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  Table of Contents    
CASE REPORT  
Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 369-372
Ossified uncertain malignant potential gastric glomus tumor with tumor thrombus


1 Department of Pathology, University of Health Sciences, Umraniye Training and Research Hospital, Elmalıkent, Ümraniye/İstanbul, Turkey
2 Department of Gastroenterology, University of Health Sciences, Umraniye Training and Research Hospital, Elmalıkent, Ümraniye/İstanbul, Turkey

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Date of Submission11-Jul-2020
Date of Decision27-Aug-2020
Date of Acceptance28-Oct-2020
Date of Web Publication9-Apr-2021
 

   Abstract 


Glomus tumor is a rare mesenchymal tumor composed of perivascular glomus bodies. The most common presentation area of these tumors is peripheral soft tissue, particularly in the distal part of extremities. They rarely can occur in the gastrointestinal tract and the most common location is the stomach. Preoperative diagnosis of this tumor can be difficult because of rarity and overlapping features with other mesenchymal lesions with regard to clinical and pathological findings. Therefore, to exclude differential diagnosis and make a definitive diagnosis is possible only with histopathological examination. In this case, we evaluated glomus tumor of stomach according to 2019 WHO Digestive System Tumors and accurate diagnosed was Uncertain Malignant Potential Gastric Glomus Tumor.

Keywords: Endoscopic ultrasound guided fine needle aspiration, glomus tumor, ossification, stomach

How to cite this article:
Guvendir I, Zemheri IE, Altundal K, Ozdil K, Kahraman R, Tosun I. Ossified uncertain malignant potential gastric glomus tumor with tumor thrombus. Indian J Pathol Microbiol 2021;64:369-72

How to cite this URL:
Guvendir I, Zemheri IE, Altundal K, Ozdil K, Kahraman R, Tosun I. Ossified uncertain malignant potential gastric glomus tumor with tumor thrombus. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 16];64:369-72. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/369/313283





   Introduction Top


Glomus tumors (GT) are rare mesenchymal neoplasms. They can occur anywhere in the body but commonly in the skin and soft tissues of distal extremities. The involvement of gastrointestinal tract is uncommon and mostly seen in the stomach.[1],[2],[3]According to 2019 WHO Digestive System Tumors, GT was codded as GT-NOS, GT-uncertain malignant potential (GT-UMP), and malignant.[4] Approximately 240 gastric glomus tumors were published between 1968 and 2020 in literature. Preoperative diagnosis of this tumor can be difficult because of rarity and overlapping features with other mesenchymal lesions with regard to clinical and pathological findings. Therefore, accurate recognition is important to appropriate therapy.[1],[2],[3]

We reported a female patient with a gastric glomus tumor of uncertain malignant potential with endoscopic ultrasound fine-needle aspiration (EUS-FNA) findings because of rarity.


   Case Presentation Top


A 62 years old female patient came into the gastroenterology department with upper gastrointestinal bleeding intermittently for 15 years. She was stable and had normal blood pressure. Endoscopic ultrasound (EUS) imaging revealed a 3.5 × 2.7 cm-sized, a subepithelial lesion at proximal corpus ongoing lesser curvature protruded into lumen, included dense calcified areas: echo patterns were hyperechoic and heterogenous with irregular contours [Figure 1]a and [Figure 1]b. The lesion was suspicious of a gastrointestinal stromal tumor (GIST) and other mesenchimal stromal tumors.Subtotal gastrectomy of the tumor was reccomended.Due to patient did not accept to get an operation at the first stage, EUS-FNA was performed and was obtained only 3 slides for smear cytology. Because of bleeding, operation was stopped and cell block biopsy was not obtained.
Figure 1: Subepithelial lesion protruded into lumen included dense calsificated areas, echo patterns were hyperechoic and heterogenous with irreguler contours on EUS imaging (a-arrow), lession on endoscopy (b)

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Cytologic examination revealed a loosely cohesive, uniform, small, round to oval cells with scant cytoplasm, indistinct cell borders and hyperchromatic nuclei with homogeneous chromatin [Figure 2]a and [Figure 2]b. Salt and pepper nuclei were not seen. Due to we could'nt obtain cell block, immunohistochemical staining could not performed. A final cytological diagnosis was suspicious of a stromal tumor of the stomach. After that, subtotal gastrectomy was performed. Macroscopically, 4.0 × 3 × 1.5 cm nodular mass arising submucosa and muscular layer were observed. The mass on cut surfaces was white, solid, rubbery, and encapsulated. Microscopically most capillary vessels lined by endothelial cells surrounded by uniform glomus cells forming nests, sheets, and trabeculae in a hyalinized and myxoid stroma. Glomus cells have a round shape with indistinct borders with rounded, sharply punched out nucleus in an amphophilic to eosinophilic cytoplasm. Chromatin is homogenous and bland with inconspicuous nucleoli [Figure 3]a. In focal area, tumor thrombus was detected in the large vessel [Figure 3]b. At the periphery of tumor, ossification was seen [Figure 4]a and [Figure 4]b. On the immunochemical (IHC) panel, the tumor was stained strongly and diffusely with SMA, Collagen IV, and focally synaptophysin. Beside that CD117, CD56, S-100, CD34, chromogranin, and desmin were stained negative [Figure 5]a and [Figure 5]b. Mitosis was 1/50 Hpf. On molecular working, BRAFV 600E mutation was not detected. Microscopic and immunohistochemical findings were consistent with a GT-UMP with ossification and vascular tumor thrombus because of large size and deep location. Recommended for close follow up of the patient. After the diagnosis, the patient underwent systemic examination, metastasis was not found despite vascular tumor thrombus. In the follow-ups, no recurrence or symptomatic complaints were observed in the patient, she is still alive for 12 months after diagnosis.
Figure 2: (a and b) Cytologic examination revealed a losely cohesive, uniform, small, round to oval cells with scant cytoplasm (Papanicalou staining)

