Endonasal Endoscopic Surgery in the Management of Sinonasal and Anterior Skull Base Malignancies

Sinonasal malignancies represent a rare subset of tumors with a wide variety of histopathologic diagnoses and overall poor prognosis. These tumors tend to have an insidious onset with non-specific symptoms which often leads to delayed diagnosis and advanced local disease at presentation. The principal goal of surgery is to obtain a negative margin resection. Open craniofacial techniques are well established in the management of sinonasal malignancies and remain the treatment of choice for many advanced tumors. Over the past couple of decades, there has been tremendous application of endoscopic techniques to skull base pathologies including sinonasal malignancies. For selected cases, endonasal endoscopic techniques can be performed with curative intent and reduced surgical morbidity and mortality. Here we discuss principles of surgical management of sinonasal malignancies, review the techniques of endonasal endoscopic resection of sinonasal malignancies, and highlight the importance of pathology in the multi-disciplinary management of patients with these complex lesions.     

Cervical paragangliomas-tumor control and long-term functional results after surgery.

Objective: To report long-term functional results of the surgical treatment of cervical paragangliomas. Patients and Methods: A retrospective review of 22 patients with 34 head and neck paragangliomas of which 27 were resected between 1981 and 2004. Of these, 16 were carotid body tumors and 11 were vagal paragangliomas. There were 13 women and 9 men with an average age of 48.6 years (range, 26 to 75 years; median, 49 years) and the mean follow-up period was 82 months (range, 3 to 184 months; median, 61 months). Results: There were 13 solitary tumors of which 5 were carotid body tumors and 8 vagal paragangliomas. Multiple head and neck paragangliomas were seen in 9 patients (41%). The incidence of associated multiple tumors was 64.3% for carotid body tumors and 38.5% for vagal paragangliomas. Complete tumor resection was achieved in all but 1 patient in whom a small intradural residual vagal paraganglioma had to be left. The internal carotid artery was preserved in all carotid body tumor resections. Lower cranial nerve deficits were sustained in 1 carotid body tumor resection only, but in all cases with multiple tumors. All patients with vagal paragangliomas had or developed a vagal nerve paralysis. In 4 cases minor complications developed postoperatively. No recurrent tumors were seen during the follow-up period. Conclusions: Even in large head and neck paragangliomas surgical treatment provides excellent tumor control with low postoperative morbidity. A wait-and-scan policy may be more appropriate for those patients with multiple tumors, advanced age, or high operative risk and for those whose tumors have recurred following radiotherapy.     

Octreotide scintigraphy in the detection of recurrent paragangliomas.

OBJECTIVE: Conventional follow-up of surgically and radiotherapy-treated paragangliomas is usually carried out with physical examinations and either magnetic resonance imaging and/or computed tomography. Up to now, octreotide has been reserved for primary diagnosis, for which it has been shown to be a reliable imaging modality. However, the postsurgical and postradiation changes seen on conventional imaging make the diagnosis of small recurrent lesions difficult. We sought to evaluate the role of octreotide scintigraphy in the detection of recurrent paragangliomas. STUDY DESIGN: We based this case series on 3 patients who were found to have recurrent paragangliomas after primary surgical resection. All patients underwent octreotide scintigraphy during routine follow-up. RESULTS: Three patients were found to have recurrent paragangliomas using octreotide scintigraphy. In one of the patients, conventional imaging (magnetic resonance imaging, computed tomography) failed to detect the recurrence. CONCLUSION: The octreotide study was able to detect the small recurrences because octreotide binds directly to receptors on the tumor and is not affected by postsurgical or postradiotherapy changes. The intrinsic resolution of most commercially available gamma cameras is about 1 cm, making it quite sensitive to detect small tumors. Although this patient did not have synchronous lesions, octreotide scintigraphy can also aide in the detection of such lesions.     

