Review of Nasal Paranasal and Skull-base Tumors Invading the Orbit

The majority of sinonasal malignancies present with advanced disease, and cure rates are generally poor because early on diagnosis is difficult. Surgical extirpation is the mainstay of treatment. Orbital interest in sinonasal malignancies, especially of the orbital apex, is associated with a significant reduction in survival.^two,10 In cases of sinonasal malignancy with orbital apex extension, tumor resection with orbital exenteration and os resection of the skull base of operations effectually the orbital apex is required to provide sufficient resection margins.

In this study, we depict our surgical strategy and technique for orbital exenteration with orbital noon resection. Nosotros likewise analyze the clinical features of and the results in patients who underwent this technique.

Methods

All patients in this case serial provided informed consent for inclusion of their clinical data in this paper. Between February 2001 and August 2012 at our establishment, 65 patients with sinonasal malignancy underwent craniofacial resection performed by a multispecialty skull base squad consisting of neurosurgeons, head and neck surgeons, and plastic surgeons. Of these 65 patients, 15 had sinonasal malignancy with orbital apex extension. These patients underwent craniofacial tumor resection with orbital exenteration and bony resection of the orbital noon. We reviewed the surgical indications, extent of resection, primary tumor location, outcome, pathological findings, and neoadjuvant and adjuvant therapies for these 15 patients.

Computed tomography and MRI were performed preoperatively in all patients to determine tumor size, tumor location, and human relationship of each tumor to next tissues.

Surgical Strategy

Indications for attempted gross-full resection of these lesions were equally follows: no metastasis to other organs, invasion of the intraorbital tissue around the orbital apex, and absence of invasion into the clangorous sinus or dura mater. A thorough evaluation of preoperative imaging was required to decide if orbital apex resection was required. This cancer invasion phase corresponds to the T4bN0M0 stage (International Union Against Cancer Staging System, 7th edition) without dura mater invasion.

Surgical Technique

A lumbar spinal bleed was inserted for CSF drainage subsequently general anesthesia had been induced.

Pace one: Observing From the Nasal Cavity

Intranasal endoscopic inspection of the tumor was starting time performed to visually decide the extent of nasal crenel invasion and to visualize the resection borders anticipated on review of CT and MRI (Fig. 1).

Step 2: Craniotomy and Exposing the Frontal Fossa

A semi-coronal pare incision, bifrontal craniotomy, and temporal craniotomy on the affected side were made, and the supraorbital bar was removed. Modifications of the pare incision and supraorbital bar removal were washed based on the variation in tumor location and size. The inductive cranial fossa was exposed to the stop of the jugum sphenoidale, exposing the optic sheath bilaterally (Fig. 2A). Opening the dura and subsequent cede of the bilateral olfactory fretfulness were performed to achieve adequate exposure of the frontal cranial fossa to the posterior border of the jugum sphenoidale. The dural defect was repaired with temporal fascia as described previously.^i,nine

Footstep 3: Exposing the Centre Fossa

Dolenc's arroyo was starting time described in 1985;³ this technique is used for exposure of the optic nerve, internal carotid artery, and ophthalmic avenue. The dura of the middle fossa was peeled from the skull base to expose the heart meningeal artery. This artery was coagulated and cut at the foramen spinosum. The superior orbital fissure and optic canal were unroofed with a high-speed drill and micro-rongeur. Epidural dissection of the lateral wall of the cavernous sinus was started past dissection at the meningo-orbital ring to the 3rd branch of the trigeminal nerve. The bleeding was controlled by gently packing with oxidized cellulose (Surgicel, Ethicon) soaked in fibrin glue. The dural dissection was extended to the anterior clinoid process. The anterior clinoid procedure was then removed to expose the optic sheath widely (Figs. 2B and C and 3).

The anterior wall of the foramen rotundum was drilled, and the second branch of the trigeminal nerve was exposed to prepare for ligation and transection of the nerve together with the dura mater. The falciform ligament was cut, and the optic sheath was longitudinally opened to expose the internal carotid avenue and ophthalmic artery (Fig. 4A). The optic nerve was then cut, followed by ligation and cut of the ophthalmic artery (Fig. 4B and C). The oculomotor nerve, trochlear nerve, outset branch of the trigeminal nervus, and abducens nerve were transected together with the dura mater at the transition between the dura mater and the periorbita near the superior orbital fissure while taking intendance not to damage the internal carotid avenue (Fig. 5A). After this transection of cranial fretfulness, the dura mater of the middle fossa floor is peeled further posteriorly to expose the superior and lateral wall of the sphenoid sinus (Fig. 5B and C). The opened dura mater effectually the optic sheath was tightly repaired with the temporal fascia before opening the sphenoid sinus. Illustration of this widely exposed eye fossa is depicted in Fig. six

