Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

The intrabulbar or extrabulbar growth pattern and its surgical outcomes of jugular foramen paragangliomas.

OBJECTIVE: This study is to define a subclassification system of jugular foramen paragangliomas (JFPs) and to demonstrate corresponding microsurgical outcomes of JFPs. STUDY DESIGN: Retrospective study. SETTING: A single-center study. METHODS: We conducted a retrospective review of the clinical data of 44 patients with JFPs who underwent surgical management. Extrabulbar(Be) tumor and intrabulbar(Bi) tumor are defined based on the growth patterns, receiver operating characteristic (ROC) curves of the imaging profile were generated and was confirmed based on intraoperative findings. Area Under Curve (AUC), accuracy, sensitivity, and specificity for diagnostic imaging were revealed. We also compared the correlation between the two growth patterns with Fisch's classification, blood loss, lower cranial nerves (LCNs) deficit. RESULTS: There are 27 (69%) cases of Bi tumor and 17 (39%) cases of Be tumor. Significant radiomics features between the two growth patterns were demonstrated, ROC curves achieved excellent AUCs for MRI sequences (T1W1 MRI, MR contrast-enhanced sequence, MR complex sequences and MR complex + DSA by 0.833, 0.833, 0.875, 0.944) and had statistically significant in diagnosis of two growth patterns (P<0.05). There was no statistical correlation between growth patterns of JFPs and intra-operative blood loss. Preoperative LCNs deficits and Fisch's classification of tumors were correlated with the growth patterns of JFPs (P < 0.05). CONCLUSION: We proposetd two growth patterns of JFPs in term of the inferior petrous sinus involvement. Identification of Bi or Be growth patterns preoperatively is helpful to design optimal surgical strategies and minimize postoperative complications.

Blue laser for the exclusive endoscopic transcanal approach to middle ear paraganglioma.

BACKGROUND: The management of glomus tympanicum tumours can be challenging. Blue laser coagulation may improve bleeding control thus facilitating an endoscopic transcanal excision. The objective of this presentation is to illustrate the authors' experience using this novel tool. METHODS: Case report of a patient that underwent exclusive endoscopic transcanal blue laser surgery of a class A2 glomus tympanicum tumour in a tertiary referral center. CONCLUSION: The present study provides evidence of the safety and efficacy of endoscopic blue laser surgery, for the minimally invasive treatment of early-stage glomus tympanicum tumours.

Glomus jugulare in a pediatric patient: case report and literature review.

PURPOSE: The jugular and tympanic glomus are rare neoplasms in the general population, being even more uncommon in the pediatric population. There is considerable morbidity associated with both disease and treatment. Treatment is essentially surgical, carried out in recent years in a multidisciplinary manner using preoperative embolization associated with microsurgery and eventually adjuvant radiotherapy. The outcome depends on the location of the lesion and its proximity to noble structures in addition to multidisciplinary monitoring in the postoperative period. METHODS: In this article, a literature review was carried out in the PubMed database, finding reports from 17 patients diagnosed with the disease. Only articles in English were considered. RESULTS: Moreover, we reported a case of a 14-year-old patient diagnosed with jugulotympanic glomus who underwent radical surgical treatment of the lesion. CONCLUSION: This is a rare case of jugulotympanic glomus in a pediatric patient, who underwent surgical treatment associated with multidisciplinary therapy, with a favorable postoperative outcome.

Surgical treatment of selected tumors via the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach without rerouting of the facial nerve.

The feasibility of a novel skull base approach - the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach (NaMIPSI-A) without rerouting of the fallopian canal for selected jugular foramen tumors (JFTs) - has been demonstrated in a neuroanatomical laboratory study. Here, we present our clinical experience with the NaMIPSI-A for selected JFTs, with a particular focus on its efficacy and safety. All patients with JFTs who were treated via the NaMIPSI-A were included in this study. The JFTs were classified according to a modified Fisch classification. The neurological and neuroradiological outcome, the extent of tumor resection, and the approach-related morbidity were examined. Five patients (two women, three men; mean age 57 years, range 48-65) were available. According to the modified Fisch classification, two JFTs were graded as C1, one as De1, and two as De2. Gross total resection (GTR) was achieved in three patients and near-total resection (NTR) in two. Postsurgically, no new neurological deficits and no approach-related morbidity and mortality occurred. One case with a postoperative cerebrospinal fluid leak was managed successfully with lumbar drainage. During the follow-up period (mean 67.6 months, range 12-119 months), tumor recurrence was noted in the NTR group but not in the GTR group. The NaMIPSI-A to the jugular foramen without rerouting of the fallopian canal is highly valuable for selected tumors of the jugular foramen. It is less invasive than other skull base approaches, and it allows safe and complete tumor removal in appropriate patients.

