Clinicopathological features of gastric glomus tumor

AIM:To study the clinicopathological features of gastric glomus tumor and review the related Chinese literature published in 1990-2010.METHODS:A case of gastric glomus tumor was re-ported.Clinicopathological findings in 56 cases of gastric glomus tumor were analyzed.RESULTS:Gastric glomus tumor was far more common in women than in men with a female to male ratio of 1.6:1.The median age of the patients was 45 years(range 28-79 years).The patients often complained of epigastric pain and bloody stool.The tumor was located in antrum of the stomach.The greatest diameter of the tumor was 0.8-11cm.Histologically,the tumor was comprised of nests of glomus cells surrounding the capillaries.Glomus cells were small,uniform and round.Vimentin,smooth muscle actin and actin were expressed in the tumor.Other markers,including S-100 protein,CD34,CD117,desmin,CD56,synaptophysin,chromo-granin A,neuron specific enolase and cytokeratin were all negative.CONCLUSION:Gastric glomus tumor is a rare benign mesenchymal neoplasm.Its diagnosis depends on pathologic examination.Differential diagnosis includes gastrointestinal stromal tumor,paraganglioma and carcinoid tumor.     

Glomus tumor of the stomach: a clinicopathologic analysis of 10 cases and review of the literature

Background/Aims

Gastric glomus tumors are extremely rare, and presurgical confirmation is often impossible. The identification of clinical and radiologic characteristics of this tumor type is important for preoperative diagnosis and treatment planning.

Methods

In this study, we analyzed 10 cases of gastric glomus tumors resected at a single institute over 9 years.

Results

Eight of the patients were men and 2 were women, with a mean age of 49 years. Five patients presented with abdominal discomfort or pain, 1 presented with anemia, and the remaining 4 cases were found incidentally during endoscopic examinations. The most common location of the tumor was the antrum (n=7), followed by the low (n=2) and high body (n=1). Although the endoscopic ultrasonography findings were variable, contrast-enhanced computed tomography generally showed a strong homogeneous enhancement. The resected tumors were well-demarcated solid masses with sizes ranging from 1.0 to 3.6 cm. Microscopically, the masses were composed of abundant vascular channels with clusters of uniform and round glomus cells. There was no evidence of recurrence after complete surgical resection.

Conclusions

Gastric glomus tumors are unusual, distinct lesions that should be considered in the differential diagnosis of a gastric submucosal mass. Unlike their deep soft tissue counterparts, most glomus tumors in the stomach are benign.     

Glomus tumor in the stomach:Computed tomography and endoscopic ultrasound findings

A 57-year-old man presented with intermittent dull abdominal pain after a period of 1 year.Abdominal computed tomography(CT) was performed.Except for the endoscopy,the work-up for possible medical causes remained inconclusive.An open-abdomen,partial surgical excision of the stomach was performed after the unsuccessful endoscopic resection.The pathology report revealed a glomus tumor of the stomach.Importantly,glomus tumors of the stomach are rare and are almost always benign.Therefore,the most important current role of imaging associated with the diagnostic approach and therapeutic plan for a glomus tumor is to differentiate it from other gastric submucosal tumors(SMTs).We report this case with representative radiologic findings,including CT and endoscopic ultrasound(EUS) reports,and also correlate them with clinical and pathologic presentations that can help in the early detection and differentiation of gastric SMTs from other SMTs.As such,the purpose of this report is to provide a better understanding of relevant CT and EUS features.Alternative treatments should be considered carefully according to the imaging results.     

Endoscopic enucleation of upper-GI submucosal tumors by using an insulated-tip electrosurgical knife

