High-frequency ultrasonography for subungual glomus tumor evaluation - imaging findings.

OBJECTIVE: This article aimed to describe the common imaging features of subungual glomus tumors. METHODS: The study involved data collected between January 2019 and December 2022. Twenty-three patients with a total of 31 glomus tumors underwent high-frequency ultrasound examinations with a 24-MHz probe. Two experienced radiologists independently evaluated the images, and only data from the more experienced radiologist were used for subsequent analyses. RESULTS: The average size of the tumors was 4.6 mm, and most of them appeared homogeneously hypoechogenic (90.3%). Bone remodeling of the distal phalanx was observed in 87.1% of cases, with an average axial circumference loss of 0.8 mm, indicating the slow and expansive growth of glomus tumors. Intense vascularization was found in 54.8% of cases on Doppler images, and the stalk sign, reflecting the vascular origin of the tumor, was present in 64.5% of cases. The most common clinical feature was pain, reported in 84.6% of cases, with a mean pain scale score of 7.0, indicating a negative impact on patients' lives despite being benign tumors. CONCLUSION: The study concludes that ultrasound evaluation is highly useful for diagnosing glomus tumors, especially when multiple findings, such as bone remodeling, hypervascularization, and the stalk sign, are present. This method allows for accurate diagnosis, observation of periungual structures, and proper surgical planning, ultimately reducing recurrence rates.

Gastrointestinal Glomus Tumors: A Single Institution, 20-Year Retrospective Study.

INTRODUCTION: Glomus tumors are rare mesenchymal neoplasms composed of cells resembling those of the thermoregulatory glomus body, typically occurring in the skin and superficial soft tissues. Rarely do they occur in the gastrointestinal tract, in particular the stomach, where they have been the subject of case reports and small series. We present our institutional experience with gastrointestinal glomus tumors. METHODS: A retrospective review of all gastrointestinal glomus tumors was conducted across all three Mayo Clinic sites in Minnesota, Arizona, and Florida from 2001 to 2021. Patient characteristics, pathologic findings, imaging features, operative reports, and clinical outcomes were abstracted. Descriptive statistics were utilized to report outcomes. RESULTS: Nine patients with glomus tumors were identified (five men and four women). The median age was 53 [interquartile range (IQR), 44-69] y. Four patients presented with abdominal discomfort, three had anemia or bleeding, and two tumors were incidentally diagnosed. Computed tomography scans identified masses thought to represent gastrointestinal stromal tumors or neuroendocrine tumors in all patients. The tumors were localized to the stomach in all cases, specifically in the gastric antrum. Seven patients underwent preoperative endoscopy, including five with endoscopic ultrasonography. Endoscopic biopsies were interpreted as glomus tumors (n = 3), neuroendocrine tumors (n = 2), and nondiagnostic (n = 2). All patients underwent open (n = 3) or minimally invasive (n = 6) margin-negative resection by wedge resection (n = 5) or distal gastrectomy (n = 4). No nodal metastases were identified radiographically or on pathologic examination. The median tumor size was 2.5 [IQR 1.3-3.4] cm. All tumors showed at least in part typical glomus tumor morphology and smooth muscle actin expression. Aberrant synaptophysin expression was present in the two tumors initially classified as NET. Using the current WHO criteria, tumors were classified as histologically malignant (n = 1) and of "uncertain malignant potential" (n = 8). At a median follow-up of 15 [IQR 1-56] mo, all patients were asymptomatic and without recurrence. Two patients died of unrelated causes. No patients received adjuvant therapies. CONCLUSIONS: Our 20-year, single institution, 3-site experience with resected gastrointestinal glomus tumors suggests the rarity, predisposition to involve the gastric antrum, and potentially an indolent clinical behavior of many of these tumors. Long-term follow-up is warranted as some previously reported gastric glomus tumors have metastasized, including cases lacking morphologic evidence of malignancy. Surgical resection, with minimally invasive wedge resection alone, is likely sufficient for the management of most gastric glomus tumors.