Three-Corridors Procedure to Operate in the Infralabyrinthine Cervico-Jugulo-Carotico-Tympanic Area.

INTRODUCTION: The aim of the study was to present the results of our experience in three-corridors procedures applied for the tumors and inflammatory lesions of the infralabyrinthine cervico-jugulo-carotico-tympanic area. METHODS: The lesions located in the infralabyrinthine cervico-jugulo-carotico-tympanic area were operated in 13 patients using the 3-corridors technique. The anatomical and functional integrity of the external and middle ears and the facial nerve (FN) could be preserved. RESULTS: The diagnoses were glomus jugulare, infralabyrinthine petrous bone cholesteatoma, jugular foramen schwannoma, and giant-cell tumor. The follow-up duration ranged from 2 to 24 months. No tumor recurrence or growth was encountered in the follow-up. The operations were uneventful. Total surgical excision could be achieved in 10 patients. A second-stage retrosigmoid approach was performed for the total removal of the intracranial tumor remnant in two patients. A wait-and-scan policy has been considered in one patient who had partial resection for a glomus jugulare tumor. CONCLUSION: Three-corridors procedure seems to be a useful technique to operate in the infralabyrinthine, cervico-jugulo-carotico-tympanic area as it takes the advantage of hearing preservation, preservation of the anatomic and functional integrity of the external and middle ear structures as well as the FN.

[Low-grade (polymorphous) adenocarcinoma of the middle ear mimicking a jugulotympanic paraganglioma. German version]. / (Polymorphes) Low-Grade-Adenokarzinom des Mittelohrs als Imitator eines jugulotympanalen Paraganglioms.

A 64-year-old female patient presented with otalgia and hearing loss in the right ear. On otoscopy, the right tympanic membrane was highly vascularized and bulged into the anteroinferior quadrant. High-resolution computed tomography revealed an osteolytic lesion with occupation of the hypotympanum extending into the petrous apex and right parapharyngeal space as well as infiltration of the wall of the right internal carotid artery. MRI strengthened the suspicion of a jugulotympanic paraganglioma. The biopsy material obtained through exploratory tympanotomy was assessed as a low-grade polymorphic adenocarcinoma. The tumor was treated with definitive chemoradiotherapy. Posttherapeutic imaging after 4 months did not show any evidence of tumor progression.

[Jugulotympane paraganglioma associated with polysegmentary congenital hemangioma]. / Yugulotimpanal'naya paraganglioma, assotsiirovannaya s polisegmentarnoi vrozhdennoi gemangiomoi.

The describes a clinical observation of a combination of jugulotympanic paraganglioma of the left temporal bone with multifocal congenital hemangioma. The interest of the case is that such a combination of pathologies is extremely rare. A comprehensive study and surgical treatment of a patient with this clinical diagnosis was carried out. In the diagnosis, objective otorhinolaryngological studies, otomicroscopy and MSCT of the temporal bones and MRI of the middle ear with contrast were used. Surgical intervention was performed under multicomponent anesthesia with tracheal intubation, transmastoid access. The postoperative management of the patient after surgical treatment is described.

Low-grade (polymorphous) adenocarcinoma of the middle ear mimicking a jugulotympanic paraganglioma.

A 64-year-old female patient presented with otalgia and hearing loss in the right ear. On otoscopy, the right tympanic membrane was highly vascularized and bulged into the anteroinferior quadrant. High-resolution computed tomography revealed an osteolytic lesion with occupation of the hypotympanum extending into the petrous apex and right parapharyngeal space as well as infiltration of the wall of the right internal carotid artery. MRI strengthened the suspicion of a jugulotympanic paraganglioma. The biopsy material obtained through exploratory tympanotomy was assessed as a low-grade polymorphic adenocarcinoma. The tumor was treated with definitive chemoradiotherapy. Posttherapeutic imaging after 4 months did not show any evidence of tumor progression.

LINAC radiosurgery for glomus jugulare tumors: retrospective - cohort study of 23 patients.

