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Glomus tumor can rarely arise in the central nervous system as a sella turcica mass. In this article, we report a case of sellar glomus tumor in a female patient who presented at the age of 8 years with visual impairment. The tumor recurred at 4 years and 26 years after initial excision and gamma knife therapy. Histologic examination showed a monotonous population of oval cells accompanied by delicate blood vessels, features mimicking pituitary adenoma. The tumor showed histologic progression at the second recurrence. Synaptophysin staining was positive, but chromogranin and CD56 were negative. The tumor cells were negative for epithelial markers but expressed actin and SMA. Awareness of the rare occurrence of glomus tumor at this region, careful analysis of morphology, and appropriate immunohistochemical workup are essential to solve this diagnostic challenge. The clinicopathologic features of all previously reported cases are reviewed.

Sinonasal tumours are rare, and among these there exist a small number of histologic subtypes that are infrequently encountered and rarely mentioned in the literature. These have been presented as either case reports or small case series, and their very low incidence makes prospective studies practically impossible. This review analyses the available literature, including our own experience and endeavours to outline management strategies, which involve a high index of suspicion and counselling of patients. In most instances, these tumours require aggressive multimodal treatment to improve survival outcomes. The overall prognosis remains dismal., (© 2020 S. Karger AG, Basel.)

Background: Endovascular embolization is an important tool in the management of multiple pathologies as a preoperative adjunct in the care of arteriovenous malformations or vascular tumors., Case Description: We report a case of delayed distal Onyx migration after surgical resection of a glomus vagale tumor, which had been preoperatively embolized. In this report, the patient underwent successful embolectomy of the migrated Onyx fragment using manual aspiration., Conclusions: This case represents the first in the literature to describe this potential delayed complication, as well as its management strategy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Glomus tumors (paragangliomas) are rare, usually benign, neuroendocrine tumors that arise from nonchromaffin cells and can be multifocal or familial. They grow slowly, often infiltratively and rarely form metastases. A distinction is made between carotid, jugular, tympanicum and vagal tumors. Depending on the location, clinical symptoms include neck swelling, hearing loss with pulse-synchronous ear noise or cranial nerve failures (IX-XI). Diagnosis is often delayed because of nonspecific symptoms. Diagnostically, tumor expansion and perfusion can be well visualized by color Doppler sonography. In the T2-weighted MRI, the tumor is hyperintense, after contrast agent administration a strong enhancement occurs due to the strong vascularization. In DSA (digital subtraction angiography), even small glomus tumors can be detected. Therapeutically, operative tumor resection after embolization with, e.g., polyvinyl alcohol particles or liquid embolization can be performed. If the patient is in poor general condition or if the tumor is inoperable, different treatment techniques (intensity-modulated radiotherapy, gamma or cyber-knife) may be considered as a conservative therapeutic alternative.

Background: Glomus tumors are benign neoplasms that most commonly present in the subungual region. Their occurrence in the oral cavity is exceedingly rare. Here, we present 2 cases from the oral cavity, detail their clinical and histopathologic features, and review the literature for solitary cases involving the oral regions., Study Design: The English language literature was queried for cases of benign glomus tumors in/around the oral cavity. Additional citations were cross-referenced from the identified sources., Results: Thirty-one cases of solitary glomus tumor in the oral and paraoral regions have been described, including the present cases. Patient age ranged from 10 to 85 years, with an average age of 47 years. In 12 of the 31 cases, the tumors occurred in the lips, 5 in the palate, 4 in the tongue, 4 in the buccal mucosa, 3 in the gingiva, and 1 each in the parotid, pterygoid fossa, and oropharynx. Only 18 of these cases had accompanying immunohistochemical stains, with 14 expressing positivity for muscle cell markers., Conclusions: Although glomus tumors have distinct histopathologic features, diagnostic confusion may exist with regard to extradigital locations. Detailed documentation and discussion of the clinical and histopathologic features of rare tumors like these are vital to understanding them., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: To describe our current multidisciplinary approach to pediatric malignant glomus tumors of the head and neck and review the current literature., Study Design: Retrospective chart review at a tertiary referral children's hospital and a comprehensive literature review., Methods: A comprehensive literature search of PubMed, Embase, Web of Science, Google Scholar, and EBSCO with respect to malignant glomus tumors of the head and neck was conducted. We obtained expert input from other pertinent specialties, including oncology, pathology, and radiology. To highlight the difficulty of evaluation and management of these patients, we also present a pediatric patient with a left neck malignant glomus tumor and lung metastases., Results: Only two cases of pediatric malignant glomus tumor (including our own) have been reported in the English literature. Overall, 14 malignant glomus tumors have been reported in the head and neck (11 primary and three metastatic). Surgical resection is the mainstay of treatment, but local recurrence is common (five of 11, 45%)., Conclusions: Malignant glomus tumor of the head and neck is an extremely rare tumor in children. Evaluation consists of imaging, and tissue biopsy is necessary for definitive diagnosis. Management options include surgical resection with or without an adjuvant chemotherapy protocol similar to those designed for sarcoma. Additional reports are necessary so that we may determine the utility, if any, of radiotherapy in the management of this tumor. Laryngoscope, 127:2873-2882, 2017., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

Glomus tumors are rare mesenchymal neoplasms with a phenotype akin to the modified smooth muscle cells of the glomus body. Most are benign, but rare examples show malignant histologic characteristics and aggressive behavior. We recently encountered a malignant glomus tumor with BRAF V600E mutation. We sought to study a large cohort for this mutation, with particular attention to associated malignant histologic characteristics. Tumors were classified based on WHO criteria as benign, uncertain malignant potential (glomus tumors of uncertain malignant potential-GT-UMP), or malignant. Tumors were screened for BRAF V600E by immunohistochemistry, and positive staining was evaluated further by Sanger sequencing. A total of 102 glomus tumors were included and classified as benign (57, 56%), GT-UMP (15, 15%) and malignant (30, 29%). Tumors occurred in patients aged 8 to 89.9 years (median: 50.2), without sex predilection (55% men). Most occurred in the superficial soft tissue (84%) and upper extremities (55%). Six of 95 tested cases had BRAF V600E mutation (6%), including 0 of 57 benign tumors, 3 of 14 GT-UMP (21%), and 3 of 24 malignant tumors (12%). Follow-up was obtained for 59 cases (median: 75.7 mo, range: 7.8 to 268.5). Three of 11 malignant tumors (27%) had progressive disease: 1 with metastasis to brain and heart, 1 with enlarging residual disease, and 1 with recurrence. Two of 4 GT-UMP (50%) had progressive disease: 1 with metastasis to lung, and 1 with local recurrence (50%). Three of 44 benign tumors (7%) had local recurrence. Two of 5 patients with BRAF V600E had progression, including 1 GT-UMP with local recurrence and 1 malignant tumor with enlarging residual disease. In summary, BRAF V600E mutation was detected in 6% of glomus tumors, all of which were malignant or GT-UMP. This mutation may be associated with a malignant phenotype, although study of additional cases is needed. In patients with progressive disease, BRAF could be a promising therapeutic target.

We present a case of a 60-year-old woman with a 2-year history of nasal obstruction and unilateral recurrent epistaxis. Anterior rhinoscopy identified a unilateral hypervascular lobular mass occupying the entire left nasal cavity. Imaging studies documented an extensive soft tissue density lesion in the left nasal cavity with complete infiltration of the anterior ethmoid, limited indentation of the medial wall of the orbit, bone demineralisation of the cribriform plate and involvement of the anterior cranial fossa. The diagnosis of locally invasive glomangiopericytoma was obtained preoperatively through biopsy of the lesion, which resulted in a self-limited epistaxis that was easily controlled by nasal packing, confirming the vascular nature of the lesion. We discuss the diagnostic work up, imaging and surgical approach of this tumour, and review the recent literature on endoscopic treatment of these lesions., (2016 BMJ Publishing Group Ltd.)