Craniofacial resection for tumors of the nasal cavity and paranasal sinuses: a 25-year experience

BACKGROUND: Craniofacial resection is the established "gold standard" for surgical treatment of tumors affecting the anterior skull base. METHODS: This study analyzed 308 patients (220 males, 88 females) who had undergone craniofacial resection for sinonasal neoplasia with up to 25-year follow-up. RESULTS: An overall actuarial survival of 65% at 5 years and 47% at 10 years was found for the cohort as a whole. For patients with malignant tumors, the 5-year actuarial survival was 59%, falling to 40% at 10 years. For patients with benign pathology, the actuarial survival was 92% at 5 years falling to 82% at 10 years. Statistical analysis again identified brain involvement, type of malignancy, and orbital involvement as the 3 most significant prognostic factors. CONCLUSION: Analysis of one of the largest single institution cohorts over a 25-year period provides a baseline against which other approaches such as an entirely endoscopic skull base resection must be judged.     

Vagal paraganglioma.

OBJECTIVES: To report the outcomes of surgical treatment of vagal paragangliomas and to define a management protocol. DESIGN: A retrospective case series. PATIENTS AND METHODS: Sixteen consecutive patients with vagal paragangliomas managed by surgical resection using a cervicoparotid approach and pericapsular dissection using microsurgical techniques between 1990 and 2003. RESULTS: All patients either had or developed a vagal palsy. Additional cranial nerve deficits were sustained in 8 patients. No patients died as a result of surgery or from their disease. CONCLUSIONS: The technique used and described in this article allowed adequate exposure of the retrostyloid parapharyngeal space for the safe removal of all vagal tumors in this series. Careful consideration must be given to the likely natural progression of these tumors before committing to surgical resection. This is particularly important in patients with multifocal disease.     

Sinonasal Hemangiopericytoma: Case Report and Literature Review

Hemangiopericytoma (HPC) is a rare vascular tumor that can arise in any organ system, but occurs most frequently in skeletal muscle. We present a case of a primary sphenoid sinus HPC unusual for its spontaneous remission after biopsy. There have been approximately 55 cases of sinonasal HPC reported in the literature, representing less than 5% of all lesions. In general, HPC behaves aggressively, demonstrating greater than 50% local recurrence and 10% metastatic disease. Although extended surgical resection is traditionally considered the most effective therapy for all HPC, critical literature review does not support this method of treatment for HPC occurring in the paranasal sinuses and skull base. Long-term follow-up indicates that the majority of sinonasal HPC have a benign clinical course regardless of treatment. As a result, we do not recommend extended resection as the initial therapy for sinonasal HPC. Instead, a period of observation coupled with serial MRI or CT scans should be used to detect tumor progression. These indolent tumors should undergo extended resection only after progression has been confirmed. In addition to the case report, a synopsis of the reviewed literature and a summary of treatment recommendations are also presented.     

Surgical management of a voluminous abdominal paraganglioma and review of the literature

The Authors report a case of a voluminous abdominal paraganglioma and a review of the literature on the surgical management of paragangliomas. CASE REPORT: A 58 years old woman was admitted to our Institution with acute endocrine impairment and simultaneous intestinal necrosis resulting from the mass effect of the tumor on the mesenteric vessels. Clinical and biochemical preoperative work up posed the suspect of paraganglioma. Previous intensive care treatment, the patient, underwent surgical resection of the tumor and left hemicolectomy, with immediate remission of both endocrine and abdominal syndromes. Histological examination confirm the preoperative clinical hypothesis. Paragangliomas are rare tumors of the extra-adrenal chromaffin tissue. A paraganglioma may be discovered in the absence of any symptom. When symptomatic, the clinical findings are related to the hypersecretion of catecholamines or to the compression of several anatomical structures from a growing mass. Combined acute syndromes of hormonal impairment and contemporaneous mass effect, as in our case, are exceptional. The diagnosis of the secreting forms may be obtained on the basis of biochemical dosage of serum and urinary catecholamines and metanephrines. The assessment of malignancy for paragangliomas is not always feasible. Several imaging, cytological and histological parameters have been proposed as predictors of malignancy, but the only element widely accepted is the existence of distant metastasis. Surgery represents the treatment of choice for paragangliomas. Benign forms, catecholamine secreting forms and tumors compressing neighbouring anatomical structures can be treated surgically with success. In general, an accurate follow-up after complete resection must be considered.     