Step 4: Resecting the Tumor With a Safety Margin

The line of resection at the frontal and middle fossa skull bases included more than 5 mm of margin and was cutting using a high-speed drill (Fig. 7A and B). In cases of sphenoid sinus tumor invasion, the posterior edge included the foramen ovale so that the resection would be posterior plenty to include the sphenoid sinus (Fig. 8A1–A3). In such cases, the root of the pterygoid procedure betwixt the foramina rotundum and ovale was drilled off to create a corridor to the posterior part of the sphenoid sinus. It was sometimes difficult to determine adequate margins from the frontal and eye fossae view. In these cases, endoscopic lighting from the nasal crenel transmitting through the ethmoid and sphenoid sinus wall served as a guide (Fig. 7B and D). If tumor did not invade the sphenoid sinus, the lateral wall of the sphenoid sinus was drilled off at the foramen rotundum to gear up the posterior boundary for excision in the anterior part of the sphenoid sinus (Fig. eight B1–B3). In our serial, nosotros divers ample margins at the skull base equally a sufficient amount of tissue left surrounding tumor to preclude exposure of the tumor during resection.

A palpebral conjunctiva incision was made (Fig. 9A), and the facial skin and subcutaneous tissue were peeled from the maxillary bone in a downward direction. The maxillary bone was cutting using a bone saw without exposing the tumor (Fig. 9B and C). The tumor along with orbital content was and then resected en bloc with a margin (Fig. 9D).

Step 5: Reconstructing the Defect After Tumor Resection and Making the Ocular Prosthesis Bed

Anterolateral thigh free flap or rectus abdominis myocutaneous costless flap was harvested and transplanted to the cavity after resection (Fig. 10B). If feasible, the ocular prosthesis bed was made at the same time (Fig. 10C).

Statistical Methods

Estimated overall survival (OS) rate and recurrencefree survival (RFS) rate were calculated using the Kaplan-Meier method. "Recurrence gratuitous" was defined as no evidence of tumor recurrence on imaging studies such as CT, MRI, or FDG PET.

Results

A summary of characteristics for fifteen patients is listed in Table 1. The patients consisted of 12 men and 3 women, with a mean historic period 47.7 years (range 14–79 years). The longest postoperative follow-up was ix.5 years, the shortest was 0.67 year, and the mean was 3.0 years. Extended orbital exenteration surgery was the outset process for tumor resection in eleven patients. In the 4 remaining patients, the surgery indexed for this report was the second surgery for ane patient and the third, the fourth, and the fifth surgery for 1 patient each. The pathology of the 2 lesions that required fourth and 5th reoperations was rhabdomyosarcoma. Ethmoid sinus was the most common primary tumor location. Tumor originated from the ethmoid sinus in 6 (40%) of fifteen patients, maxillary sinus in v (33%), nasal crenel in 2 (13%), and orbital cavity and maxillary os in 1 patient each (vii%). Histological analysis of tumor specimens revealed squamous cell carcinoma in nine patients (60%), rhabdomyosarcoma in 2 (13%), and small cell carcinoma, mucoepidermoid carcinoma, adenoid cystic carcinoma, and Ewing sarcoma pathologies in 1 patient each (7%). 13 patients received neoadjuvant chemotherapy; ii patients did not, and their pathologies consisted of mucoepidermoid carcinoma and adenoid cystic carcinoma. Patients with squamous cell carcinoma and rhabdomyosarcoma (11 patients total) underwent preoperative radiation therapy, and the remaining patients with different pathologies (4) did not receive preoperative radiations. Simply ii patients (Cases 2 and viii) experienced early recurrence at 5 and 1 months; these patients died at 10 and 5 months later on surgery, respectively. Case 2 was a 79-yearold man with small-scale cell carcinoma, and Example 8 was a lx-yr-one-time man with squamous prison cell carcinoma. The patient in Case 2 had tumor invasion of the dura mater and brain that was observed intraoperatively just was not seen on preoperative CT or MRI studies. In this serial of avant-garde-stage patients, estimated 5-twelvemonth RFS was 86.7% and estimated v-year Os was 86.two% (Fig. 11). In addition, there was no perioperative mortality. All of the patients without recurrence at eight months remained disease free. Univariate analysis of factors, including historic period (p = 0.11), histology (p = 0.20), extent of resection (with or without the foramen ovale; p = 0.xx), and intracranial invasion (p = 0.37) as the outcome predictors, revealed no significance.

Table 1

Summary of clinical characteristics in 15 patients with sinonasal malignancy

Case No.	Age (yrs), Sex	Main Tumor Site	Histology	Prior Treatment	Adjuvant Therapy	Ocular Prosthesis Bed/Ocular Prosthesis	Complication	Recurrence/Outcome	Follow-Up (mos)
1	58, M	Ethmoid sinus	Mucoepidermoid carcinoma	—	CT+RT (50 Gy)	+/−	Infection	−/alive	114
2	79, M	Ethmoid sinus	Modest cell carcinoma	CT	CT	−/−	—	+/dead	10
3	34, F	Ethmoid sinus	Squamous cell carcinoma	SR+CT+RT (35 Gy)	—	+/+	Infection	−/live	80
4	77, M	Maxillary sinus	Squamous prison cell carcinoma	CT+RT (50 Gy)	—	−/−	Infection	−/alive	35
v	sixty, M	Maxillary sinus	Squamous prison cell carcinoma	CT+RT (50 Gy)	—	−/−	—	−/alive	58
6	21, G	Orbital cavity	Rhabdomyosarcoma	SR×4+CT+RT (48 Gy)	CT	+/+	—	−/alive	53
vii	45, M	Ethmoid sinus	Squamous cell carcinoma	CT+RT (40 Gy)	—	+/+	—	−/alive	54
viii	lx, Yard	Nasal crenel	Squamous cell carcinoma	CT+RT (65 Gy)	CT	−/−	—	+/dead	5
ix	fifty, Thousand	Ethmoid sinus	Adenoid cystic carcinoma	SR×ii	RT (l Gy)	+/+	—	−/alive	34
10	14, F	Nasal crenel	Rhabdomyosarcoma	SR×3+CT+RT (50 Gy)	—	+/−	—	−/alive	viii
eleven	xvi, M	Maxillary bone	Ewing sarcoma	CT	CT+RT (56 Gy)	+/−	—	−/alive	22
12	31, F	Maxillary sinus	Squamous cell carcinoma	CT+RT (50 Gy)	—	+/−	Infection	−/alive	26
13	55, M	Maxillary sinus	Squamous prison cell carcinoma	CT+RT (fifty Gy)	—	+/+	—	−/alive	18
14	62, M	Maxillary sinus	Squamous cell carcinoma	CT+RT (40 Gy)	—	−/−	—	−/alive	xx
15	54, M	Ethmoid sinus	Squamous cell carcinoma	CT+RT (lxx Gy)	—	+/−	Infection	−/alive	17

CT = chemotherapy; RT = radiation therapy; SR = surgery; − = no; + = yes; — = not applicable.

Five patients suffered infectious complexity effectually the graft transplanted to the cavity later resection. This infection originated from the ocular prosthesis bed in all 5 of these patients. This infection resolved with irrigation of the abscess in iii patients, and i patient had a small incision made at the infection site prior to irrigation. Ane patient needed to undergo removal of the infected skull bone flap and irrigation of the abscess ane month after surgery. No patient suffered neurological or any other complication attributable to the surgical procedure.

Discussion

Cancers of the nasal and paranasal sinuses are rare and reported to be but 3%–5% of all head-and-cervix cancers.^2,4 The asymptomatic growth of these tumors into the air-filled nasal and ethmoidal sinus spaces makes their early diagnosis difficult. Hence, many patients are admitted to the infirmary with an avant-garde stage of the disease. Craniofacial surgery for this type of sinonasal malignancy is difficult to perform adequately and requires a squad consisting of neurosurgeons, head and neck surgeons, and plastic surgeons.

Five-year Os for patients with sinonasal malignancy who have undergone anterior craniofacial resection has been reported to range betwixt forty% and 58%.^{ane,ii,five–8,ten} 5-twelvemonth RFS has been reported every bit 24.4%–52.8%.^1,2,5,7 The perioperative death rate has been reported equally 3.6%–4.7%.^ii,7–ix These data betoken that anterior craniofacial surgery is still a challenging functioning.

Suarez et al. reported that survival for patients with sinonasal tumors treated using craniofacial resection was 40% at five years and that the clinical outcome for these patients with Phase T2 and T4 affliction was about the aforementioned.¹⁰ These authors likewise showed that 5-year survival was significantly affected past tumor histological findings, with 5-year survival rates of 71% in patients with esthesioneuroblastomas, 65% in those with squamous prison cell carcinoma, 31% in those with adenocarcinoma, 17% in those with undifferentiated carcinoma, and 0% in those with melanoma. Patel et al. showed that the histology of the chief tumor, the extent of intracranial extension, and the status of the surgical margins were meaning independent predictors of RFS.^vii

The poor prognosis associated with malignant tumors of the paranasal sinuses is mainly a consequence of local recurrences in the skull base. Some reports accept shown that orbital involvement significantly affects survival, especially if the orbital noon is involved.^1,vii,10 These studies have suggested that orbital apex involvement positively correlates with college recurrence rates and shorter survival. Therefore, nosotros believe that en bloc resection with margins, specially at the skull base of operations effectually the orbital apex, is necessary to avoid local recurrences and contributed to our high estimated OS and RFS rates. Suarez as well reported that involvement of the lateral wall of the sphenoid sinus was i of the factors affecting survival;¹⁰ therefore, it is also of import to make a sufficient resection margin at the deepest part in the sphenoid sinus to prevent recurrence, as nosotros did in this study.

In our series, the estimated 5-twelvemonth Os for patients with sinonasal malignancy who had undergone extended orbital exenteration was 86.2%, and estimated five-year RFS was 86.7%. These survival rates are better than those in previous reports.^2,six–8,10 Furthermore, our v-year OS rate was better than the 70.3% v-yr OS in the 50 sinonasal malignancy patients (mean age 49.1 years) with no invasion of the orbital noon who also underwent craniofacial resection in the same time period at our establishment. The surgical strategy for patients without orbital noon invasion was as follows: When the tumor did not invade orbital bone, the periorbita and orbital contents were preserved, and the medial side of orbital os was resected with the tumor. When the tumor invaded orbital bone, the orbital contents, excluding the orbital noon, were resected with the tumor. Histological analysis of specimens from these 50 patients without orbital apex resection revealed olfactory neuroblastoma in 15 patients (thirty%), squamous cell carcinoma in thirteen (26%), adenoid cystic carcinoma in half dozen (12%), rhabdomyosarcoma in 4 (8%), adenocarcinoma in ii (4%), osteosarcoma in 2 (4%), and other pathologies in 8 (16%). Patients with olfactory neuroblastoma have better outcomes than patients with other sinonasal malignancies. When we excluded the xv patients with olfactory neuroblastoma from these 50 patients, v-year OS decreased to 58.7%. Yet, this rate is still better than previously reported rates only worse than the rate for our fifteen cases with orbital apex tumor extension who had undergone extended orbital exenteration. Although these results were initially surprising, we believe that our orbital apex resection allowed for more than complete resection of tumor from the sphenoid sinus. Tumor resection from the sphenoid sinus is i of the most technically difficult parts of the excision in virtually cases simply is also one of the most crucial steps in preventing recurrence. Total tumor resection with ample safety margins became feasible using our surgical technique of extended orbital apex resection, providing wide exposure of the posterior office of the sphenoid sinus. In that location was no perioperative mortality in our patient series, and the postoperative infectious complication rate was 33.3%. All of these infections involved the lacrimal gland effectually the ocular prosthesis bed. However, these infections were easily controlled in most cases. Irrigation alone cleared the infection in 4 patients, and only 1 patient required an boosted performance such as removal of the infected bone flap. There were no neurological and systemic complications. This perioperative complexity rate is within an acceptable limit for this type of surgery. There were no factors that independently predicted outcome. However, the number of patients was too small (but 2 of 15 patients died) to depict whatever unequivocal conclusions.

A limitation of this study is the short mean follow-up menstruation of 3.0 years while calculating the estimated 5-year Bone and RFS rates. Even so, v patients are alive and take been recurrence free for more than than 50 months after tumor resection. The OS and RFS were not decreased after a few years postresection, as shown in Fig. 11. The use of historical controls to compare outcome is also a limitation of this written report. We intend to accumulate more cases and monitor them for a longer catamenia.

Conclusions

We are the first to describe the surgical strategy and procedures for extended orbital exenteration with skull base os resection around the orbital noon having a sufficient resection margin for sinonasal malignancy with orbital apex extension. The estimated 5-year Bone and RFS were high, and the perioperative complication rate was acceptably low because information technology was possible to make sufficient resection margins using this procedure. We plan further characterization of the effectiveness of this technique with longer patient follow-ups and the accumulation of more than case experience.

Acknowledgment

We thank Robert Ayer, MD (Department of Neurosurgery, Academy of California Irvine, Orange, California), for assisting with this publication.

References

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