A rare primary sacral glomus tumor presenting as intradural-extramedullary tumor: A Case report and review of literature.

Background: Glomus tumors are very infrequent in the spine where they can grow intraosseously at any level. We were able to identify only eight such cases in the literature, with only one occurring in the sacrum. Here, a 48-year-old male with sacral S1/S2 radiculopathy was found to have a rare glomangioma/glomus tumor of the sacral region. Case Description: A 48-year-old male presented with left-sided S2 radiculopathy characterized by left lower extremity weakness/paresis. The magnetic resonance showed an intradural extramedullary mass measuring 1.8 × 1.9 × 4.3 cm at S1-S2 extending through the left foramen, inhomogeneously enhanced with contrast. He underwent an S1-S2 durotomy with gross total excision of the mass. Pathologically, it proved to be a glomus tumor. Two months postoperatively, he ambulated without the support and demonstrated no tumor recurrence at 1 postoperative year. Conclusion: Glomus tumors involving the sacral region are rare and can be successfully excised resulting in good clinical outcomes.

Additional Role of 3D ASL Perfusion in Skull Base Lesions.

Background Arterial spin labeling (ASL) perfusion imaging is widely used since its main advantage is that no intravenous contrast is needed. Given that perfusion is a crucial biological characteristic for identifying tumor lesions, the qualitative noncontrast perfusion characteristics of these lesions were examined. Aim We attempted utilizing the three-dimensional (3D) ASL technique to characterize skull base lesions and to highlight its crucial role in differentiating lesions. Methods and Material 3D ASL imaging of 20 patients with posterior skull base lesions was performed in a 3-T magnetic resonance (MR) system (Siemens Healthineers, Skyra, Erlangen, Germany). The common differential diagnoses of skull base lesions could be distinguished based on this qualitative evaluation. Results and Conclusions Glomus tumor has a strikingly increased perfusion when compared to meningiomas. The perfusion characteristics of metastasis depends on the primary tumor. Chondrosarcomas have a heterogeneously increased perfusion. Chordomas have variable perfusion, which helps in prognosticating the tumors. ASL benefits pediatric patients and in renal failure as well since it avoids the ethical ambiguity associated with contrast agents.

Point Tenderness for 35 Years: A Rare Presentation of a Glomus Tumor in the Proximal Upper Extremity.

Glomus tumors are rare benign neoplasms that are commonly found on the fingers and distal extremities. Clinically, they are often associated with a symptom triad of moderate pain, cold sensitivity, and point tenderness. These tumors are often not considered during a clinical workup due to their rarity and can be misdiagnosed due to their diverse clinical presentations. Glomus tumors are made up of mesenchymal cells derived from glomus bodies, which are specialized arteriovenous (AV) anastomoses primarily responsible for thermoregulation. Microscopically, they present as intricate nests of endothelial cells surrounding glomus bodies, which can clinically manifest as point tenderness. Glomus tumors are usually benign and are commonly found in locations with a high concentration of glomus bodies such as the fingers. Extradigital tumors are very rare and usually not considered in primary diagnosis. This can lead to patients experiencing years and, in this case, decades of unexplained pain. The diagnostic workup for glomus tumors should include an initial Doppler ultrasound and a definitive diagnosis via immunohistochemistry (IHC). They can be completely cured with surgical excision. Although most glomus tumors are benign and easily treatable, they are often not considered in differential diagnoses when assessing for point tenderness.  This case illustrates an atypical presentation of a glomus tumor that caused 35 years of chronic pain and was incidentally misdiagnosed on imaging, leading to treatment delay by an additional eight months. This exemplifies the necessity of including glomus tumors within the differential diagnosis and diagnostic workup for point tenderness and soft tissue masses of the upper extremity.

Diagnostic and Therapeutic Insights into Spinal Glomangioma of a Unique Intradural, Extramedullary Presentation-Systematic Review.

Contemporary literature lacks examples of intradural, extramedullary spinal glomangiomas. Moreover, glomus tumors in general are exceedingly rare among benign spinal tumors and are mostly located within epidural space or within intervertebral foramen, and only a few cases have been documented to date. This report provides a detailed analysis of the clinical presentation, imaging characteristics, surgical intervention, and pathological findings of a 45-year-old patient experiencing progressive locomotor deterioration. The tumor was surgically excised, and subsequent histological examination identified it as a representative of glomus tumors-a glomangioma. Notably, this represents a unique case as it was the first example of such a tumor being discovered intradurally. Radical surgical excision remains the modality of choice in most benign spinal tumors of this localization. Although the malignant transformation of glomus tumors within the spine has not been documented thus far, cases have arisen in other areas. Consequently, we will investigate potential oncological treatments for cases with malignant potential and highlight advancements in surgical techniques for benign intradural spinal tumors.