BACKGROUND: Glomus jugulare tumors (GJTs) are uncommon and locally disruptive tumors that usually arise within the jugular foramen of the temporal bone. Surgery was the treatment of choice up until recently. In the last decades, however, radiosurgery has surfaced as a promising alternative treatment by providing excellent tumor control with low risk of cranial nerve injuries. Our aim was to examine the results of radiosurgery specifically, linear accelerator stereotactic radiosurgery (LINAC SRS) for GJT treatment. We hypothesized that radiosurgery will reduce the size of the tumor and improve neurological symptoms. DESIGN AND METHOD: Between January 1, 1994 and December 31, 2013, 30 patients with GJTs were treated in Sheba Medical Center using LINAC SRS treatment. Comprehensive clinical follow-up was available for 23 patients. Sixteen patients were female and seven males with a median age of 64 years, with a range of 18-87 years. In 19 of the patients, LINAC SRS was the primary treatment, whereas in the remaining four cases, surgery or embolization preceded radiosurgery. The median treated dose to tumor margin was 14 Gy (range 12-27 Gy), and the median tumor volume before treatment was 5 ml (range 0.5-15 ml). RESULTS: Following the LINAC SRS treatment, 14 of 23 patients (60%) showed improvement of previous neurological deficits, nine patients (40%) remained unchanged. At the end of a follow-up, tumor reduction was seen in 13 patients and a stable volume in eight (91% tumor control rate). Two cases of tumor progression were noted. Three patients (13%) had post- SRS complications during the follow-up, two of which achieved tumor control, while in one the tumor advanced. CONCLUSIONS: LINAC SRS is a practical treatment option for GJTs, with a high rate of tumor control and satisfactory neurological improvement.

Treatment of patients with glomus jugulare tumours (GJT) and its subjective effect on quality of life (QoL) measures.

Cerebellopontine angle (CPA) tumours account for 6-10% of intracranial tumours. The most common CPA tumours are vestibular schwannomas (VS), also known as acoustic neuromas, benign tumours of the vestibulocochlear nerve. Less common but symptomatic skull base lesions are glomus jugulare tumours (GJT), of which approximately 40% are identified as CPA tumours. Initial symptoms for GJT may include hearing loss and tinnitus and progress to various cranial nerve dysfunctions. Three well-accepted treatment modalities for such tumours include surgical resection, radiotherapy and/or conservative management employing serial MR or CT imaging. Patients' quality of life may be impacted by different treatment methods, so treatment decisions should be client centered.

Complete embolization of jugular paragangliomas by direct puncture. Technical note.

Objective: Intra-arterial embolization of jugular paragangliomas is an established endovascular technique. Intratumoral embolization by direct puncture has been proposed, prior to surgical treatment or radiosurgery to reduce the risk of cranial nerve deficits. Methods: We examined the technical aspects of two patients with jugular paragangliomas embolized with liquid embolic agent by direct puncture of the lesion, as sole treatment. Results: Two patients with jugular paragangliomas presented with lower cranial nerve deficits. The first patient showed an extended lesion (55-mm) and was treated with partial intra-arterial embolization plus direct puncture and injection of Squid 18 and a second staged embolization by direct puncture and filling of the remainder of the lesion. The second patient with a smaller jugular paraganglioma (33-mm) was treated by single embolization by direct puncture of the tumor and injection of Squid 12 and Squid 18 obtaining complete filling of the lesion. No procedural complications were observed. Both patients showed no residual and initial improvement of the neurological deficits. Conclusion: The intratumoral embolization by direct puncture of jugular paragangliomas, under accurate radiological control is a safe procedure, and complete exclusion of the lesion can be obtained in selected cases. A staged particle embolization of the lesion by direct puncture can be proposed for large lesions. Only further studies with larger series and long-term follow-up will be able to define, if this strategy can be curative avoiding additional surgical or radio-surgical treatment.

Preservation of the facial and lower cranial nerves in glomus jugulare tumor surgery: modifying our surgical technique for improved outcomes.

PURPOSE: To describe the neurological results obtained in six patients with large Fisch C glomus jugulare tumors (GJT) in which a less aggressive, nerve-preserving surgical strategy was used to reduce surgical morbidity. METHODS: Prospective study of six patients with Fisch C GJT who underwent surgery in a tertiary care referral center from February 2015 to August 2017 with an average follow-up of 18 months. The intervention is the surgical technique used and the main outcome measures are recurrence and the functional preservation of the facial and lower cranial nerves. RESULTS: Gross total removal was obtained in the six patients with preservation of the medial wall of the jugular bulb protecting the lower cranial nerves. After follow-up, we obtained a House-Brackmann (H-B) grade II in three patients who were managed with an inferior facial nerve transposition. One patient managed with a facial bridge technique preserved a normal facial function and two patients who presented a H-B III before surgery went to H-B V after surgery and recovered to a H-B III after 4 months. Four patients were presented with dysphagia after surgery and required nasogastric tube placement. The average time for removal with return to normal oral feeding was 4.3 weeks. Three patients with preoperative Xth nerve dysfunction showed an adequate compensation of the opposite vocal fold in the postoperative period without dysphonia or aspiration. CONCLUSION: The surgical techniques used in these patients provided good functional preservation without recurrence after an 8-30-month follow-up.