Glomus Tumor of the Stomach: A Systematic Review and Illustrative Case Report.

INTRODUCTION: Glomus tumor (GT) is a rare mesenchymal neoplasm that can be found anywhere throughout the body, including the stomach. Our goal was to present a case and a systematic review of the literature, reporting clinical, radiological, surgical, and pathological features of the disease. METHODS: We reviewed Pubmed and SCOPUS for all case reports and case series published after 2000. Papers written in languages different from English and letters to the editor were excluded. Screening and data extraction were performed following the PRISMA guidelines. RESULTS: A total of 89 studies were included in the systematic review, consisting of 187 cases of gastric glomus tumor. Mean age was 52 (18-90); most patients were female (61%). The most common clinical presentation was epigastric pain (33.9% of cases). The gastric antrum was the most frequently involved site (75.3%). Mean tumor size was 2.82 cm (0.8-17). Preoperative diagnosis was achieved in 22 cases, mostly by endoscopic ultrasound (EUS)-guided biopsy. Wedge resection was performed in 62% of treated patients. Smooth muscle actin was expressed in all cases with available immunohistochemistry. Malignant GT was reported in 11 cases. DISCUSSION: Epigastric pain and bleeding were the most common symptoms at presentation in patients with diagnosis of glomus tumor. EUS-guided fine needle aspiration can be useful for preoperative diagnosis. Endoscopic elastosonography is a promising tool for the differential diagnosis of gastric submucosal lesions, including glomus tumors. The treatment of choice is wedge resection with adequate free margins. A laparoscopic approach is warranted when technically feasible. Since malignant gastric GTs have been described, long-term follow-up is suggested after surgical excision.

Glomangioma in the hand: diagnosis, treatment, and challenges.

INTRODUCTION: In this paper, we have analysed all hand glomangioma cases referred to our clinic in the context of symptoms, time to diagnosis, and the role of surgical resection of the lesion. MATERIAL AND METHODS: We have collected the following data: the presence of risk factors, manifestation, time to diagnosis, the treatment applied, and follow-up of patients. RESULTS: We have collected medical records from six patients, three males and three females. The median age was 45 (IQR: 29.5-65.75). The main symptom in all patients was severe pain and tenderness. The first-choice physician(s) were: general practitioners, general surgeons, and neurologists. The median time to diagnosis was 7 (IQR: 5-10) years. The main complaint of our patients was severe pain - 9 (IQR: 9-10) on the VAS scale, which was significantly alleviated after surgical treatment - 0 (IQR: 0-0; p = 0.043). CONCLUSIONS: Extremely long times to final diagnosis, and excellent outcomes of surgical treatment, highlight the necessity of raising awareness of glomangiomas among clinicians.

[Glomuvenous malformation: a clinicopathological analysis of 31 cases].

Objective: To investigate the clinicopathological features of glomuvenous malformation (GVM). Methods: Thirty-one cases of GVM diagnosed at the Henan Provincial People's Hospital from January 2011 to December 2021 were collected. Their clinical and pathological features were analyzed. The expression of relevant markers was examined using immunohistochemistry. The patients were also followed up. Results: There were 16 males and 15 females in this study, with an average age of 11 years (range, 1-52 years). The locations of the disease included 13 cases in the limbs (8 cases in the upper limbs, 5 cases in the lower limbs), 9 cases in the trunks, and 9 cases in the foot (toes or subungual area). Twenty-seven of the cases were solitary and 4 were multifocal. The lesions were characterized by blue-purple papules or plaques on the skin surface, which grew slowly. The lumps became larger and appeared to be conspicuous. Microscopically, GVM mainly involved the dermis and subcutaneous tissue, with an overall ill-defined border. There were scattered or clustered irregular dilated vein-like lumens, with thin walls and various sizes. A single or multiple layers of relatively uniform cubic/glomus cells were present at the abnormal wall, with scattered small nests of the glomus cells. The endothelial cells in the wall of abnormal lumen were flat or absent. Immunohistochemistry showed that glomus cells strongly expressed SMA, h-caldesmon, and collagen IV. Malformed vascular endothelial cells expressed CD31, CD34 and ERG. No postoperative recurrence was found in the 12 cases. Conclusions: GVM is an uncommon type of simple venous malformation in the superficial soft tissue and different from the classical glomus tumor. Morphologically, one or more layers of glomus cells grow around the dilated venous malformation-like lumen, which can be combined with common venous malformations.

