Outcomes Following Excision of Toe Glomus Tumors.

BACKGROUND: Glomus tumors are uncommon tumors and their occurrence in the foot is even less common. Glomus tumors of the toes are often missed, causing delays in diagnosis and treatment. We report an ambispective observational study of glomus tumors of the toes that were treated at our institution. METHODS: We reviewed the records of all the patients who underwent excision of toe glomus tumors in our department from January 2010 to September 2022. The follow-up data were collected from the outpatient records and by telephonic interview. Single Assessment Numeric Evaluation (SANE) score, Foot and Ankle Outcome Score (FAOS), and the Foot Function Index (FFI) were collected. RESULTS: Out of all the patients treated for glomus tumors, we found that 7 patients had glomus tumors of the toes. Of the 7 patients, 6 were women and 1 was a male. The mean follow-up of our patients was 66.4 months (range, 7-109 months). Of the 7 patients, 1 presented with recurrent glomus tumor 30 months following the primary operation, for which she underwent excision again, after which she was symptom free. Another patient who developed recurrent symptoms on telephonic interview refused any further treatment. Among the 6 patients who were symptom-free at follow-up (including the patient who underwent excision for the recurrent tumor), the median SANE score, and FFI were 99.5 (IQR, 96-100) and 0.5 (IQR, 0-2) respectively. The mean FAOS was 96 (SD, 3.3). CONCLUSION: Surgical excision of the subungual toe glomus tumors can be curative. Recurrence of toe glomus tumors was noted in 2 patients (29%), one of whom refused further surgery. Re-excision in the other patient resulted in complete resolution of symptoms. LEVEL OF EVIDENCE: Level III, ambispective observational study.

Radiotherapy and Anrotinib in Malignant Glomus Tumor of the Bladder: A Case Report and Literature Review.

Background: Malignant glomus tumors (MGTs) are rare malignancies, which grow rapidly and are aggressive. Surgical resection has been regarded as the standard management, but treatment options for those unresectable tumors are limited, resulting in a high recurrence rate and poor prognosis. Case Description: An 85-year-old man presented with gross hematuria and was diagnosed with MGTs of bladder. The patient achieved long-term local control after multimodal therapy comprising radiotherapy, iodine-125 seeds brachytherapy, transcatheter arterial chemoembolization, and antiangiogenic targeted therapy. Conclusion: MGTs occurring in the bladder are clinically rare and refractory. The case presented here highlights the importance of multidisciplinary diagnosis and treatment, providing evidence that radiotherapy and antiangiogenic therapy may play an important role in unresectable bladder MGT.

The clinicopathologic and immunohistochemical features of 60 cutaneous glomus tumor: a retrospective case series study.

BACKGROUND: Glomus Tumor (GT) are benign neoplasms that originate from mesenchymal cells. It presents as tenderness and cold allodynia in the digits, especially in the subungual region. There are a few studies that investigated the mechanism of pain. OBJECTIVES: To analyze the clinical-pathologic characteristics of GT and to identify the expression of IL-1ß, IL-6, and CGRP in it, further, to explore the possible mechanism of pain. METHODS: The clinical and pathological data of 60 GT patients were retrospectively analyzed. Tissue microarrays and immunohistochemistry were used to measure the expression of IL-1ß, IL-6 and CGRP. RESULTS: GT is more common in females and the ratio of male to was near to 1:2, mostly in middle-aged people. It often occurs in fingertips, especially the thumbs. Patients often present with spontaneous pain, tenderness, and cold hypersensitivity. Both the two pain mediators IL-1ß and IL-6 were highly expressed in GT cells of patients with and without cold hypersensitivity. While CGRP was not expressed in GT. STUDY LIMITATIONS: Low sample size and further research is needed to explore the specific mechanism. CONCLUSIONS: IL-1ß and IL-6 were highly expressed in GT cells, suggesting that IL-1ß and IL-6 have certain nociceptive roles in GT. In the 4 patients with cold intolerance, the intensity of IL-1ß and IL-6 staining was also strong, suggesting that they may not play a role in the cold hypersensitivity. However, since there are only 4 patients with cold intolerance, it's necessary to conduct further in-depth research using a larger sample size. The specific role of CGRP in GT has not been found yet.