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Figure 3: Glomus tumor with capillary sized vessels surrounded by uniform glomus cells forming nests (a). Tumor trombus in the large vessel (arrow) (b)

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Figure 4: (a and b) Ossification at the perihery of the glomus tumor

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Figure 5: Tumor stained with diffuselly SMA (a) and focally synaptophysin (b)

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   Discussion Top


Glomus tumor is a mesenchymal tumor composed of perivascular glomus bodies. They are modified smooth muscle cells. The most common presentation area of these tumors is peripheral soft tissue, particularly in the distal part of extremities. They rarely can occur in the gastrointestinal tract and the most common location is the stomach. They grow as submucosal masses and can detect into the lumen or out onto the serosa.[5]

On differential diagnosis: Gastrointestinal stromal tumor, leiomyoma, ampullary carcinoma- neuroendocrine types, neuroendocrine tumor G1, hemangiopericytoma or ectopic pancreas can be made according to imaging features.[6],[7]

Clinical presentation of these tumors is variable like bleeding, hematemesis/melena, dyspepsia, and epigastric discomfort.[5],[8]

The preoperative diagnosis is difficult because of intramural location of tumor and thus generally, endoscopic biopsy is not useful. Current imaging studies such as computed tomography (CT), magnetic resonance imaging (MRI) and EUS are limited because of similarities of other submucosal lesions. Glomus tumor has dense homogeneous enhancement in the arterial phase and continuous enhancement in the delayed phase on CT like as GIST and neuroendocrine tumors.[9] EUS imaging can help detection of lesion location but in diagnose of glomus tumor have not specific findings. But EUS-guided aspiration with cytologic and immunohistochemical evaluation can make the diagnosis easier.[9] However, EUS has major limitations such as obtaining small amount of tissue and risk of bleeding because of highly vascular tumors[8] In this case, we could not obtain enough material by virtue of bleeding.

Glomus tumors are typically composed of 3 components: glomus cells, vasculature, and smooth muscle cells . They are categorized into three subtypes on light microscopy as (1) Solid glomus tumors: this type comprises approximately 75% of glomus tumors and is composed of nests of glomus cells surrounding capillary-sized vessels. (2) Glomangioma: this type comprises approximately 20% of glomus tumors and is characterized by cavernous hemangioma-like vascular structures surrounded by small clusters of glomus cells. (3) Glomangiomyoma with more prominent vascular and smooth muscle components. In this case the tumor was concordant with solid subtype, it comprised typically capillary vessels lined by endothelial cells surrounded by uniform glomus cells forming nests, sheets, and trabeculae in a hyalinized stroma.

The cytomorphology of glomus tumor is poorly defined because of rarity. To date, there have a few reports in the English literature relevant to cytology of glomus tumors.[9] Cytologically, glomus tumor reveals well-demarcated nests with small, uniform, round/oval-shaped epithelioid cells with round nuclei and scanty, ill-defined cytoplasmic borders.[9] But, because of similarities between glomus tumors and neuroendocrine tumors in cytologically, diagnosis is very difficult. So EUS-FNA needs to use together with Hematoxylin&Eosin/Papanicolaou staining and IHC analysis such as positivity for smooth muscle actin (SMA) and type IV collagen and negativity for desmin, cytokeratin, S-100, CD-117, CD34, DOG1 protein and chromogranin A. Sometimes, focal positivity for synaptophysin can be seen.[8],[9],[10] In our case, cytologic findings were not specific. Because there were no cells with salt pepper nuclei, so we got away from neuroendocrine neoplasia. We didn't make immunohistochemical staining on cytologic material because could'nt obtain cell block from inadequacy material. But we applied IHC on resection material and the tumor was stained with SMA, type IV collagen and focally synaptophysin.

On electron microscopy, the cytoplasm is found to contain myofilamentous dense bodies, with connecting structures between adjacent cells and the basement membrane around the cells.[11]Since there is no electron microscope in our laboratory, we couldn't examine the tumor with it.

Secondary changes such as hemorrhage, cystic change, and calcification on pathologic examination can be seen.[8],[11] In our case beside calcification areas, with osteoblastic cells, ossifying processing was seen.

Although glomus tumors are usually benign in the stomach, uncertain malignant potential and malignant gastric glomus tumors rarely were reported. Malignancy criteria are atypical mitosis, moderate to high nuclear grade, mitotic activity of more than five mitoses/10 mm2, tumor size more than 2 cm and deep location.[4] Glomus tumor of uncertain malignant potential was codded but not defined by 2019 WHO Digestive System Tumors. According to Fletcher et al., of the above malignant indicators, if the tumor has only >5 mitotic cells/50 HPF and the location is superficial, or if it only has a large size or only a deep location, it can be classified as a glomus tumor with uncertain malignant potential.[11] In this case, mitosis was one/50Hpf, there is no atypical mitosis, nuclear grade was very low, but because of its large size (4 × 3 × 1.5 cm) and deep location , diagnosed as UMP-GT. Although vascular invasion is an important finding for other malignancies, it is frequently seen in benign gastric glomus tumors. Miettinen was detected vascular invasion in 11 of 14 cases, and only 1 patient died of metastatic disease to liver at 50 months.[5] In addition focal atypia commonly was seen in benign glomus tumor of stomach.[5]

About half of benign and malignant glomus tumors have the fusion of NOTCH family gene rearrangements, especially fusion of NOTCH2 to MIR143. In addition, BRAFV 600E mutation was detected in 6% of glomus tumors which had features of malignant and uncertain malignant potential.[12] We didn't detect BRAFV 600E mutation despite diagnosed as GT-UMP in this case.

A surgical wedge resection is the best treatment since glomus tumor is a mesenchymal tumor with potential malignant behavior and long-term follow-up and monitoring is suggested.[2] Enucleation surgery is usually followed by recurrence.[13] In the existence of BRAFV600E mutation, BRAF is a potential therapeutic marker.[4],[12]

Gastrointestinal glomus tumors occur commonly in the stomach and they have a good prognosis, but in a small part, unpredictable potential for malignant behavior can be seen. Patients with large tumors, high heteromorphism, and active mitosis should undergo long-term follow-up to accumulate additional data for the diagnosis of malignant GGTs.[11] The preoperative diagnosis of this tumor can be difficult because of rarity and overlapping features with other mesenchymal lesions andneuroendocrine tumors. Accurate recognition is important to appropriate therapy.

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.
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. 1
    
2.
Papadelis A, Brooks CJ, Albaran RG. Gastric glomus tumor. J Surg Case Rep 2016;11:1-2.  Back to cited text no. 2
    
3.
Chen K-B, 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. 3
    
4.
Nagtegaal ID, Odze RD, Klimstra D, Paradis V, Rugge M, Schirmacher P, et al. Mesenchimal Tumors of the Digestive System. WHO Classification of Tumours Editorial Board. The 2019 WHO Classification of Tumours of the Digestive System. 5th ed.. World of Health Organization; 2019. p. 473. doi: 10.1111/his. 13975.  Back to cited text no. 4
    
5.
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. 5
    
6.
Vig T, Bindra MS, Kumar RM, Alexander S. Gastric glomus tumour misdiagnosed as gastric carcinoid: An unfamiliar entity with aids to diagnosis and review of literature. J Clin Diagn Res 2017;11:ED32-3.  Back to cited text no. 6
    
7.
Jagtap SV, Jokhi CD, Patil DB, Jagtap SS. Adenocarcinoma of ampulla vater presented as recurrent jaundice.Annals of pathology and laboratory medicine. Ann Pathol Lab Med 2018;5:82-4.  Back to cited text no. 7
    
8.
Kang G, Park JH, Kim JY, Choi D, Min BH, Lee JH, et al. Glomus tumor of the stomach: A clinicopathologic analysis of 10 cases and review of the literature. Gut Liver 2012;6:52-7.  Back to cited text no. 8
    
9.
Kato S, Kikuchi K, Chinen K, Murakami T, Kunishima F. Diagnostic utility of endoscopic ultrasound-guided fine-needle aspiration biopsy for glomus tumor of the stomach. World J Gastroenterol 2015;21:7052-8.  Back to cited text no. 9
    
10.
Morte D, Bingham J, Sohn V. Gastric glomus tumor: An uncommon source for an acute upper GI bleed. Case Rep Gastrointest Med 2018;2018:1-3. doi: 10.1155/2018/7961981.  Back to cited text no. 10
    
11.
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;2020:1-6. doi: 10.1155/2020/5637893.  Back to cited text no. 11
    
12.
Karamzadeh Dashti N, Bahrami A, Lee SJ, Jenkins SM, Rodriguez FJ, Folpe AL, et al. BRAF V600E mutations occur in a subset of glomus tumors and are associated with malignant histologic characteristics. Am J Surg Pathol 2017;11:1532-41.  Back to cited text no. 12
    
13.
Pidhorecky I, Cheney RT, Kraybill WG, Gibbs JF. Gastrointestinal stromal tumors: Current diagnosis, biologic behavior, and management. Ann Surg Oncol 2000;7:705-12.  Back to cited text no. 13
    

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Correspondence Address:
Irem Guvendir
Elmalikent Blvrd. Alemdag St. No:1, Postal Code:34764 Umraniye/Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_374_20

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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