Background: Glomuvenous malformations (GVMs) (previously known as glomus tumours) are uncommon, benign, vascular neoplasms. Current treatments include surgical excision and sclerotherapy, often with high recurrence rates and poor cosmetic results., Objective: We sought to use a nonsurgical approach for treatment of a GVM., Methods: We present a patient with an acquired, biopsy-proven GVM of the heel unamenable to surgical excision, treated with a long pulsed 1064-nm neodymium-doped yttrium aluminum garnet (Nd:YAG) laser., Results: Excellent cosmesis and long-term remission were achieved after several treatment sessions., Conclusion: Our experience provides further evidence to support the safety and effectiveness of the 1064-nm Nd:YAG laser in the management of large and surgically challenging GVMs., (© The Author(s) 2015.)

Glomus tumors are very infrequent in the spine. The lesions can grow intraosseously along the entire spinal axis. A single female presenting with back pain from the upper thoracic spine is reported on. Removal of this lesion may require reconstruction of the anterior column with posterior fixation resulting in significant blood loss. The current report describes an embolization procedure prior to removal in order to reduce the significant blood loss that occurs with removal of this lesion, and summarizes the clinical and pathological characteristics of this rare tumor. A single, recent case and removal of an intraosseous tumor arising from the upper thoracic vertebra of T2-T4 is described. A 45-year-old female presenting with symptoms secondary to a glomus tumor of the upper thoracic vertebra of T2-T4 underwent resection of the lesion followed by reconstruction of the anterior column following preoperative emobolization. She had neurological symptoms for 3 years, and an irregular crescent-shaped lesion was seen going through the foramen at T3 to the chest cavity in the MRI scans. The operation was performed with a posterior approach in a single stage. The use of preoperative embolization of the T2-T4 segmental arteries resulted in significantly less blood loss as compared to without an embolization procedure. It was confirmed by histopathological examination that the glomus tumor rose from the smooth muscle cells in the right paravertebral muscles of T2. The glomus tumor has not recurred in the MRI during the five-year follow-up. Intraosseous glomus tumors are rare lesions that may extend into the epidural space and through the neural foramina and chest compartments resulting in neurological compromise. Over time, they may grow very large. Radiotherapy can be useful for eradication of this rare lesion. However, it can reoccur requiring extensive surgery resulting in significant blood loss. Preoperative embolization results in a reduction of blood loss and can be a very useful technique when performing the resection of large lesions suspected to be glomus tumors.

Benign lesions comprise a majority of soft tissue tumors. It has been estimated that their incidence outnumbers that of malignant tumors by a factor of at least 100 [1]. While history and physical examination can start the diagnostic process, imaging including the use of magnetic resonance imaging can be more helpful. Biopsy of these tumors is sometimes necessary and can be performed in a number of ways, often in conjunction with definitive treatment. Specific diagnostic and treatment strategies for a number of the more commonly encountered benign soft tissue tumors including lipomas, pigmented villonodular synovitis and hemangiomas are reviewed. An algorithm for the management of benign soft tissue tumors is discussed.

We report the cases of two women aged 28 and 34 years who presented recurrent glomus tumors of the hand after surgery for marginal resection of the tumor mass. The pathological study of the surgical specimen confirmed the diagnosis of recurrent glomus tumor. Due to the vascular origin of this tumor, sclerotherapy was delivered. The functional outcomes were good with dramatic pain relief within a few days. At 3-year mean follow-up, the cosmetic and functional results were very satisfactory., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Objective: Surgical resection of complex glomus vagale tumors can be complicated by extensive blood loss and might require surgical sacrifice of an encased internal carotid artery., Methods: A young patient presented with mass effect from glomus valage tumor. Computerized tomography angiography showed an encased internal carotid artery. Cerebral angiography demonstrated a highly vascular tumor. A literature review was performed for endovascular treatment options for neck tumors., Results: Staged preoperative embolization of feeder arteries via internal maxillary artery, and thyrocervical trunk with onyx was performed. A covered stent was implanted in the cervical internal carotid artery to the common carotid artery; this resulted in complete devascularization of the tumor with exclusion of external carotid artery from the circulation. This is followed by surgical resection of the tumor., Conclusion: Preoperative embolization with onyx decreased the amount of blood loss intra-operatively. The implantation of a covered stent in the cervical internal carotid artery through the common carotid artery contributed for further devasculatization of the tumor bed, as well as provided a lumen continuity in case iatrogenic carotid injury is encountered intra-operatively.

The glomus tumor is a rare, benign, but painful vascular neoplasm arising from the neuromyoarterial glomus. Primary intraosseous glomus tumor is even rarer, with only about 20 cases reported in the literature so far, 5 of which involved the spine. Surgical resection is currently considered the treatment of choice. We herewith present an uncommon case of primary intraosseous spinal glomus tumor involving the right pedicle of the eleventh thoracic vertebra (T11). To our knowledge, this is the first case of primary intraosseous spinal glomus tumor successfully treated by percutaneous CT-guided radiofrequency ablation (RFA).

Purpose: Glomus tumors are rare tumors, highly vascular and typically radiosensitive. Therapeutic options include surgery, radiation therapy (RT), embolisation or any combination of them, but the appropriate treatment still remains a challenge. The purpose of this study was to report the results of local control of 7 patients with glomus tumors treated with surgery and external beam RT (EBRT)., Methods: All of the patients underwent primary surgery and then postoperative EBRT. Follow-up was calculated from the date of initiation of EBRT and ranged from 3 to 15 years (mean 7.14, median 6.2). The likelihood of local control was analysed using the Kaplan-Meier product limit method. We also analysed the average duration of response between two groups of patients with different doses of EBRT as well as the presence of acute and late EBRT complications., Results: Local control was obtained in 6/7 (85.7%) patients. Moreover, local control was achieved in 3/4 (75%) patients with recurrent glomus tumors, while in patients with postoperative residual disease local control was obtained in 3/3 (100%) of them. Patients who received <50 Gy (n=2) had shorter average duration of response compared to patients who received >50 Gy (n=5; p=0.248). There were no severe treatment complications., Conclusion: Surgery and RT represent an appropriate treatment approach for advanced glomus tumors with acceptable complications.

Glomus tumors are mesenchymal neoplasms and are rarely seen in visceral organs like the kidney. Our patient presented with a metastatic, malignant, and highly aggressive glomus tumor in the kidney. In our extensive literature review, we did not come across even a single case of malignant glomus tumor arising in the kidney. We report the clinical presentation, radiologic, and pathological features of our case. Immunohistochemical findings that distinguish our case from other reported cases of glomus tumors arising from the kidney have been discussed. We have also reviewed the criteria for malignancy and other reported malignant glomus tumors., (Copyright © 2011 Elsevier Inc. All rights reserved.)

A 66-year-old man presented with acute respiratory distress due to a tracheal tumor involving the posterior wall of the upper trachea, with nearly complete airway obstruction. Partial debulking of the tumor's endoluminal component, via rigid bronchoscopy and yttrium-aluminum-perovskite laser, allowed timely and effective airway restoration. The diagnosis was benign tracheal glomus tumor. Two weeks later, elective tracheal sleeve resection with end-to-end anastomosis allowed complete resection of the lesion. No tumor recurrence was found at 21-month follow-up. We describe the multidisciplinary management of this extremely rare tracheal tumor, and review its features.

Background: Glomus tumours are benign painful tumours of the glomus body, a thermoregulatory shunt in the digits. Glomus tumours of the fingers and toes are associated with the monogenic disorder neurofibromatosis type 1 (NF1) and are recently recognised as part of the NF1 phenotype., Methods and Results: A multi-institutional experience with 15 individuals with NF1 and glomus tumours of the fingers or toes is reported. The majority of individuals presented with at least two of the symptoms in the classic triad of localised tenderness, severe paroxysmal pain, and sensitivity to cold. Appearance of the nail and finger or toe is often normal. Women are affected more often than men. Multifocal tumours are common. There is often a delay in diagnosis of many years and clinical suspicion is key to diagnosis, although magnetic resonance imaging may be useful in some scenarios. Surgical extirpation can be curative; however, local tumour recurrence and metachronous tumours are common. Three of our patients developed signs and symptoms of the complex regional pain syndrome., Conclusions: Glomus tumours in NF1 are more common than previously recognised and NF1 patients should be specifically queried about fingertip or toe pain.

This article discusses the epidemiology, etiology, presentation, pathology, evaluation and staging, and treatment of unusual skin tumors, such as Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

Objective: To analysis the effectiveness of preoperative digital subtraction angiography (DSA) on glomus jugularis tumors., Methods: A retrospective study of 14 cases of glomus jugularis tumors which received preoperative DSA was carried out. Among them, 10 cases were accepted balloon occlusion test and selective embolization of supplied vessels., Results: Hypervascular changes was found in all 14 cases and compression of internal carotid artery was found in 5 cases. Ascending pharyngeal artery was the main supplied vessel. Balloon occlusion test was applied in 10 cases, 2 were intolerable and 8 were passed occlusion test. Ten cases were accepted embolizations and tumor blood supply was conspicuous decreased. Neurovascular complications did not occurred after DSA but postoperatively in 2 cases. Intraoperative bleeding was less in embolization patients than that of without embolization., Conclusions: Preoperative DSA association with balloon occlusion test and embolizations might be a safe option on surgery of glomus jugularis tumors.

Soft-tissue glomus tumors (or glomangiomas) are unrelated to neuroendocrine paragangliomas (glomus tympanicum, jugulare, and vagale). The authors present the first reported case of an orbital soft-tissue glomus tumor in a child. An 8-year-old girl developed rapidly progressive right-eye blindness, proptosis, and a sixth cranial nerve palsy. Magnetic resonance imaging demonstrated a homogeneously enhancing lesion extending from the right orbit through the superior orbital fissure to the cavernous sinus and middle cranial fossa. A biopsy specimen demonstrated the lesion to be a soft-tissue glomus tumor. Following angiography and embolization, a gross-total resection of the tumor was achieved. The patient was treated with adjuvant proton-beam radiotherapy. At 24 months follow-up her proptosis and sixth cranial nerve palsy had resolved and there was no evidence of tumor recurrence.

According to many practitioners, any vascular swelling is a "Hemangioma". This generic term leads to much confusion: some lesions present a spontaneous regression while the others tend to worsen all the life long. The only common feature is the histological pattern. The vast majority of the vascular lesions are benign and the malignant tumors are extremely rare. However, if some vascular lesions have minor consequences and can be easily managed, in many cases a curative treatment cannot be assessed and some complex lesions can threaten life. An International Classification has been established. It allows appreciating the differences between the various entities - malformations and tumors - their evolutive features and helps to decide the most appropriate investigations and treatment. Now, every surgeon should learn and use this classification, to achieve an accurate diagnosis and to improve the management of these vascular anomalies.

The authors present a case of multiple glomus tumors (GTs) of the gastrointestinal tract, representing the type of a gastric glomus tumor proper and large bowel glomangiomyomas with myopericytoma-like features, observed in a 46-year old female, with multifocal perivascular proliferations of primitive cells and hepatic involvement. Histologically, the multilobular gastric tumor and hepatic lesions corresponded to a typical glomus tumor, while the tumor situated in the transverse colon, up to 7 cm in diameter, presented as a glomangiomyoma infiltrative (with myopericytoma-like foci), and satellite tumors in the large bowel mucosa, 0.5-0.7 cm in diameter, represented small glomangiomyomas. In addition, the patient demonstrated two types of concomitant vascular lesions: 1/ intravascular spread in the form of neoplastic plugs that obliterated the lumen of medium-size veins, and 2/ microscopic perivascular proliferation of primitive, small cells seen in the vicinity of the main tumor and in the adjacent adipose tissue. The patient has been ill for 2.5 years; she has been subjected to a partial colectomy with a resection of the small intestinal loop, greater omentum and the right ovary, followed by chemotherapy. At present, she is stable, and the infiltration--especially in the epigastric region--has decreased. The picture may confirm the theory that multiple GTs develop in association with multifocal proliferation of perivascular stem cells, as well as that their ability to penetrate into the lumen of large vessels gives origin to satellite tumors, which are not necessarily metastatic. It seems that at present, the group of perivascular SMA+ tumors may include infantile-type myofibromatosis in adults, myopericytoma, glomangio(myo)pericytoma, glomangiomyoma, glomus tumor proper, and glomangioma. Most likely, also some tumors previously classified as hemangiopericytomas belong to this group. The distinctive feature present in at least some of the above listed perivascular tumors is their synchronous or metachronous growth in a particular region and their ability to occupy intravascular space as nodules or solid bands, which in turn may give origin to satellite tumors. Multifocal lesions associated with a short survival in a given patient will obviously support the presence of metastatic disease. In the remaining cases, determination whether the patient has metastatic disease requires deep consideration and caution, also while deciding on treatment to be employed.

Objective: To review a large series of extradigital glomus tumors in order to gain a better understanding of their presentation and provide guidelines to aid in their diagnosis and treatment., Patients and Methods: We performed a retrospective review of all extradigital glomus tumors seen at our institution during a 20-year period (1985-2005) to document the incidence of the classic triad of symptoms, the duration of symptoms, the contribution of imaging to making a definitive diagnosis, and the effectiveness of treatment., Results: Fifty-six different patients with extradigital glomus tumors presented as follows: glomus tumors in the hand (3), wrist (4), forearm (11), elbow (4), arm (4), shoulder (2), buttock (1), thigh (5), knee (10), leg (3), ankle (2), foot (2), back (1), nose (1), cheek (1), ear lobe (1), and trachea (1). Forty-eight patients presented with pain and localized tenderness, but only 1 patient presented with cold Intolerance. The average duration of symptoms was greater than 7 years, with most patients being evaluated previously and having their conditions misdiagnosed. Magnetic resonance imaging proved to be the most useful modality for localization of these lesions. Surgical resection was the definitive treatment and generally provided immediate and sustained pain relief., Conclusions: Extradigital glomus tumors are not a rare subgroup of glomus tumors. Treatment outcomes are excellent, but misdiagnosis and delayed diagnosis are common. Improved guidelines regarding symptoms and diagnosis of these neoplasms may reduce the morbidity, ensuing chronic pain, and psychiatric consequences of delayed diagnosis or misdiagnosis.

Nail abnormalities are varied and numerous. They include warts, keratoacanthomas, onychomatricomas, fibrokeratomas, osteochondromas, tumors (ie, glomus, giant cell, Koenen's, and others), and Bowen's disease. Although the gravity of these conditions may vary, prompt diagnosis and treatment is of the utmost importance. This article discusses the most common defects associated with the nail unit and its surrounding tissue, as well as the differential diagnosis and treatment of these conditions.

A 62-year-old female was admitted to our clinic in October 1999 with a 6-year history of hearing loss, tinnitus and otorrhea in the right ear. Physical examination showed a polypoid mass arising from the right middle ear and extending into the external auditory canal. Serohemorrhagic otorrhea was also observed. Serious hemorrhage occurred while taking a biopsy. The histopathologic diagnosis was a glomangioma. MRI demonstrated a hypervascular mass (10 x 15 mm(2)) completely filling the right middle ear. Preoperative embolization of the ascending pharyngeal artery was carried out. The tumor was completely resected via a transmastoid approach. No complications were observed postoperatively. At the most recent follow-up examination, 72 months after the operation, no sign of disease was present. The modified radical mastoidectomy approach used in this case proved to be a safe and efficacious method for removing the glomangioma.

The primary goals of preoperative embolization of glomus tumors are to reduce the blood loss in the surgical field, minimize the risk of operative complications, and prevent recurrence by contributing to complete resection. Obliteration of a vascular channel may provide additional help in symptomatic relief by decreasing the tumor volume. Selective transarterial embolotherapy outcomes of 17 patients, most of whom had inoperable tumors and were unable to have surgery/radiotherapy because of poor general health, were evaluated to investigate the therapeutic contributions, efficacy, and safety of embolization techniques in the treatment of different glomus tumors and to examine the role of the embolotherapy in the treatment algorithm of such lesions. The pattern of vascular supply of these tumors was also documented. Eleven glomus jugulotympanicum, 4 glomus caroticum, and 2 glomus vagale tumors were embolized for palliative or curative purposes between 1992 and 2000. Coils and combination of coils plus polyvinyl alcohol were used for embolization. Relief of symptoms and patient satisfaction were analyzed within 3 months after the intervention by a questionnaire combined with full ear, nose, and throat and neurological examinations. The results indicate that the use of embolotherapy in the treatment of glomus tumors must be basically preoperative but is not curative.

Glomus tumors of the head and neck are rare tumors of adulthood which arise from paraganglia or glomus cells within the carotid glomus, vagus nerve, middle ear or jugular foramen. The diagnosis of these mostly benign lesions is predominantly done with CT and MRI. DSA can provide important additional information. Besides surgical resection, one therapy option is radiological intervention with tumor embolisation. Because of the typical radiological imaging and the "salt and pepper appearance" in MRI, glomus tumors can be differentiated from other lesions in the head and neck. This review gives a survey of the classification, diagnosis and therapy of paragangliomas with images to demonstrate characteristic features.

Hereditary multiple glomus tumours constitute an autosomal dominant skin disease which is known to demonstrate cutaneous mosaicism typified by type 1 and 2 segmental arrangements. We report a patient with type 2 segmental multiple glomangiomyomas who was disturbed by the pain of her lesions. A symptomatic lesion was successfully treated with the pulsed dye laser and to date there has been no recurrence of the pain. Possible explanations for the clinical response are discussed.

Objective: To describe the new L95P mutation of the paraganglioma 1 gene for glomus tumors in a Dutch paraganglioma 1 family with six affected family members and to report the clinical findings and results of treatment in nine glomus tumors with a maximum follow-up of 34 years., Setting: Tertiary referral centers., Results: Mutation analysis of the SDHD gene of paraganglioma 1 showed the L95P mutation in six affected family members and two nonaffected carriers protected from becoming affected by genomic imprinting. In six affected family members, nine glomus tumors (five glomus caroticum tumors, two glomus vagale tumors, and two glomus jugulare tumors) were traced. The ages at presentation varied from 25 to 61 years. In two of six affected family members with a total of four tumors, all the tumors were traced in the extended family study, using magnetic resonance imaging; at that time these tumors were silent. After radiotherapy in one patient at the age of 34 years, a T4 planocellular carcinoma of the tongue occurred within the previous radiation field 27 years later, when the patient was 61 years old. Volume measurements of three untreated glomus tumors (two glomus vagale tumors, one glomus caroticum tumor) during 25 months showed an increase in two tumors (left glomus caroticum, left glomus vagale tumor) and a decrease in one tumor (right glomus vagale tumor). Surgery to remove two bilateral and one unilateral glomus caroticum tumors was successful. A wait-and-see policy is being applied to two glomus vagale tumors., Conclusions: In family members of paraganglioma 1 patients, mutation analysis can be used to make an early diagnosis of glomus tumors. Radiotherapy may have induced a carcinoma. Modalities of treatment can include a wait-and-see policy. Long-term follow-up studies on the natural course of glomus tumors are needed to improve decisions about treatment modalities.

