Glomus Tumor Research Articles

Infiltrative fibrous lesion of the facial nerve mimicking a facial nerve tumor.

To report three cases of facial nerve lesions that were clinically expected to be facial nerve tumors but showed fibrotic infiltration without any apparent signs of a specific tumor on histopathological findings. We also aimed to investigate the clinical characteristics of these cases. Medical records of patients who underwent surgery for facial nerve lesions were reviewed. All three cases initially had House-Brackmann (HB) grade IV-V facial nerve palsy. On radiological imaging, schwannoma or glomus tumor originating from the facial nerve was suspected. All patients underwent complete surgical removal of the neoplasm followed by facial nerve reconstruction using the sural nerve. The lesions were histologically confirmed as infiltrative fibrous lesions without tumor cells. In two cases, facial nerve palsy improved to HB grade III by nine months post-surgery, and there were no signs of recurrence on follow-up MRI. The other case, after 1year of follow-up, showed persistence of HB grade V facial nerve palsy without any evidence of recurrence. Fibrotic lesions of the facial nerve could mimic primary facial nerve tumors. Clinicians should consider this condition even when a facial nerve tumor is suspected.

Glomus tympanicum removal using transcanal endoscopic assisted surgery: An experience with six cases.

To evaluate an endoscopic approach in the management of glomus tumor, and also to investigate and evaluate its appropriateness and feasibility. Glomus tumors, also known as paragangliomas, are benign primary tumors of the middle ear. The advent of endoscopic ear surgery has provided new dimensions to the management of this highly vascular tumor. Retrospective analysis of six patients of glomus tympanicum, operated between July 2014 and June 2019, with modified Fisch classification Type A and B1, who were managed by a retroauricular transcanal endoscopic approach. Preoperative and postoperative analysis was done for these patients. The chief complaint was pulsatile tinnitus, which disappeared in five cases and reduced in severity in one of them. Hearing was improved with reduction in air-bone gap in all the cases. No major complications or recurrence were observed in any of the patients after 12 months of follow-up. This endoscopic approach serves as a safe and reliable technique for tumor removal. It thus provides postoperative comfort for most of the patients.

Gene fusions in superficial mesenchymal neoplasms: Emerging entities and useful diagnostic adjuncts.

Cutaneous mesenchymal neoplasms are diagnostically challenging because of their overlapping morphology, and, often, the limited tissue in skin biopsy specimens. Molecular and cytogenetic techniques have identified characteristic gene fusions in many of these tumor types, findings that have expanded our understanding of disease pathogenesis and motivated development of useful ancillary diagnostic tools. Here, we provide an update of new findings in tumor types that can occur in the skin and superficial subcutis, including dermatofibrosarcoma protuberans, benign fibrous histiocytoma, epithelioid fibrous histiocytoma, angiomatoid fibrous histiocytoma, glomus tumor, myopericytoma/myofibroma, non-neural granular cell tumor, CIC-rearranged sarcoma, hybrid schwannoma/perineurioma, and clear cell sarcoma. We also discuss recently described and emerging tumor types that can occur in superficial locations and that harbor gene fusions, including nested glomoid neoplasm with GLI1 alterations, clear cell tumor with melanocytic differentiation and ACTIN::MITF translocation, melanocytic tumor with CRTC1::TRIM11 fusion, EWSR1::SMAD3-rearranged fibroblastic tumor, PLAG1-rearranged fibroblastic tumor, and superficial ALK-rearranged myxoid spindle cell neoplasm. When possible, we discuss how fusion events mediate the pathogenesis of these tumor types, and we also discuss the related diagnostic and therapeutic implications of these events.

Gastric glomus tumor on EUS-FNA-based cytology: clinicopathologic and immunohistochemical features of 4 cases, including 1 case with associated MIR143HG-NOTCH2 fusion gene.

