Tactics of surgical treatment of recurrent glomus tumors of the skull base: A clinical case.

AIM: The purpose of the study is to build effective tactics of surgical treatment of pathology, and to increase the effectiveness of surgical treatment of patients with this diagnosis by optimising preoperative preparation, and the correct sequence of actions that will help prevent relapse. METHODS: The leading approach to the study is the description of a clinical case, which will help to carefully consider this issue from all angles, analyse the methodology of patient examination, and build highly effective tactics of surgical intervention to eliminate tumour-like masses, minimising the risks of recurrence. The second clinical case was also considered, and a comparative analysis was carried out on certain parameters. RESULTS: The paper presents a clinical case with a practical aspect of surgical treatment of the pathology. The issue of detailed symptoms and manifestations of the disease is disclosed, and the course of surgical interventions is described in stages, in which no nerve is damaged, no complications are caused, and the desired result is achieved. The peculiarity of this case is the recurrence of the disease, and the ineffectiveness of previous treatment methods, including embolisation of the neoplasm and antromastoidotomy. CONCLUSIONS: The results obtained in the course of this study and the formulated conclusions are of great importance for surgeons who are faced with patients with the stated diagnosis.

Oesophageal glomus tumours: rare neoplasms with aggressive clinical behaviour.

AIMS: Glomus tumours are neoplasms with perivascular smooth muscle differentiation, which rarely occur in the oesophagus and may behave aggressively in this site based upon prior case reports. This study describes the clinicopathologic features of three oesophageal glomus tumours diagnosed at two large academic institutions between 1984 and 2022. METHODS AND RESULTS: Three cases of oesophageal glomus tumours were identified. Patients included two females and one male, with an age range of 19-65 years. All three tumours behaved in a malignant fashion, with metastases to various sites (lymph nodes, lung, pericardium, pleura, diaphragm, scalp). One patient developed an aorto-oesophageal fistula, resulting in a fatal haemorrhage. Tumours ranged in size from 4.5 to 8.1 cm. Histologically, all tumours had a multinodular, perivascular growth pattern. The neoplasms showed varying degrees of cytologic atypia and spindling, elevated mitotic activity (2-12 mitotic figures per 10 high-power fields), and necrosis was seen in in two cases. All tumours expressed smooth muscle actin by immunohistochemistry, and harboured NOTCH gene alterations (MIR143::NOTCH2 fusion in two cases; NOTCH3 rearrangement and NOTCH1 point mutation in one case). An ATRX splicing mutation in exon 10 was also identified in one case. CONCLUSION: Oesophageal glomus tumours pose diagnostic challenges, given their rarity at this site, but can be recognised by their characteristic perivascular growth pattern, round central nuclei, and supportive ancillary studies. Given the propensity for aggressive behaviour in this location, we recommend management by a multidisciplinary sarcoma team for optimal outcome.

Gastric glomus tumour: A case report.

Glomus tumours (GTs) are rare, mesenchymal neoplasms arising from the glomus body. Atypical sites, like the stomach, have been reported with extreme rarity since the usual location lies under the nail beds. We report a rare case of gastric GT in a 28-year-old female presenting with upper abdominal pain, intermittent haematemesis and melena. Contrast-enhanced computed tomography indicated a well-defined, heterogeneously enhancing lesion in the antral region of the stomach and a possibility of gastrointestinal (GI) stromal tumour (GIST). Upper GI endoscopy showed a wide-based lesion in the sub-mucosal plane with ulcerated mucosa. Laparoscopic excision of the tumour with primary closure of the defect was performed, with an uneventful post-operative course. Histopathological and immunohistochemical findings confirmed a gastric GT. Follow-up clinical and endoscopic examinations were normal. Gastric GTs should be a rare differential in patients with suspected GIST and upper GI bleed.

Glomangiomyoma of the neck in a child in Nepal: a rare case report and literature review.

BACKGROUND: Glomangiomyoma is a rare histological variant of glomus tumour. Clinically, it mimicks as a haemangioma and is challenging to diagnose. Its occurrence in the neck of a child has not been previously described. CASE PRESENTATION: A 3 year old girl presented with the complaints of painless progressive neck swelling in the right side for one and half year. Sonography, computed tomography (CT), magnetic resonance imaging (MRI), CT neck angiography and fine needle aspiration cytology (FNAC) were suggestive of vacular malformation i.e. giant haemangioma or arteriovenous malformation. The mass was removed in toto under general anaesthesia without postoperative complications. The histopathology confirmed it to be glomangiomyoma with haemangiopericytoma like features. CONCLUSION: It's an extremely rare variant of glomus tumour and may be the first report of a glomangiomyoma in the neck of a child. Despite a rare entity, it should be borne in mind during differential diagnosis.