BACKGROUND: Submucosal tumors often are found incidentally at upper endoscopy. Although the majority of the ones less than 5 cm in size are benign, periodic monitoring for incremental changes in size, which can be stressful and bothersome to patients, is essential. If relatively easy and safe, an endoscopic method of removal would be optimal treatment. Conventional endoscopic enucleation technically is difficult and often leads to serious complications, such as bleeding and perforation. A novel method for endoscopic enucleation of submucosal tumors with an insulated-tip electrosurgical knife is reported. METHODS: En bloc enucleation was attempted by using an insulated-tip electrosurgical knife in 15 patients (10 men, 5 women; median age 48 years) with submucosal tumors of the esophagus (5) or stomach (5 cardia/fundus, 4 body, one antrum). EUS was performed to determine the layer of origin and the exact size of the submucosal tumor. RESULTS: Tumors arose in the muscularis propria in 11 cases, submucosa in two, and muscularis mucosa in one. Enucleation was relatively easy and successful in 14 cases. In one case, however, piecemeal resection was unavoidable because of fibrotic adhesions with the surrounding tissue; this tumor later was confirmed to be a glomus tumor. Median procedure time was 35 minutes (8-180 minutes) and median size of the submucosal tumors was 2x1.7 cm. The largest lesion, located in the esophagus, measured 6x3 cm. Histopathologic diagnoses included leiomyoma (9), GI stromal tumor (4), stromal tumor of unknown malignant potential (1), and glomus tumor (1). A small perforation occurred in one patient with a 2.5-cm tumor in the anterior wall of proximal gastric body but was managed successfully by endoscopic clip application. Follow-up endoscopy at 2 months in this patient revealed no tumor recurrence and complete healing of the treatment-related ulcer. CONCLUSIONS: En bloc endoscopic enucleation of submucosal tumors by using an insulated-tip electrosurgical knife appears to be safer, easier, and less time consuming compared with previously described methods. However, further investigation and comparative studies are required to confirm the safety and efficacy of this method.     

A rare large tracheal glomus tumor with postoperative haematemesis

Glomus tumors are uncommon benign tumors which usually arise from the distal portion of the digits. A tracheal glomus tumor with large size is extremely rare. We present a case of a large tracheal glomus tumor that was resected using posterolateral thoracotomy and successful primary reconstruction of the trachea was achieved. Severe haematemesis happened after the patient was discharged. An emergency exploratory thoracotomy was performed but no signs of anastomotic bleeding were observed, while intraoperative gastroscopy revealed plenty of blood and blood clots in the patient’s stomach. Medical treatments targeting hemorrhage of upper digestive tract were given and the patient finally recovered.KEYWORDS : Glomus tumor, trachea, haematemesisGlomus tumors are rare, benign but often painful tumors arising from modified smooth muscle cells (SMCs) of the glomus body, generally much less than 1 cm in diameter. These tumors are usually found in the distal portion of the digits, especially under the fingernails. They are morphologically characterized by round, slightly elevated, red-blue, firm nodules (1). However, these tumors can also occur occasionally at sites such as the chest wall, bone, gastrointestinal tract, nerve, eyelid, mediastinum, and trachea, where normal glomus bodies may be few or even absent (2). We reported a rare tracheal glomus tumor which was successfully resected.     

A gastric glomus tumor resected using non-exposed endoscopic wall-inversion surgery

Gastric glomus tumors are extremely rare, constituting approximately 1% of gastric mesenchymal tumors. We report the case of a 45-year-old female patient in whom upper gastrointestinal endoscopy revealed a 3-cm submucosal tumor with a bridging fold in the posterior wall of the antrum of the stomach. Contrast-enhanced computed tomography revealed the tumor to be hypervascular in the arterial phase and exhibit continuous enhancement in the post-venous phase. Dynamic contrast-enhanced magnetic resonance imaging showed that the tumor was hypervascular in the early phase and persistently enhanced in the late phase. Endoscopic ultrasonography revealed a mosaic echo pattern. Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed. Immunohistochemical examination revealed that the tumor was positive for α-smooth muscle actin and synaptophysin, but negative for CD56, chromogranin A and C-kit. Furthermore, 1–2% of the tumor cell nuclei were Ki-67-positive. Thus, the preoperative diagnosis was a benign glomus tumor. The patient then underwent non-exposed endoscopic wall-inversion surgery (NEWS), and the tumor was completely resected. In conclusion, NEWS is an effective method for the treatment of gastric glomus tumors.     

