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Glomus tumors are typically benign, soft tissue neoplasms composed of thermoregulatory glomus bodies. The more common varieties, such as subungual, are treated surgically and typically have a very low mortality rate. Malignant glomus tumors are very rare, and their pathogenesis is poorly understood. As such, treatment options and prognosis are unclear. We present a 67-year-old female diagnosed with a chest wall glomus tumor with biopsy-proven metastases to her lungs. Her treatment course included neoadjuvant radiation therapy followed by immunotherapy with pembrolizumab. After completion of the initial radiation therapy, imaging showed disease regression. Interval imaging after seven months of immunotherapy showed the resolution of all lung nodules with no reported concerns for disease recurrence. Pembrolizumab was discontinued due to concerns for dermatologic and renal adverse events, and the patient continues to be monitored off therapy. The metastatic glomus tumor described in this case had several unique qualities, including its initial presentation on the chest wall as an aggressive lesion, as well as its spread to multiple locations in the lungs. Glomus tumors are not normally as aggressive as seen in this case, but the genetic profile with high tumor mutational burden allowed for guided treatment. Radiation is often used as neoadjuvant treatment in higher risk glomus tumors, but the addition of immunotherapy such as pembrolizumab represents a potential avenue to manage these patients when surgery is not an option. Malignant glomus tumors are exceptionally rare occurrences that, by nature of their rarity, require protocols or therapies that are not specifically designed for their treatment. The clinical course of these tumors is difficult to predict as most cases of metastatic spread have few examples from which to draw conclusions. This case provided encouraging results for treatment with radiation and, potentially, immunotherapy. Each instance of a malignant glomus tumor and its genetic profile should be closely examined and documented so that sufficient data can be accumulated to guide treatment for this rare cancer.
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Purpose: Paragangliomas represent a surgical challenge due to their hypervascularization. The preoperative selective embolization of these tumors significantly decreases intraoperative blood loss. However, the literature on preoperative embolization in glomus tumors is limited. The aim of this study is to contribute additional evidence regarding the role of preoperative embolization, as well as to evaluate risks and complications in the treatment of glomus tumors. Methods: A retrospective evaluation of all the embolizations of glomus tumors from 2009 to 2023 was conducted. The primary outcome parameter was the rate of devascularization after embolization and the occurrence of significant perioperative hemorrhages. The secondary outcome was embolization-related complications. Results: Twenty-one embolizations in 20 patients were investigated in the study. In 43% of the cases more than 90% devascularization was achieved by embolization, while in the remaining cases, 80 to 90% devascularization was reached. In one case (5%), significant perioperative bleeding after embolization occurred. In one case (5%), a symptomatic complication occurred periinterventionally due to the brief dislocation of the coaxial and microcatheter into the internal carotid artery (ICA), which led to fresh punctate DWI lesions on the subsequent MRI. No patients developed nerve palsy following embolization. Conclusions: The preoperative embolization of glomus tumors can lead to significant tumor devascularization and a reduction in perioperative bleeding, with a low complication rate.
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Glomus tumors arise from a neuromyoarterial plexus origin, typically demonstrating branching vascular channels and aggregates of specialized glomus cells. They are characteristically identified in the subungual region of the hand, with the presentation of firm, red or blue nodules that are painful and sensitive to temperature. However, very few cases of malignant glomus tumors of the skin have been reported in the medical literature, making this case quite unique. This case report aims to identify an additional case of this rare condition, discuss the treatment plan pursued, and bring awareness to this distinct oncological entity. A 41-year-old female patient initially presented to her primary care physician with an enlarging and painful nodule on her left temple that had been present for the last two months. The patient was then referred to plastic surgery for excision and pathological workup of the mass. The pathology report of the excised specimen revealed a malignant glomus tumor of the skin. The patient was subsequently scheduled for an oncologic workup with medical oncology and radiation oncology, and a wide excision was performed after the pathologic diagnosis. With negative margins from the wide excision and no evidence of disease spread, it was determined that there was no role for systemic therapy or radiation therapy at that time. Biannual dermatologic examination and monitoring are indicated for follow-up. With very few reports of similar cases and little information in the medical literature on the treatment of this type of neoplasm, this is a unique and rare case that warrants discussion and exposure. Making the correct diagnosis, performing a complete workup, and removing the malignant glomus tumor are all essential parts of medical management in this case, and this type of neoplasm should not be excluded when evaluating the presentation of unusual cutaneous lesions.
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Glomus tumors (GT) are very rare mesenchymal neoplasms arising from glomus bodies, arteriovenous structures located in the dermis and involved in thermoregulation. Although most are benign, they may occasionally present malignant histological features associated with aggressive clinical behavior, metastatic spread, and poor response to conventional chemotherapy. The BRAF V600E mutation has been identified in a subset of malignant GT, highlighting a promising therapeutic target. Here, we report the impressive clinical and morpho-metabolic response of a metastatic BRAF V600E-mutated glomangiosarcoma after treatment with encorafenib and binimetinib.
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Protocolos de Quimioterapia Combinada Antineoplásica , Benzimidazóis , Carbamatos , Tumor Glômico , Mutação , Proteínas Proto-Oncogênicas B-raf , Sulfonamidas , Humanos , Proteínas Proto-Oncogênicas B-raf/genética , Carbamatos/administração & dosagem , Tumor Glômico/genética , Tumor Glômico/patologia , Sulfonamidas/administração & dosagem , Sulfonamidas/farmacologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Benzimidazóis/administração & dosagem , Benzimidazóis/farmacologia , Resultado do Tratamento , Masculino , Pessoa de Meia-Idade , FemininoRESUMO
Objective: To investigate head and neck paraganglioma cases treated at a tertiary center from 2007 to 2023. The research includes a thorough examination of published studies that have focused on long-term outcomes. The additional goal is to contribute to the existing knowledge on head and neck paraganglioma, with a particular emphasis on refining diagnostic algorithms, treatment selection, and follow-up procedures. Methods: A total of 44 patients were retrospectively analyzed, and 39 were included. Demographic information, symptoms, radiological examination results, types, stages, and postoperative complications were recorded. A review was conducted to select articles that reported single-center experiences with large cohorts, long follow-ups, and different treatment modalities since 2010. Results: The mean age of the patients was 54 years, and the female/male ratio was 3.55:1. Among the 39 cases examined, 18 and 19 were identified as cervical paraganglioma and 19 as temporal bone paraganglioma. All patients initially underwent surgical resection. The mean follow-up duration was 5.42 years. Four residual cases and two recurrences were identified postoperatively, and a Gamma Knife was used as additional treatment. Subsequently, 17 articles were selected and summarized, and then a flowchart was prepared showing the possible options for diagnosis, treatment, and follow-up. Conclusions: Preoperative staging is essential for surgical planning and predicting potential intraoperative complications. Based on our findings and review of the articles, we have prepared a flowchart that includes all possibilities depending on the tumor stage to help in the diagnosis, treatment, and follow-up of head and neck paragangliomas.
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Vascular anomalies (VA) are classified as either vascular tumors or vascular malformations. Vascular tumors are characterized by the neoplastic proliferation of endothelial cells, whereas vascular malformations result from defects in the pathways that regulate the development of vascular channels during embryogenesis. Dermatological ultrasound has emerged as a critical diagnostic tool for distinguishing VA from other conditions and differentiating among various types of VA by providing detailed imaging characteristics. In this study, we present cases of less common VA identified and characterized using dermatological ultrasound to illustrate the utility of this modality in clinical practice. Patients were randomly selected from clinical records (February 2021 to February 2024) with dermatological VA confirmed by histology, spanning ages 10 to 72 years. Inclusion required complete clinical records and ultrasound reports. The sample represents six different VA and illustrates a spectrum of complications observed in clinical practice. The selected cases include five vascular tumors: pyogenic granuloma, cutaneous angiosarcoma, extradigital glomus tumors, cherry angiomas, and angiokeratoma, and one vascular malformation: verrucous venous malformation. Despite their varied clinical presentations, dermatological ultrasound demonstrated distinct features for each type of anomaly, proving to be a valuable tool in the multidisciplinary management of cutaneous VA. This imaging modality enables precise evaluation and guides effective treatment strategies.
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Introduction: This case report documents an exceptionally rare hepatic glomus tumor, contributing valuable insights to the diagnosis and differential diagnosis of such tumors. Main symptoms and important clinical findings: A 58-year-old male was admitted in 2023 due to unexplained elevation of bilirubin discovered during routine laboratory tests. The patient denied any symptoms, and physical examination revealed no positive findings. Laboratory tests indicated elevated bilirubin, while serum tumor markers remained within normal ranges. Contrast-enhanced computed tomography revealed an indistinctly bordered hepatic mass, displaying uneven and marked enhancement in the arterial phase and sustained enhancement in the venous phase. The main diagnoses therapeutic interventions and outcomes: The patient underwent laparoscopic partial hepatectomy, and pathological examination suggested a spindle cell tumor of mesenchymal origin. Immunohistochemistry confirmed positive staining for smooth muscle actin, leading to the final diagnosis of a hepatic glomus tumor with undetermined malignant potential. Follow-up at 6 months postoperatively showed no signs of metastasis or recurrence. Conclusion: This case underscores the subtle radiological distinctions of hepatic glomus tumors, resembling hemangiomas but manifesting unique features. Additionally, the crucial role of immunohistochemistry in achieving a definitive diagnosis is emphasized in this report.
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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.
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INTRODUCTION: Glomus tumors are rare benign tumors arising from glomus bodies that are responsible for thermoregulatory control. Their typical location is the subungual area of the digits, and extra-digital glomus tumors are very rare, leading to misdiagnosis and delayed treatment due to the absence of typical symptoms. CASE: Here, we report the case of a 49 years old male patient with a long history of localized right thigh pain who was found to have an extra-digital glomus tumor of the thigh after surgical excision. DISCUSSION: A comprehensive physical examination, detailed medical history, in depth imaging and early surgical excision upon clinical suspicion may prevent delayed or incorrect diagnosis. The treatment of glomus tumor is surgical excision providing immediate relief from pain, however if the lesion is not palpable, it can be easily missed or confusing with other diagnoses such as schwannoma, neuroma or arteriovenous malformation. CONCLUSION: Glomus tumors of the thigh represent an exceptional location for extra digital glomus tumors. The aim of this report was to make the surgical community more aware of this entity to prevent delayed treatment and misdiagnosis. Glomus tumor should be kept in mind in the differential diagnosis of all painful subcutaneous lesions.
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Glomus tumors are rare, benign vascular hamartomas of the glomus apparatus with unknown etiology. They can arise from anywhere in the body. However, up to 90% of them are located in the subungual region of the fingers, as in the case of our patient. These tumors typically present with the classic triad of pain, cold sensitivity, and point tenderness. Characteristic US and MRI findings aid the clinical diagnosis; nevertheless, a histopathologic examination is confirmatory. There is a well-documented mean delay in diagnosis of around 7 years, due to the rarity, benignity, small size, and lack of proper knowledge about the condition. However, we reported a case with a delay in diagnosis that reached 40 years, which is much longer than what is documented in the literature. A high index of suspicion is required for early diagnosis and management of glomus tumors to relieve the patient's long-term suffering and prevent possible secondary nail deformities. The curative treatment of glomus tumor is complete surgical excision, which is crucial to prevent recurrence and relieve the patient's symptoms.
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The glomus tumor is a rare neoplasm that is typically found subungually in the extremities and functions as a specialized neurovascular organ. An extremely rare site for glomus tumors is the breast, with only a few reported cases. Breast glomus tumors present with three typical clinical signs: dull pain, focal tenderness, and cold sensitivity. Less than 10% of all glomus tumors are malignant. We herein present a case of a malignant glomus tumor originating in the breast. Distant metastasis was ruled out, and the tumor was completely resected. However, the patient unexpectedly developed rapid systemic metastasis, detected 5 weeks after tumor removal. Despite the administration of analgesics and targeted therapy, the patient died 1 month later. When treating patients with undiagnosed breast tumors, clinicians should pay attention to unexplained and repeatedly reported symptoms and consider the possibility of a rare disease. Our literature search revealed no cases of malignant glomus tumors originating in the breast, making this case the first of its kind.
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Neoplasias da Mama , Tumor Glômico , Humanos , Tumor Glômico/patologia , Tumor Glômico/diagnóstico , Tumor Glômico/cirurgia , Feminino , Neoplasias da Mama/patologia , Neoplasias da Mama/cirurgia , Neoplasias da Mama/diagnóstico , Pessoa de Meia-Idade , Evolução Fatal , Progressão da DoençaRESUMO
Upper arm glomus tumors are uncommon, benign vascular growths originating from skin glomus bodies. They typically cause severe, paroxysmal pain triggered by cold, pressure, or touch. Diagnosis can be complex due to mimicry of other conditions, but histopathological examination confirms the presence of glomus cells. In a case study, a 46-year-old woman with an Upper arm glomus tumor experienced pain and a lump in her left arm. Ultrasound identified a well-circumscribed, heterogeneous, hypoechoic, pedunculated mass with internal vascularity and MRI showed a lobulated soft-tissue mass with skin involvement and polypoid projection, alongside axillary lymphadenopathy and skin thickening. Following an open biopsy that confirmed the diagnosis of a glomus tumor, the patient experienced complete resolution of symptoms after surgical excision, with no recurrence during the 24-month follow-up period.
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Glomus tumors are rare mesenchymal tumors involving cells from the glomus body, smooth muscle, and vasculature, typically found in distal extremities' skin. This case describes a 54-year-old woman with a history of hypothyroidism and hyperlipidemia, incidentally discovered to have a four-centimeter calcified renal tumor. Surgery was performed due to suspected malignancy. Immunohistochemical staining confirmed a renal glomus tumor, positive for muscle actin and smooth muscle actin (SMA). The tumor was benign, and no adjuvant therapy was needed. The patient remained recurrence-free during follow-up. Renal glomus tumors are predominantly benign, with surgical resection as the primary treatment.
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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.
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Glomus tumor (GT) is a neoplastic lesion of mesenchymal origin arising from the neuromyoarterial canal or glomus body. Although most GT occur in the peripheral soft tissue and extremities, these tumors can grow anywhere in the body. Here, we describe an uncommon case of GT involving the prostate.
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Tumor Glômico , Neoplasias da Próstata , Humanos , Masculino , Tumor Glômico/patologia , Tumor Glômico/cirurgia , Tumor Glômico/diagnóstico por imagem , Neoplasias da Próstata/patologia , Neoplasias da Próstata/cirurgia , Pessoa de Meia-IdadeRESUMO
This is a case report on the treatment of tracheal glomus tumor, which is a rare tumor originating from the glomus bodies in the trachea and form spherical or nodular masses. These tumors can lead to obstruction and narrowing of the tracheal lumen, causing symptoms such as dyspnea. The standard treatment for tracheal glomus tumors is typically surgical resection. In this case, the patient underwent a procedure involving low tracheostomy with closure of the airway and mechanical ventilation through the stoma, followed by endoscopic-assisted subglottic tracheal glomus tumor resection using plasma-assisted radiofrequency.
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Protection of cranial nerves is one of the major challenges in the resection of paragangliomas of head and neck, especially in complex paragangliomas. We report a case of bilateral jugular tumor with unilateral carotid body tumor. Baroreflex failure syndromeï¼BFSï¼ occurred after staged resection of bilateral lesions. There is still a lack of effective treatment for this complication. More prudent and reasonable treatment strategy is important to reduce the incidence of BFS.
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Neoplasias de Cabeça e Pescoço , Paraganglioma , Humanos , Neoplasias de Cabeça e Pescoço/cirurgia , Paraganglioma/cirurgia , Barorreflexo , Complicações Pós-Operatórias/etiologia , Tumor do Corpo Carotídeo/cirurgia , Masculino , Feminino , Pessoa de Meia-Idade , Síndrome , AdultoRESUMO
To investigate the clinical experience of glomus jugulare paraganglioma by presenting a case of giant glomus jugulare paraganglioma. The clinical data of 1 case of giant glomus jugulare paraganglioma with unilateral anacousia and pulsatile tinnitus admitted to our department was retrospectively analyzed, and the relevant literature was reviewed to summarize the characteristics of the disease. The tumor tissue in the jugular venous foramen region was completely resected, with complete preservation of the facial nerve during the operation. There was no tumor recurrence during the 2-year postoperative follow-up period. With nonspecific clinical symptoms and a high rate of early misdiagnosis The giant glomus jugulare paraganglioma case only manifested as symptoms of unilateral anacousia and pulsatile tinnitus is clinically rare. The intraoperative safe resection of the tumor, maximum preservation of facial nerve function remains the focus of surgery.
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Tumor do Glomo Jugular , Humanos , Tumor do Glomo Jugular/cirurgia , Paraganglioma/cirurgia , Paraganglioma/diagnóstico , Masculino , Forâmen Jugular , Pessoa de Meia-Idade , Feminino , Adulto , Zumbido/etiologia , Estudos Retrospectivos , Veias JugularesRESUMO
Background: Nail glomus tumor is a well-known tumor, with well-defined clinical characteristics and surgical treatment; however, some of these lesions occur in different locations and sizes with difficult surgical resolution. Summary: Clinical and imaging tests help in the diagnosis and tumor localization. Key Message: Adequate surgical knowledge for these cases ensures lower rates of recurrence and nail dystrophy.