Kaohsiung J Med Sci June 2010 • Vol 26 • No 6 321 Glomus tumors are rare benign tumors arising from
the glomus body, which plays a role in regulating arterial blood flow, and is sensitive to temperature variation. Glomus bodies are commonly observed wherever arteriovenous anastomoses are present. The first clinical and pathological description of a glomus tumor was described by Barre and Masson in 1924 [1].
Glomus tumors are commonly noted in the extremities and are rarely found in visceral organs, although tumors in the mediastinum, trachea, kidney, uterus, vagina and stomach have been described. Since Kay et al first reported three glomus tumors of the stomach in 1951 [2], fewer than 200 cases have been published in the literature in any language, and less than 100 cases have been published in English-language journals. The inci-dence of gastric glomus tumors is much less common than that of gastrointestinal stromal tumors (GISTs), with only 1 in 100 GISTs being gastric glomus tumors [3]. Because of the limited number of cases experienced at a single institute, we have presented our case and reviewed all five cases reported in Taiwan to date, and compare these cases with those of other countries. Received: Sep 23, 2009 Accepted: Oct 28, 2009
Address correspondence and reprint requests to: Professor Jaw-Yuan Wang, Department of Surgery, Kaohsiung Medical University Hospital, and Department of Surgery, Faculty of Medicine, College of Medicine, Kaohsiung Medical Univer-sity, 100 Tzyou 1stRoad, Kaohsiung 807, Taiwan.
E-mail: [email protected]
R
EVIEW OF THE
L
ITERATURE
Chuang-Chi Huang,1Fang-Jung Yu,2Chang-Ming Jan,2,3Sheau-Fang Yang,4,5Yu-Ting Kuo,6 Jan-Sing Hsieh,1,7and Jaw-Yuan Wang1,7
Departments of 1Surgery, 2Internal Medicine, 5Pathology and 6Radiology, Kaohsiung Medical University Hospital, and Departments of 3Internal Medicine, 4Pathology, and 7Surgery, Faculty of
Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Glomus tumors are usually thought of as benign tumors although some malignant cases have been reported. These tumors arise from the glomus body and are commonly observed in the der-mis or subcutis, but rarely in visceral organs. Here, we report a 37-year-old female who initially presented with epigastric discomfort. The preoperative diagnosis was a gastrointestinal stromal tumor. A minilaparotomy was done with an incision length of 4 cm followed by wedge resection. The final pathologic diagnosis was a gastric glomus tumor. We have reviewed the only five cases of gastric glomus tumors that have been reported to date in Taiwan, including the present case, and compare these cases with those reported in other countries. The age of onset ranged from 35 to 69 years (median, 41 years) with female dominance (4 females and 1 male). Two of the five cases presented with gastrointestinal bleeding with an ulcerative tumor, and the others only had epigastric discomfort. The tumors were located around the prepyloric antrum of the stomach. No definite diagnosis was reached before surgery in any of the five cases, and all of the tumors were considered likely to be benign lesions. Clinicians who treat such patients should be aware of this problem because of the difficulty in accurate preoperative diagnosis.
Key Words:endoscopic ultrasonography, glomus tumor, minilaparotomy, stomach (Kaohsiung J Med Sci 2010;26:321–6)
C
ASEP
RESENTATIONA 37-year-old female visited our institution for inter-mittent epigastric discomfort over a 2-month period. No nausea or vomiting was noted and her physical examination was normal. Panendoscopy revealed an antral submucosal tumor with an intact surface (Figure 1). Abdominal computerized tomography (CT) re-vealed a hypervascular submucosal tumor at the poste-rior, medial and inferior wall of the antrum, near the pylorus ring (Figure 2). Endoscopic ultrasonography (EUS) showed that the tumor was within the muscu-laris propria layer and had a heterogeneous appearance (Figure 3). Initial laboratory tests revealed the follow-ing: serum aspartate aminotransferase level of 55 IU/L, serum alanine aminotransferase level of 67 IU/L, and serum hemoglobin level of 11.9 g/dL. Because we ini-tially suspected antral GIST, a minilaparotomy was done with an incision length of 4 cm. At operation, a submucosal tumor of about 2.0× 1.5 cm in size was found at the posterior wall of the antrum, and a wedge resection of the tumor was done. She was discharged 5 days later with an uneventful postoperative course. The pathology revealed that the tumor cells were sur-rounded by hyperplastic smooth muscle fibers of the muscularis propria. The tumor had sheets of round or epithelioid tumor cells with small, uniform nuclei and sharp cell borders surrounding dilated thin-walled blood vessels (Figure 4). Immunohistochemistry re-vealed the tumor was positive for smooth muscle actin, but negative for cytokeratin and CD56 (Figure 5). Thus, the final diagnosis was gastric glomus tumor.