[Vascularized tumor of the retroauricular skin]. / Gefäßreicher Tumor der retroaurikulären Haut.

An 84-year-old female patient with loss of hearing and otorrhoea went to an otolaryngologist. On examination, a polypoid mass in the auditory canal and a retroauricular skin lesion, possibly seborrheic keratosis, were found. Only the skin was submitted for histological examination and showed a cutaneous highly vascular lesion with an associated "zellballen" of clear cells without atypia. The initial diagnosis was that of a cutaneous glomangioma. Immunohistochemical findings favored diagnosis of a paraganglioma. Additional clinical information revealed a large mastoid tumor mass. Therefore, the findings were compatible with an jugulotympanic paraganglioma with infiltration of the overlying skin. This possibility was initially not considered in differential diagnosis, as cutaneous glomangiomas are relatively common (typically located on the hands). Cutaneous paragangliomas, however, are not reported.

Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. DESIGN: A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P < .05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P < .05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P < .05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. CONCLUSIONS: An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group.

Surgery, radiotherapy or a combined modality for jugulotympanic paraganglioma of Fisch class C and D.

OBJECTIVES: To identify the risks associated with surgery, radiotherapy or a combined treatment approach for Fisch class C and D jugulotympanic paraganglioma, in order to develop an individualised approach for each patient depending on Fisch class, age, mutation presence, tumour size growth rate and presenting symptoms. DESIGN: A retrospective multicenter cohort study with all patient records of patients with a head and neck paraganglioma in the Radboudumc, Nijmegen and the St. Elisabeth Hospital, Tilburg, the Netherlands. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: We found highest local control rates after tumour debulking with postoperative radiotherapy in case of residual tumour growth, referred to as the combined treatment group, (100%; n = 19), which was significantly higher than the surgical group (82%; n = 17; P = 0.00), but did not differ from the radiotherapy group (90%; n = 29). There were significantly less complications in the radiotherapy group, when compared to surgery (63 vs 27%; P = 0.002) and the combined group (44 vs 27%; P = 0.016). Furthermore,: using a logistic regression model, we found that pretreatment tumour growth was a negative predictor for post-treatment cranial nerve function recovery (OR = 50.178, P = 0.001), reducing the chance of symptom recovery (67.3% vs 35.7%) post-treatment. CONCLUSIONS: Radiotherapy should be the treatment of choice for the elderly. For younger patients, tumour debulking should be considered, with potential radiotherapy in case of residual tumour growth.

Temporary noncicatricial focal alopecia following Gamma knife radiosurgery: Case series and review of literature.

OBJECTIVE: The aim of this study is to report the pattern, timing, responsible radiation parameters, and dosimetric results on the outcome of alopecia following gamma knife radiosurgery (GKRS) for various intracranial pathologies. A literature review of radiation-induced alopecia and observation of this complication with GKRS are also included. MATERIALS AND METHODS: The authors report 6 cases of GKRS-induced focal temporary alopecia without dermal fibrosis or clinical scarring and with no long-term squeal. RESULTS: In all the cases, the scalp received ≥3 Gy radiation exposure. Post GKRS alopecia is a temporary, noncicatricial, focal, reversible phenomenon observed within 2 weeks of the treatment. This acute complication is dose-dependent and reflects damage to the hair follicle. It is only seen with treated volumes in the superficial location. Hair regrowth occurs within two months with no long-term complications and change in hair quality being noted. CONCLUSION: Compared to whole brain radiotherapy, chances of alopecia are significantly less after GKRS. Despite a sharp dose fallout, a single fraction high-dose radiosurgery with GKRS may expose the skin appendages to more than 3 Gy radiation exposures, which may injure the hair follicles irrespective of the growth phase (anagen, telogen, or catagen) they are in. A careful planning that includes sparing of the dermal appendages and hair roots up to 4-6 mm depth in the skin may prevent this complication. Patients with superficial lesions should be well informed about the reversible nature of this possible complication, with near-normal hair growth being established within 2 months of treatment.

[A glomus tumour of the bulb of the jugular vein: complications during the postoperative period]. / Glomusnaia opukhol' lukovitsy iaremnoi veny: oslozhneniia posleoperatsionnogo perioda.

The article presents a clinical case that reflects the difficulties of postoperative management of patients after the removal of the jugular glomus tumor.