Gastric glomus tumor (GT) is a rare submucosal tumor for which the preoperative diagnosis can be challenging. We report the cytomorphologic and immunohistochemical features of 4 gastric GTs diagnosed by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) cytology. Files were searched to identify gastric GTs diagnosed by EUS-FNA between 2018 and 2021. A total of 4 cases of gastric GTs (3 men and 1 women; mean age, 60 years) were included. Three GTs were located in the gastric antrum and one in the gastric body. Their size ranged from 2 to 2.5 cm. Three patients presented with epigastric discomfort and one with chest wall discomfort. Rapid on-site evaluation was performed for 3 cases; the findings for all 3 were indeterminate. The smears were moderate to highly cellular and showed loose clusters of evenly distributed small- to medium-size bland tumor cells. The tumor cells had centrally located round to oval nuclei with inconspicuous nucleoli and scant to moderate amount of eosinophilic to clear cytoplasm. Examination of the cell blocks revealed branching small vessels surrounded by small- to medium-size cells. The neoplastic cells were positive for smooth muscle actin and synaptophysin and negative for AE1/AE3 and S-100. C-KIT and CD34 were variably positive. Ki-67 was <2% positive. In 1 case, the fusion panel-solid tumor (50 genes) revealed the MIR143HG-NOTCH2 fusion gene. Smears and cell block preparation revealed angiocentric sheets of uniform, small round to oval tumor cells with pale to eosinophilic cytoplasm, intermingled with endothelial cells. The differential diagnosis of gastric GTs on rapid on-site evaluation includes neuroendocrine tumors and epithelioid or spindled cell neoplasms. Immunohistochemical and molecular studies can be helpful in the preoperative diagnosis of gastric GT.

Glomus jugulare tumor presenting as papilledema – A rare case report

A 54-year-old male presented with headache and blurring of vision in both eyes for 2 months. Ocular examination showed bilateral lateral rectus palsy with papilledema, suggestive of raised intra-cranial pressure. A computed tomography scan of the brain revealed an expansile infiltrating soft tissue lesion causing lysis of the right petrous and jugular fossa, suggestive of a Jugular fossa tumor. The patient’s blood pressure was high at all times despite regular medications because of the glomus tumor’s supposed release of catecholamines. Elective surgery was planned; however, the patient developed a fatal tumor rupture within 4 months of diagnosis, for which emergency neurosurgical intervention and intensive care support were given, but he succumbed to death. Tumors obstructing the jugular outflow can cause increased intra-cranial pressure and vision loss, requiring prompt neurosurgical intervention.

Glomus tumor mimicking as cervical radiculopathy

Glomus tumors are rare, painful, and usually benign neoplasms typically occurring at the subungual aspect of digits. Patients usually complain of extremely painful episodes. Symptoms occur spontaneously or elicited by local compression and cold environment, in special if the tumor has an acral location. We describe a case of cervical radiculopathy caused by glomus tumor which developed spontaneously causing pain at the right index finger. Pain was excruciating, which increased on touching and sudden striking on objects. There was no neck pain, no sensory loss, and no weakness. Pain radiates to the elbow and sometimes shoulders. The patient was given trial of neuropathic medications without much relief. Nerve conduction studies were normal. Magnetic resonance imaging spine minimal disk bulge at C5–C6 level with no neural compression. On examination, the neck movements were normal and power, tone, and reflexes were normal. Neurological examination was normal. Severe tenderness on palpating the right index finger nail. MRI of the right index finger revealed a glomus tumour. Glomus tumor was diagnosed and the patient underwent surgical excision. There can be a delay in diagnosis in glomus tumours.Cervical radiculopathy may be diagnosed erroneously in patients if careful physical examination and investigations are not done.

Glomus Tumor of the Index Finger: Multimodality Imaging in a Case Report

Glomus tumors are benign lesions developed at the neuro-myo-arterial glomus of the dermal-epidermal tissue and were first described in 1924 by Masson. This is a rare pathology, progressing very slowly, generally unrecognized, affecting young adults with a female predominance. These tumors are most commonly located in the fingers and represent 1-5% of soft tissue tumors of the hand. Imaging is important in the diagnosis and follow-up and mainly based on Doppler ultrasound and MRI. However, confirmation of the diagnosis needs a systematic anatomopathological analysis after surgical excision. We report the case of a 41-year-old woman, without any particular pathological history, who had a painful swelling of the distal index finger for 1 year. The diagnosis of glomus tumor was suggested by multimodal imaging, and surgical excision was performed to confirm the diagnosis.