[A Case of Primary Liver Cancer and Gastric Glomus Tumor Diagnosed Preoperatively and Treated with Liver Segmentectomy and Local Gastrectomy].

A man in his 70s was concurrently suspected of having a submucosal tumor(SMT)of the stomach and a liver tumor during a medical examination. Abdominal contrast-enhanced CT scan revealed S8 hepatocellular carcinoma(HCC)and an SMT of the stomach, which was strongly enhanced from the early to the later phase. Upper gastrointestinal endoscopy revealed a 20 mm SMT in the antrum of the stomach. Endoscopic ultrasonography showed a hyperechoic tumor in the fourth layer of the gastric wall. T2-weighted MRI showed a 25 mm SMT in the antrum of the stomach with a faint high signal intensity compared with that of the gastric wall. The patient was diagnosed with HCC and gastric glomus tumor, and a liver segmentectomy and a local gastrectomy were performed. Immunohistochemistry of the SMT revealed the expression of α-SMA but no expression of desmin, c-kit, CD34, or S-100. Therefore, a diagnosis of a Glomus tumor of the stomach was made. Gastric Glomus tumors are very rare; therefore, we have reviewed some citations and would like to discuss our case.

A rare combination of tumor-induced osteomalacia caused by sinonasal glomangiopericytoma and coexisting parathyroid adenoma: case report and literature review.

BACKGROUND: Tumor-induced osteomalacia (TIO) is a rare, acquired disease of renal phosphate wasting and disturbed vitamin D homeostasis as a result of the action of a phosphaturic protein - FGF-23, produced by a neoplasm. Although the clinical and biochemical profile of the syndrome is characteristic, it remains underreported and unrecognized by clinicians. Hyperparathyroidism is rarely associated with oncogenic osteomalacia, but it should be considered because of potentially life-threatening hypophosphatemia caused by both conditions. CASE PRESENTATION: We report a case of a 42-year-old woman admitted to the Department of Otolaryngology of the Military Institute of Medicine in Warsaw for the endoscopic resection of hormonally active glomangiopericytoma extending into the anterior skull base. She presented with a 5-year history of musculoskeletal pain and progressive weakness of the extremities which finally led her to become bedridden. After the excision of the tumor her symptoms and laboratory results gradually improved except increasing PTH serum levels. Further examination revealed a parathyroid proliferative tumor, which was surgically removed. The patient walked without aids at follow-up 16 months after the surgery. CONCLUSIONS: This case is unusual because of tumor-induced osteomalacia and parathyroid adenoma occurring concomitantly. Further investigations of FGF-23 and PTH interplay should be conducted to elucidate the pathogenesis of hyperparathyroidism and tumorigenesis in some cases of TIO. By presenting this case, we wanted to remind clinicians of a rare and misdiagnosed paraneoplastic syndrome and highlight the importance of monitoring PTH concentrations during the follow-up of patients with TIO.

Clinico-epidemiological profile and management outcome of subungual digital glomus tumor-Indian experience.

BACKGROUND: Glomus tumors are rare tumors arising from the mesenchymal smooth muscle cells of the glomus body. They are extremely painful tumors but because of their subungual location, remain mostly underdiagnosed. AIM: To characterize the demographic, clinical, onychoscopic, radiological features and management outcome of subungual glomus tumor. Material & methods- 15 patients with a total of 16 subungual glomus tumors were evaluated and their demographic data, history, clinical features, investigations, treatment, and follow-up were analyzed. RESULTS: Glomus tumors had a female preponderance (11/15) with thumb being the commonest site. All patients presented with intractable pain. Nail discoloration was observed in 11/16 (68.8%) lesions and nail plate deformity in 6/16 (37.5%) lesions. Common features on onychosocpy were pink glow and linear vascular structures. Doppler sonography and/or magnetic resonance imaging confirmed the diagnosis of glomus tumor in all the lesions. Surgical excision was done under local anesthesia using a trans-ungual approach and no recurrence was seen. CONCLUSIONS: High index of suspicion, meticulous clinical assessment along with radiological investigations can help in the early diagnosis. Complete surgical excision is the treatment of choice to prevent recurrence.

Primary Adrenal Glomus Tumor: The First Case Report.

We report a unique case of a 25-year-old man who was incidentally identified to have a mass in the left adrenal gland region by computerized tomography scan. The image revealed a 1.8 × 1.6 cm, well-defined adrenal mass with moderately homogeneous enhancement. The mass was laparoscopically completely removed, and the diagnosis of a solid variant glomus tumor of the adrenal gland was rendered. There were no histologic features indicating atypia or malignance and no primary tumors anywhere else. The patient was free of disease at the 6-month follow-up. To our knowledge, this is the first case of primary adrenal glomus tumor described in the literature.

Risk Factors Associated With Misdiagnosis of Digital Glomus Tumor: A Retrospective Cohort Study.

OBJECTIVE: Glomus tumors are benign with unique triad of symptoms; however, the delayed diagnosis of these tumors is common. We investigated the possible risk factors for the misdiagnosis of digital glomus tumors, with an aim to treat these patients on time. METHODS: We conducted a retrospective cohort study of 104 patients with digital glomus tumors from October 2009 to February 2021. Data pertaining to sex, age, tumor locations, symptoms, imaging modalities, and clinical departments visited by the patients were extracted and analyzed through logistic regression. RESULTS: The duration of delayed diagnosis ranged from 3 months to 40 years (mean, 5.5 ± 6.5 years). The total misdiagnosis and recurrence rate are 34.6% and 3.8%, respectively. On the multivariate logistic regression, the misdiagnosis of digital glomus tumor was significantly associated with the clinical departments visited by the patients ( P < 0.001). The risk of misdiagnosis of nonhand surgery department visit is 179.741-fold higher than that of hand surgery department visit. CONCLUSIONS: The misdiagnosis rate of digital glomus tumor was closely related to the clinical departments visited by the patients. Hand surgeons are the first choice for the treatment of the tumor.

Glomus tumors around or in the knee: a case report and literature review.

BACKGROUND: Glomus tumors commonly affect the extremities, especially subungual. And glomus tumors rarely occur around knee, which are often misdiagnosed. A lack of experience with glomus tumors is likely the cause. CASE PRESENTATION: A 42-year-old female presented with continuous dull pain of right knee for the past 7 years. Severe pain was experienced after walking a few hundred meters or climbing up or down stairs. The patient had a slight limp, and the lateral superior aspect of her right knee was tender to palpation. The range of motion and skin around her right knee were normal. Magnetic resonance imaging revealed a well-defined abnormal lesion confluent with the periosteum on the femoral lateral supracondylar. She was finally diagnosed with glomus tumor according to pathological results. After surgery, the pain disappeared, and the patient was discharged three days postoperatively. At the 18-month follow-up visit, the patient reported sustained pain relief, and regular follow-ups were continued. Additionally, 30 published reports documenting 36 cases of glomus tumors around the knee were reviewed, which showed that 20% of all reported cases of glomus tumor around the knee had a history of trauma. The median age for male with glomus tumor was greater than that of female; however, the median duration of illness between the two groups was equivalent. The mean diameters of glomus tumors ranged from 4 to 65 mm, and locations around the knee included the knee joint cavity, soft tissue (e.g. popliteal fossa, patellar tendon, iliotibial band, and Hoffa's fat pad), distal femur, and proximal tibia. CONCLUSION: Literature review demonstrated that no significant differences were found between male and female with glomus tumor in regard to location (left or right side) and illness duration. It was noting that a history of trauma may be a cause of glomus tumor and approximate 94.4% of glomus tumors was benign. The most effective therapy accepted for glomus tumors is complete surgical excision, and recurrence was rare after complete surgical excision.

Tracheal Glomus Tumor: A Case Report with CT Imaging Features.

BACKGROUND AND OBJECTIVES: Glomus tumors are rare benign tumors. The majority of them affect the skin the most and are rarer in the trachea, where the glomus bodies may not be present. Only scarce reports of tracheal glomus tumors have been reported solely with case reports of relevant articles. MATERIALS AND METHODS: A 53-year-old man, with a free previous medial history, presented to our hospital with tracheal mass which was incidentally found. He did not complain of any specific symptoms associated with the tracheal tumor. The contrast-enhanced chest computed tomography (CT) revealed an avid enhancing nodular lesion, which is similar to blood vessels, in the trachea, 3 cm above the carina level without definite airway obstruction. RESULTS: Successful tracheal resection and end-to-end anastomosis were performed on the patients; therefore, the final post-operative pathologic findings revealed a benign tracheal glomus tumor. The follow-up CT scan four months after operation showed complete removal of the tumor. CONCLUSION: Tracheal glomus tumors, even rare entities, could be considered as a differential diagnosis if a highly enhancing mass appears on CT images.

Minimally Invasive Transungual Tumors Enucleation With Nail Preservation for Subungual Glomus Tumors.

BACKGROUND: Glomus tumors are benign neurovascular neoplasms, most commonly located in the subungual region. These subcutaneous nodules are characterized by intense pain and temperature sensitivity. Although surgical excision is the curative treatment, permanent nail deformity and recurrence are not uncommon after conventional surgery. OBJECTIVE: This study evaluated the long-term efficacy of a modified transungual approach with nail preservation for removing subungual glomus tumors. MATERIAL AND METHODS: Thirty-nine patients with clinically diagnosed and histopathologically proven glomus tumors treated at a medical center over a 13-year period (2007-2019) were retrospectively evaluated. RESULTS: The 39 patients included 28 women and 11 men (ratio, 2.5:1), with a mean age of 48.9 years. Thirty-four tumors were located in the fingers and 5 in the toes. All patients returned to normal activity soon after the surgery. At a mean follow-up of 28.8 months, all were pain free or experienced minimal pain and were satisfied with treatment outcomes. One patient (2.6%) experienced recurrence. No nail deformity was observed. CONCLUSION: The transungual approach with nail plate preservation is a novel and effective method for removing subungual glomus tumors with reduced postoperative pain and rapid healing, while preserving or improving the configuration of the nail.

Glomangiomatosis of the Upper Extremity.

We describe a 43-year-old woman with a 23-year history of recurrent extradigital glomus tumors (glomangiomatosis) of the hand and forearm. She presented with a typical presentation of pain, tenderness to palpation, and hypersensitivity of the affected regions. After surgical resection, she continued to present with new locations of tumor burden and progressively malignant features of the tumors. This exceedingly rare presentation highlights the importance of vigilantly monitoring patients for recurrence with glomangiomatosis.

[Cardiac glomus tumor: An unusual localization of pericytic (perivascular) tumor]. / Tumeur glomique intracardiaque : une tumeur péricytaire (périvasculaire) de localisation inhabituelle.

Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization. However visceral locations have been described. Cardiac location is exceptional with only one case reported in literature. Here, we report the case of cardiac glomus tumor (glomangiomyoma) developed in the left ventricle in a 34 year-old patient, diagnosed after chest pain. The length of tumor was 4cm in greatest dimension. Histologically, the tumor concerned both round glomus cells and smooth muscle cells with prominent branching thin-walled vessels. By immunohistochemistry, these two contingents exhibited diffuse expression of smooth muscle actin and heterogeneous expression of H-caldesmone whereas cytokeratins, melanocytic markers and chomogranine were negative. Next Generation molecular analysis using RNA sequencing highlighted the characteristic MIR143-NOTCH gene fusion witch supports the diagnosis of glomus tumor. In this observation, we recall histological and immunohistochemistry features of glomus tumor and we make a synthesis concerning the molecular data recently described in sporadic glomus tumor.

[Gastric glomus tumors. Interest of ultrasound endoscopic biopsies for the diagnosis. About two cases]. / Les tumeurs glomiques gastriques. Intérêt des biopsies sous échoendoscopie pour le diagnostic. À propos de deux cas.

Glomus tumors are rare mesenchymal tumors, mostly benign, although a few malignant cases have been described in the literature. These tumors are usually localized subcutaneously or subcutaneously, however they may exceptionally be gastric localized. We report two new observations diagnosed on biopsies performed by echo endoscopy. The first was a 66-year-old man and the second a 78-year-old man. In both cases, the lesions were on astral, nodular, and very limited location. The biopsies revealed a tumor proliferation of trabecular architecture, composed by monomorphic cells, with eosinophilic cytoplasm, medium size, with a rounded, regular nucleus, without mitosis or necrosis, with distended and branched capillaries on the periphery. In immunohistochemical studies, tumor cells expressed smooth muscle actin and caldesmone. The proliferation index KI 67 was very low. In practice, however, preoperative diagnosis remains difficult because there is no typical appearance in imaging or echo-endoscopy to distinguish them from other gastric parietal tumors. To date, there is no consensus on the therapeutic management of gastric glomus tumors. However, endoscopic resection by dissection under the mucosa associated with endoscopic monitoring seems to be a method of choice as well as a way to postpone open or laparoscopic surgery, especially when these tumors are small (<30mm).

Eponychial flap elevation method for surgical excision of a subungual glomus tumor.

The clinical symptoms of glomus tumors are pain, tenderness, nail discoloration, and cold intolerance. The only treatment for subungual glomus tumors is surgical removal. In this clinical study, we present an eponychium preserving method as a new innovative surgical approach for subungual glomus tumor. We retrospectively reviewed the data of 27 patients with subungual glomus tumors who underwent tumor resection using the eponychial flap elevation method between March 2010 and 2019. After the excision of the subungual glomus tumor using the eponychial flap elevation method, wound problems, severe pain, and nail deformity other than a minimal scar were not noted. The innovative eponychial flap elevation method is a simple and straightforward method for the management of subungual glomus tumors; the tumors can be sufficiently exposed and completely excised using this method while avoiding secondary nail dystrophy. This method can be another option among several methods of subungual glomus tumor removal.

Ultrasound features of the subungual glomus tumor and squamous cell carcinomas.

BACKGROUND: Nail tumors often pose a significant challenge for the clinician, as they are burdened by a high delay in diagnosis and paucity of clinical signs in the early stage. In most cases, the onset of pain or nail plate dystrophy leads to imaging investigations such as plain radiography, magnetic resonance imaging, and ultrasonography (US) when surgery is pending. MATERIAL AND METHODS: A 2-year monocentric study evaluated the histologically confirmed cases of subungual glomus tumor (GT) and squamous cell carcinoma (SCC) with a pre-surgical US assessment, and reviewed their sonography features. A total of 7 digital GTs and 6 SCCs of the nail were collected. RESULTS: GT and SCC are easily distinguishable on US. Compared to SCC, GT was positively associated with an ovoid shape, well-defined borders, bone cup-scalloping without cortical erosion, and a rich intralesional vascular pattern (P < 0.05). Vice versa, SCC was significantly correlated with irregular shape, ill-circumscribed margins, an infiltrative growth pattern with peripheral hypoechoic foci, posterior acoustic shadow, cortical erosion, and a highly vascular multipolar peripheral pattern (P < 0.05). CONCLUSION: The rapid assessment of GT and SCC sonographic features may contribute to reduce diagnostic delay, expedite management, and decrease the risk of surgical relapse.