Mucosa-associated Lymphoid Tissue Misdiagnosis as Glomus Tympanicum.

Middle ear tumors are diverse, but relatively uncommon. The most frequent tumor in the middle ear is glomus tumor, followed by others such as schwannoma and cholesteatoma. We experienced a case of Mucosa-associated lymphoid tissue hyperplasia as a middle ear tumor. The mass behind tympanic membrane appeared a hypervascular tumor, mimicking a glomus tumor, but the form of multiple separate masses in middle ear and mastoid cavity was the distinguishing feature that set it apart from a glomus tumor. Additionally, another characteristic was its tendency to easily shrink under pressure. This characteristic should be considered when encounter a hypervascular looking middle ear mass. Laryngoscope, 134:1894-1896, 2024.

A Rapidly Expanding Hemorrhagic BRAF-Mutant Orbital Atypical Glomus Tumor.

A healthy 32-year-old woman presented with the acute onset of left sided eye pain, upper eyelid fullness, and binocular diplopia during light weightlifting. Examination elevated intraocular pressure, proptosis, upper eyelid ptosis, and motility deficits. CT demonstrated a well-circumscribed, homogeneous-appearing extraconal mass in the superior left orbit. The patient underwent an urgent orbitotomy with the excision of a hemorrhagic mass. Histopathology showed a glomus tumor with atypical features and hemorrhagic infarction, best classified as having uncertain malignant potential. A B-Raf proto-oncogene V600E mutation was detected with immunohistochemistry, which suggests a more aggressive tumor behavior yet presents an opportunity for targeted primary or adjunctive therapy. This is the first reported case of a B-Raf proto-oncogene-mutant atypical glomus tumor arising in the orbit.

Novel ATG7::RAF1 gene fusion in malignant glomus tumor.

Glomus tumors are classified as members of the perivascular myoid family of tumors. Nearly half of these show NOTCH-gene fusions and a smaller subset has BRAF V600E mutations. Here, we report a novel ATG7::RAF1 fusion in malignant glomus tumor occurring in a 40-year-old female which has not been reported in the malignant glomus tumor before. A 40-year-old female presented with a persistent lateral heel pain and an increase in the size of a mass along the lateral ankle for nearly 10 years. Resected specimen showed a well circumscribed lesion composed of spindled and epithelioid cells with moderate nuclear atypia and mitotic figures (7/10 high-power fields) including atypical forms without any necrosis, lymphovascular, or perineural invasion. The tumor was positive for smooth muscle actin, smooth muscle myosin heavy chain, H-caldesmon, collagen type IV, and discovered on gastronintestinal stromal tumors-1 but negative for AE1/3, desmin, S-100, CD34, and CD117. RNA sequencing showed presence of ATG7-RAF1 fusion. This fusion has not been reported in the malignant glomus tumor before. Future studies on larger cohorts are needed to ascertain the biological significance of these tumors with novel gene fusions.

A case of cutaneous malignant glomus tumor.

Glomus tumors are well-known but relatively rare vascular neoplasms, with their malignant counterparts still being rarer. There are very few reports of cutaneous malignant glomus tumors, and the current limited evidence suggests that they follow a more indolent course than deep-seated malignant glomus tumors. Herein, we are reporting a case of cutaneous malignant glomus tumor. A 94-year-old male presented with a right-sided ulcerated scalp lesion, which, on biopsy, showed a diffusely infiltrative epithelioid malignancy with considerable pleomorphism and a notable perivascular growth pattern. The tumor cells were positive for smooth muscle actin (SMA) and h-caldesmon, and negative for cytokeratin MNF116, CK5, p40, S100, SOX10, HMB45, Melan-A, ERG, CD31, CD45, CD3, CD20, ALK, desmin, CD68, CD34, and HHV8. A diagnosis of cutaneous malignant glomus tumor was made, and the patient underwent a wider excision. Cutaneous malignant glomus tumors are extremely rare and should be considered when examining unusual cutaneous mesenchymal tumors.