GASTRIC LIPOMA SUCCESSFULLY REMOVED BY LAPAROSCOPIC INTRAGASTRIC SURGERY

Lipomas are found very rarely in the stomach, where they account for only 3% of benign gastric tumors. Here, we report a case of large gastric lipoma removed successfully by laparoscopic intragastric surgery. A 45‐year‐old woman with no symptoms was incidentally diagnosed with a yellowish submucosal gastric tumor, about 3 cm in diameter, in the prepyloric antrum by upper gastrointestinal endoscopy. Pathological findings of biopsy specimens showed no evidence of malignancy. Computed tomography showed an ovoid and well‐circumscribed intramural mass, measuring 35 mm, which was of fat density. These findings suggested that the tumor was a lipoma that we felt should be treated, because large lipomas can give rise to gastrointestinal bleeding. We performed a laparoscopic intragastric enucleation for this tumor. Postoperatively, the tumor was confirmed pathologically to be a lipoma of the stomach. This laparoscopic procedure for benign non‐epithelial gastric tumors, such as lipomas, presented here is technically feasible, safe, and less stressful for the patient than gastrectomy.     

Late recurrence of a cardiac glomus tumor

Glomus tumors, also known as paragangliomas or chemodectomas, arise from well-differentiated mesenchymal cells that are known to be benign. These tumors account for 2% of all soft tissue tumors. The first case of a cardiac glomus tumor was reported in 1924 by Masson.1 Glomus tumors located within the pericardial tissue are extremely rare. We present the case of a recurrent cardiac glomus tumor. The preoperative investigation, diagnostic problems and surgical treatment of this case will be highlighted. Moreover we wish to emphasize the importance of such an entity and to remind readers that it must be included in the differential diagnosis of a pericardial tumor.     

Plexiform fibromyxoma: Case report and literature review

Plexiform fibromyxoma (PF) is a rare mesenchymal neoplasm which can be misdiagnosed as the gastrointestinal stromal tumor. This tumor almost formed a lobulated intramural/submucosal mass in the gastric antrum and prepyloric area. It was considered as a benign tumor that exhibited no recurrence, metastasis, or tumor-related mortality. In this study, we reported 2 cases of gastric PF. The first case was a PF patient coexisting with gastric adenocarcinoma. The second case occurred in the gastric upper body close to gastric fundus. They underwent distal gastrectomy and laparoscopic partial gastric resection, respectively. Both of them exhibited a plexiform growth pattern in the submucosa, muscularis propria, and subserosal adipose tissues. The nodules were composed of abundant myxoid or fibromyxoid matrix riching in small thin-walled blood vessels and bland-looking spindle cells. The first case partially showed staggered growth pattern of PF and adenocarcinoma. Immunohistochemically, the spindle cells were diffusely immunoreactive for SMA and vimentin, and focally immunoreactive for CD10. It was important to distinguish the PF from other spindle cell tumors involving the stomach.     

Massive Glomus Tumor of the Stomach: 20-Year Follow-up and Autopsy Findings

A massive glomus tumor of the greater curvature of the stomach was removed in 1961. The patient made an uneventful recovery and died 21 years later of causes unrelated to the tumor. The literature of glomus tumors of the stomach is discussed. The pathology of the lesion is described and the autopsy findings are presented.     

A rare case of iron deficiency

Giant lipomas of the stomach are very rare, accounting for less than 3% of all benign tumors of the stomach. A clear-cut endoscopic differentiation between gastric lipomas and other submucosal neoplasms is not feasible, because routine endoscopic gastric biopsies do not reach the submucosal layer. Gastric submucosal lipomas can cause gastric ulceration as in the case presented below and in rare instances this may in turn promote gastric cancer. Therefore, complete pretreatment diagnostic evaluation is needed. We present a 52-year-old man with a 6-month history of epigastric discomfort, early satiety, decreased appetite, and dyspepsia. His weight was noted to be stable and he was iron deficient (hemoglobin 11.5 g/dl and ferritin of 5 g/dl). His past history included a gastric ulcer found on endoscopy 5 years ago for which he was on omeprazole 40 mg once a day, hypertension, hypercholesterolemia, and diabetes. Clinical examination revealed central obesity with divarification of recti muscles. He underwent a colonoscopy that was normal, and an oesophago-gastro-duodenoscopy that revealed a smooth extrinsic indentation of the anterior aspect of the distal stomach at around 50 cm. Biopsies of this were normal. A computed tomography scan was obtained () that demonstrated a 14 by 15-cm fatty tumor arising from the distal stomach with a couple of 5-mm nodes adjacent to tumor and no distant metastasis representing either a lipoma, liposarcoma or gastrointestinal stromal tumours. He subsequently underwent a subtotal gastrectomy. Macroscopically, the antrum was distorted by a huge submucosal intramural tumor mass. The antral mucosa was stretched over its surface and bore a central 15-mm ulcer surrounded by a raised border (). Microscopic examination confirmed an ulcerated benign submucosal lipoma. Our patient was symptomatic with a large gastric lipoma that necessitated surgical excision. Following surgery his postoperative recovery was uneventful, and he was asymptomatic when reviewed 4 weeks later. This case demonstrates a rare case of gastric lipoma causing gastric epithelial ulceration leading to iron deficiency.     

Glomus tumor of the stomach: a case treated by endoscopic submucosal dissection

Glomus tumors, as a type of quite rare neoplasms, originate from modified smooth muscle cells of the glomus body whose function is to regulate blood flow within arteries according to the body temperature. Although these tumors most commonly occur in the peripheral soft tissues, especially in the distal parts of extremities, there have been rare reports of visceral involvement (Lorber et al., 2005) [1]. We report a case of gastric glomus tumor, which was preoperatively diagnosed by ultrasonic endoscopy as a gastric stromal tumor and treated by endoscopic submucosal dissection (ESD).     

Antigen shared by HeLa-like human cell line and gastric mucosa

An antigen of human gastric mucosa immunologically related to the antigen of established HeLa-like cell lines (CL-GMA) is described. In gel-immunodiffusion test the antigen was revealed in 10/10 samples of normal gastric mucosa (including all parts of stomach), in 15/16 samples of cancer patients' gastric mucosa 5-10 cm distant from tumor and in 2/2 samples of ulcer patients' mucosa 5-10 cm distant from the ulcer. However, the antigen was undetectable at a distance 1-2 cm from ulcer. Homogenates of 39 embryonic organs and tissues were screened for the presence of CL-GMA. CL-GMA was detected in 7/7 samples of gastric mucosa. The antigen was revealed in trace amounts in 1/4 samples of small intestine mucosa and in 1/4 samples of spleen. Screening of 66 human tumors revealed CL-GMA in 13/16 samples of gastric cancer and in trace amounts in 2 tumors of non-stomach localization (larynx and rectum). Analysis of aceton-fixed paraffin sections by means of immunofluorescence revealed be CL-GMA in all parts of stomach. CL-GMA localized in the basal area of high columnar epithelial cells. The antigen was almost or totally undetectable in poorly differentiated adenocarcinomas of stomach and in tumors of other localization. We could not detect CL-GMA in the sera of various cancer patients by means of immunodiffusion and/or dot-blotting.     

Leiomyosarcoma of the stomach

Summary The literature is reviewed, and 10 cases of leiomyosarcoma of the stomach are presented. Nine of these were encountered during the 10-year period of 1948–58. The clinical features and roentgenologic diagnosis are discussed. Two cases were of microscopically benign leiomyomas that eventually metastasized. One case involved a gastric adenocarcinoma. A case with gastric fistula leading to the center of a large, necrotic, exogastric leiomyosarcoma is presented. There were 3 long-term survivals, 1 of five years and 2 of ten years. Emphasis is placed on the slow rate of metastasis and the excellent prognosis of long-term survival when the tumors are thoroughly removed.It is suggested that leiomyosarcoma be considered in those cases where (a) gastric tumor, or (b) gastric fistula, or (c) barium outside the confines of the stomach is radiogrphaically shown in the examination of the gastrointestinal tract, in patients whose clinical picture is one of (a) anemia secondary to blood loss, (b) vague epigastric complaints, and (c) long duration of symptoms.     