Transungual Excision of Glomus Tumors: A Treatment and Quality of Life Study.

BACKGROUND: Glomus tumors are rare benign painful tumors, frequently found in the subungual region. Complete surgical excision is essential for relief of symptoms. The main postoperative complications are tumor recurrence and nail dystrophy. OBJECTIVE: To evaluate the long-term outcome and the impact on quality of life (QoL) of glomus tumors after a transungual approach. MATERIALS AND METHODS: A retrospective cohort study was conducted. Twenty-six patients underwent transungual excision of subungual glomus tumors. A self-administered questionnaire was sent to evaluate the postoperative outcome. Glomus tumor-related QoL was investigated using modified nail psoriasis (NPQ10) and onychomycosis questionnaires. RESULTS: A response rate of 85% was achieved. After a mean follow-up of 63 months after transungual excision of the tumor, the mean Numeric Pain Rating Score had improved from 7.9 (±SD 1.8) preoperatively, to 0.8 (±SD 1.9) (p < .000). Quality of life improved significantly: the mean NPQ10-score improved from 5.5 (±SD 3.4) to 0.64 (±SD 2.1) (p < .000). Nail-related sequelae were not reported in any of the patients. CONCLUSION: Our study showed that glomus tumors cause impairment on QoL, mostly due to severe pain. Surgical excision with the transungual approach is an effective treatment, without permanent damage to the nail unit that gives relief of pain and improves QoL.

[Clinicopathological characterization of primary pulmonary and tracheal glomus tumors].

Objective: To investigate the clinical pathological features of primary pulmonary and tracheal glomus tumors. Methods: The clinical and pathological features of 11 cases (4 cases from Shanghai Pulmonary Hospital, Tongji University School of Medicine, China and 7 cases from Fudan University Shanghai Cancer Center, China) of respiratory glomus tumor diagnosed from 2010 to 2019 were analyzed, and reviewed in light of the relevant literature. Results: In the 11 cases, there were 5 males and 6 females, with the onset ages of 29‒66 years (median age of 43). Six tumors were located in the lung, and 5 in the trachea. The tumor diameters ranged 1.0‒7.5 cm, with the average diameter of 2.6 cm. At low magnification, the tumors were diffuse or lobulated in shape. The tumor cells composed of sheets of oval to short spindle cells, with sharply defined cell border and prominent branching thin-walled vessels. Among the 4 benign glomus tumors, one was classified as benign symplastic glomus tumor owing to the hyperchromatic or degeneration nuclei. Two cases were classified as glomus tumors of uncertain malignant potential, on the account of cellular atypia and rare atypical mitotic figures. Five cases were classified as malignant glomus tumors, owing to the tumor necrosis, vascular invasion, marked nuclear atypia, prominent nucleoli and brisk mitoses (2-20/10HPF) including pathological mitotic figures. The tumor cells showed strong immunostaining for SMA, vimentin, type Ⅳ collagen and caldesmon to different extents, while CD34, cytokeratin and S-100 stains were negative. One of the cases was positive for desmin, and one case positive for synaptophysin. Follow-up information was available in 8 patients with the duration ranging from 6 to 95 months. At the end of the follow-up, 6 patients were alive without recurrence or metastasis, and two of the patients with malignant glomus tumors died. Conclusions: Primary pulmonary and tracheal glomus tumors is rare. Among the reported cases, malignant glomus tumor is the most frequent, followed by benign glomus tumors and uncertain malignant potential glomus tumors. Glomus tumors show sheet-like growth pattern and clusters of round epithelioid cells with numerous vascular spaces. They can be easily misdiagnosed as carcinoid tumor. The final diagnosis should be combined with immunohistochemical staining, such as SMA, caldesmon and vimentin.