Stereotactic radiosurgery for hypervascular intracranial tumors.

OBJECTIVE: To evaluate the safety and efficacy of SRS treatment of central neurocytomas (CNCs), cavernous sinus hemangiomas (CSHs), and glomus tumors (GTs); and to compare upfront stereotactic radiosurgery (SRS) and adjuvant SRS in the treatment of these hypervascular tumors. METHODS: This was a retrospective review of consecutive CNCs, CSHs, and GTs treated with SRS between 1993 and 2017. Tumor response was categorized based on volumetric analysis on magnetic resonance imaging: (1) tumor regression [> 10% decrease in tumor volume (TV)]; (2) stable tumor (≤ 10% change in TV); or (3) tumor progression (> 10% increase in TV). RESULTS: Sixty hypervascular tumors (CNC: 28; CSH: 16; GT: 16) underwent SRS between 1993 and 2017. Margin doses were 13 Gy, 12 Gy, and 14 Gy for CNCs, CSHs, and GTs, respectively. Tumor regression was observed in 54 tumors (90%) with initial SRS after a median follow-up of 82 months. Relative reductions in TVs for CNCs were 38%, 52%, and 73% at 12, 24, and 48 months, respectively. Relative reductions in TVs for CSHs were 51%, 68%, and 88% at 12, 24, and 48 months, respectively. Relative reductions in TVs for GTs were 22%, 31%, and 47% at 12, 24, and 48 months, respectively. Upfront SRS was performed in 26 patients (43%). No difference in relative TV reductions were found between upfront and adjuvant SRS. Adverse radiation effects were observed in five patients. No mortality was encountered. CONCLUSION: SRS for hypervascular tumors is associated with high rates of tumor regression with low risk of complications. No significant difference in rates of relative TV reduction were found between upfront and adjuvant SRS. Hence, upfront SRS may be considered in select patients.

Glomuvenous malformations: dual PDL-Nd:YAG laser approach.

Glomuvenous malformations are uncommon simple vascular malformations that might be present at birth or appear during childhood that have been classically classified as a subtype of venous malformations. Sclerotherapy and surgery have been used in the past as treatments for this condition although with disappointing results in large glomangiomas. The treatment of these lesions has still not been standardized. We conducted a retrospective study of 17 patients treated with dual wavelength PDL-Nd:YAG. The majority of the patients experience a reduction of at least a 60% in their glomuvenous malformations. Treatment was well-tolerated, and adverse effects were rare.

A malignant glomus tumor in the nasal cavity responding to radiation therapy.

Malignant glomus tumors of the head and neck are extremely rare, and to our knowledge, a response to high-dose radiation has not been described previously. We report one case in an 80-year-old woman with right nasal cavity mass. Histological examination revealed sheets of atypical round glomus cells. The presence of increased mitotic activity (25 per 10 high-power fields), cellular atypism, and tumor necrosis suggested malignancy. The smooth muscle actin, vimentin, and h-caldesmon immunohistochemistry stains the tumor cells. Two cycles of doxorubicin and cyclophosphamide chemotherapy were done and the tumor size was slightly increased. Salvage radiation therapy (RT) was delivered to the primary mass over 4 weeks (50 Gy in 20 fractions) and leading to nearly complete regression of tumor. Additional investigations are warranted so that we may determine the usefulness of RT in the management of this rare tumor.

Radiotherapy of benign intracranial tumours.

We present the updated recommendations of the French Society for Radiation Oncology on benign intracranial tumours. Most of them are meningiomas, vestibular schwannomas, pituitary adenomas, craniopharyngiomas, and glomus tumours. Some grow very slowly, and can be observed without specific treatment, especially if they are asymptomatic. Symptomatic or growing tumours are treated by surgery, which is the reference treatment. When surgery is not possible, due to the location of the lesion, or general conditions, radiotherapy can be applied, as it is if there is a postoperative growing residual tumour, or a local relapse. Indications have to be discussed at a multidisciplinary panel, with precise evaluation of the benefit and risks of the treatments. The techniques to be used are the most modern ones, as multimodal imaging and image-guided radiation therapy. Stereotactic treatments, using fractionated or single doses depending on the size or the location of the tumours, are commonly realized, to avoid as much a possible the occurrence of late side effects.

Glomuvenous Malformations (Familial generalized multiple glomangiomas).

A 15-year-old boy with a diagnosis of generalized multiple glomangiomas was referred for evaluation and treatment of enlarging and increasingly painful lesions on his right ankle. The patient underwent a series of two treatments with long-pulsed KTP 1064 nm laser that resulted in substantial improvement in appearance and decreased pain. Generalized glomuvenous malformations, or multiple glomangiomas, are the less common presentation of proliferation of glomus cells and may have extracutaneous involvement. Whereas surgical management is often employed and definitive for solitary lesions, interventions such as laser therapy, may be beneficial for improvement of functional impairment and cosmesis as was observed in our patient.

Intracranial soft-tissue glomus tumor (glomangioma) in a young-woman. A case report and review of the literature.

BACKGROUND: Glomus tumors, or glomangiomas, are benign vascular tumors typically seen at distal extremities. These tumors differ from paragangliomas and classically present in the female population between the 4th and 5th decade. Intracranial localizations have not been described in literature in the adult population. CASE DESCRIPTION: We present a case of a 32 year-old woman with a 3 months history of progressive left-sided visual loss and headache. A pre-operative MRI showed a homogeneously enhancing lesion extending from the left cavernous sinus to middle cranial fossa at first suspected to be a cavernous sinus meningioma. Eventually, histopathological analysis concluded for a glomangioma diagnosis. Post-operative RT was also performed. CONCLUSIONS: From our experience it is very important for clinical management considering glomangiomas in differential diagnosis of a homogeneously enhancing extra axial mass. Subtotal resection followed by radiation therapy determined no recurrence of the disease up to 7 years. KEY WORDS: Glomangioma, Glomus tumor, Soft-tissue tumor.

Radiosurgery for Glomus Tumors.

Glomus tumors of the head and neck typically compress adjacent blood vessels and cranial nerves and result in varied clinical presentations. Moreover, they are seldom encountered, even at large medical centers, and specialists in neurosurgery, otolaryngology, and radiation oncology have yet to reach a generalized consensus regarding the optimal management approach. In an effort to summarize the available data and better elucidate optimal treatment and management strategies for glomus tumors, we conducted a review of the published literature on this topic with emphasis on stereotactic radiosurgery.

Gamma Knife radiosurgery for glomus tumors: Long-term results in a series of 30 patients.

BACKGROUND: Glomus tumors are rare and benign hypervascular tumors. Surgery represented the mainstay of their treatment, even if it has been associated with high morbidity and mortality rates. Recently, the treatment shifted to a multimodal approach and Gamma Knife radiosurgery represents one of the treatment options. METHODS: Authors retrospectively analyzed the clinical and radiological outcome of a series of patients who underwent Gamma Knife radiosurgery for glomus tumors. RESULTS: Thirty patients underwent Gamma Knife radiosurgery. Mean tumor volume was 7.69 cc (range 0.36-24.6). Mean tumor margin dose was 16 Gy (range 13-18). Median follow-up was of 91 months (mean 90; range 11-172). Overall clinical control rate was 100%; overall volumetric tumor control rate was 96.6%. Patients' and tumors' characteristics, treatment data, and outcome have been analyzed. CONCLUSION: Gamma Knife radiosurgery represents a safe and effective treatment for glomus tumors. Longer follow-up and larger cohort studies are needed to definitively outline the role of Gamma Knife radiosurgery for glomus tumors.

Less common indications for stereotactic radiosurgery or fractionated radiotherapy for patients with benign brain tumors.

Microsurgical resection remains the mainstay of treatment for truly benign brain tumors that can be safely resected because of the potential for permanent cure with most histologic findings, including most of the histologic findings discussed in this article. Physicians must keep in mind the indolent nature of many of the benign brain tumors and realize that many patients are likely to live out normal life spans if tumor control is achieved. Therefore, it is not sufficient simply to consider local tumor control rates and short-term toxicity risks when choosing between surgery, stereotactic radiosurgery, and fractionated radiotherapy. Patients need to be apprised of all therapeutic options and to make their decisions with all information required to evaluate the risks and benefits. For benign brain tumors, these decisions may have consequences that last for decades.

Role of radiosurgery in the management of glomus tumors.

BACKGROUND: Glomus tumors are benign slow-growing hypervascular neoplasms. The role of radiosurgery for the treatment of these tumors has increased. The purpose of this study was to show our experience with glomus tumors and to analyze different prognostic factors. METHODS: Data from 39 adult patients were retrospectively analyzed. All of them underwent head frame stereotactic radiosurgery with linear accelerator (LINAC). Tumor and symptomatic control were calculated using the Kaplan-Meier method. Bivariate statistical analyses were performed to examine different prognostic factors. RESULTS: The median follow-up was 71 months. The radiological local and symptomatic control was achieved in 37 patients (94.8%) and 29 patients (74.3%), respectively. Toxicity was detected in 4 patients (10%). In the bivariate analysis, dose coverage and maximum dose were associated with tumor control with an odds ratio (OR) of 5.29 (p = .041) and 2.67 (p = .056), respectively. CONCLUSION: Stereotactic radiosurgery is a safe and efficient treatment for glomus tumors that is associated with high probability of radiological and symptomatic control and low incidence of morbidity. © 2015 Wiley Periodicals, Inc. Head Neck 38: E798-E804, 2016.

Unusual eustachian tube mass: glomus tympanicum.

SUMMARY: A case of recurrent glomus tympanicum presenting with epistaxis is described. CT and MR imaging revealed a homogeneously enhancing mass extending along the entire course of the eustachian tube, with a portion protruding into the nasopharynx. Glomus tumors tend to spread along the path of least resistance and may extend into the eustachian tube. The unique imaging appearance should place a glomus tumor high on the list of differential diagnoses.