Malignant glomus tumor of the head and neck: A National Cancer Database analysis.

e18078 Background: Malignant glomus tumors of the head and neck are rare malignancies originating from glomus bodies found within the skin, soft tissues, and mucosa of the head and neck. Malignant glomus tumors often arise from benign glomus tumors and rarely metastasize. In cases where metastasis had ocurred it was typically fatal. Another review of the malignant glomus tumor cases indicated that only a minority developed metastases. These findings, along with a general lack of knowledge regarding demographic and socioeconomic factors on glomus tumors warrants further investigation. The National Cancer Database (NCDB) was analyzed to identify demographic factors among patients and better understand the epidemiology of these tumors. Methods: A retrospective cohort analysis was performed using the 2004–2020 National Cancer Database including patients with a histologically confirmed malignant glomus tumor diagnosis (N = 42). Demographic factors such as race, age, sex, Hispanic status, insurance status, Charlson-Deyo score, facility type, and distance to facility were analyzed by descriptive statistics. Results: A total of 42 patients with a confirmed malignant glomus tumor diagnosis were identified in the National Cancer Database (NCDB) between 2004 – 2020. The average age of diagnosis was 60.9 years (SD = 13.7, range = 36 - 90 years). The aortic body and other paraganglia were the most common primary tumor sites (57.1%). Most patients were White (69.0%) or Black (28.6%). Most individuals (88.1%) had a Charlson-Deyo comorbidity score of 0. A higher percentage of patients were in the bottom quartile of income earners (31.4%). Fewer patients lived in metropolitan counties with populations less than 1 million compared to those that lived in counties with a population greater than 1 million (52.5%). More patients were privately insured (47.6%) relative to those who were insured by Medicare/Medicaid or uninsured. The most common treatment facility type was an academic/research program (57.5%) and patients lived an average of 40.1 miles (SD = 65.6, range = 0.6 – 265.4) from their treatment facility. Conclusions: Before this study, there had been no previous report on the socioeconomic profile of patients with malignant glomus tumors. However, our findings indicate that these patients are more prone to having a low income and that White patients are more likely to be affected. No obvious connection exists between metastases and racial minority status. Additional research is needed to grasp how socioeconomic factors, such as low income and minority status, affect the risk, diagnosis, treatment, and overall survival of individuals with malignant glomus tumors.

ID: 216029 Digital Nerve Block for a Painful Glomus Tumor of the Finger Tip: A Case Report

Neurofibromatosis type 1 (NF1) is an autosomal dominant, tumor predisposition disorder that commonly occurs approximately once every 2,500th live births. Mutations of the NF1 gene, especially of neural crest cells, result in functional changes to growth and cellular regulators of neurofibromin, leading to uncontrolled cellular proliferation and increased cancer risk (4). One particular concern for NF1 is the generation of peripheral nerve tumors, including glomus tumors. The classic triad of symptoms of glomus tumors include paroxysmal spontaneous pain, point tenderness, and cold hypersensitivity (10). Generally, the primary management of these tumors is complete resection while attempting to preserve residual neurological function. However, surgical involvement may result in life-changing complications, requiring significant specialization of hand and foot surgeries. Thus, more conservative management should be considered.

Comparing Ultrasound with Magnetic Resonance Imaging in the Evaluation of Subungual Glomus Tumors and Subungual Myxoid Cysts

Introduction: Subungual myxoid cysts and subungual glomus tumors demonstrate characteristic features on magnetic resonance imaging (MRI) and ultrasound (US). It is not yet well-established whether US is congruent to MRI in diagnostic evaluation of these subungual lesions. Methods: Participants with clinical suspicion for subungual glomus tumors or subungual myxoid cysts were recruited. After clinical evaluation, participants underwent radiography, MRI, and US plus biopsy, aspiration, or excision where possible. Differential diagnoses were revised after review of imaging, and imaging findings were compared to definitive diagnosis by pathology, aspiration, or clinical course. Results: All lesions were visible on both US and MRI and size estimates agreed between the two modalities. US and MRI findings of subungual glomus tumors and subungual myxoid cysts agreed with their known respective imaging characteristics. Conclusions: Diagnosis of subungual myxoid cysts and subungual glomus tumors agreed between US and MRI. We provide sample MRI and US imaging parameters for optimal evaluation of subungual myxoid cysts and glomus tumors. We demonstrate that subungual MRI evaluation can be performed without special equipment, allowing for evaluation by most radiology departments. Lastly, US is user-dependent and may be non-inferior for a sonographer familiar with subungual US.

