (musclaris propria) having distinct borders,[2],[5],[7] with characteristic peripheral halo sign – tumour cells surrounded by muscularis propria. Mass appears heterogeneous due to increased vascularity, haemorrhage and calcification.[5]

As GTs are intramural, endoscopic biopsies are often inconclusive. Alternatively, EUS-guided fine needle aspiration cytology (FNAC) has been tried with varying success.[5] FNAC helps distinguish GT from more aggressive tumours rapidly and pre-operatively thus helps avoid major surgery. However, it can lead to a false diagnosis of leiomyoma or NET.[7] Being vascular tumour bleeding is a major complication.[5]

Clinicoradiological presentations in our case were similar to that reported in the literature. As mentioned in various studies, a pre-operative diagnosis of GT was difficult as biopsy was inconclusive, and radiologically, it was suspected to be a GIST.[2],[5],[8],[10] Rarely, malignant transformation can occur in GT. Therefore, surgical resection is in most cases the diagnostic and therapeutic option.[7],[10] Hence, a laparoscopic partial gastrectomy was performed in this case.

Grossly, GT presents as white-tan intramural circumscribed masses, ulcerated or polypoidal, with a mean size of 3 cm (range 0.8–22 cm),[9],[10],[11] commonly in antrum. Microscopically, tumour nodules are composed of solid sheets, nests of uniform round cells with sharply defined cell membranes, round nuclei and delicate chromatin, clear to amphophilic cytoplasm arranged around medium and large vessels.[6],[9],[11] GT can be divided into three subtypes based on the proportion of components: (i) solid GT (75%) are composed of nests of glomus cells surrounding capillary-sized vessels; (ii) glomangioma (20%) is characterised by haemangioma-like vessels surrounded by small clusters of glomus cells; and (iii) glomangiomyoma is the rarest, with a transition between typical cells and spindle cells.[2],[6] Our case had features of glomangioma.

Hyaline or myxoid degeneration of the stroma often occurs with occasional calcification and ossification.[6],[9],[10],[11] Reticulin stain demonstrates reticulin fibres around tumour cells and blood vessels.[6] Focal nuclear atypia and vascular invasion are relatively common and are not prognostically adverse.[11] Furthermore, gastric GT almost exclusively arises in the muscularis propria and usually lacks sub-mucosal involvement, which helps differentiate from NET which arise in the deep lamina propria.[5]

The following criteria have been proposed for defining malignancy in GT: deep location, size >2 cm, atypical mitotic figures, moderate-to-high nuclear grade with >5 mitoses/50 high-power fields.[12] Some workers had suggested the criterion 'deep location' does not apply to gastric GT with bad prognosis and a size of 5 cm is a more appropriate indicator of malignancy.[1]

In our case, tumour was 6 cm in size with characteristic histological features of glomangioma. No other risk factor for malignancy was seen. However, this being a rare presentation, IHC was done for confirmation of diagnosis, and for ruling out other differential diagnoses, namely, GIST, NET, leiomyomas and paragangliomas.[2],[3],[9]

Gastric GT cells show diffuse strong positivity with characteristic accentuation of cell borders for SMA in virtually all cases, h-Caldesmon in >60% of cases.[2],[5],[6],[9],[11] SMA and h-caldesmon were strongly positive in this case. Glomangiomas are also positive for calponin and collagen IV but negative for desmin, CD34, CD117, S-100, chromogranin and CK.[2],[3],[7],[9]

In contrast to GISTs, GT are consistently negative for CD 117, DOG1 and CD 34. Focal expression of synaptophysin was observed in our case, which has been described in literature;[3],[5],[6],[8],[11] however, lack of chromogranin, CK expression and SMA positivity ruled out NET. Paragangliomas can mimic a solid variant of GT, but they are strongly positive for chromogranin A, synaptophysin and S-100.[2],[3],[6] Leiomyomas have spindle cells in fascicles and are positive for both SMA and desmin.[3] CK, desmin, CD34, CD117 and chromogranin were negative in this case ruling out the other possibilities.

Most gastric glomangiomas are benign and segmental resection, or partial gastrectomy with clear margins is the treatment of choice and is curative.[5],[6],[7],[8],[10],[11] Unlike GIST, if complete removal of a glomangioma is verified histologically, there is no indication for further specific therapy. Endoscopic follow-up for early detection of recurrence can be done.[10] Our patient is on regular follow-up as the initial size of tumour was large.

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 Top

1.
Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumors: A clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. Am J Surg Pathol 2002;26:301-11.  Back to cited text no. 1
    
2.
Fang HQ, Yang J, Zhang FF, Cui Y, Han AJ. Clinicopathological features of gastric glomus tumor. World J Gastroenterol 2010;16:4616-20.  Back to cited text no. 2
    
3.
Mengoli MC, Nosseir S, Mataca E, Bordi P, Lupi M, Cavazza A, et al. Epidemiologic, clinicopathological, immunohistochemical and molecular analysis of gastrointestinal glomus tumors. J Clin Exp Pathol 2014;4:189-93.  Back to cited text no. 3
    
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Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med 2008;132:1448-52.  Back to cited text no. 4
    
5.
Duan K, Chetty R. Gastric glomus tumor: Clinical conundrums and potential mimic of gastrointestinal stromal tumor (GIST). Int J Clin Exp Pathol 2017;10:7905-12.  Back to cited text no. 5
    
6.
Lin J, Shen J, Yue H, Li Q, Cheng Y, Zhou M. Gastric glomus tumor: A clinicopathologic and immunohistochemical study of 21 cases. Biomed Res Int 2020;2020:5637893.  Back to cited text no. 6
    
7.
Chen KB, Chen L. Glomus tumor in the stomach: A case report and review of the literature. Oncol Lett 2014;7:1790-2.  Back to cited text no. 7
    
8.
Rosales-López E, Salceda-Otero JC, Angulo-Molina D, Posada-Torres JA, Canchola-Aguilar MG, Lozoya-Gonzalez D. Gastric glomangioma, differential diagnosis of gastrointestinal stromal tumors. Rev Gastroenterol Mex (Engl Ed) 2018;83:72-4.  Back to cited text no. 8
    
9.
Noffsinger AE, Grant NG. Mesenchymal tumors. In: Noffsinger AE, editor. Fenoglio – Preiser's Gastrointestinal Pathology. 4th ed. Philadelphia: Wolters Kluwer; 2017. p. 1190-1.  Back to cited text no. 9
    
10.
Matevossian E, Brücher BL, Nährig J, Feußner H, Hüser N. Glomus tumor of the stomach simulating a gastrointestinal stromal tumor: A case report and review of literature. Case Rep Gastroenterol 2008;2:1-5.  Back to cited text no. 10
    
11.
Thway K, Doyle LA, Fukayama M. Mesenchymal tumors of the digestive system. In: World Health Organization Classification of Tumors of Digestive System. 5th ed. Lyon, France: IARC; 2019. p. 473-4.  Back to cited text no. 11
    
12.
Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: Analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1-12.  Back to cited text no. 12
    


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