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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.)

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.

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.

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.

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.

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.

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.