Endoscopic resection of gastric glomus tumor: a case report and literature review

Abstract Objectives Gastric glomus tumor (GGT) is a rare mesenchymal tumor, mostly located in the gastric antrum region, and occasionally displays malignant behavior. Due to the lack of typical endoscopic or clinical features that distinguish GGT from other gastric submucosal tumors, the diagnosis is based primarily on postoperative histopathological and immunohistochemical findings. The first-line treatment is either local laparoscopic resection or open surgical intervention. Endoscopic resection techniques can also be used to successfully treat such lesions. Case presentation This case report presented a 39-year-old woman suffering from anorexia for the past 15 days. Barium upper gastrointestinal radiography suggested a benign space-occupying lesion in the antrum region. Endoscopically, a non-ulcerative, smooth submucosal lesion was observed in the greater curvature of the antrum. Endoscopic ultrasound (EUS) examination revealed a hypoechoic lesion with an anechoic zone in the center, originating from the muscularis propria layer of the stomach wall, which could be a gastrointestinal stromal tumor (GIST). Conclusions The lesion was successfully removed via submucosal tunneling endoscopic resection (STER). Based on histopathological and immunohistochemical findings, the patient was diagnosed with GGT.

Dermatologic Manifestations of Neurofibromatosis Type 1 and Emerging Treatments

Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome that increases one’s risk for both benign and malignant tumors. NF1 affects every organ in the body, but the most distinctive symptoms that are often the most bothersome to patients are the cutaneous manifestations, which can be unsightly, cause pain or pruritus, and have limited therapeutic options. In an effort to increase awareness of lesser-known dermatologic associations and to promote multidisciplinary care, we conducted a narrative review to shed light on dermatologic associations of NF1 as well as emerging treatment options. Topics covered include cutaneous neurofibromas, plexiform neurofibromas, diffuse neurofibromas, distinct nodular lesions, malignant peripheral nerve sheath tumors, glomus tumors, juvenile xanthogranulomas, skin cancer, and cutaneous T-cell lymphoma.

Cover Image

The cover image is based on the Original Article Oesophageal glomus tumours: rare neoplasms with aggressive clinical behaviour by Jacqueline E Birkness-Gartman et al., https://doi.org/10.1111/his.14888. Image Credit: Norm Barker.

Endoscopic Resection of a Gastric Glomus Tumour Using a Hybrid Approach of Endoscopic Submucosal Dissection and Endoscopic Full Thickness Resection: A Case Report

Endoscopic Resection of a Gastric Glomus Tumour Using a Hybrid Approach of Endoscopic Submucosal Dissection and Endoscopic Full Thickness Resection: A Case Report

Line-field confocal optical coherence tomography of cutaneous vascular lesions: Morphological assessment and histopathological correlations.

Cutaneous vascular lesions (VLs) are benign or malignant processes involving blood and/or lymphatic vessels, usually readily diagnosed with dermoscopy. However, cases showing unclear clinical/dermoscopic findings may require further investigations. Line-field confocal optical coherence tomography (LC-OCT) is a new, non-invasive imaging technique displaying high resolution and deep penetration. The aim of this study was to describe the LC-OCT features of the most common benign and malignant VLs and to correlate them with histopathological substrates. Clinical, dermoscopic, LC-OCT and histopathological images of VLs were retrospectively collected. Detailed LC-OCT description and histopathological correlations were produced for different types of VLs. The study included 71 VLs belonging to 50 caucasian patients [31 (62%) females; median age 56.8 (30-83) years] study lesions included 25 cherry haemangiomas, 15 angiokeratomas, 10 thrombosed haemangiomas, six pyogenic granulomas, five venous lakes, four targetoid haemosiderotic haemangiomas, four Kaposi's sarcomas and two extraungual glomus tumours. LC-OCT detected increased dermal vascularity, assuming different size and shape according to the particular type of VLs. LC-OCT criteria correlated well to established histopathologic findings. The results of our preliminary observations indicate that in vivo evaluation with LC-OCT may provide practical clues for the identification of the vascular nature of a lesion and its differential diagnosis.

