Craniofacial resection for malignant paranasal sinus tumors: Report of an International Collaborative Study

BACKGROUND: Malignant tumors of the superior sinonasal vault are rare, and, because of this and the varied histologic findings, most outcomes data reflect the experience of small patient cohorts. This International Collaborative study examines a large cohort of patients accumulated from multiple institutions experienced in craniofacial surgery, with the aim of reporting benchmark figures for outcomes and identifying patient-related and tumor-related predictors of prognosis after craniofacial resection (CFR). METHODS: Three hundred thirty-four patients from 17 institutions were analyzed for outcome. Patients with esthesioneuroblastoma were excluded and are being reported separately. The median age was 57 years (range, 3-98 years). One hundred eighty-eight patients (56.3%) had had prior single-modality or combined treatment, which included surgery in 120 (36%), radiation in 79 (23.7%), and chemotherapy in 56 (16.8%). The most common histologic findings were adenocarcinoma in 107 (32%) and squamous cell carcinoma in 101 (30.2%). The margins of resection were close or microscopically positive in 95 (30%). Adjuvant radiotherapy was given in 161 (48.2%) and chemotherapy in 16 (4.8%). Statistical analyses for outcomes were performed in relation to patient characteristics, tumor characteristics, including histologic findings and extent of disease, surgical resection margins, prior radiation, and prior chemotherapy to determine predictive factors. RESULTS: Postoperative mortality occurred in 15 patients (4.5%). Postoperative complications occurred in 110 patients (32.9%). The 5-year overall, disease-specific, and recurrence-free survival rates were 48.3%, 53.3%, and 45.8%, respectively. The status of surgical margins, histologic findings of the primary tumor, and intracranial extent were independent predictors of overall, disease-specific, and recurrence-free survival on multivariate analysis. CONCLUSIONS: CFR for malignant paranasal sinus tumors is a safe surgical treatment with an overall mortality of 4.5% and complication rate of 33%. The status of surgical margins, histologic findings of the primary tumor, and intracranial extent are independent predictors of outcome.     

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.     

Metastases to nasal cavity and paranasal sinuses

The sinonasal cavities are rare locations for metastases. Metastases to these locations are usually solitary and produce similar symptoms to those of a primary sinonasal tumor. Nasal obstruction and epistaxis are the most frequent symptoms. The maxillary sinus is most frequently involved. The most common primary tumor sites to spread to this region originate in the kidney, breast, thyroid, and prostate, although any malignancy could potentially lead to a metastasis to the paranasal sinuses. The patient's prognosis is usually poor because of the fact that the sinonasal metastasis is usually associated with widespread disseminated disease. In the majority of patients, palliative therapy is the only possible treatment option. Nevertheless, whenever possible, surgical excision either alone or combined with radiotherapy may be useful for palliation of symptoms and, rarely, to achieve prolonged survival. This review considers the most interesting cases reported in the literature that presents metastases to the sinonasal cavities. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1847–1854, 2016     

CRANIOFACIAL RESECTION OF ANTERIOR SKULL BASE TUMOURS: FACTORS CONTRIBUTING TO SUCCESS

Background: A case series of 20 consecutive patients followed for a minimum of 54 months following craniofacial resection of anterior skull base tumours is presented, with the aim of identifying complications and factors influencing survival. Methods: A retrospective review of a case series of 20 consecutive patients was carried out. Results: Dural invasion was significantly correlated with poorer survival. There was also a tendency for tumour grade and positive resection margins to be associated with poorer outcome. Most local recurrences occurred within 6 months of surgery, with the exception of adenocarcinoma of the ethmoids, in which recurrence occurred up to 36 months postoperatively. A variety of complications were encountered, with a marked decrease in serious complications from midway through the series. Conclusions: The change in pattern of complications may be indicative of a learning curve, or the discontinuation of the use of lumbar drainage.     

Verrucous carcinoma of the paranasal sinuses: case report and clinical update

BACKGROUND: Verrucous carcinoma is a low-grade malignancy that has been reported to occur in all anatomic sites of the head and neck. Fourteen cases of verrucous carcinoma of paranasal sinus origin have been reported to date in the English literature. METHODS: Case report and retrospective review of all cases of verrucous carcinoma of the paranasal sinuses in the English literature. All authors were contacted to provide missing data and long-term follow-up. RESULTS: Five of the eight authors contacted responded, and the most current data from all 15 cases was compiled. Eleven of the 15 patients (73%) were men and ranged in age from 35 to 81 years (median, 68 years). The maxillary sinus is the most common paranasal sinus involved (93%). Presentation often occurred late, with 12 of 15 (80%) initially being seen at stage T3 or higher. Surgical excision was the treatment of choice, and median disease-free survival was 54 months. CONCLUSIONS: Verrucous carcinoma of the paranasal sinuses is a rare but potentially curable disease. Treatment is surgical, and prognosis is good with early intervention.     

Increased safety in robotic paranasal sinus and skull base surgery with redundant navigation and automated registration

We present an advanced version of our robotic setup for paranasal sinus surgery that was developed at the Department of Otorhinolaryngology, Head and Neck Surgery in Erlangen, Germany. The system was interconnected with a redundant navigation system for increasing intraoperative safety while performing telemanipulatory as well as fully automated maneuvers.In contrast to the previous "all in one" version, we built a modular three component setup. The basic feature of the computer navigation system is the "CAPPA ENT" station. The system references by automatically detecting a referencing frame mounted on a non-invasive upper jaw mouthpiece. Software components of both systems, navigation and robotics were combined on one user interface. Accuracy as well as clinical applicability studies were carried out. For better surveillance and increased safety, we decided to evaluate the robots reproducibility errors and overall stereotactic accuracy by means of redundant navigational control on a phantom model for paranasal sinus and skull base surgery. Multiple measurements from 14 CT-markers were taken representing different surgical approaches.A modular setup was designed and was deemed feasible in its size and weight dimensions as well as its maneuvrability for application in a routine operating room environment. The navigational feedback is integrated in real time in the robots user interface. In case of blocked visibility to the Dynamic Referencing Frame the robot powers down and activates the force torque sensor, thus softening all articulating joints. We found only adequate accuracies in pinpointing a specific CT-marker both in telemanipulatory and fully automated maneuvers. No significant offsets were observed evaluating accuracies for different surgical approaches.By using redundant navigation feedback, we were able to add another safety feature, the "loss of control" function, which shuts down any robotic action. However, no increase of the absolute accuracy was observed by adding this feature. We conclude that redundant navigational control does not make the robot more accurate, but it adds a potent safety feature to the system.     

Experience with mucosal melanoma of the nose and paranasal sinuses

Purpose: Sinonasal mucosal melanoma is a rare and aggressive disease and its incidence does not mimic that of its cutaneous counterpart in the Australian population. The present study examines one unit's experience with the disease and proposes a treatment strategy. The significance of macroscopic widespread mucosal melanosis and histological melanoma in situ is considered in the present study to be crucial in overall survival and the main cause of local failure and is specifically addressed. Methods: The present study represents the retrospective experience of the multidisciplinary Head and Neck Clinic at the Prince of Wales Hospital over a 30‐years period (from 1970 to end 1999) in the management of the disease, including both primary and salvage treatment approaches. The study includes 27 patients treated with surgery with or without postoperative radiation therapy. Management of recurrence was also considered. Results: The mean time to local recurrence was 14.7 months and the mean time to distant metastases was 23.2 months. Mean survival time was 52 months and mean time from local recurrence to death was 75 months. Overall, disease free and disease specific survival and survival post‐recurrence were analysed by the Kaplan?Meir method. A cancer specific 5 years survival of 46% was achieved, which compares favourably with recent international series. Conclusion: Sinonasal mucosal melanoma remains an aggressive disease with the possibility of local recurrence years after initial treatment, however, initial radical surgery encompassing the primary lesion and distant in situ or satellite disease and postoperative radiotherapy can offer long‐term control, as can reoperation for local recurrence where appropriate.     

Primary yolk sac (endodermal sinus) tumor of the prostate: case report and review of the literature.

A primary yolk sac (endodermal sinus) tumor of the prostate is described in a 40-year-old male. This is the second documented case of a yolk sac tumor occurring in the prostate gland. Treatment included surgical removal and subsequent combination chemotherapy. Four months following diagnosis, the patient died of complications. At autopsy, no residual tumor was detected. The role of combination chemotherapy as potentially curative therapy in the treatment of extragonadal yolk sac tumors is discussed.     

Prognostic factors in sinonasal tumors involving the anterior skull base

BACKGROUND: Anterior craniofacial resection is a standardized procedure for the treatment of ethmoid and frontal orbital tumors with intracranial invasion. METHODS: A retrospective review of 100 patients with sinonasal tumors involving the anterior skull base who underwent combined craniofacial surgery at the Hospital Central de Asturias. RESULTS: The most frequent pathologic entity was adenocarcinoma (53 cases) and other epithelial tumors (29 cases). Five-year actuarial survival according to the Kaplan-Meier method was 40%. Factors such as involvement of surgical margins, orbital periosteum involvement, frontal sinus invasion, or spread into the dura had no significant effect on survival. Survival, however, was affected by the histologic findings of the tumor (p=.03), brain involvement (p=.04), deep soft tissue involvement of the orbit (p=.003), involvement of the sphenoid sinus (p=.001), previous treatment (p=.05), and postoperative recurrence (p=.0000). Neither the INT staging system nor the UICC system showed statistical prognostic significance. After multivariate analysis and Cox regression analysis, only recurrence after craniofacial resection, involvement of soft tissues of the orbit, and invasion of the sphenoid sinus significantly influenced survival. CONCLUSIONS: Standard staging systems did not show statistical prognostic significance. Only involvement of some critical areas was reliable as predictor of an unfavorable outcome.     

Malignant tumors of the nasal cavity and paranasal sinuses

PURPOSE: To evaluate the role of radiation therapy in patients with nasal cavity and paranasal sinus tumors. MATERIALS AND METHODS: Between October 1964 and July 1998, 78 patients with malignant tumors of the nasal cavity (48 patients), ethmoid sinus (24 patients), sphenoid sinus (5 patients), or frontal sinus (1 patient) were treated with curative intent by radiation therapy alone or in the adjuvant setting. There were 25 squamous cell carcinomas, 14 undifferentiated carcinomas, 31 minor salivary gland tumors (adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma), 8 esthesioneuroblastomas, and 1 transitional cell carcinoma. Forty-seven patients were treated with irradiation alone, 25 with surgery and postoperative irradiation, 2 with preoperative irradiation and surgery, and 4 with chemotherapy in combination with irradiation with or without surgery. RESULTS: The 5-year actuarial local control rate for stage I (limited to the site of origin; 22 patients) was 86%; for stage II (extension to adjacent sites (eg, adjacent sinuses, orbit, pterygomaxillary fossa, nasopharynx; 21 patients) was 65%; and for stage III (destruction of skull base or pterygoid plates, or intracranial extension; 35 patients) was 34%. The 5-year actuarial local control rate for patients receiving postoperative irradiation was 79% and for patients receiving irradiation alone was 49% (p =.05). The 5-, 10-, 15-, and 20-year ultimate local control rates for all 78 patients were 60%, 56%, 48%, and 48%, respectively. The 5-, 10-, 15-, and 20-year cause-specific survival rates for all 78 patients were 56%, 45%, 39%, and 39%, respectively. The 5-, 10-, 15-, and 20-year absolute survival rates for all 78 patients were 50%, 31%, 21%, and 16%, respectively. Of the 67 (86%) patients who were initially seen with node-negative disease, 39 (58%) received no elective neck treatment, and 28 (42%) received elective neck irradiation. Of the 39 patients who received no elective neck treatment, 33 (85%) did not experience recurrence in the neck compared with 25 (89%) of 28 patients who received elective neck irradiation. Most patients who received elective neck irradiation (57%) had stage III disease. Twenty-one (27%) of 78 patients had unilateral blindness develop secondary to radiation retinopathy or optic neuropathy; the complication was anticipated in most of these patients, because the ipsilateral eye was irradiated to a high dose. Four patients (5%) unexpectedly had bilateral blindness develop because of optic neuropathy. All four of these patients received irradiation alone. CONCLUSION: Surgery and postoperative radiation therapy may result in improved local control, absolute survival, and complications when compared with radiation therapy alone. Elective neck irradiation is probably unnecessary for patients with early-stage disease.     

Which classification for ethmoid malignant tumors involving the anterior skull base?

BACKGROUND: The purpose of this study was to compare three systems of classification for malignant ethmoidal tumors in patients undergoing anterior craniofacial resection. METHODS: A radiologic locoregional evaluation of 241 patients with malignant ethmoid tumors was performed before patients underwent an anterior craniofacial resection. Disease in each case was staged according to the American Joint Committee on Cancer-Union Internationale Contre le Cancer (AJCC-UICC) 1997 classification, the AJCC-UICC 2002 classification, and the Istituto Nazionale Tumori (INT) classification. Kaplan-Meier curves and Cox models were used to investigate the prognostic value of each classification system on disease-free survival (DFS) and overall survival (OS). The classifications were compared in terms of prognostic discrimination capability, measured by use of an index of agreement between each classification and DFS or OS time. RESULTS: All three classification systems yielded statistically significant results in the Cox analysis, both for DFS and OS. In the AJCC-UICC 2002 system, minor differences were observed between T1 and T3 tumors. The INT classification showed a progressive worsening of the prognosis with increasing stage. The index of prognostic discrimination favored the INT classification over both the 1997 and 2002 AJCC-UICC classifications. CONCLUSIONS: Both the 1997 and 2002 AJCC-UICC classifications seemed to have limited prognostic value. By contrast, the INT classification satisfied one of the main goals of tumor staging, demonstrating the progressive worsening of prognosis with different tumor classes.     

Primary aspergillosis of the sphenoid sinus with pituitary invasion – a rare differential diagnosis of sellar lesions

Summary Aspergillosis belongs to the group of mycotic diseases of paranasal sinuses. The invasive forms, and particularly the fulminant forms, are potentially fatal. Isolated aspergillosis of the sphenoid sinus or the clivus is a difficult diagnosis, since the often misleading clinical manifestations of this rare disease develop late. These patients become apparent by neurological signs such as cavernous sinus syndrome, pseudotumor of the pituitary or the orbit. Diagnosis is often made intra-operatively or on histological examination. We report a case of invasive aspergillosis uniquely involving the sellar area revealed by clinical features suggesting a pseudotumor of the pituitary. Although such lesions are almost always seen in immune suppressed subjects, in our case, the patient was immune competent and had no past history of sinusitis. The question of whether, and when to perform limited or extensive surgery remains an issue for discussion, owing to the rarity of this disease honed by lack of experience. It depends on several factors: the kind of disease, the immunity, the subtype of invasive fungal sinusitis and the degree of tissue invasion.