Frontal Sinus Malignancies 19

Contents

Introduction . . . . 165

The University of Florida Staging System . . . . 166

Pathology . . . . 166

Epithelial Malignancies . . . . 167

Minor Salivary Gland Tumors . . . . 168

Sarcoma . . . . 168

Lymphoma . . . . 169

Miscellaneous Tumors . . . . 169

Introduction Malignant neoplasms originating from the paranasal sinuses account for 3% of all malignancies arising from head and neck sites [21]. Within the paranasal sinuses, the maxillary sinus is the primary site of 60%–70% of malignancies, followed by the nasal cav- ity in 20%–30% and the ethmoid sinuses in 10%–15% [26, 35]. The frontal sinus is the primary site of malig- nancy in less than 2% of cases, more often being in- volved by extension of a tumor arising elsewhere [3, 25, 36, 48]. Because of their rarity, institutional expe- riences with malignancies arising from the frontal si- nus remain largely anecdotal, with no large series re- ported to date. Malignancies arising from the frontal sinus are not included in the staging system for paranasal si- nus neoplasms promoted by the American Joint Committee on Cancer (AJCC) [2]. The most com- monly used staging system currently applied to cases of frontal sinus malignancies was developed at the University of Florida for tumors of the nasal cavity, sphenoid, and frontal sinuses [25].

Frontal Sinus Malignancies

19

Treatment . . . . 170

Surgical Approaches . . . . 172

Complications . . . . 175

Outcomes . . . . 176

Conclusion . . . . 176

References . . . . 176

The University of Florida Staging System

쐽 Stage I: tumors limited to the site of origin

쐽 Stage II: extension to adjacent sites (orbit, par- anasal sinuses, skin, nasopharynx, pterygo- maxillary fossa)

쐽 Stage III: base of skull or pterygoid plate de- struction and/or intracranial extension

The majority of frontal sinus malignancies are stage II or III at presentation. The lymphatic drainage of the frontal sinus occurs via lymphatic channels that drain the skin and the anterior nasal vault. As a re- sult, metastases are rare without tumor involvement of the overlying skin or extension into the anterior nasal mucosa. Regional metastases occur in 10%–

15%, and distant metastases are present at the time of diagnosis in 10% of patients with frontal sinus malig- nancies [36]. Despite advances in anterior craniofa- cial surgery and in multimodality therapy, local re- currence remains the most significant cause of treat- ment failure and death. The histologic grade and the extent of disease appear to be the most important factors associated with prognosis of tumors of this region [3, 48].

Pathology

The frontal sinus is lined by an epithelium often termed the Schneiderian membrane, which is de- rived from ectoderm and consists of pseudostratified columnar epithelium and seromucinous salivary glands. Not surprisingly, the most common neo- plasms arising in the frontal sinus are epithelial tu- mors arising from the pseudostratified columnar epithelium (squamous cell carcinoma) and adeno- carcinomas arising from the minor salivary seromu- cinous glands.

Frontal sinus malignancies are seen more com- monly in men, with a male: female preponderance of 5 : 1 and a peak incidence in the 5th and 6th decades of life [43]. Both tobacco smoke and certain occupa- tional exposures are associated with an increased risk of developing malignancy of the sinonasal tract, which presumably holds true for the frontal sinus as well. The latter group includes nickel workers, in whom the incidence of squamous cell carcinoma is 28-fold greater than that of the general population;

leather workers, who are exposed to tannins and chromate; and wood workers, who are exposed to formaldehyde-based adhesives, wood dust, and pre- servatives such as creosote, and have been reported to have a 500-fold increased risk for the development of adenocarcinoma [34, 44, 50].

An increased incidence of paranasal sinus malig- nancy has been reported in textile workers, petrole− um refinery workers, welders, blast furnace opera- tors, and individuals exposed to ionizing radiation [49, 50].

Exposure to Thorotrast (thorium dioxide), a ra- dioactive contrast agent that was commonly instilled into the maxillary sinuses until 1950, has been asso- ciated with the development of maxillary and frontal sinus cancer after a mean latent period of approxi- mately 15 years [49].

The most common types of sinonasal malignancies are [36]:

쐽 Squamous cell carcinoma (60%)

쐽 Minor salivary gland malignancies (adenocar- cinoma, adenoid cystic carcinoma, and mu- coepidermoid carcinoma) (20%–30%)

쐽 Esthesioneuroblastoma (10%)

쐽 Lymphoma (5%)

쐽 Sarcoma (5%)

쐽 Other less common malignancies including sinonasal undifferentiated carcinoma, melano- ma, plasmacytoma, malignant meningioma, and infraclavicular metastases

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In most cases, involvement of the frontal sinus occurs secondary to tumor spread, usually from the ethmoid sinuses, rather than occurring primarily.

The most common types of sinonasal neoplasms arising primarily from the frontal sinus are (Ta- ble 19.1) [1, 3, 10, 11, 15, 17, 25, 42, 43, 47]:

쐽 Squamous cell carcinoma, accounting for 90%

of cases

쐽 Adenocarcinoma

쐽 Rare reports of lymphoma, sarcoma, menin- gioma, and infraclavicular metastases

Epithelial Malignancies

Squamous cell carcinoma is the most common ma- lignancy reported to arise from the frontal sinuses, accounting for 90% of all cases [5]. Bone erosion with hyperostosis has been reported in 48% of cases, and the true incidence of bone involvement may be

underestimated, as the majority of cases described in the literature predate the use of CT scanning [5, 11].

The clinical presentation may mimic that of a muco- cele with swelling of the overlying skin, but evidence of infection is usually absent and the degree of bone destruction and hyperostosis has been described as out of proportion to sinus expansion [43]. The 5-year actuarial survival for patients with squamous cell carcinoma of the paranasal sinuses ranges from 25%

to 50%, but the prognosis for patients with squamous cell carcinoma of the frontal sinus is poor, and the majority of patients present at an advanced stage [3, 4, 23, 30]. Combined modality treatment consisting of radical surgery with postoperative radiation is most commonly employed, with irradiation alone reserved for patients with inoperable disease [25]. There is limited data to support the use of adjuvant chemo- therapy in the management of sinus carcinoma, but because of favorable results seen with the use of adju- vant chemotherapy in other head and neck sites, con- sideration should be given to the use of chemothera- py concurrent with irradiation [11, 25].

Basaloid squamous cell carcinoma and verrucous carcinoma have both been reported to arise from the sinonasal tract. Basaloid squamous cell carcinoma is a rare tumor of the paranasal sinuses, with only 14 cases reported to date, none of which have been re- ported to arise from the frontal sinus [52]. A histolog- ically distinct variant of squamous cell carcinoma, basaloid squamous carcinoma is an aggressive, high- grade tumor that is deeply invasive, multifocal, and often metastatic: the prognosis is poor because of ad- vanced stage at presentation secondary to the tumor’s aggressive characteristics. In contrast, verru- cous carcinoma is a low-grade variant of squamous cell carcinoma that is associated with a more favor- able prognosis and outcome when aggressive treat- ment is employed [37]. There have been two reports of verrucous carcinoma involving the frontal sinus, and despite low-grade histology, intracranial exten- sion was present in both cases, which underscores the need for aggressive treatment in such cases de- spite the presence of more favorable histology [37].

As for squamous cell carcinoma, irradiation alone is used for patients with inoperable disease or who re- fuse surgery.

Inverted papilloma may involve the frontal sinus in 11%–16% of patients and is associated with malig-

Table 19.1.Malignant tumors reported to arise from the frontal sinuses.

Histology

Squamous cell carcinoma Verrucous carcinoma

Malignant transformation of inverted papilloma Minor salivary gland malignancies

Adenocarcinoma

Mucoepidermoid carcinoma Sarcoma

Lymphoma Other

Plasmacytoma Hemangiopericytoma Malignant melanoma Malignant meningioma

Metastases (infraclavicular primary) Based on [1, 3, 5, 10, 11, 17, 25, 36, 42, 43, 47]

nant transformation to squamous cell carcinoma in 5%–15% [19]. Rarely, inverted papilloma may origi- nate from the frontal sinus [36].While inverted papil- loma of the frontal sinus may be treated using an en- doscopic modified Lothrop approach, the presence of malignancy mandates an external, en bloc resection [28].

Minor Salivary Gland Tumors

Adenocarcinoma is the second most common malig- nancy of the frontal sinus, accounting for approxi- mately 10% of reported cases, and is associated with a better prognosis than squamous cell carcinoma.

There is a known association between the develop- ment of sinonasal adenocarcinoma and exposure to the wood, leather, and textile industries as mentioned previously. The 5-year actuarial survival rate for pa- tients with adenocarcinoma of the paranasal sinuses ranges from 40% to 60%, with the best prognosis seen in patients with papillary histology [23, 30, 48].

Multimodality therapy employing surgery and irra- diation is most often used in treatment. Adjuvant chemotherapy appears to have a beneficial effect on disease control, with impressive local responses, and the 5-year disease-free survival for paranasal sinus adenocarcinoma is reported to be as high as 65%–

87% [1, 27]. The prognosis for patients with disease limited to the frontal sinus is unknown because of small numbers.

Adenoid cystic carcinoma of frontal sinus origin has not been reported, but may involve the frontal si- nus as a result of direct extension from the ethmoids.

Adenoid cystic carcinoma of the paranasal sinuses is associated with an increased incidence of local re- currence compared to other head and neck sites and poor long-term survival rates due to the develop- ment of distant metastases. The 5-year survival rates range from 17% to 53%, with local recurrence occur- ring in 50%, regional recurrence in 20%, and distant metastasis in 30% [30, 40]. Histologically, adenoid cystic carcinoma may manifest as cribriform, tubu- lar, or solid variants: the solid variant has been re- ported to have an increased incidence of local recur- rence, but the majority of tumors have a mixed histo-

logic pattern [40, 41]. Aggressive surgical resection combined with postoperative irradiation improves local control rates but has no effect on survival, be- cause of the high incidence of distant metastatic dis- ease [40]. Perineural invasion is a hallmark of this tu- mor and has been reported to occur in as many as 91% of paranasal sinus lesions: the presence of peri- neural invasion does not correlate with local control for sinonasal disease [40, 41]. Fast-neutron radio- therapy has been shown to provide higher local con- trol rates than mixed beam or photon irradiation and can provide excellent palliation, but does not influ- ence survival [14, 22].

Mucoepidermoid carcinoma of the paranasal si- nuses is rare, and only one case arising from the fron- tal sinus has been reported to date [3, 25, 43, 47, 48].

While there is a paucity of data regarding paranasal sinus mucoepidermoid carcinoma, aggressive surgi- cal treatment followed by irradiation is generally em- ployed.

Sarcoma

Sarcomas comprise approximately 5% of paranasal sinus malignancies with a variety of histologic types described, including rhabdomyosarcoma, fibrosarco- ma, angiosarcoma, leiomyosarcoma, chondrosarco- ma, and osteosarcoma [47]. Occurrence in the frontal sinus is rare, with four cases reported to date: two represented osteosarcomas, one chondrosarcoma, and one unspecified [18, 45, 47]. The treatment of sar- comas is primarily surgical, with postoperative irra- diation reserved for high-grade lesions, inadequate surgical margins, or inoperable recurrences [45]. Ra- diation therapy is often required in the postoperative setting because of the difficulty in obtaining clear margins at the skull base. Because sarcomas are rela- tively radioresistant, there is no role for preoperative radiation therapy, which has been shown to be of no benefit [45]. Fast-neutron radiotherapy may improve locoregional control [29]. The role of chemotherapy in conjunction with radiation therapy is unproven but may improve local control rates: regimens used include cyclophosphamide, vincristine, methotrex- ate, doxorubicin, and actinomycin D [45].

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Lymphoma

Lymphomas arising from the paranasal sinuses are most commonly found in the nasal cavity (68%), fol- lowed by the maxillary sinus (21%), ethmoid sinuses (9%), and least often in the frontal sinus (2%) [20].

These represent extranodal non-Hodgkin’s lym- phomas, with survival dependent on the immunolog- ic subtype [15, 20]:

Diffuse B-cell lymphomas are reported to have a 55% 5-year survival in comparison to T-cell lympho- mas, which have a 33% 5-year survival.

Treatment is non-surgical, consisting of combined chemotherapy and irradiation. It has been suggested that sinonasal non-Hodgkin’s lymphomas may be less responsive to traditional chemoradiation re- gimes [6]. The role of surgery is confined to biopsy for tissue diagnosis and can often be accomplished endoscopically, although an external approach may be required to obtain adequate tissue [16].

Miscellaneous Tumors

A variety of unusual tumors have been described originating from the frontal sinuses in rare instances.

Mucosal melanoma of the sinonasal tract is rare and most commonly arises from the lateral nasal wall, al- though origin in the frontal sinus has been described [24]. Long-term survival is rare, with most patients succumbing to disease within 5 years. Prognosis ap- pears unrelated to the site of origin, size of the pri- mary, local recurrence or treatment [9]. Most mor- bidity is due to distant disease. Treatment consists of radical surgery with adjuvant irradiation if an initial metastatic workup is negative.

Meningiomas may arise in the frontal sinus as ei- ther a direct extension of intracranial disease, or be truly extracranial, resulting from entrapment of arachnoid cells outside of the cranial cavity during bone fusion or in association with arterial sheaths

that extend through the dura and the bone [39]. Sur- gical excision is the mainstay of treatment, and un- like most sinonasal malignancies, the prognosis is fa- vorable for extracranial meningiomas. Intracranial meningiomas are associated with a high recurrence rate. Radiation therapy is not effective [39].

Plasmacytoma and metastases from infraclavicu- lar primary sites including the breast, lung, and kid- ney have been reported in the frontal sinus [12, 33, 51].

Plasmacytoma responds to either surgery or radia- tion therapy, and disseminated disease is treated with chemotherapy. The overall 10-year survival is report- ed to be 50% [51]. Metastases to the frontal sinus from infraclavicular neoplasms are poorly understood, but are associated with distant metastases at other sites and a poor prognosis.

Diagnosis

The frontal sinus is a relatively sequestered and quiescent location and as a result, early lesions of the frontal sinus may be asymptomatic or associated with vague, nonspecific complaints.

Early signs associated with frontal sinus neo- plasms include: a sensation of sinus pressure or dis− comfort and nasal discharge (which may or may not be bloody).

Early diagnosis is uncommon. In most reported cases, patients present with soft tissue swelling of the forehead or medial upper eyelid secondary to tumor erosion through bone, and may be misdiagnosed as having frontal sinusitis with osteomyelitis of the frontal bone. (Fig. 19.1) Absence of cellulitis, fever, and purulent discharge may aid in distinguishing in- fection from neoplasm, although an infectious com- ponent may exist secondary to tumor obstruction of sinus drainage. Proptosis, blepharoptosis, epiphora, and diplopia are signs of orbital involvement.

The presence of the following symptoms should alert the physician to the possibility of neoplasia:

쐽 Unilateral nasal obstruction

쐽 Anosmia

쐽 Intranasal mass

쐽 Proptosis

Imaging studies assist in making a correct diagnosis.

Axial and coronal computed tomography (CT) scans will demonstrate the degree of sinus aeration as well as any soft tissue or bony erosion. CT scanning is the diagnostic modality of choice to assess for bone in- volvement. CT is inferior to magnetic resonance im- aging (MRI) in delineating soft tissue characteristics of tumor, particularly in large lesions in which the tu- mor margin can be difficult to distinguish from the fascia of adjacent orbital and scalp musculature, and in determining the presence and extent of intracrani- al extension. MRI with gadolinium enhancement and fat suppression can distinguish between inspissated secretions and soft tissue masses, differentiate

between postsurgical changes and tumor, and dem- onstrate perineural enhancement associated with tu- mor spread (Fig. 19.2). The information obtained from both CT and MRI are complementary, and both should be obtained in the evaluation of extensive le- sions or when there is suspicion of malignancy.

Biopsy of tumors of the frontal sinus usually re- quires trephination of the floor of the frontal sinus for access in the absence of extension of disease into the ethmoid sinuses or nasal cavity. This can be at- tempted endoscopically but may require an external approach.

Treatment

Surgical resection is the most successful primary therapeutic modality for cancer involving the frontal sinuses [26]. The aggressive application of craniofa- cial approaches for tumor resection has significantly improved survival and local control rates. Surgery alone may be adequate treatment for early-stage sin- onasal cancer but is most commonly combined with postoperative irradiation because of the high inci- dence of advanced stage disease at presentation. The addition of postoperative radiation to craniofacial resection results in improved survival rates that are comparable to less advanced stage tumors and super- ior to single-modality treatment alone [4, 48]. The use of radiation as a sole initial treatment modality been described but is associated with poorer 5-year disease-specific survival rates of approximately 21%–40% [3, 25]. Salvage surgery after initial radia- tion failure is associated with a 5-year disease-specif- ic survival rate of 28% [13].

Regional metastases are uncommon for tumors of the paranasal sinuses, and elective treatment of the neck does not affect the survival or regional recur- rence rate and is therefore not indicated [25]. The ap- plication of concurrent chemotherapy with irradia- tion in the management of head and neck cancer arising from other sites suggests that the use of adju- vant chemotherapy should be considered in the treatment of sinonasal cancer, particularly in the treatment of epithelial malignancies [25]. However, there is inadequate data to support the routine use of adjuvant chemotherapy in the management of fron- tal sinus malignancies.

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Fig. 19.1.Medial left upper eyelid swelling and proptosis secon- dary to medial and downward displacement of the globe by tu- mor

Fig. 19.2.

Coronal T1 (A), T2 (B), gadolinium contrasted fat suppression T2 (C), and T1 (D)MRI images distinguish between secretions and tumor. Note tumor enhancement with gadolinium

Surgical Approaches

Endoscopic approaches to the frontal sinus have been successfully used in the treatment of benign disease. There is a lack of data regarding the efficacy of endoscopic approaches to the frontal sinus in the

treatment of malignant disease. An endoscopic ap- proach can be used for biopsy of inferiorly- or medi- ally-based tumors or tumors involving the nasofron- tal duct, but an open approach is indicated for surgi- cal extirpation of malignancy. The extent of resection required dictates the surgical approach that is used.

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Fig. 19.2C,D

The rare case of an early tumor confined to the frontal sinus with intact bony walls or located in the most inferior aspect of the frontal sinus or nasofron- tal recess can be approached through a limited exter- nal incision [36]. Access is accomplished with a 2–3- cm incision made above the medial aspect of the upper eyelid, just below the eyebrow and medial to the supraorbital nerve in the superomedial aspect of the orbit to expose the floor of the frontal sinus. The periosteum is elevated and the floor of the frontal si- nus can then be entered with a cutting burr. When exposure of the anterior ethmoids or nasofrontal re- cess is required, a Lynch frontoethmoidectomy inci- sion can be utilized, which extends the infrabrow in- cision medially and inferiorly to the level of the me- dial canthus and can be extended laterally to the lat- eral aspect of the brow. Attempts are made to pre- serve the integrity of the supraorbital neurovascular bundle when the anterior wall or floor of the frontal sinus is intact. When the intersinus septum has been penetrated by tumor and access to the contralateral frontal sinus is required, a gull-wing incision can be utilized, which involves a horizontal incision across the nasion connecting the incision to a contralateral Lynch incision. A major disadvantage of the gull- wing incision is the sequelae of bilateral forehead an- esthesia when both supraorbital nerves are divided.

A Silastic drainage tube is inserted at the completion of the procedure from the frontal sinus into the mid- dle meatus and confirmed endoscopically. The tube is sutured in place and removed after normal drain- age is restored, usually in 2–3 months. Prophylactic dacrocystorhinostomy is indicated when the lacrimal gland and/or duct is involved.

Larger tumors or those with involvement of the walls of the frontal sinus require a coronal approach for exposure, which can be combined with a bifrontal craniotomy and if needed, a lateral rhinotomy. If bi- frontal craniotomy is not required, a 6-foot Caldwell- Luc x-ray of the frontal sinuses is helpful for use as a template in determining the location of the frontal si- nus prior to making cuts in the frontal bone, to avoid inadvertent entry into the anterior cranial fossa. The coronal incision extends from the level of the zygo- ma, in the preauricular crease anterior to the tragus and extends over the vertex of the scalp above the hairline. The preauricular incision is extended verti- cally to the level of the vertex of the scalp to avoid di-

vision of the anterior branch of the superficial tem- poral artery. The lateral aspect of the incision is car- ried down to the level of the deep temporal fascia:

staying deep to this plane avoids injury to the super- ficial temporal artery, which travels within the tem- poroparietal fascia above the deep temporal fascia, as well as the temporal branches of the facial nerve which travel in the superficial muscular aponeurotic system (SMAS). Medially, the incision is usually car- ried down to the level of the pericranium, which is el- evated with the scalp flap to prevent desiccation and tearing of the flap in the event it is needed for recon- struction. The blood supply to the pericranial flap is based on the supraorbital and supratrochlear vessels, and its availability for reconstruction is dependent on the ability to preserve these vessels during tumor ablation.

The coronal flap is elevated to the level of the su- praorbital rims, leaving the periosteum on the bone, with care taken to avoid injury to the supraorbital and supratrochlear vessels which exit at the level of the supraorbital rim. The periorbita can then be ele- vated from the superior, lateral, and medial walls of the orbit. At this point the frontal sinus can be en- tered if craniotomy is not planned (Fig. 19.3). When the posterior wall of the frontal sinus is suspected preoperatively to be involved by tumor, a unilateral

Fig. 19.3.Exposure of anterior and posterior walls of frontal si- nus with a coronal approach. Note tumor obstructing the right nasofrontal duct

or bifrontal craniotomy results in excellent exposure of the posterior wall of the frontal sinus and assess- ment of dural invasion. Frontal lobe dura is elevated from the posterior wall of the frontal sinus or resect- ed if involved; the extent of resection is dictated by the extent of disease. The dura can be reconstructed with a pericranial flap, which is elevated from the scalp flap, rotated into position, and sutured to the dural

defect (Fig. 19.4). When pericranium is not available, other options include pericardium, cadaveric dura, or a tensor fascia lata graft. Posterior frontal bone de- fects require cranialization of the remaining sinus.

The nasofrontal ducts must be obliterated with mus- cle plugs to prevent cerebrospinal fluid leakage. Ante- rior wall defects can be reconstructed using split cal- varial bone grafts, titanium mesh, methylmethacry-

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Fig. 19.4.A pericranial flap is elevated from the scalp flap (A)and used for reconstruction of a dural defect following bifrontal craniotomy (B)

Fig. 19.5.

Reconstruction of an extensive fron- tal bone defect with split thickness calvarial grafts

late, or a free flap (Fig. 19.5).When tumor involvement necessitates resection of the overlying forehead skin, the resulting defect can be closed with a rotational flap if small (less than 2 cm), but larger defects usual- ly require free flap reconstruction because of the lim- ited mobility of the scalp skin.

Management of the orbit is a topic of some debate, largely because of the emotional issues surrounding the loss of an eye versus the oncologic safety of pre- serving the orbit and the functional outcome of a preserved eye [23]. The presence of orbital involve- ment, including invasion of the periosteum alone, ad- versely affects survival compared with patients with- out orbital or periosteal involvement [8, 30]. Howev- er, when survival of patients undergoing periosteal resection with preservation of the eye is compared with that of patients undergoing exenteration, pres- ervation of the eye is not associated with a poorer outcome, suggesting that the orbit can be preserved in such cases [8, 30]. Tumors that invade periorbita have higher potential for invasion and a more dismal prognosis [8]. Involvement of the periorbita has tra- ditionally been considered an indication for orbital exenteration, but several studies have suggested that limited involvement of the periorbita can be resected with orbital preservation, using frozen section con- trol to achieve negative margins without compromis- ing outcome [23, 31, 38]. Extensive periorbital involve- ment, defined as unresectable full-thickness invasion of the periorbita, or extension into the ocular fat, muscles, or orbital apex are indications for exentera- tion [23].

Preservation of the eye has been reported to result in preservation of functional vision postoperatively in 91% of patients; however, 41% developed ocular se- quelae, the most common of which were due to ab- normalities of globe position (enopthalmous) par- ticularly if rigid bony reconstruction was not per- formed [23]. Ocular function is adversely affected by extent of resection of supporting structures, particu- larly the floor of the orbit [8]. Rigid reconstruction of the walls of the orbit is recommended for subtotal or total floor defects, defined as greater than 80% of the surface area, or multisegment defects. The function of a preserved eye is significantly worsened when postoperative radiation is used. In one large series of patients who underwent orbital preservation, radia- tion-induced blindness occurred in the ipsilateral

eye in 35% and in the contralateral eye in 8% [25].

Overall, radiation-induced functional ocular seque- lae occur in more than 50% of patients treated with postoperative irradiation with complications that in- clude ectropion, conjunctivitis, exposure keratitis, epiphora, blindness, and cataract formation. [23].

However, the sequelae related to orbital preservation may be acceptable to patients given the aesthetic val- ue of a preserved eye, particularly when some func- tional vision can be preserved. Bilateral orbital exen- teration is considered by many surgeons to be a con- traindication to surgical resection.

Complications

Complications arising from surgical ablation of fron- tal sinus carcinoma are related to the approach and extent of resection and range from wound infection to meningitis and death. Both intracranial and extra- cranial approaches are associated with a good cos- metic result, particularly when bony reconstruction

Fig. 19.6.Same patient depicted in Fig. 19.1, 3 months after bi- frontal craniotomy with left lateral rhinotomy and irradiation

is employed for defects of the anterior frontal sinus wall and orbit (Fig. 19.6). Extracranial approaches are associated with a low incidence of complications, with wound infection and cerebrospinal fluid leak most common. Intracranial resection is associated with a higher incidence of complications, with men- ingitis and cerebrospinal fluid leak most commonly reported [30, 47]. The overall complication rate is less than 20%, with perioperative mortality following intracranial approaches ranging from 0% to 13% [3, 4, 30, 32, 36]. The postoperative complication rate fol- lowing preoperative irradiation is significantly high- er, ranging from 24% to 100% [13, 36]. Mental status changes may result from significant frontal lobe re- section or excessive retraction.

Outcomes

The overall disease-specific 5-year survival rates for patients with paranasal sinus malignancy range from 24% to 69% [3, 4, 26, 30, 32, 48]. Local recurrence is the major cause of mortality in patients with sinona- sal malignancy and occurs in 38%–89% of patients [3, 4, 32]. Results of salvage resection following failure of initial treatment are poor [36]. The incidence of lo- cal recurrence appears to be independent of the stat- us of surgical margins following craniofacial resec- tion and is similar in patients with both positive and negative margins [32, 46]. More than 50% of patients with negative surgical margins experience local re- currence, likely because of the difficulty in achieving wide en bloc resections and evaluating resection margins at this site, particularly when disease has spread to involve the adjacent ethmoid sinuses, orbi- tal walls, and cranial vault [32, 47, 48].

Advanced tumor stage, histology, skull base and dural involvement, and orbital involvement are asso- ciated with a poor prognosis [3, 4, 30, 32, 48]. Squa- mous cell carcinoma in particular has been associat- ed with diminished 5-year survival rates of 10%–32%

compared to other histologic tumor types [30, 32].

Dural and orbital involvement similarly are associat- ed with reduced 5-year survival rates of 23%–29%

compared with 48%–69% in patients without inva- sion of these structures [30, 48].

Conclusion

The development of craniofacial approaches to sin- onasal malignancies has resulted in improvement in local control rates, and significant palliation can be achieved with acceptable morbidity and mortality [26]. However, with improved local control, a greater proportion of patients succumb to distant metastat- ic disease, which is reported to develop in as many as one-third of patients [30]. As is true for cancers of the head and neck involving other sites, disease- specific survival for frontal sinus cancer is unlikely to improve without innovations in novel systemic ther- apies.

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