Tumor glômico em antebraço direito: uma apresentação atípica / Glomus tumor in the right forearm: an atypical presentatio

Introdução: Tumor glômico é uma neoplasia mesenquimal rara que compreende menos de 2% dos tumores de partes moles. Trata-se de um tumor com comportamento clínico benigno, de maior incidência no sexo feminino, entre a segunda e quarta década de vida. Objetivo: Apresentar um caso clínico de tumor glômico. Material e Método: Estudo tipo relato de caso. Relato: Homem de 35 anos de idade com nódulo em antebraço direito há 5 anos. Tratava-se de um nódulo, não ulcerado, eritemato-acastanhado, medindo cerca de 3 milímetros de diâmetro, doloroso a palpação e sensível ao contato com líquidos em baixas temperaturas. Após biópsia incisional da lesão, o exame foi compatível com o aspecto histopatológico de Tumor Glômico, com a presença de vasos sanguíneos dilatados e circundados por pequenas células glômicas com núcleos ovalados, citoplasma eosinofílico e bordas regulares, o que permitiu o correto diagnóstico e o planejamento terapêutico. Até a conclusão do trabalho, o paciente não havia realizado a remoção cirúrgica do tumor, porém não apresentou limitações ou queixas importantes. Conclusão: O estudo do caso do tumor glômico permitirá maior elucidação de novos casos, tendo em vista a raridade do tumor, o quadro clínico pouco específico e escassez de literatura disponível

Introduction: Glomus tumor is a rare mesenchymal neoplasm that comprises less than 2% of soft tissue tumors. It is a tumor with a benign clinical behavior, with a higher incidence in females, between the second and fourth decade of life. Objective: To present a clinical case of glomus tumor. Material and Method: Case report study. Report: 35-year-old man with a nodule on his right forearm for 5 years. It was a non-ulcerated, erythematous-brown nodule, measuring approximately 3 millimeters in diameter, painful on palpation and sensitive to contact with liquids at low temperatures. After incisional biopsy of the lesion, the examination was compatible with the histopathological appearance of Glomus Tumor, with the presence of dilated blood vessels surrounded by small glomus cells with oval nuclei, eosinophilic cytoplasm and regular borders, which allowed the correct diagnosis and planning therapeutic. Until the conclusion of the work, the patient had not undergone surgical removal of the tumor, but did not present any limitations or major complaints. Conclusion: The study of the glomus tumor case will allow greater elucidation of new cases, given the rarity of the tumor, the non-specific clinical picture and the scarcity of available literature

Introducción: El tumor glómico es una neoplasia mesenquimatosa rara que comprende menos del 2% de los tumores de tejidos blandos. Es un tumor de comportamiento clínico benigno, con mayor incidencia en el sexo femenino, entre la segunda y cuarta década de la vida. Objetivo: Presentar un caso clínico de tumor glómico. Material y Método: Estudio de caso clínico. Informe: Hombre de 35 años con nódulo en antebrazo derecho desde hace 5 años. Se trata de un nódulo de color marrón eritematoso, no ulcerado, de aproximadamente 3 milímetros de diámetro, doloroso a la palpación y sensible al contacto con líquidos a bajas temperaturas. Luego de la biopsia incisional de la lesión, el examen fue compatible con el aspecto histopatológico de Tumor Glómico, con presencia de vasos sanguíneos dilatados rodeados de pequeñas células glómicas con núcleos ovalados, citoplasma eosinofílico y bordes regulares, lo que permitió el correcto diagnóstico y planificación terapéutica. Hasta la conclusión del trabajo, el paciente no había sido sometido a extirpación quirúrgica del tumor, pero no presentaba limitaciones ni quejas importantes. Conclusión: El estudio del caso de tumor glómico permitirá un mayor esclarecimiento de nuevos casos, dada la rareza del tumor, el cuadro clínico inespecífico y la escasez de literatura disponible

A malignant glomus tumor in the nasal cavity responding to radiation therapy.

Malignant glomus tumors of the head and neck are extremely rare, and to our knowledge, a response to high-dose radiation has not been described previously. We report one case in an 80-year-old woman with right nasal cavity mass. Histological examination revealed sheets of atypical round glomus cells. The presence of increased mitotic activity (25 per 10 high-power fields), cellular atypism, and tumor necrosis suggested malignancy. The smooth muscle actin, vimentin, and h-caldesmon immunohistochemistry stains the tumor cells. Two cycles of doxorubicin and cyclophosphamide chemotherapy were done and the tumor size was slightly increased. Salvage radiation therapy (RT) was delivered to the primary mass over 4 weeks (50 Gy in 20 fractions) and leading to nearly complete regression of tumor. Additional investigations are warranted so that we may determine the usefulness of RT in the management of this rare tumor.

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.

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.

Tumor glómico del espacio masticatorio que imita patología mandibular / Glomus tumour of the masticatory space mimicking oral mandibular pathology

El tumor glómico es una neoplasia mesenquimal derivada de la célula muscular lisa modificada del aparato glómico. Solo se han descrito 39 casos en la cavidad oral. Una mujer de 32 años presentó dolor intenso e hipoestesia en el área del nervio mandibular. El examen físico oral reveló un abultamiento suave sensible a la palpación. La ortopantomografía mostró una imagen radiolúcida y bien definida en la rama mandibular que sugiere un trastorno óseo. Sin embargo, el TC y el angio-TC mostraron una lesión hipervascular en el espacio masticatorio derecho. Se optó por un abordaje quirúrgico intraoral de la lesión. El análisis histopatológico mostró una positividad difusa y fuerte para la vimentina. La actina del músculo liso, la actina específica del músculo y la cadena pesada de miosina del músculo liso también fueron positivas, pero la inmunorreactividad para los marcadores varió en extensión e intensidad entre las diferentes áreas tumorales. Calponina mostró inmunotinción focal y débil. El índice de proliferación (Ki67-MIB1) fue inferior al 1 %.Basado en estos hallazgos morfológicos e inmunohistoquímicos, el tumor se diagnosticó como un tumor glómico (tipo sólido). Esta primera descripción de un tumor glómico en el espacio masticatorio nos hace incluirlo en el diagnóstico diferencial de las neoplasias en esta área. (AU)

Glomus tumour is a mesenchymal neoplasm from modified smooth muscle cell of the glomus apparatus. Only 39 cases have been reported in the oral cavity. A 32-year-old female presented with intense pain and hypoaestesia in the mandibular nerve area. Oral physical examination revealed a soft bulging tender to palpation. Orthopantomography showed a radiolucent, well-defined image in the mandibular ramus suggesting an osseous disorder. However, CT and angio-CT showed a hypervascular lesion in the right masticatory space. An intraoral surgical approach to the lesion was chosen. Histopathological analysis showed diffuse and strong positivity for vimentin. Smooth muscle actin, muscle specific actin, and smooth muscle myosin heavy-chain were also positive, but immunoreactivity for markers varied in extension and intensity between different tumour areas. Calponin showed focal and weak immunostaning. Proliferation index (Ki67-MIB1) was less than 1 %.Based on these morphological and immunohistochemical findings, the tumour was diagnosed as a glomus tumour (solid type). This first description of a glomus tumour in the masticatory space makes us include it in the differential diagnosis of neoplasms in this area. (AU)

Tumor glómico del septum nasal / Glomic tumor of the nasal septum

Presentamos el caso clínico de una mujer de 58 años que consulta por episodios de epis-taxis autolimitadas por fosa nasal derecha asociados a hipersensibilidad al manipular dicha fosa nasal. A la exploración se evidenció en el septum nasal una lesión mucosa nodular, de consistencia dura y aspecto violáceo. Se llevó a cabo exéresis de la misma por vía endonasal, con resultado histopatológico de tumor glómico (TG) del septum nasal. El TG es una neoplasia benigna perivascular, derivada de las células musculares lisas presentes en el cuerpo glómico; estructura con función termorreguladora, formada por un shunt entre arteriolas y vénulas, sin presencia de red capilar. Los cuerpos glómicos están presentes, mayoritariamente, en extremidades (lecho ungueal). Los tumores glómicos son, verdaderamente, excepcionales en el área ORL, existiendo sólo una treintena de casos publicados hasta la fecha a nivel nasosinusal. La mayoría de casos se presentan entre la 5ᵃ y 6ᵃ década de la vida y más, frecuentemente, en mujeres. Plantea diagnóstico diferencial, fundamentalmente, con dos entidades: el paraganglioma (con el que no debe ser confundido desde el punto de vista terminológico ni histológico), y con el tumor fibroso solitario (antiguo hemangiopericitoma). El comportamiento es benigno y el tratamiento es la cirugía

We present the clinical case of a 58-year-old woman who consulted for episodes of self-limited epistaxis in the right nostril associated with hypersensitivity to manipulation of this nostril. Examination revealed a nodular mucosal lesion in the nasal septum, with a hard consistency and purplish appearance. The lesion was excised endonasally, with histopatho-logical result of a glomus tumor (GT) of the nasal septum. The GT is a benign perivascular neoplasm, derived from smooth muscle cells present in the glomus body; structure with thermoregulatory function, formed by a shunt between arterioles and venules, without the presence of capillary network. Glomus bodies are mostly present in the extremities (nail bed). Glomus tumors are truly exceptional in the ENT area, with only about thirty cases published to date at the nasosinusal level. Most cases occur between the 5th and 6th decade of life and more frequently in women. It raises differential diagnosis fundamentally with two entities: paraganglioma (with which it should not be confused from the terminological or histological point of view) and with solitary fibrous tumor (former hemangiopericytoma). The behavior is benign and the treatment is surgery.

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.

Diagnosis and surgical treatment of benign nail unit tumors.

Little is known about benign non-melanocytic nail tumors, probably due to their low pathogenicity. They are commonly misdiagnosed as inflammatory or infective diseases. They have various features, depending on the type of tumor and its location in the nail apparatus. The typical sign of a tumor is the presence of a mass and/or secondary nail changes from damaged nail structures. In particular, if a single digit is affected by a dystrophic sign or a symptom is reported without any explanation, the presence of a tumor should always be ruled out. Dermatoscopy helps to enhance visualization of the condition and in many cases supports the diagnosis. It may also assist in identifying the right place to biopsy, but it never replaces surgery. Most common non-melanocytic nail tumors are analyzed in this paper, including glomus tumor, exostosis, myxoid pseudocyst, acquired fibrokeratoma, onychopapilloma, onychomatricoma, superficial acral fibromyxoma and subungual keratoacanthoma. The aim of our study is to review the main clinical and dermatoscopic characteristics of the most common benign non-melanocytic nail tumors, to correlate them with the histopathology and to advise practitioners of the best surgical management.

Giant thigh glomus tumor of uncertain malignant potential: Case report with pathologic-radiologic correlation.

INTRODUCTION: Glomus tumors (GT) are rare, benign tumors that arise from glomus bodies and usually develop in digital areas. Extradigital GT are exceptional and thigh location is infrequent. CASE REPORT: We report a case of a GT of the thigh in a 79-year-old male patient that measured 9.5 cm in maximum size. The GT lay above the muscular fascia without infiltrating it. Internal hypervascularity was seen by spectral Doppler ultrasound. Magnetic resonance image showed a heterogeneous mass with hyperintense and hypointense components and internal lobes with liquid-liquid levels. Histopathology revealed a monotonous round-cell proliferation with central nuclei without atypia or mitotic figures, around small-caliber vessels. These cells expressed smooth muscle actin and pericellular collagen IV. GT of uncertain malignant potential was diagnosed. The mass was completely removed. The patient did not experience local relapse nor distant metastasis. CONCLUSION: GT are rare soft tissue tumors whose diagnosis of unusual giant masses in uncommon locations may be delayed and misdiagnosed given the low suspicion.

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.