Multiple Carcinoid Tumors of the Stomach with Hypergastrinemia

A 42-yr-old woman presented with multiple carcinoid tumors in her stomach and a markedly elevated serum gastrin level. Total gastrectomy was performed, and 22 small carcinoid tumors were found in the gastric fundus and body. A high serum gastrin level was revealed in the gastric drainage veins; still more gastrin was detected in the carcinoid tumors by the immunohistochemical method, and many secretory granules were found in the tumor cells with an electron microscope. The fundic gland showed marked atrophy, and there was some conglobation of endocrine cells (ECL cells). This case suggests a hypothetic sequence of anacidity due to atrophic gastritis----hypergastrinemia----proliferation of ECL cells----multiple carcinoids. A search of the Japanese literature revealed that 26 cases of multiple carcinoid tumors in the stomach have been reported so far, and most of them support this hypothesis.     

Benign Granular Cell Tumor ("Myoblastoma") of the Stomach

Two cases of benign granular cell tumor of the stomach which were found incidentally are reported. A review of the literature disclosed 10 case reports and three other cases which were mentioned. Despite the small size of the tumors eight of the 10 cases produced gastrointestinal signs or symptoms. A brief discussion of the histogenesis of the lesion is presented.     

Malignant glomus tumor in pleural cavity

Glomus tumors, an uncommon hypervascular tumor, arise from modified smooth muscle cells of the glomus body that plays a significant role in the regulation of skin circulation. The tumors are usually located in the extremities, typically in the subungual region of the fingers. Primary glomus tumors of the chest are extremely rare, and to our knowledge, there are no cases have been described in thoracic cavity to date. We here report a case of intrathoracic glomus tumor in a 31-year-old man who presented with a persistent chest pain. Chest computed tomography scans demonstrated an irregularly shaped mass in the left thorax. Left thoracotomy was performed under the suspicious diagnosis of unexplained thorax tumor, and a tumor located in the left upper portion of thorax was founded. Complete resection of tumor along with the partial structure of chest wall was performed. Postoperative diagnosis was malignant glomus tumor.     

Cardiac glomus tumor. Light- and electron microscopic investigations (author's transl)]

A cardiac glomus tumor, first described by Masson, was observed in a 28 year old woman who presented symptoms of cardiac dysfunction. Electron microscopic studies disclose a wide range of differentiation of glomus cells from smooth muscle cells to epitheloid cells. The common feature of both cell types include cytoplasmic microfibrils, fusiform condensations and small vesicles. The endothelial cells also show some structural similarities to these glomus cells. The histogenesis of this cardiac tumor is discussed on the basis of its ultrastructure and its uncommon localization. It is concluded that cardiac glomus tumors arise from primitive mesenchymal cells.     

Ulceration of intramural gastric neoplasms

A 37-year-old male with an ulcerated glomus tumor of the stomach and a 59-year-old male with a bleeding gastric carcinoid tumor are presented. The cases are illustrative of the common mode of clinical and roentgenographic presentation of intramural gastric neoplasms of varied histology. The lesions, by virtue of their plane of growth, stretch and thin overlying gastric mucosa producing ulceration.     

Gastric glomus tumor: Analysis of endosonographic characteristics and computed tomographic findings

Among submucosal tumors (SMT), gastric glomus tumors are rare vascular tumors, and only a few cases where glomus tumors have been differentiated from other mesenchymal tumors by endoscopic ultrasonography (EUS) or computed tomography (CT) have been reported. In this case series, we aimed to analyze the EUS and CT characteristics of gastric glomus tumors. Seven patients with gastric glomus tumors were retrospectively enrolled in this study. EUS showed that all the tumors were located in the fourth EUS layer (muscularis propria) and had distinct borders. The tumors had a heterogeneous appearance with either hypo‐ or hyperechogenicity, and all the tumors except one had the characteristic peripheral halo around them. On CT scans,all the tumors appeared as well‐defined SMT with a clear margin and showed the same characteristics in the different phases of CT. During dynamic contrast‐enhanced CT, the tumors showed strong enhancement in the arterial phase and prolonged enhancement in the delayed phase. This case series shows the characteristic EUS findings and the distinguishing features of CT scans for gastric glomus tumors; these findings will help in differentiating gastric glomus tumors from other SMT.