A 65-Year-Old Man Presenting to the Emergency Department with Gastric Hemorrhage Caused by a Glomus Tumor.

BACKGROUND Glomus tumor is a benign but rapidly growing mesenchymal tumor that is a rare in the gastrointestinal tract, can be locally invasive due to its rapid growth, and can result in perforation of a viscus. We report a 65-year-old man presenting as an emergency with gastric hemorrhage and gastric glomus tumor. CASE REPORT A 65-year-old man came to our hospital for a life-threatening upper digestive hemorrhage. The preoperative examinations (digestive endoscopy without sampling of biopsy fragments and contrast-enhanced computer tomography) led to the presumptive diagnosis of gastrointestinal stromal tumor. Wedge resection of the gastric wall was performed. The histopathological examinations revealed a proliferation of round-oval cells of medium size with a solid disposition and in nests. This proliferation dissected the muscular tunic and caused ulceration of the gastric mucosa. Immunohistochemical tests confirmed the diagnosis of glomus tumor and excluded other diagnoses (neuroendocrine tumor or gastrointestinal stromal tumor). The postoperative evolution was favorable, and at the time of discharge, the biochemical test values normalized. CONCLUSIONS Pathologists are faced with a challenging task due to the deceptive appearance that can be presented by such a rare tumor. Histopathological and immunohistochemical examinations are essential in achieving a precise diagnosis and assessing the biological potential of the glomus tumor. Even if it is a benign tumor, the clinical picture it causes can still be a major risk to the patient's life. Consequently, ensuring effective case management becomes crucial, as it requires a thorough comprehension of all conditions encompassed in the differential diagnosis.

A rare cause of AA amyloidosis: Glomus tumor and treatment with anakinra-Case report and literature review.

Systemic AA amyloidosis is associated with poorly controlled chronic inflammatory disorders. Chronic infections and inflammatory arthritis are the most common causes; however, they can also rarely occur as a complication of neoplastic disorders. The development of AA amyloidosis secondary to paraganglioma, which is a rare type of tumor, has rarely been reported in the literature. In this case, an 85-year-old female patient with a glomus tumor in the neck, who has been followed up over 50 years, applied with complaints of loss of appetite, nausea, and diarrhea for 5-6 months. While evaluating the patient, who had high levels of acute phase reactants, amyloidosis was diagnosed by salivary gland biopsy. No other cause was found to explain amyloidosis. The patient, who could not tolerate treatment with colchicine and azathioprine, is successfully treated with the interleukin-1 inhibitor anakinra. A rare relationship, systemic AA amyloidosis, which is thought to have developed as a result of long-standing jugular paraganglioma, is presented in this article. In addition, publications showing an association between paragangliomas and amyloidosis were reviewed.

Metastatic Malignant Glomus Jugulare Tumor: A Rare Case Report with Clinical Manifestations and Treatment Approach.

BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.

Glomus Tumor of the Stomach Presenting With Upper Gastrointestinal Bleeding: A Case Report.

Gastric glomus tumor is a rare mesenchymal tumor of the gastrointestinal tract, accounting for approximately 1% of all gastrointestinal soft tissue tumors. We describe a unique case of a 27-year-old female patient who presented with recurrent episodes of overt gastrointestinal bleeding requiring multiple blood transfusions. The patient was diagnosed with a gastric ulcer detected on esophagogastroduodenoscopy (EGD), which was grossly suggestive of an ulcerated gastrointestinal stromal tumor (GIST). Preoperative diagnosis was difficult, requiring laparoscopic robotic-assisted local wedge resection of the gastric mass. Pathological diagnosis and immunohistochemical (IHC) studies were consistent with a glomus tumor. We emphasize that the gastric glomus tumor might present with life-threatening recurrent gastrointestinal hemorrhage. In addition, it might mimic GIST and require surgical resection. Pathological diagnosis and IHC studies are needed to confirm the diagnosis.

Glomus Tumor Within the Tensor Fascia Lata: A Case Report.

BACKGROUND Glomus tumors are rare, benign, soft-tissue lesions, usually occurring in the hand, but they can occur in other regions of the body, such as the thigh. Most of the time, extradigital glomus tumors are difficult to diagnose, and symptoms can persist for a long time. The usual clinical presentations consist of pain, tenderness at the site of the tumor, and hypersensitivity to cold. CASE REPORT We report a case of a GT of the proximal thigh in a 39-year-old man with left thigh pain without palpable mass for several years, without clear diagnosis. He had pain and hyperesthesia exacerbated by running. The patient was diagnosed initially by ultrasound imaging, which revealed a round, solid, hypoechoic, homogeneous mass in the left upper thigh. Magnetic resonance imaging (MRI) with contrast showed a well-defined intramuscular lesion in the tensor fascia lata. A percutaneous biopsy was done through ultrasound guidance, followed by excisional biopsy and immediate pain relief. CONCLUSIONS Glomus tumors of the thigh are a rare neoplasm, especially in the proximal thigh; they are difficult to diagnose and are associated with morbidity. Diagnosis can be made through a systematic approach and simple investigation, such as via ultrasonography. A percutaneous biopsy can help in drawing up a management plan, and malignancy must be considered if the lesion is suspicious. Symptoms can persist in case of incomplete resection or unrecognized synchronous satellite lesions; thus, symptomatic neuroma should be considered.

Post-traumatic glomus tumor of the left anterior supraclavicular nerve: a case report.

OBJECTIVES: Peripheral nerve glomus tumors are extremely rare and occur with typical symptoms of peripheral neuropathic pain. Clinicians hardly consider this entity when faced with the swelling of a peripheral nerve and the diagnosis is reached only with histological examination. Nerves of limbs are usually affected and the solid glomus tumor is the most frequent histological variant. CASE DESCRIPTION: A 55-year-old man presented with a glomus tumor of the anterior supraclavicular nerve of the left cervical plexus, misdiagnosed clinically and radiologically as neuroma. Despite the preoperative suspicion and the intraoperative appearance, the histological examination revealed a glomus tumor with a prevalent muscular component, a glomangiomyoma. Once the tumor was removed, pain regressed completely. CONCLUSIONS: Because of its rarity, pre-operative diagnosis of glomus tumors is still a challenge, especially when arising from peripheral nerves. In the presence of chronic localized neuroma-type pain and sensitivity, glomus tumors should be considered in the pool of differential diagnosis, even if the imaging is not conclusive.

Tracheal Glomus Tumor Complicated with Asthma Exacerbation in a Pregnant Woman.

A 34-year-old pregnant woman in the 34th week of gestation with uncontrolled asthma was admitted because of asthma exacerbation. Although she received bronchodilators and systemic corticosteroids, respiratory failure rapidly progressed. Chest computed tomography revealed a mass occluding approximately 80% of the tracheal lumen. After urgent Caesarean section, endobronchial resection was performed. The pathological findings of the resected tumor were compatible with tracheal glomus tumor. Tracheal tumors are often misdiagnosed as asthma, but its complication with asthma is rare. Even if the diagnosis of asthma is definitive, clinicians should consider coexisting diseases, including tracheal tumors, when asthma control is poor.

Posttraumatic Glomus Tumor of the Digital Nerve with Atypical Presentation: A Case Report.

CASE: A 53-year-old man sustained an injury to his left index finger and underwent presumably traumatic neuroma excision from the radial digital nerve 2 years ago. He presented with a painful mass distal to the prior site of neuroma excision with distinctly different symptoms from those that led to his index surgery. Thus, he underwent excisional biopsy of the mass which was adherent to his radial digital nerve consistent with a benign glomus tumor on histologic examination. CONCLUSION: Digital nerve glomus tumors are rare. In most of the cases, some portion of the digital nerve requires excision but decreased pain can be expected.

A rare combination of tumor-induced osteomalacia caused by sinonasal glomangiopericytoma and coexisting parathyroid adenoma: case report and literature review.

BACKGROUND: Tumor-induced osteomalacia (TIO) is a rare, acquired disease of renal phosphate wasting and disturbed vitamin D homeostasis as a result of the action of a phosphaturic protein - FGF-23, produced by a neoplasm. Although the clinical and biochemical profile of the syndrome is characteristic, it remains underreported and unrecognized by clinicians. Hyperparathyroidism is rarely associated with oncogenic osteomalacia, but it should be considered because of potentially life-threatening hypophosphatemia caused by both conditions. CASE PRESENTATION: We report a case of a 42-year-old woman admitted to the Department of Otolaryngology of the Military Institute of Medicine in Warsaw for the endoscopic resection of hormonally active glomangiopericytoma extending into the anterior skull base. She presented with a 5-year history of musculoskeletal pain and progressive weakness of the extremities which finally led her to become bedridden. After the excision of the tumor her symptoms and laboratory results gradually improved except increasing PTH serum levels. Further examination revealed a parathyroid proliferative tumor, which was surgically removed. The patient walked without aids at follow-up 16 months after the surgery. CONCLUSIONS: This case is unusual because of tumor-induced osteomalacia and parathyroid adenoma occurring concomitantly. Further investigations of FGF-23 and PTH interplay should be conducted to elucidate the pathogenesis of hyperparathyroidism and tumorigenesis in some cases of TIO. By presenting this case, we wanted to remind clinicians of a rare and misdiagnosed paraneoplastic syndrome and highlight the importance of monitoring PTH concentrations during the follow-up of patients with TIO.

An unusual case of chest wall glomus tumor presenting with axillary pain: a case report and literature review.

BACKGROUND: Glomus tumor is an uncommon soft tissue tumor. However, as the tumor causes significant disability, its early diagnosis is essential. It involves subungual areas of fingers and toes in most cases, and its extra-digital involvement is rarely seen. To the best of the authors' knowledge, only a few chest wall involvement cases have been reported in the literature. CASE PRESENTATION: In this paper, we describe a 63-year-old patient with a chest wall glomus tumor presenting with axillary paroxysmal pain and limitation in his shoulder range of motion that had been missed for nearly 15 years. His symptoms were relieved immediately following surgical excision. CONCLUSION: Glomus tumors may involve any part of the human body. It is curable with surgical excision in most cases. Therefore, a correct early diagnosis has paramount importance. A high index of suspicion is needed for early diagnosis, especially when the tumor involves uncommon anatomic areas.

Unilateral lower limb atrophy associated with glomus tumors: a case report.

BACKGROUND: Glomus tumors are soft tissue neoplasms comprised of glomus cells, vasculature, and smooth muscle cells, which occur commonly in a single subungual area of the digits, and their main clinical features include severe paroxysmal pain, localized tenderness, and cold hypersensitivity. CASE PRESENTATION: A 47-year-old Japanese man had suffered from chronic progressive paroxysmal shooting pain in his right leg since childhood. He avoided putting weight on his right foot whenever he walked. The frequency of paroxysmal pain and the number of tender points both gradually increased with age, and his right leg gradually atrophied. Magnetic resonance imaging of the lower extremity demonstrated multiple gadolinium-enhanced nodules that corresponded with his tender points. Excisional biopsy relieved his pain and provided a histopathological diagnosis of glomus tumors. CONCLUSION: This case suggests that small glomus tumors located in deep tissue may cause disuse atrophy because of their long delay before diagnosis. Clinicians should consider the potential for glomus tumors when patients exhibit unilateral lower limb muscular atrophy with pain.

Mácula telangiectásica como forma de presentación de un tumor glómico solitario / Solitary Glomus Tumor Presenting as a Telangiectatic Macule

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Transungual Excision of Glomus Tumors: A Treatment and Quality of Life Study.

BACKGROUND: Glomus tumors are rare benign painful tumors, frequently found in the subungual region. Complete surgical excision is essential for relief of symptoms. The main postoperative complications are tumor recurrence and nail dystrophy. OBJECTIVE: To evaluate the long-term outcome and the impact on quality of life (QoL) of glomus tumors after a transungual approach. MATERIALS AND METHODS: A retrospective cohort study was conducted. Twenty-six patients underwent transungual excision of subungual glomus tumors. A self-administered questionnaire was sent to evaluate the postoperative outcome. Glomus tumor-related QoL was investigated using modified nail psoriasis (NPQ10) and onychomycosis questionnaires. RESULTS: A response rate of 85% was achieved. After a mean follow-up of 63 months after transungual excision of the tumor, the mean Numeric Pain Rating Score had improved from 7.9 (±SD 1.8) preoperatively, to 0.8 (±SD 1.9) (p < .000). Quality of life improved significantly: the mean NPQ10-score improved from 5.5 (±SD 3.4) to 0.64 (±SD 2.1) (p < .000). Nail-related sequelae were not reported in any of the patients. CONCLUSION: Our study showed that glomus tumors cause impairment on QoL, mostly due to severe pain. Surgical excision with the transungual approach is an effective treatment, without permanent damage to the nail unit that gives relief of pain and improves QoL.

Glomus tumors of the head and neck: thirteen years' institutional experience and management.

Background: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. Aims/objectives: This study aimed to express the safety of paraganglioma surgery regarding complications, treatment, and outcomes of patients with head and neck glomus tumors. Materials and methods: Medical records of patients who were operated because of head and neck paraganglioma between 2006 and 2016 were reviewed. Results: The study group consisted of 49 patients (M/F: 6/43). The patients were distributed as follows: 22 glomus caroticum (GC) (44.8%), 8 glomus jugulare (GJ) (16.3%), 10 glomus tympanicum (GT) (20.4%), 4 glomus vagale (GV) (8.1%), 2 GC + GV (4%), 2 bilateral GC (4%) and 1 thyroidal glomus tumor (2%). All GC and GV tumors were resected via cervical approach. Three of GJ tumors were resected through transmastoid approach while five of them were resected through both transmastoid and cervical approach. Nine GT tumors were resected via transmastoid approach. One patient received cyberknife. Thyroid paraganglioma was diagnosed incidentally after total thyroidectomy. Mean follow-up period was 61.92 ± 35.11 months (1-124 m). Conclusions and significance: The choice of treatment depends on the size, location and biologic activity of tumor as well as the physical condition of the patient. Our results show that glomus tumors can be resected with low mortality and morbidity rates due to developing imaging and microsurgical methods.

Malignant glomus tumor of the thyroid gland: a case report.

OBJECTIVE: Primary malignant glomus tumors of the thyroid gland are a rare occurrence. METHODS: A 68-year-old man found a mass on the right side of his neck in October 2017. An X-ray examination on 9 January 2018 showed multiple round reinforced masses in both sides of the lung. Computed tomography imaging of the neck showed a low-density mass on the right side of the thyroid gland. RESULTS: The immunohistochemistry results were positive for smooth muscle actin, calponin, collagen IV, and Ki-67 60%. The patient received chemotherapy starting on 17 January 2018, 8 February 2018, and 11 March 2018. The chemotherapy drugs included ifosfamide, epirubicin, and cisplatin. However, the patient subsequently developed multiple organ failure and died in April 2018, approximately 6 months after the initial discovery of the mass in his neck. CONCLUSIONS: Primary malignant glomus tumors of the thyroid gland are rare, and examination of their pathology and immunohistochemistry is vital for making an accurate final diagnosis. This case also indicates that primary malignant glomus tumors of the thyroid gland may have a poor prognosis, despite chemotherapy.