D
ISCUSSIONWe searched Medline for all cases of gastric glomus tumors reported up to May 2009, and found no more than 200 cases in all languages and less than 100 cases in English-language journals. To date, only five cases have been reported in Taiwan, including the present case. The clinical presentations, diagnostic tools, surgi-cal techniques, preoperative tentative diagnosis, and the characteristics of the specimens are listed in the Table [4–7]. The clinical presentations showed that Figure 1.A submucosal tumor was found near the prepyloric
antrum by panendoscopy (arrow).
C A
B
Figure 2.Abdominal computed tomography revealed a submu-cosal tumor at the posterior, medial and inferior wall of the antrum near the pylorus ring (arrow). (A) Unenhanced; (B) arterial phase; and (C) delayed phase.
Figure 5.Immunohistochemistry confirmed the tumor was posi-tive for smooth muscle actin (original magnification, 100×).
Figure 3.The submucosal tumor was noted in the muscularis propria layer and had a heterogeneous appearance under endo-scopic ultrasound (arrow).
A
B
Figure 4. (A and B) The tumor was surrounded by hyperplastic smooth muscle fibers of the muscularis propria. The tumor has sheets of round or epithelioid tumor cells with small, uniform nuclei and sharp cell borders around the dilated thin-walled blood vessels. (Hematoxylin and eosin; original magnification, (A) 20× and (B) 100×).
these gastric glomus tumors in Taiwan have a female predominance (4/5 cases, 80%), which is similar to the incidence in other countries: 76.9% in Korea [3] and 60% in Canada [8]. The age range (35–69 years) of the cases in Taiwan was also similar to that in Korea and Canada, although a wider age range was reported in Canada [8]. Gastrointestinal (GI) bleeding with an ulcerative tumor was observed in two cases, while three patients with a non-ulcerative tumor presented with epigastric discomfort. Consequently, GI bleeding with hematemesis/melena and epigastric discomfort are the most two common initial symptoms/signs, and GI bleeding can be life-threatening or lead to chronic anemia. No incidental case have been reported in Taiwan, which is similar to that in Korea [3], although five of 31 cases were incidental in Canada [8].
In terms of preoperative evaluations, EUS was per-formed in three of the five cases, and fine needle aspi-ration (FNA) was done in one case. However, a definitive diagnosed was not reached before surgery in any case, even in the case that underwent FNA. Until now, only three articles have reported a preoper-ative definite diagnosis with FNA [9–11]. The findings of EUS were usually heterogeneous tumors between the submucosal and muscularis propria layer and these findings may be confused with malignant GIST or leiomyosarcoma, which is also represented by a heterogeneous tumor on EUS [12,13]. Our case also had a heterogeneous submucosal tumor within the fourth layer. One case underwent subtotal gastrec-tomy with Billroth I anastomosis because the EUS findings suggested malignant GIST. Abdominal CT
T
able.
Demographic and clinical description of the five gastric glomus tumors in T
aiwan Case 1 [4] Case 2 [5] Case 3 [6] Case 4 [7] Pr esent case Y ear of publication 1993 1999 2005 2007 2009 Sex F F M F F Age (yr) 41 35 60 69 37 Location Antr um Lower body Antr um Antr um Antr um (gr eater curvatur e) (less curvatur e) (less curvatur e) Ulceration Y es Y es No No No Symptom/sign Melana Hematemesis, melana Epigastric discomfort Intermittent Epigastric discomfort epigastric discomfort Size (cm) 3.0 × 2.5 × 2.5 2.5 × 3.0 2.0 2.0 2.0 × 1.5 EUS ND Heter ogeneous with ND Heter ogeneous, Heter ogeneous hyper echoic and
hypoechoic mass with
anechoic internal hyper echoic spots FNA ND ND ND Nondiagnostic ND Operative pr ocedur e Distal hemigastr ectomy Subtotal gastr ectomy W edge r esection W edge r esection W edge r esection and Billr oth I anastomosis and Billr oth I anastomosis T entative diagnosis Car cinoid tumor Leiomyosar coma GIST , car cinoid, Not diagnostic GIST by endoscopic glomus tumor biopsy Immunor eactive stains SMA, vimentin ND SMA SMA SMA CT findings ND ND W ell-defined Homogenous Significant hypervascular tumor enhancement
enhancement during arterial phase
Other examination No No MRI: Power Doppler No T1-slightly hypointense sonogram: pr ominent T2-slightly hyperintense
vascular signals within the mass
EUS
=
Endoscopic ultrasonography; FNA
=
fine needle aspiration; CT
=
computerized tomography; MRI
=
magnetic r
esonance imaging; ND
=
not done; SMA
=
smooth muscle actin;
GIST = gastr ointestinal str omal tumor .
[6] also underwent magnetic resonance imaging, but the imaging was unable to differentiate between GIST and a carcinoid tumor. The tumors were located around the prepyloric antrum in all five cases, and the size of the tumors was approximately 2–3 cm.
Gastric glomus tumors are commonly benign le-sions but are difficult to distinguish from GISTs or carcinoid tumors by radiography, without specific immunohistochemical staining of FNA samples. In the National Institute of Health classification, tumor size and mitotic count are the major criteria indicat-ing the malignant potential of GISTs. Shah et al pro-posed that EUS characteristics, including tumor size, extraluminal border, depth and heterogeneity, could be used to predict the malignant potential of GISTs [10]. Thus, definite diagnosis before operation may be necessary and repeated FNA must be considered if the initial FNA does not provide definitive diagnosis. Debol et al reported a gastric glomus with definite pre-operative diagnosis through repeated FNA, and the tumor was resected by partial gastrectomy [9]. In our case, EUS revealed a heterogeneous submucosal tumor mimicking a GIST and a partial gastrectomy was sub-sequently performed because of very low malignant potential. Laparoscopic resection and endoscopic enu-cleation of gastric glomus tumors have been reported and may offer an alternative surgical procedure [14,15]. Most gastric glomus tumors have benign character-istics, but malignant cases have been reported. Folpe et al proposed a classification scheme with criteria for malignant glomus tumors, including deep location, tumor size more than 2 cm, or the presence of atypical mitotic cells, but it is unclear whether these criteria are suitable for gastric glomus tumors [16]. Furthermore, no long-term follow-up data were available for these cases, meaning criteria for the malignant potential of gastric glomus tumors remain poorly defined.
Here, we report our clinical experience of the fifth gastric glomus tumor in Taiwan, and also review the demographic and clinical presentation of this and four previous cases. The clinical characteristics of gastric glomus tumors, including female dominance, age range, tumor size, and EUS and CT findings were similar to those reported in other countries. It is diffi-cult to differentiate between glomus tumors, GIST and carcinoid tumors by CT, magnetic resonance imaging and EUS. Therefore, invasive and repeated FNA may
R
EFERENCES1. Barre JA, Masson P. Etude anatoclinique de certaines tu-meurs sousungueales douloureuses (Tutu-meurs du glomus neuromyo-arterial des extremities L). Bull De Soc Francaise
de Dermologie et Sypheligraphie 1924;31:148. [In French]
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and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors.
收文日期： 98 年 9 月 23 日 接受刊載： 98 年 10 月 28 日 通訊作者：王照元教授 高雄醫學大學附設醫院外科 高雄市三民區自由一路 100 號
胃脈絡球腫瘤－病例報告及文獻回顧
黃仲琦1 余方榮2 詹昌明2,3 楊曉芳4,5 郭禹廷6 謝建勳1,7 王照元1,7 高雄醫學大學附設醫院 1外科 2內科 5病理科 6放射線科 高雄醫學大學 醫學院醫學系 3內科 4病理科 7外科 Glomus tumors 通常發生在四肢的甲床下，很少會發生在臟器，我們報告在本院發 現的第一個胃部的 glomus tumor。這位病患是 37 歲的女性因上腹部間斷性疼痛而 至本院胃腸內科接受內視鏡檢查，因而發現在胃竇有一黏膜下的腫瘤，經內視鏡超音 波及電腦斷層檢查，發現這是一個血流豐富的腫瘤，但手術前無法確定腫瘤類別。經 由外科以約 4 公分的手術傷口施行迷你剖腹手術，並施行局部胃腫瘤切除手術，最後 病理報告的免疫染色呈現 SMA（Smooth Muscle Actin） 陽性，確定是發生在胃的glomus tumor。同時我們回顧過去曾在臺灣報告過的 4 個病例，並與其他國家的胃
部 glomus tumor 做比較並回顧文獻。
關鍵詞：內視鏡超音波，脈絡球腫瘤，迷你剖腹手術，胃 （高雄醫誌 2010;26:321–6）