Glomus tumours: Outcome based on tumour location in hand.

OBJECTIVE: To determine the clinical outcome of patients with glomus tumour in nail matrix and nail bed that were excised with transungual approach. METHODS: The case series was conducted at the Allied and District Headquarter Hospital, Faisalabad, Pakistan,from January 2012 to June 2014, and comprised patients with glomus tumour at finger tipswhose diagnosis was confirmed with biopsy results. Variables noted were the location of the tumour in the nail i.e. either nail matrix or the nail bed. RESULTS: Of the 8 patients in the study, 5(62.5%) had tumours located in the nail matrix and 3(37.5%) in the nail bed. Three (60%) patients with nail matrix tumour had prolonged pain sensation, whereas none (with nail bed lesion had prolonged pain sensation. CONCLUSIONS: There was no recurrence but anatomic location of the subungual glomus tumour at initial presentation can predict postoperative prolonged pain.

The use of ionising radiation for the purpose of arresting external haemorrhage from an intracranial mass lesion.

External bleeding from any form of tumour of the central nervous system is unusual. We present a case where this difficult problem was controlled by the use of Gamma Irradiation from a focused delivery system.

[Solitary subungual glomangioma]. / Glomangiome sous-unguéal solitaire.

BACKGROUND: Glomangiomas are benign vascular tumours. They are usually multiple, painless and extradigital. Herein we report a case of a solitary subungual glomangioma. PATIENTS AND METHODS: This 65-year-old woman presented with a history of bluish, asymptomatic, subungual lesions located in the lunula of her right thumb. Surgical exploration by a transungual approach showed a large bluish, well-circumscribed tumour, which was completely excised. Histological examination revealed numerous dilated blood vessels surrounded by aggregates of glomus cells, which was consistent with the diagnosis of glomangioma. DISCUSSION: Glomus tumours are benign tumours arising from glomus cells. Histopathologically, based on the predominant tissue type present, glomus tumours are classified as solid glomus tumours, glomangiomas or glomangiomyomas. The classical form usually consists of a painful erythematous nodule with exaggerated sensitivity to cold and pressure. The nails are frequently involved, with two sites of predilection: the matrix and the nail bed. Vascular forms of glomus tumours or glomangiomas have a different clinical presentation and are usually multifocal, bluish, painless and extradigital. Diagnosis is frequently based on histological examination. Our observation raises the question of differential diagnosis with regard to matrix melanocytic tumours (blue nevi or melanomas). CONCLUSION: We report the case of a solitary subungual glomangioma. Histological examination is necessary to rule out a clinically indistinguishable benign or malignant melanocytic tumour.

Extramural recurrence of tracheal glomus tumour following resection by rigid bronchoscopy.

Glomus tumour of the trachea is very rare neoplasm that is generally benign and arises most commonly from the distal portion of the respiratory tree. This report presents the case of a 67-year-old man who was referred to our institute for excision of a tracheal mass that had been found incidentally, and subsequently recurred extramurally. Initial contrast-enhanced computed tomography images of the chest revealed a nodular lesion in the trachea, 2.5 cm above the carina, that demonstrated strong enhancement similar to blood vessels. The tumour was excised by rigid bronchoscopy, but an extramural tracheal lesion was detected 18 months later. Tracheal resection and end-to-end anastomosis were performed, and histopathological examination confirmed the extramural lesion as recurrence of the tracheal glomus tumour. The histologic features and treatment are discussed.

Outcomes of the Transungual Approach in 56 Consecutive Digital Subungual Glomus Tumours.