Jugulotympanic paraganglioma (glomus tumour) presenting with recurrent epistaxis.

A case is presented where a left jugulotympanic paraganglioma (JTP) extended to the nasopharynx and the patient presented with recurrent epistaxis. Although initial biopsy of an aural polyp had been suggestive of the diagnosis several years previously, the diagnosis was not confirmed until the patient presented with recurrent epistaxis and severe anaemia. To the best of our knowledge, this is the first case reported of such a presentation of JTP.

Primary radiation therapy in the management of glomus tumours.

The records of five patients who received local irradiation for incompletely excised or inoperable glomus tumour were retrospectively reviewed. Age ranged from 25 to 46 years. There were two female and three male patients. Two patients who were solely treated by radiation therapy remain clinically disease free at 30 and 42 months respectively. Of the remaining three patients, where radiotherapy was delivered post operatively, one achieved complete remission and is well and alive at 60 months, whereas the other two patients were lost to follow up at 22 and 26 months respectively after showing an early clinical improvement. We advocate the efficiency of radiation therapy as primary treatment in the management of primary glomus tumour.

A glomus tumour involving the base of the skull.

The glomus tumours can invade considerable areas of the base of the skull with no presenting symptoms or if any they can hardly be guiding. The diagnosis in most of the cases is made 5 to 8 years after the onset of the disease. Operative treatment may be efficient, but depends on the operability of the case. In our case of an inoperable glomus tumour involving the middle and posterior cranial fossae, tinnitus appeared as an initial symptom. A week later peripheral facial palsy developed. On the 15th day the patient's deglutition was impaired. At the end of the fifth month bleeding from the ear appeared. Otoscopy of the outer ear showed an irregular, granular formation in the anterior quadrants. The biopsy proved alveolar paraganglioma. The size of the tumour was evaluated with computerized tomography with and without contrast medium and digital subtraction angiography. We report the case because of the long latent asymptomatic course of the disease with penetration in the skull base. The first symptoms, manifested at the inoperable stage, was suggestive of involvement of the middle and inner ear.

[The treatment of glomus tympanicum tumors in the years 1962-1992 in the Krakow Otolaryngological Clinic]. / Guzy klebków bebenkowych w materiale Kliniki Otolaryngologii AM w Krakowie w latach 1962-1992.

Between the years 1962-1992 25 woman were treated surgically with glomus tympanicum tumors. All those patients were irradiated postoperatively and 80% of 5 years survival were achieved. According to our experience and data of the literature the method of choice in treatment those tumors should consist of surgery and radiation therapy postoperatively.

Treatment of head and neck paragangliomas with external beam radiation therapy.

PURPOSE: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. METHODS AND MATERIALS: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm(3) (range, 0.9-243 cm(3)) and 116 cm(3) (range, 24-731 cm(3)), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). RESULTS: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. CONCLUSION: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.

Glomus jugulare: high index of clinical suspicion is important for optimal management.

Glomus tumour is a type of extra-adrenal chemodectoma or paraganglioma, originating within the wall of jugular bulb. We report a case of a 60-year-old woman who presented with hearing loss and pulsatile tinnitus. High index of suspicion and appropriate use of imaging led to early diagnosis. However, the patient did not give consent for surgery and was managed satisfactorily with radiotherapy.

[Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan?]. / Endokrine Chirurgie zervikaler Paragangliome: Operation, Strahlentherapie oder "wait and scan"?

BACKGROUND: Head and neck paraganglioma (HNP) represent rare endocrine tumors. Therapy is decided on genetic findings, tumor characteristics (e.g. tumor size, localization and dignity), age of patient and symptoms. In terms of local control radiation therapy is as equally effective as surgery but surgical morbidity rates secondary to cranial nerve injuries remain high. PATIENTS: Based on 6 patients with 11 solitary (4 patients) and multiple (2 patients) HNP (8 carotid body tumors, 1 vagal, 1 jugular and 1 jugulotympanic paraganglioma) the specific characteristics of the need for surgery as well as correct choice of treatment in cases of sporadic succinate dehydrogenase (SDH) negative and hereditary SDH positive HNP will be exemplarily demonstrated. RESULTS: A total of 6 carotid body tumors (four sporadic, two hereditary) were resected in 4 patients, five as primary surgery and one as a revision procedure. In one case a preoperative embolization was performed 24 h before surgery. Malignancy could not be proven in any patient. The 30-day mortality was zero. In the patient with bilateral hereditary carotid body tumors, unilateral local recurrent disease occurred. After resection of the recurrent tumor permanent unilateral paralysis of the laryngeal nerve, glossopharyngeal nerve and hypoglossal nerve occurred. All patients were followed-up postoperatively for a mean of 64 months (range 23-78 months) with a local tumor control rate of 100%. The overall survival rate after 5 years was 100%. CONCLUSIONS: Given a very strict indication with awareness of surgical risks selective surgery has a key position with low postoperative morbidity in the treatment of HNPs. We prefer surgery for small unilateral paraganglioma, malignant or functioning tumors.