Challenges in the diagnosis and management of tumor-induced osteomalacia: A case report.

The present case report is aimed to highlight the difficulty and the reason for the delayed diagnosis of phosphaturic mesenchymal tumors, emphasizing the need of standardized protocols for diagnosis, surgery and follow-up in high-volume hospitals. The clinical signs and symptoms, diagnostic and therapeutic procedures, immunohistological features were analyzed. Delayed diagnosis of phosphaturic mesenchymal tumor was primarily due to non-specific clinical symptoms such as fatigue, muscular and bone pain, and multiple fractures. This cryptic clinical picture made the diagnosis tricky that led to treatment of patient for non-specific pain and stress fractures before to consider the tumor-induced osteomalacia syndrome. Some well-documented studies were found in the literature in which the history of trauma is a critical trigger of glomus tumors. Extra-subungual tumors most frequently occur in the knee and ankle regions, particularly among young adults, and the diagnosis is typically made approximately 7.2 years after initial symptom onset. The difficult tumor localization represented an additional obstacle to the prompt treatment, leading to delayed curative surgery.

Combined treatment for a rare malignant glomus tumor of the esophagus with pulmonary and liver metastases: a case report and review of literature.

Background: Glomus tumors are typically benign soft tissue tumors that occur at the extremities; malignant and viscerally occurring cases are extremely rare. Case presentation: We report a 49-year old male patient with a malignant esophageal glomus tumor that was complicated by lung and liver metastases. Genetic test results guided the patient's individualized treatment. Consequently, treatment with Anlotinib combined with Tislelizumab achieved significant clinical benefits. Conclusion: Our case report demonstrates that immunotherapy combined with anti-angiogenic therapy in patients with malignant esophageal glomus tumors can achieve significant efficacy and suggests the potential value of next-generation sequencing (NGS) detection in guiding personalized treatments in patients with malignant esophageal glomus tumors.

Glomus tumor of the foot dorsum: A case report of a neglect podiatric entity.

Key Clinical Message: Podiatrists and orthopedists should be vigilant for chronically evolving, hyperalgic soft lumps in the foot with vascular radiological features, prompting early detection of glomus tumor, timely mass removal, providing pain relief and improving patient's quality of life. Abstract: Glomus tumors refers to a rare group of benign perivascular neoplasms that originate from a neuromyoarterial structure called a glomus body. These tumors are characterized by their painful nature and predominant distribution in the extremities mainly the fingers, the hands and the feet. Nonetheless, the diagnosis is usually made after several years of symptoms experience as the lesions are mostly small, not palpable, and have variable presentations. Radiological workup especially with magnetic resonance imaging is very useful for diagnosing the tumoral process, however, confirmation can only be obtained by histological analysis. The treatment is purely surgical, and it is successful in most cases. Herein, we describe a case of glomus tumor of the foot dorsal side among a middle age male patient.

Primary glomus tumor of the thymus in a 66-year-old patient.

We report a unique case of a 66-year-old man who was incidentally identified to have a mass in the thymus region by computerized tomography scan. CT revealed a well-defined 1.6 × 1 × 0.9 cm thymus mass with moderate uniform enhancement. Thoracoscopic thymectomy was performed, and the pathological diagnosis was primary glomus tumor of the thymus. There were no atypia or malignant histological features, and no primary tumors in other sites. To our knowledge, this is the first case of primary thymic glomus tumor reported in the literature.

Glomus jugulotympanicum tumor treated with radiation therapy: A case report with review of literature.

Glomus tumors (or paragangliomas) are rare, benign tumors of neuroendocrine origin that appear in tissues of paraganglionic origin. This clinical entity poses a significant treatment challenge due to its proximity to critical neurovascular structures, thus the potential of morbid functional damage caused by disease progression and/or treatment approach. While surgery remains the standard of care for such cases, there has been an increasing trend toward management with radiotherapy or close observation. Here, we present a case of a large and irregularly shaped glomus jugulotympanicum tumor that was treated with volumetric arc radiotherapy. Given the risk of cranial neuropathy with surgery, radiation was the preferred treatment modality. This case demonstrated the safety and efficacy of volumetric arc radiotherapy in the management of a large glomus tumor with a complex shape.