Glomangiomas are characterized by cavernous vascular channels surrounded by glomus cells. Multiple glomangiomas, although usually painless, can be a few centimetres in size and appear as blue phlebectatic lesions. Surgical excision of multiple glomangiomas can lead to scarring and recurrences. Laser treatment using vascular lasers and CO2 lasers has been useful in small, superficial lesions. We present the successful sclerotherapy treatment of multiple glomangiomas using sodium tetradecyl sulphate in a 59-year-old man who presented with postoperative recurrence of multiple lesions.

Glomus tumors (glomangiomata) are benign tumors arising from glomus cells. Multiple glomangiomata are less frequent and less painful than the solitary variant, which is usually located subungually. Nonetheless multiple glomangiomata--sometimes being sensitive to pressure and changes in temperature--may cause considerable discomfort. Treatment of multiple glomangiomata is problematic because of the often large number of tumors. Sclerotherapy represents an alternative to surgical and cryosurgical therapy. We report on sclerotherapy in a 35 year old female patient with multiple hereditary glomus tumors.

Hereditary multiple glomangiomas are an interesting rare problem which can either present as multiple painful cutaneous lesions or, rarely, a cosmetic challenge due to site, size or number of lesions. It may also pose a diagnostic dilemma, which can only be settled by biopsy. Malignant change in glomangioma is extremely rare and only a few cases are well documented in literature. A case report of this condition, along with extensive review of the literature, is presented. The prognosis of various forms of glomangiomas and efficacy of diagnostic and therapeutic tools is discussed.

Objective: To determine if technetium Tc 99m-labeled macroaggregated albumin selective angioscintigraphy enables estimation of microparticle-arteriovenous shunt in branchial paragangliomas and nasopharyngeal angiofibromas., Design: Technetium Tc 99m-labeled macroaggregated albumin particles with calibrated diameters between 25 and 50 microns were administered during selective catheterization of the feeding arteries in 4 branchial paragangliomas (glomus tumors) and 4 nasopharyngeal angiofibromas. Preliminary measures of the activity of the tumor region and of the lungs were obtained in the angiography suite using a hand-held detector. Without changing the position of the microcatheter, definitive embolization was performed. After endovascular treatment, scintigraphy was performed and the pulmonary shunt index was calculated with the use of a gamma camera and a dedicated computer program., Results: The pulmonary shunt index varied between 0% and 36% for branchial paragangliomas (glomus tumors) and between 82% and 95% for angiofibromas., Conclusions: Our results provide new evidence that the incidence of microparticle-arteriovenous shunt in nasopharyngeal angiofibromas is much higher than in paragangliomas.

Purpose: To present and evaluate a devascularization technique for hypervascular tumors of the head and neck by direct tumor puncture., Methods: Tumor puncture was performed percutaneously or via natural orifices (nose and mouth). In one case, an intrasellar tumor was embolized via a transseptosphenoidal surgical approach. The embolization material used was NBCA, lipiodol, and tungsten in the majority of tumors (14 out of 17) and alcohol for 3 metastases of the calvarium. We used this technique to embolize 10 nasopharyngeal fibromas, 4 tumors of the calvarium (3 metastases and 1 hemangiopericytoma), 1 intrasellar hemangiopericytoma, and 2 glomus tumors. Reflux of blood was obtained in every case after direct puncture of the tumor. Direct injection of contrast agent into the tumor revealed local parenchymography followed by local and regional venous drainage without extravasation., Results: Total devascularization was obtained in 14 cases, and devascularization greater than 90% was obtained in 3 cases. Thirteen tumors were totally resected without requiring blood transfusion. During surgery, the limits of the exsanguinated tumor were very well defined in every case by the black staining induced by tungsten. Of the 4 tumors embolized but not operated on (3 metastases and 1 glomus tumor), 2 metastases needed retreatment after 6 and 8 months of remission, respectively. The other metastasis is still in remission after 3 months, and the volume of the glomus tumor decreased by 80% remains unchanged after 8 months., Conclusion: This technique was initially used to devascularize tumors with difficult or dangerous intravascular access, but in view of the hemodynamic and surgical results obtained, we believe that the indications for this technique can be extended to hypervascular tumors accessible to conventional embolization.