Background: Glomus tumour is a painful small tumour of the glomus body commonly located under the nail bed. The aim of this study is to evaluate the correlation of clinical diagnosis with MRI findings, determine the prevalence of the tumour at different subungual locations and determine the differences in outcomes (if any) between a longitudinal and a transverse nail bed incision for excision of the tumour. Methods: This retrospective study of 56 subungual glomus tumour was conducted from May 2010 to December 2021. Data with regard to gender, age at presentation, digit involved, presenting symptoms, duration of symptoms, clinical signs, need for MRI, anatomical location, surgical approach (longitudinal versus transverse), histopathology result, period of follow-up and complications were recorded. Results: All 56 (100%) patients presented with classic triad of symptoms. The average duration of symptoms was 52.9 months (range: 3-204 months). Eleven (20%) tumours were in the sterile matrix, 38 (68%) at the junction of sterile and germinal matrix and 7 (12%) in the germinal matrix. The tumours were excised through the longitudinal incision in 31 (55.3%) patients and transverse incision in 25 (44.7%). One (1.8%) tumour was intraosseous that was diagnosed intraoperatively and excised successfully. Average follow-up was 35.4 months (range: 6-120 months). There was no difference in outcomes (pain or nail deformity) between the two incisions. One patient (1.8%) has persistent pain that was due to a missed satellite lesion in the same digit. This was excised later with resolution of symptoms. There were no recurrences and all patients were cured after excision of tumour. Conclusions: Diagnosis of glomus tumour is usually clinical, and most are located at junction of sterile and germinal matrix. Tumour can be excised either by longitudinal or transverse nail bed incisions without any change of treatment outcome. Level of Evidence: Level IV (Therapeutic).

Unlocking the diagnostic riddle: Beyond arthritis in DIP joint deception.

Glomus tumour is a rare benign neoplasm arising from specialized neuromyoarterial plexus situated in the subungual region. Here, we present a 36-year-old male who had severe pain in the distal part of left third finger mimicking distal interphalangeal joint arthritis (DIP). On evaluation, he was found to have severe tenderness just distal to the DIP joint line. Also, he had positive cold sensitivity test. MRI showed high-signal intensity lesion in the dorsal aspect of left third digit. Glomus tumour was suspected based on these findings. Surgical excision was done followed by histopathological examination, confirmed the diagnosis. We present this case to raise awareness about this rare condition and the possibility of misinterpreting distal pain as DIP arthritis.

Glomus tumour: an institutional experience of 31 cases.

BACKGROUND: Glomus tumour is an uncommon soft tissue tumour which commonly occurs in the distal extremities, particularly the subungual region of the finger. Due to its rarity, there is a paucity of literature concerning glomus tumour. Therefore, this paper aims to report a case series based on our institution's experience. METHODS: A retrospective cross sectional study was performed in a single tertiary institution in Singapore. All patients diagnosed with glomus tumour confirmed on histology from January 2019 to October 2022 were included in the study. Patient demographics and clinical information (presenting signs and symptoms, tumour parameters and presence of recurrence) were retrieved from existing medical records. RESULTS: A total of 31 cases of glomus tumour were diagnosed from January 2019 to October 2022, and the relevant demographics and clinical presentation were reported. Majority of glomus tumours occurred in the finger (61.3%). Pain was present in almost all the cases (96.8%), while a lump was visible in less than half (48.4%). An average of 44.0 months elapsed before patients were properly diagnosed and treated. There were no cases of recurrence despite involved margins in three cases. CONCLUSION: Glomus tumour can be easily missed if clinicians do not have an index of suspicion for it, resulting in delayed treatment. Once diagnosed, glomus tumour can be treated with complete excision with good outcomes.

Genotype-phenotype associations in paragangliomas of the temporal bone in a multi-ethnic cohort.

BACKGROUND: Temporal bone paragangliomas are rare tumours with variable presentation that can be hereditary. Identification of clinical and genetic factors of aggressive tumour behaviour is important. OBJECTIVE: To determine the underlying genetic mutations and genotype/phenotype correlations in a multi-ethnic population of South Florida with sporadic temporal bone paragangliomas. METHODS: In a cohort of glomus tympanicum (GT) and glomus jugulare (GJ) cases, we assessed the frequency of pathogenic single nucleotide variants, insertions, deletions, and duplications in coding exons of genes that have been associated with paragangliomas (SDHB, SDHC, SDHD, SDHA, SDHAF2, RET, NF1, VHL, TMEM127, and MAX). RESULTS: None of the 12 GT cases had mutations. Among 13 GJ cases, we identified four mutation carriers (31%); two in SDHC, one in SDHB, and one in SDHD. All patients with pathogenic mutations were of Hispanic ethnicity, presented at a younger age (mean 27.5 versus 52.11 years), and with more advanced disease when compared to mutation-negative GJ cases.Conclusions and Significance: Mutations in the SDH genes are found in 31% of sporadic GJ. SDH-associated GJ had advanced disease and a 50% risk of metastasis. Our data supports emerging recommendations for genetic screening in all populations with GJ tumours as the genetic status informs management.

Glomus tumour of the nipple in a male patient.

A glomus tumour is a benign mesenchymal tumour. It is extremely rare in the breast. We report a case of glomus tumour of the nipple in a 54-year-old man. To the best of the authors' knowledge, this is the first case report of a glomus tumour of the nipple. We describe the different presenting symptoms, method of diagnosis and treatment.

Glomus tumour of the iris: A case report.

INTRODUCTION: Glomus tumours are rare benign tumours formed by modified smooth muscle cells arising from the glomus body. Glomus tumours occurring in the iris have not been previously reported. CASE DESCRIPTION: A 32-year-old woman presented with a 9-day history of blurred vision in her right eye. Ultrasound, computed tomography and magnetic resonance imaging confirmed the presence of a mass lesion within the iris. Surgery of the iris was performed and the tumour was removed. Histopathological analysis confirmed a glomus tumour. The patient remains clinically stable 5 months following surgery and has experienced no tumour recurrence. CONCLUSION: The findings from this case suggest that the typical symptoms of a glomus tumour may be absent in some cases, and that imaging examinations can help in understanding the extent of the lesion and the involvement of adjacent structures. Moreover, pathology and immunohistochemistry are crucial to confirm the diagnosis.

Glomic Tumour of the Stomach: A Rare Cause of Anaemia.

Glomus tumour is a rare benign mesenchymal neoplasm usually located in the dermis and subcutis, and most commonly found in the extremities. Glomus tumour in the gastrointestinal system is rare and its symptoms unspecific. The diagnosis of gastric glomus tumour is challenging due to the lack of specific findings on imaging and blood analysis, and so is usually based on histology and immunochemistry. We describe the case of a 22-year-old man admitted for pallor and anaemia, the diagnostic path, treatment and follow-up. The present case suggests that this rare entity should be considered in the differential diagnosis of gastric lesions. LEARNING POINTS: Glomus tumour is a rare benign mesenchymal neoplasm most commonly found in the extremities.Gastric glomus tumour (GGT) is rare and its symptoms unspecific.The diagnosis of GGT relies on histology and immunohistochemistry as imaging lacks accuracy.Treatment is usually surgical but follow-up should be considered due to its malignant potential.

Combination of cardiac and carotid glomus tumour: a rare case report.

The most common tumour of the heart is myxoma but paraganglioma (also called glomus tumour in extracardiac sites) in the cardiac position is the rarest of them. While this tumour accounts for 0.8% of all primary benign tumours, the combination of both neoplasms is an exceedingly rare occurrence. Herein, we present a case of combined carotid glomus tumour and left atrial paraganglioma tumour in which respiratory distress was the presenting symptom of cardiac type but carotid tumour was asymptomatic. The case underwent a two-step resection of the neck and cardiac mass with an uncomplicated postoperative course and in the 1year follow-up, no recurrence of tumour in both sites was found on physical exam and imaging studies.

Hand and Foot Glomus Tumors: Significance of MRI Diagnosis Followed by Histopathological Assessment.

Introduction Glomus tumors are benign lesions with hamartomatous proliferation in the neuromyoarterial glomus bodies. Glomus tumors are small, reddish, painful blue nodules usually underneath the fingernail. Objectives This study is mainly focusing on magnetic resonance imaging (MRI) findings of glomus tumors on T1, T2, short inversion time inversion recovery (STIR), and post-gadolinium images. Further study of clinical and histopathological findings to support the radiological diagnosis. Material & methods The retrospective study included an elaborate study of MRI imaging findings of 24 cases of glomus tumors of the hand and leg at a tertiary care center in Udaipur. Patients with imaging findings confirmed on histopathology were included in the study. MRI study was conducted using a 3-T MR unit and a high-spatial-resolution module. Results A total of 24 cases of glomus tumors were diagnosed during the six years between January 2015 and November 2020. Out of 24 patients, 14 were female and 10 were male. The most common site of involvement was a hand, followed by a foot. All 24 cases showed isointense to hyperintense lesions on T2-weighted images with a conspicuous hyperintense lesion on STIR images. Further histopathological examination confirmed the diagnosis showing nests of monomorphic tumor nuclei arranged in a perivascular pattern. Conclusion Glomus tumors can present with variable pain. A high index of suspicion is needed for diagnosis. Contrast MRI has a significant role in the diagnosis of glomus tumors. The clinical and histopathological picture further confirms the diagnosis. MRI imaging is further supportive to differentiate postoperative fibrosis from residual or recurrent tumors.

Glomus Tumour of Hand--A Commonly Misdiagnosed Pathology: A Case Series.

Introduction: Glomus tumours are benign neoplasms arising from the glomus body, a network of specialized neuromyoarterial structures containing arteriovenous anastomosis and regulating temperature. It is often misdiagnosed as paronychia, arthritis, traumatic sequelae, and many other conditions including psychiatric misdiagnosis. A typical triad of paroxysmal pain, point tenderness, and cold intolerance characterize it. Surgical excision is the treatment of choice, either by transungual or lateral approaches. Materials and Methods: We carried out a retrospective study of cases operated in our department in the last 4 years. Patients are evaluated based on either clinical parameters (Love test, Hildreth test, and cold insensitivity) or radiological parameters (X-ray and magnetic resonance imaging). Parameters such as age, gender, tumour side, presenting complaints, duration of symptoms, diagnostic modality, follow-up duration, recurrence, and postoperative nail deformity were analysed. Visual analogue scale (VAS) score was the primary statistical parameter, and the change in VAS score following surgery was analysed with paired t test. Result: Mean preoperative VAS was 7.75 ± 0.5; in a total of four patients, and following surgery, VAS was reduced to a mean of 1 ± 1.5. Paired t test on the change of VAS score following surgery showed a significant difference in the VAS score (P = 0.002838). The average age was 39.25. The male-to-female ratio was 1:3, and the mean follow-up was 16 months (range 2-48 months). The mean duration of symptoms was 5.75 years (range 2-10 years). Two cases were in the right-hand side, and two cases were on the left-hand side; the thumb was most commonly involved with 50% (n = 2) incidence. Conclusion: Glomus tumours are often misdiagnosed and are intervened with different treatment options before being surgically intervened. With complete surgical excision, recurrence is nil but postoperative nail growth requires nearly 10 months. Level of Evidence: Level IV.

CT angiography and MRI of hand vascular lesions: technical considerations and spectrum of imaging findings.

Vascular lesions of the hand are common and are distinct from vascular lesions elsewhere because of the terminal vascular network in this region, the frequent hand exposure to trauma and microtrauma, and the superficial location of the lesions. Vascular lesions in the hand may be secondary to local pathology, a proximal source of emboli, or systemic diseases with vascular compromise. In most cases, ischaemic conditions are investigated with Doppler ultrasonography. However, computed tomography angiography (CTA) or dynamic contrast-enhanced magnetic resonance angiography (MRA) is often necessary for treatment planning. MR imaging is frequently performed with MRA to distinguish between vascular malformations, vascular tumours, and perivascular tumours. Some vascular tumours preferentially affect the hand, such as pyogenic granulomas or spindle cell haemangiomas associated with Maffucci syndrome. Glomus tumours are the most frequent perivascular tumours of the hand. The purpose of this article is to describe the state-of-the-art acquisition protocols and illustrate the different patterns of vascular lesions and perivascular tumours of the hand.

Primary glomus tumour of the pituitary gland: diagnostic challenges of a rare and potentially aggressive neoplasm.

Primary non-neuroendocrine tumours of the pituitary gland and sella are rare lesions often challenging to diagnose. We describe two cases of clinically aggressive primary glomus tumour of the pituitary gland. The lesions occurred in a 63-year-old male and a 30-year-old female who presented with headache, blurred vision and hypopituitarism. Neuroimaging demonstrated large sellar and suprasellar tumours invading the surrounding structures. Histologically, the lesions were characterised by angiocentric sheets and nests of atypical cells that expressed vimentin, smooth muscle actin and CD34. Perivascular deposition of collagen IV was also a feature. Case 2 expressed synaptophysin. INI-1 (SMARCB1) expression was preserved. Both lesions were mitotically active and demonstrated a Ki-67 labelling index of 30%. Next-generation sequencing performed in case 1 showed no mutations in the reading frame of 37 commonly mutated oncogenes, including BRAF and KRAS. Four pituitary glomus tumours have previously been reported, none of which showed features of malignant glomus tumour. Similar to our two patients, three previous examples displayed aggressive behaviour.