Glomus tumor of volar pulp of little finger: A rare presentation

Abstract Glomus tumors are tumors with unknown etiology. Though the most common location is subungual region of distal phalanx of hand, occurrence in the volar pulp region is a rare presentation and less commonly reported. Herewith, we are reporting a case of glomus tumor of the volar pulp region of hand.

Clinical Characteristics and Results of Transungual Excision of Subungual Glomus Tumor

IntroductionGlomus tumor is rare and subungual location of the distal phalanx is the most common site. Surgical excision is the mainstay of treatment to relieve the symptoms. The objective of this study was to analyze the clinical characteristics and result of transungual excision of the tumor.MethodsA retrospective cross sectional study of was conducted among 15 cases of subungual glomus tumors operated with transungual excision over 8 years period in the department of Orthopedics. The clinical and demographic characteristics were noted from the case record. The outcome variables were assessed from case records, OPD visits, and telephone interviews and the results analyzed were pain relief, postoperative recurrence, nail deformity, and patient satisfaction. Data was analyzed by using descriptive statistical tools in SPSS-20.ResultsThe mean age of the patients was 32.6 years with the mean duration of symptoms 1.8 years. The mean tumor size was 3.4 mm. Pain and pinpoint tenderness were present in all 15 cases and positive cold sensitivity test and positive Hildreth’s test in 13 (86.7%) and 10 (66.7%) cases respectively. All cases had complete relief of pain within 2 weeks of the operation. There was no case of recurrence and nail deformity till a minimum of 1 year of follow up.ConclusionsThe diagnostic clinical triad of pain, point tenderness, and cold sensitivity are the most common findings. The transungual excision of the tumor can give rise to excellent results with complete relief of pain in all cases with no recurrence or nail deformity.

A Case of Glomus Tumor; Missed Diagnosis as Enchondroma For Previous 13 Years

Rare benign tumors called subungual glomus tumors that develop from the glomus body can cause sharp discomfort under the nail. This case involves a 38-year-old female patient who has been complaining of severe pain above her left thumbnail for 13 years. Under general anesthesia, a transungular technique was used to remove the tumor mass, and histology revealed a subungual glomus tumor. To lessen the damage and deformity to the nail bed, the tumor mass must be completely removed. To get the right diagnosis, it's crucial to consider specific clinical traits such as intense pain, regional soreness, and cold sensitivity. The diagnosis can be verified, and the tumor mass can be located with the use of a physical examination and imageology.

Lower extremity peripheral nerve pathology: Utility of preoperative ultrasound-guided needle localization before operative intervention.

Historically, the use of ultrasound (US) in the management of peripheral nervous system (PNS) pathology has been limited to diagnostic confirmation or guidance for interventional injections. This technical case series will demonstrate the utility and versatility of preoperative US-guided needle localization for the excision of lower extremity neuromas and other pathology of the PNS. Five patients with symptomatic lower extremity PNS tumors were retrospectively reviewed. This case series corroborates the technical nuances of localizing lower extremity neuromas by US-guided needle and wire placement prior to operative excision. This was achieved by a multidisciplinary team that included plastic surgery, neurosurgery, and radiology. Five patients had US-guided needle localization of a lower extremity PNS target prior to operative intervention. Three patients had lower extremity neuromas of varying origins, including the lateral femoral cutaneous nerve (LFCN), saphenous nerve, and sural nerve. The remaining two patients had a sciatic nerve sheath Schwannoma and a femoral nerve glomus tumor. Under sonographic visualization, a needle was advanced to the target perimeter and withdrawn, leaving behind a percutaneous guidewire. This technique simplified the marking of the nerve course prior to dissection and led to efficient intraoperative identification of all five PNS tumors without any complications. Preoperative US-guided needle localization led to safe, accurate, and efficient perioperative and intraoperative identification of neuromas and other PNS tumors of the lower extremity prior to excision. By reducing the challenges of nerve identification in a scarred tissue bed, this multidisciplinary approach may decrease postoperative patient morbidity.

Lower Four Cranial Nerves in the Management of Glomus Jugulare: Anatomical Study.

Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.