Sinonasal hemangiopericytoma: case report and literature review

Hemangiopericytoma (HPC) is a rare vascular tumor that can arise in any organ system, but occurs most frequently in skeletal muscle. We present a case of a primary sphenoid sinus HPC unusual for its spontaneous remission after biopsy. There have been approximately 55 cases of sinonasal HPC reported in the literature, representing less than 5% of all lesions. In general, HPC behaves aggressively, demonstrating greater than 50% local recurrence and 10% metastatic disease. Although extended surgical resection is traditionally considered the most effective therapy for all HPC, critical literature review does not support this method of treatment for HPC occurring in the paranasal sinuses and skull base. Long-term follow-up indicates that the majority of sinonasal HPC have a benign clinical course regardless of treatment. As a result, we do not recommend extended resection as the initial therapy for sinonasal HPC. Instead, a period of observation coupled with serial MRI or CT scans should be used to detect tumor progression. These indolent tumors should undergo extended resection only after progression has been confirmed. In addition to the case report, a synopsis of the reviewed literature and a summary of treatment recommendations are also presented.     

Tailored endoscopic surgery for the treatment of sinonasal inverted papilloma

BACKGROUND: This retrospective study was designed to evaluate the efficacy of tailored endoscopic surgery. Tailored endoscopic surgery aims at resecting the inverted papilloma completely with a customized surgical approach, especially when an en-bloc excision cannot be comprehensively or routinely achieved because of the immense extent of the tumor. METHODS: Between November 1991 and March 2002, 43 patients with sinonasal inverted papillomas were treated by tailored endoscopic surgery. The average duration of follow-up for this population was 25.3 months (range, 9-150 months). A staging system developed by Krouse was adopted for tumor grading. On the basis of tailored endoscopic surgery, 15 localized lesions and 12 smaller extensive lesions (Krouse stages 1 and 2) were treated by ordinary endoscopic resection, whereas 16 larger extensive lesions (Krouse stages 3 and 4) in which the tumors were immense were subjected to sequential segmental endoscopic surgery (SSES). Seven of these 16 larger extensive lesions combined with endoscopic medial maxillectomy because of extensive encroachment of maxillary sinus antrum. RESULTS: Four patients (9.3%) had residual disease, each requiring one revision surgery. All tumors were successfully resected. No patient required lateral rhinotomy or midfacial degloving procedure. No major complications were encountered in any of the patients. None of the patients had residual disease at the time of this writing. CONCLUSIONS: Tailored endoscopic surgery is a safe and effective treatment that obviates the need for more extensive surgery for the management of inverted papilloma. Proper preoperative evaluations, intraoperative determination of extent and attachment of the tumor, close endoscopic follow-up, and expert application of endoscopic techniques are the keys to the successful use of tailored endoscopic surgery.     

Endoscopic nasal and anterior craniotomy resection for malignant nasoethmoid tumors involving the anterior skull base.

Objectives: The traditional approach to sinonasal tumors involving the skull base has been the anterior craniofacial resection. The first report by Ketcham et al (American Journal of Surgery, 1963;106:698-703) documented their experience with 17 anterior craniofacial resections for malignant tumors of the sinonasal tract. Later experience with this technique at several centers has resulted in the publication of many refinements of technique and further reduction in the morbidity and mortality associated with this procedure. In our hands, endoscopic techniques have allowed us to approach the intranasal aspect of skull base lesions without external incisions and yet still achieve an en bloc resection. The type of lesions suitable for this approach and the associated technical issues are discussed in this article. Methods: Between 1999 and 2004, 18 patients with malignant nasoethmoid tumors underwent endoscopic nasal and anterior craniotomy resections. The average age of the patients 60.2 years, with a male-to-female distribution of 15 to 3. Mean follow-up period was 25.1 months. Results: Two patients died from postoperative complications, three died from recurrent disease and two from unrelated causes. Eleven patients are free of disease with a mean survival of 19.8 months. Conclusions: Although we do not consider this approach a replacement for the traditional anterior craniofacial resection, it is an important adjunct in the skull base surgeon's armamentarium.     

Preoperative embolization in the management of a mediastinal paraganglioma

Parangliomas are rare and highly vascular tumors of neuroendocrine cell origin which are treated by complete surgical resection. Preoperative embolization to reduce perioperative bleeding complications, although described in paragangliomas of the neck and carotid body, has never before been described in the case of a mediastinal paraganglioma. The following is a presentation of such a case of mediastinal paraganglioma, in which embolization was used successfully before surgical resection.     

Thoracoscopic removal of intrathoracic neurogenic tumors: a combined Chinese experience

OBJECTIVE: To review the surgical and clinical results of minimally invasive resection of intrathoracic neurogenic tumors using a video-assisted thoracoscopic technique. SUMMARY BACKGROUND DATA: Thoracoscopy has emerged as a possible means for diagnosing and managing various intrathoracic disorders. Benign intrathoracic tumors often are ideal lesions for resection using a video-assisted technique. The authors report on their combined experience with the thoracoscopic resection of 143 intrathoracic neurogenic tumors. METHODS: Between March 1992 and February 1999, 143 patients with intrathoracic neurogenic tumors were diagnosed and underwent resection using video-assisted thoracoscopic techniques in three teaching centers. Case selection, surgical technique, and clinical results were reviewed. RESULTS: The average age of the patients was 40.8 years; 57.3% were male. Thirty-eight patients (27%) had symptoms attributable to the masses. Seventy-two masses were neurofibromas, 33 were neurilemmomas, 7 were paragangliomas, and 31 were ganglioneuromas. All but seven tumors were located in the posterior mediastinum. The masses were on average 3.5 cm in greatest diameter. The mean surgical time was 40 minutes, and the average hospital stay was 4.1 days. There were no major postoperative complications or recurrences to date. Nine patients reported paresthesias over the chest wall during a mean follow-up of 29 months. CONCLUSIONS: Resection of intrathoracic neurogenic tumors using a thoracoscopic technique based on standard surgical indications would seem to be appropriate. Most of these masses are benign and readily removed. For dumbbell tumors, a combined thoracic and neurosurgical approach is mandatory.     

Duodenal gangliocytic paraganglioma,successfully treated by local surgical excision-a case report

IntroductionDuodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy.Presentation of caseA 67 year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3.1 cm intraluminal mass situated grossly at the junction of the third with the fourth portion of the duodenum. The tumor was found to be situated near the ampulla of Vater and was excised through a longitudinal duodenotomy followed by myotomy of the sphincter of Oddi.DiscussionComplete resection of duodenal gangliocytic paragangliomas by surgical or endoscopic means is the only potential cure. Endoscopic removal is the first option and is both safe and adequate. Ηowever, localized excision may be utilized instead in those cases in which endoscopic removal is not possible or cannot achieve negative margins. Recurrent disease after complete resection is unlikely.ConclusionCases of duodenal gangliocytic paragangliomas are best managed with endoscopic resection. However, local surgical excision remains as a second-choice procedure. Adjuvant chemotherapy and radiotherapy are unnecessary after complete excision.     

Primary intraosseous paraganglioma of the sacrum: a case report

BACKGROUND CONTEXT: Paragangliomas are neuroendocrine tumors that most frequently arise in the adrenal medulla, carotid body, and glomus jugulare. However, they have been reported in many other sites throughout the body. Within the central nervous system, the overwhelming majority of paragangliomas arise intradurally in the area of the cauda equina. We report a case of a woman who presented with back pain and radiculopathy and was found to have an intraosseous sacral mass on magnetic resonance imaging (MRI). Operative biopsy revealed that this lesion was a paraganglioma. Chest, abdomen, and pelvis computed tomography scans revealed no other tumors. This lesion was treated with limited resection, decompression, stabilization, and radiation therapy. PURPOSE: To report a rare case of an intrasacral paraganglioma that presented with back pain and radiculopathy and was treated with intralesional decompression, stabilization, and radiation. STUDY DESIGN: Observational case report. METHODS: A 69-year-old woman presented with back and leg pain. An MRI scan revealed a grade II spondylolisthesis at L5/S1 and a left-sided intraosseus mass at S1 with significant obstruction of the left S1 nerve root foramen. The patient underwent initially an open biopsy of this lesion that revealed a paraganglioma. Subsequently, she underwent transarterial embolization of this lesion, partial resection with decompression of the nerve root, and stabilization and arthrodesis from L4 to the ilium. RESULTS: Intraoperatively, a highly vascular lesion was encountered. The patient's back and leg pain improved significantly after surgery. She was referred for intensity-modulated radiation therapy. One year after surgery, she was neurologically intact without evidence of growth of the lesion. CONCLUSIONS: Only two cases of primary intraosseous sacral paragangliomas were reported in the past. We believe that paragangliomas should be included in the differential diagnosis of intraosseous sacral tumors. Optimal treatment of the lesions can be difficult because of their hypervascular nature and the high morbidity associated with complete surgical resection. Treatment should combine preoperative embolisation, surgical resection if possible, and radiation therapy.     

Endoscopic and endoscope-assisted resections of inverted sinonasal papillomas.

OBJECTIVE: To evaluate the success of endoscopic and endoscope-assisted resection of inverted sinonasal papillomas and to assess the indications for adjunctive external procedures. STUDY DESIGN: Retrospective chart review. RESULTS: Fifty patients were treated surgically during the 10-year study period and had greater than 1 year of follow-up care. These patients had a recurrence rate of 14% (7/50) with a mean follow-up of 31.1 months. Of these 7 patients with recurrences, 3 were treated primarily at this institution, and 4 had been treated prior to referral. Recurrence was 11% (3/27) for primary resections and 17% (4/23) for secondary resections. Average time to recurrence was 11.7 months. Of the patients, 38% (19/50) had adjunctive external procedures either during the initial resection or for the management of recurrent disease. CONCLUSIONS: Endoscopic and endoscope-assisted resections of inverted papilloma are effective techniques for managing inverted papilloma. The intraoperative findings, most importantly the site(s) of tumor attachment, dictate whether an endoscopic procedure is sufficient to complete resect the inverted papilloma or whether an adjunctive external procedure is required. Appropriate patient selection and an aggressive surgical approach are necessary for the management of these neoplasms.     

Large gangliocytic paraganglioma of the duodenum: A rare entity

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.     

Malignant carotid paraganglioma. A case report

The most common sites of paragangliomas occurring in the head and neck region are the carotid body and vagal glomus. The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination. Few cases of malignant carotid body paraganglioma have been reported in the literature. We report a case managed in the ENT Department of Grenoble university hospital in 1993. A 53-year-old woman presented a bilateral functional (dopamine secretion) carotid tumor, stage II on the right side and stage I on the left side according to the Shamblin classification. Two successive excisions with complete resection were performed without complication. Two years after the second surgical excision, bone and liver metastases were diagnosed and visualized with a Ostreoscan scintigraphy. Clinical course was rapidly unfavorable and the patient died a few weeks later. Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy. Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential. Histology in needed to establish the diagnosis of metastasis. A histology specimen may not be possible for a functional paraganglioma. In this case scintigraphy can provide the diagnosis. Malignant forms are more frequent in case of functional paraganglioma, more particularly in case of dopamine secretion.     

Aggressive endoscopic resection of inverted papilloma: an update.

OBJECTIVE: Endoscopic resection has been proposed for sinonasal inverted papilloma (IP). Our objective was to determine the efficacy of aggressive endoscopic resection of IP. METHODS: Retrospective analysis was performed on patients undergoing endoscopic resection of IP at the University of Virginia between 1990 and 1996. Total ethmoidectomies, wide maxillary antrostomies, frontal recess explorations, sphenoidotomies, and turbinate resection were performed as required. Once all visible papilloma was removed, residual mucosa was removed by using a diamond burr to polish bone at the site of origin. RESULTS: Twenty-one patients were treated with endoscopic resection of IP. Only 1 of 21 patients had an adjunctive external procedure (an osteoplastic flap without obliteration). Average follow-up was 41.9 months after initial aggressive endoscopic resection at the ureterovesical angle. Recurrences occurred in 19% (4/21) of patients. One of the 4 had two recurrences. Recurrences occurred in 16 months or less, except for one noted at 35 months and another at 56 months. CONCLUSIONS: Aggressive endoscopic resection of IP by experienced rhinologists is an acceptable treatment.     

Combined endovascular and surgical treatment of head and neck paragangliomas--a team approach

BACKGROUND: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. METHODS: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. RESULTS: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. CONCLUSIONS: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen.