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Purpose of Review: Review the recent literature regarding the management of orbital invasion in sinonasal malignant tumors., Recent Findings: There is a recent trend in preserving the orbit in cases of minimal invasion of periosteum and limited periorbit involvement, as well as in presence of good response to neoadjuvant chemotherapy, mainly in squamous cell carcinoma and neuroendocrine histologies., Summary: The decision about orbital exenteration in cases of sinonasal malignancies is facilitated if the patient already has clear clinical signs of intraconal invasion such as visual loss, restriction of ocular mobility or infiltration of the eyeglobe. However, in borderline situations, confirmation of orbital involvement should be performed intraoperatively. In selected cases with minimal orbital invasion without functional compromise, orbit sparing surgery can be done with acceptable oncological outcomes.

Background: Indications for preservation of orbital contents in case of malignant sinonasal tumors invading the orbit remain controversial., Methods: A retrospective consecutive review was conducted of patients in a tertiary care center over a 15-year period., Results: Ninety-three patients were diagnosed with a malignant tumor invading the orbit. Eighty-three were treated with curative intent of which 58 underwent surgery. Mean follow-up was 45 months. Orbital preservation was feasible in 66% of cases, whereas orbital clearance was performed in cases of locally advanced disease (invasion of extraocular muscles, ocular globe, or orbital apex). Local control rate was 70% for patients treated with orbital clearance and 74% for those with orbital preservation, with no statistical difference. Five-year survival and 5-year relapse-free survival were similar in both groups., Conclusion: Using our strategy, the eye can be spared in more patients than what is currently proposed, without oncologic or survival disadvantages. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1708-1716, 2016., (© 2016 Wiley Periodicals, Inc.)

Objective: To investigate the effect of endoscopic transnasal surgery for malignant sinus tumors involving the orbit, and to analyse the factors affecting the prognosis., Methods: Thirty-four cases of malignant sinus tumors involving the orbit, who underwent surgical treatment in Chinese people's liberation army general hospital between January 2009 and April 2015, were reviewed retrospectively. The 5-year survival rate, the recurrence rate and the complications was analysed, the factors affecting prognosis were discussed., Results: Among 34 patients, 2 patients were lost to follow-up. Follow-up time of 32 patients ranged 7 to 75 months. The 5-year overall survival(OS)in this group was 53%. Twelve cases recurred, the recurrence rate was 35%. Eye function improved in 26 cases, the improvement rate was 76.5%. Four cases developed diplopia and peri-orbital swelling after surgery, and simple peri-orbit swelling in 25 cases. The 5-year survive rate of stage 1, stage 2 and stage 3 were 80%, 36% and 25%, respectively, with significant difference(χ(2)=6.643, P=0.036)., Conclusions: Endoscopic transnasal surgery for malignant sinus tumors is effective, minimally invasive and accurate. The prognosis is closely related to the clinical stages and the pathological type.

Background: The purpose of this study was to analyze the oncologic and functional outcomes of the preserved eye in malignant sinonasal tumors with orbital involvement., Methods: In a retrospective study of 19 consecutive patients who underwent craniofacial resection or maxillectomy with preservation of orbital contents and adjuvant radiotherapy, the oncological outcome in terms of local recurrence and survival was analyzed. The functional outcome in the preserved eye was analyzed for the cases that did not recur in the orbit., Results: Nineteen patients were analyzed for their oncologic and functional outcomes. Fifteen patients underwent immediate reconstruction of the orbital support. Eight patients had local recurrences; 11 patients were disease free at the end of the follow-up. Squamous cell carcinoma has a high propensity for local recurrences (ie, 5 of 8 local recurrences were squamous cell carcinoma). Sixteen patients, in whom a satisfactory orbital tumor clearance was obtained, remained recurrence free in the orbit. All the preserved eyes retained adequate function after adjuvant radiotherapy., Conclusions: Adequate local control with preserved visual function can be obtained with surgery and adjuvant radiation in appropriately selected malignant sinonasal tumors with orbital involvement. The preserved eye, if reconstructed appropriately, maintains good overall function with acceptable morbidity., (Copyright © 2013 Wiley Periodicals, Inc.)

Purpose: To identify the type and frequency of ocular and orbital complications observed following treatment for primary rhabdomyosarcoma of the paranasal sinuses or the orbit., Methods: An institutional review board-approved, Health Insurance Portability and Accountability Act-compliant retrospective chart review was conducted to identify all patients treated at one institution from 1966 to 2005 with biopsy-proven primary paranasal sinus or orbital rhabdomyosarcoma. Pretreatment, treatment, and follow-up data were collected., Results: Forty-four patients (25 male) of a median age of 7.8 years (range 1.0-18.0 years) with primary paranasal sinus (17) or orbital (27) rhabdomyosarcoma were treated and followed for a median period of 5.3 years (range 0.6-32.0 years). The three most frequently observed ophthalmic complications were persistent eyelid erythema or cellulitis (12), epithelial keratitis (7), and conjunctival injection (6) in the paranasal sinus and epithelial keratitis (18), conjunctival injection (11), and cataract (10) in the orbit. Overall survival after treatment trended higher for the orbital rhabdomyosarcoma group (23 of 27) than the paranasal sinus group (9 of 17)., Conclusions: The most frequently observed treatment-induced ophthalmic complications in the paranasal sinus group were manageable with minimal patient morbidity, as in the patients with orbital disease. Vision-threatening complications were infrequently encountered. Despite maximal therapy, the mortality rate was higher in the paranasal sinus rhabdomyosarcoma group than in the orbital rhabdomyosarcoma group.

Background/aims: To describe the clinical features, imaging, histologic spectrum, treatment and prognosis of patients with malignant orbital lymphoproliferative disorders extending from the paranasal sinuses., Methods: Patients were culled from the University of British Columbia Orbit Clinic (1977-2004) and their clinical charts, imaging and pathology specimens reviewed., Results: The study included 6 patients with non-Hodgkin's lymphoma (NHL) and 5 multiple myeloma (MM). Orbital symptoms at presentation were periorbital swelling, diplopia, proptosis and pain. Major clinical findings were globe displacement, abnormal ocular movements, periorbital fullness, fifth nerve hypoesthesia and inflammatory signs. Imaging showed a soft tissue mass in one or more sinuses invading the orbit. Three NHL patients were still alive at last follow-up with one patient lost to follow-up. Two NHL patients and all MM died., Conclusion: Presenting symptoms of malignant orbital lymphoproliferative disorders extending from the paranasal sinuses include mass effect occasionally with infiltrative and inflammatory characteristics accompanied by symptoms of sinus disease. On imaging, a soft tissue mass in the sinuses and orbit with bone destruction is commonly seen. Biopsy is essential for conclusive diagnosis. Treatment should be initiated promptly as local symptoms are frequently relieved and survival is possible, especially in cases of NHL.

Purpose: The aim of this study is to illustrate our personal experience concerning the diagnostic and therapeutic management of primary and secondary orbital melanomas., Patients and Methods: Nine patients (five men and four women) with a histological diagnosis of orbital malignant melanoma were surgically treated in our department during the last 10 years (1995 - 2005)., Results: All the patients had a unilateral orbital malignant melanoma. A primary tumour was diagnosed in one case. There was metastatic orbital localisation of a cutaneous malignant melanoma in two cases. In six cases, a secondary melanoma originated from the uveal tract (three cases), conjunctiva (two cases), or paranasal sinuses (one case). All the patients underwent surgical treatment of the tumour (exenteration in 5 cases; subtotal exenteration in one case; subtotal excision in two cases; and craniofacial resection in one case) combined with immunotherapy in one case. The median age at surgery was 66 years, with a mean postoperative follow-up of 17 +/- 14 months. Four patients died of widespread dissemination of the melanoma after a mean time of 13 +/- 7 months. Two more patients died of causes other than melanoma. Three patients were still living at 9, 33 and 45 months after surgery. None of the patients presented with a local relapse of the melanoma during follow-up., Conclusion: Although different approaches have been proposed, the prognosis of orbital melanoma remains poor. In our experience too, the patients with longest survival were those whose tumours were exenterated.

Purpose: To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens., Design: Retrospective observational case series., Participants: Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26-65)., Methods: We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein-Barr virus-encoded mRNA in each patient, were reviewed., Main Outcome Measurements: Time of survival from presentation to last known follow-up and tumor-related death., Results: Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein-Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up)., Conclusions: Natural killer/T-cell orbital lymphoma is a rare Epstein-Barr virus-associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.

Purpose: In order to further assess the survival value of orbital exenteration in malignant orbital and periorbital tumors., Methods: The charts of 44 patients exenterated for a neoplasm of the orbit or periorbit were reviewed in a retrospective study., Results: The overall 4-year survival was 45%. 26 patients had free margins on histological examination and 24 patients had developed local recurrence or metastasis. Local recurrence or metastasis were significantly more common in the group with transected margins than in the group with free-margins (p= 0.01). Survival between the group of patients with local recurrence or metastasis and the group without local recurrence or metastasis showed statistically significant difference (p=0.0025). In contrast, survival between the group of patients with free margins and the group with transected margins did not show statistically significant difference (p=0.13)., Conclusion: Surgical free margins section is a key element in successful cancer surgery but seems not the only prognosis variable.

Objective/hypothesis: The treatment of squamous cell carcinoma (SCC) of the sinonasal tract has evolved from routine exenteration of the orbital contents to sparing of the orbit when the SCC does not transgress the periorbita. Nonetheless, the influence of this change in treatment over the rate of local recurrence or survival has not been clearly elucidated. The objective was to ascertain whether orbital sparing surgery for the treatment of SCC of the paranasal sinuses influences the rate of local recurrence or survival., Study Design: This is a retrospective study of patients presenting with SCC arising in the sinonasal tract, treated primarily at the University of Pittsburgh Medical Center from 1977 to 1990, including meta-analysis of the English literature regarding SCC of the sinonasal tract., Methods: Review of medical records regarding demographics, histology, extension of tumor, pathologic results, type of surgery, adjunctive therapy, and outcome. Articles for meta-analysis were identified by Medline search and cross-referencing., Results: Fifty-eight patients with orbital invasion, including bone and/or soft tissue invasion, were included in the study. Patients presenting with invasion of the bony orbit without soft tissue invasion were treated with maxillectomy and/or ethmoidectomy, sparing the orbital contents. Patients presenting with invasion of the orbital bones and soft tissues were treated with ethmoidectomy or maxillectomy, including orbital exenteration. At 3 years' follow-up, 52% of the patients whose orbit was exenterated were alive and without evidence of disease, compared with 59% of the patients whose orbit was spared. This difference was not statistically significant. Similarly, the rate of local recurrence was not statistically significant (P > .05). A meta-analysis of the literature revealed similar results., Conclusions: Our data suggest that sparing of the soft tissues of the orbit when the periorbita has not been transgressed by SCC does not downgrade the rate of cure or local control.

Fifteen patients at the University of Mainz were diagnosed as having T3 or T4 carcinomas of the paranasal sinuses that invaded the orbit. Treatment involved surgical resections of tumor with adjacent periorbita but without enucleation in an attempt to preserve vision and/or cosmesis. Survival rates and especially the rate, time and locations of recurrences were investigated. Analysis of the data revealed that preservation of the eye in these cases with advanced sinus carcinomas with orbital tumor involvement did not seem to worsen prognosis. The individual gain in terms of quality of life through the preservation of vision and cosmesis seems to justify the surgical approach used.

Transfacial approaches, traditionally used for malignant tumors of the paranasal sinuses, provide limited exposure when several sinuses are involved and are unsuitable for tumors that erode through the floor of the anterior cranial fossa. A transcranial approach may aid in the removal of such lesions. To better understand the risks and benefits of this surgical approach, we reviewed all patients (n = 76) who underwent a transcranial approach as part of the excision of paranasal sinus lesions between 1984 and 1993 at our institution. The spectrum of disease included adenocarcinoma (13 patients), squamous cell carcinoma and olfactory neuroblastoma (11 patients each), adenoid cystic carcinoma and poorly differentiated forms of carcinoma (6 patients each), melanoma (5 patients), and miscellaneous others (24 patients). Most patients had ethmoid sinus involvement; tumors were also commonly found in the cribriform plate, sphenoid sinus, and nasal fossa. In each patient, a bifrontal craniotomy was performed with extradural dissection to the floor of the anterior fossa and osteotomies for resection of involved elements. In 47 patients (62%), disease in the orbit, the anterior nasal cavity, or the soft tissues of the face required transfacial as well as transcranial resections. Bony defect in the anterior fossa floor was repaired with a pedicled pericranial flap. Patients with major complications included six patients with epipericranial and/or epidural hematomas requiring evacuation, three with transient cerebrospinal fluid leaks, two who developed bifrontal cerebral infarcts, and one who died soon after surgery. No meningitis was seen. To date, 26 patients (34%) have died; of those living (mean follow-up, 34 mo), 42 (84%) remain in full remission. The transcranial approach can achieve removal of erosive, invasive tumors from this area with predictable morbidity and may be considered whenever sinus tumors breach the anterior cranial base or extend beyond the reach of conventional transfacial approaches.

Between 1987 and 1994 we performed 103 anterior cranio-facial resections in patients affected by tumors involving the ethmoid, the nasal cavities and, sometimes, the orbit, the maxillary and sphenoid sinuses. The cibriform plate was always involved. The tumor invaded the frontal dura in 20 patients without intradural neoplastic vegetations. These were present in 6 cases. Ninety-one of these patients had a malignant tumor; from the histologic point of view we had 50 adenocarcinomas, 16 epidermoid carcinomas, 8 estesioneuroblastomas, 6 adenoid cystic carcinomas, 5 melanomas and 6 infrequent types. The surgical technique became simplier in the second half of our patients. Now we perform a coronal skin incision and prepare a pericranial flap without the galea and use it to reconstruct the cranial base defect without bone or alloplastic material. The frontal craniotomy is rectangular, low and made by an oscillating saw without trephine holes. The posterior section of the skull base for a typical ethmoid tumor is always on the sphenoid roof and the lateral ones on the medial third of the orbital roof, al least in the more interested side. The anterior section is on the frontal sinus floor. The osteotomies may be enlarged according to tumor extension. Our facial incision is paranasal without splitting of the upper lip, but sometimes we used wider skin incisions and osteotomies for tumors involving the maxillary sinus and palate. We had many important complications in the first half of out patients with 7 postoperative deaths but none in the second half. Fifty-five percent of the adenocarcinomas, 7% of the epidermoid carcinomas, 75% of the estesioneuroblastomas, 100% of the adenoid cystic carcinomas and 0% of the melanomas are alive and well. Forty-six patients were previously treated elsewhere and 45 were untreated. The cure rate of these two groups of patients is very different: 38.1% of the first versus 61.9% of the second ones are alive and free of disease. Our experience proves that every transfacial or transnasal resection of an ethmoidal tumor involving the cribriform plate must be avoided.

Chondrosarcomas of the skull base are indolent, locally invasive tumors with a marked tendency to recur. Surgery is the mainstay of treatment because these tumors are generally resistant to other forms of treatment. A surgical approach with wide access to the skull base and one that is easily repeatable is required, because recurrence is common. We have used the LeFort I maxillotomy or mobilization of the zygoma at the time of craniotomy to obtain wide access to the skull base in eight cases of chondrosarcoma. Three patients have undergone subsequent procedures by us for recurrent disease. One patient died 30 days after the operation, and one has required an open repair of a cerebrospinal fluid leak. Good palliation of symptoms has been achieved in all survivors. These approaches fulfill the criteria for the surgical management of these difficult tumors by allowing excellent exposure, safe repetition if required, satisfactory palliation, and acceptable morbidity.

Teamwork between neurosurgeons and head and neck surgeons has increased the possibility of managing tumours involving the skull base. The authors distinguish the anterior half of the skull base into a median and two lateral portions and describe the surgical approaches utilized to expose it. The authors present their clinical experience in 27 patients with extensive lesions of the anterior half of the skull base and their results.

A seven year experience with twenty patients undergoing the craniofacial approach for excision of tumours of the paranasal sinus and orbit is presented. Results indicate a low postoperative morbidity and mortality with excellent cosmesis. Good palliation is achieved in the presence of extensive disease, while large tumours which would have been considered inaccessible can be resected. Its advantage recommend it as the treatment of choice for tumours of the orbit and paranasal sinuses.