Success and safety of endoscopic versus microscopic resection of temporal bone paraganglioma: a meta-analysis.

PURPOSE: Temporal bone paraganglioma (TBP) are the most common tumors of the middle ear. They pose a challenge in otologic surgery due to their extensive vascularity and intricate location within the middle ear. This meta-analysis aimed to compare the safety and efficacy of two surgical approaches, microscopic middle ear surgery (MMES) and endoscopic middle ear surgery (EMES), in the resection of TBP. METHODS: Eligible studies published after 1988 were identified through systematic searches of "PubMed", "Scopus" and "Google Scholar". Retrospective studies and randomized/non-randomized control trials reporting on surgical approaches for TBP with a minimum of five adult patients were included. RESULTS: A total of 595 records were initially identified. After removing 229 duplicates, 349 articles were excluded based upon article subject, title and abstract. Following the review of full texts, 13 articles were assessed for eligibility. The pooled analysis included a total of 529 ears, with a complication rate of 7.8% for EMES and 14.2% for MMES. Subgroup differences indicated no significant variation between the two methods (p = 0.2945). CONCLUSION: Both EMES and MMES demonstrated favorable surgical outcomes with low complication rates for TBP resection. These findings suggest that EMES is a safe and effective method for TBP resection and one that is comparable to MMES. Since the risk of bleeding is significant in these tumors, a third-hand technique, endoscopic bipolar cautery or laser-assisted hemostasis should be considered. Conversion to MMES is another option when visibility is critically affected by bleeding.

Relationship between dermoscopic and histopathological findings of extradigital glomus tumors.

In extradigital glomus tumors, (1) noncontact and (2) contact dermoscopy show (1) a central purplish-white area corresponding to tumor nests surrounding enlarged vessels, a peripheral yellow-white area corresponding to mucin deposition, melanin granules, and fibrous tissue, and (2) white reticular and linear cord areas corresponding to pseudocapsules and collagen fibers.

Malignant glomus tumor of the breast: a case report.

Malignant glomus tumor (MGT) is a rare mesenchymal neoplasm. It is rarely located in the breast. We present a case of a 57-year-old female patient presenting with complaints of a progressively growing mass found in her left breast. Though multiple imaging examinations have been performed, especially multimodal ultrasound examinations, an accurate diagnosis still cannot be determined. Finally, the lesion was confirmed to be a MGT of the breast by postoperative pathological diagnosis. In conclusion, MGT originating from breast is extremely rare. No such case has ever been described before. This study demonstrates the imaging characteristics of a patient with MGT of the breast in order to provide more extensive insights to consider the differential diagnosis of breast lesions.

Thoracoscopic enucleation of an esophageal glomus tumor in the prone position: a case report and literature review.

BACKGROUND: Glomus tumors (GT) generally occur in the skin. However, esophageal GT, an extremely rare condition, has no established standardized treatment guidelines. Herein, we report the case of an esophageal GT successfully removed by thoracoscopic enucleation in the prone position using intra-esophageal balloon compression. CASE PRESENTATION: A 45-year-old man underwent an annual endoscopic examination and was found to have a submucosal tumor in the lower esophagus. Endoscopic ultrasound (EUS) revealed a hyperechoic mass originating from the muscular layer. Contrast-enhanced computed tomography identified a 2 cm mass lesion with high contrast enhancement in the right side of the lower esophagus. Pathologic findings of EUS-guided fine needle aspiration biopsy (EUS-FNA) revealed round to spindle shaped atypical cells without mitotic activity. Immunohistochemically, the tumor was positive for alpha-smooth muscle actin, but negative for CD34, desmin, keratin 18, S-100 protein, melan A, c-kit, and STAT6. He was diagnosed with an esophageal GT and a thoracoscopic approach to tumor resection was planned. Under general anesthesia, a Sengstaken-Blakemore (SB) tube was inserted into the esophagus. The patient was placed in the prone position and a right thoracoscopic approach was achieved. The esophagus around the tumor was mobilized and the SB tube balloon inflated to compress the tumor toward the thoracic cavity. The muscle layer was divided and the tumor was successfully enucleated without mucosal penetration. Oral intake was initiated on postoperative day (POD) 3 and the patient discharged on POD 9. No surgical complications or tumor metastasis were observed during the 1-year postoperative follow-up. CONCLUSIONS: As malignancy criteria for esophageal GT are not yet established, the least invasive procedure for complete resection should be selected on a case-by-case basis. Thoracoscopic enucleation in the prone position using intra-esophageal balloon compression is useful to treat esophageal GT on the right side of the esophagus.

Diagnosis and Management of Longitudinal Erythronychia: A Clinical Review by an Expert Panel.

Longitudinal erythronychia (LE) is defined as a longitudinal red band of the nail(s) and is classified as localized (involvement of one nail) or polydactylous (involvement of more than one nail). The differential diagnosis is distinct for these classifications. The etiologies of localized longitudinal erythronychia (LLE) are most frequently benign subungual neoplasms, and less often malignancies. Polydactylous longitudinal erythronychia (PLE) is typically secondary to regional or systemic diseases, including lichen planus and Darier disease. LE is a common, but underrecognized clinical finding. Increased dermatologist awareness of the clinical characteristics and differential diagnosis for LE is necessary given the possibility for malignancy and associated systemic disease. In this clinical review, the clinical features, differential diagnosis, evaluation, and management of LE are described.

Neurofibroma with glomus-like bodies: A novel association-Thoughts about origin.

A neurofibroma with focal glomus-like body differentiation is an unusual phenomenon recently encountered in an excision specimen from the right lateral distal forearm of a 26-year-old man. Glomus cells are modified smooth muscle cells normally present in glomus-like bodies but can also be found in glomus tumors (GT) or lesions considered in the spectrum of GT, including myopericytoma, myofibroma, and angiolipoma. Neurofibromas are peripheral nerve sheath tumors derived from the neural crest cells. While both GT and its variants and neurofibroma are thought to be derived from different cell types, there is growing evidence that glomus cells have a neural crest origin. This is based on multiple theories, with some overlapping pathways, including neural crest cell differentiation, Schwann cell reprogramming, VEGF expression, and NF1 gene biallelic inactivation. This report adds to the growing evidence of possible neural crest origin for glomus cells and would help explain finding glomus-like bodies scattered through a neurofibroma.

Tumor glómico causante de dolor de dedo crónico: la prueba de detección de isquemia es un signo clínico fiable para su diagnóstico. Informe de un caso y revisión de la literatura / Glomus tumor causing chronic finger pain: Ischemia test is a reliable clinical sign for the diagnosis. Case report and review of literature

Los tumores glómicos (TG) son neoplasias benignas raras, que se derivan de la estructura neuroarterial denominada cuerpo glómico, un shunt arteriovenoso especializado implicado en la regulación de la temperatura. Representan menos de 2% de los tumores del tejido blando, y entre 1 y 4,5% de los tumores de la mano. Aun cuando sus primeras descripciones aparecieron hace casi 100 años, son comunes la demora y la ausencia diagnósticas, las cuales originan un sufrimiento terrible. La tríada diagnóstica clásica consiste en dolor espontáneo, sensación de presión y sensibilidad, e hipersensibilidad al frío. La imagen de resonancia magnética (IRM) sigue siendo la modalidad de imagen más útil. La supresión del dolor tras inflar un esfigmomanómetro por encima de los niveles de la presión arterial sistólica (prueba de detección de isquemia) es altamente diagnóstica, por lo que sugerimos el uso rutinario de esta prueba simple en los casos de dolor en la extremidad superior de etiología desconocida. La resección quirúrgica es el tratamiento de elección, y es curativa. (AU)

Glomus tumors are a rare benign neoplasm arising from the neuroarterial structure called the glomus body, a specialized arteriovenous shunt involved in temperature regulation. They represent less than 2% of soft tissue tumors and between 1 and 4.5% of hand's tumors. Even though its first descriptions appeared almost 100 years ago, late and missed diagnoses are common, which leads to terrible suffering. The classic diagnostic triad consists of spontaneous pain, pressure sensation and tenderness, and cold hypersensitivity. Magnetic resonance imaging stills the most useful imaging modality. Abolition of pain after inflating a blood pressure cuff above systolic blood pressure levels (ischemia test) is highly diagnostic, so we suggest the routine use of this simple test in cases of upper limb pain of unknown etiology clear. Surgical excision is the treatment of choice and is curative. (AU)

Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas.

OBJECTIVE: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. STUDY DESIGN: Retrospective study. METHOD: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. RESULT: Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. CONCLUSIONS: Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature.