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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 38-41

Anterior craniofacial resection for squamous cell carcinoma ethmoid sinus involving anterior skull base


1 Department of Otorhinolaryngology and Head Neck Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Neurosurgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission26-Apr-2020
Date of Acceptance23-Jan-2021
Date of Web Publication03-Jul-2021

Correspondence Address:
Dr. Saurabh Varshney
Department of Otorhinolaryngology and Head Neck Surgery, All India Institute of Medical Sciences, Rishikesh - 249 203, Uttarakhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aiao.aiao_5_20

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  Abstract 


Sinonasal squamous cell carcinoma (SCC) is a rare form of head-and-neck malignancy, with an incidence of 3%, while SCC of the ethmoid sinus is even more infrequent with relatively few cases cited in the literature. Management of anterior skull base tumors is complex due to the anatomic detail of the region and the variety of tumors that occur in this area. Currently, the “gold standard” for surgery is the anterior craniofacial resection. A 61-year-old man with a locally advanced SCC of the ethmoid sinus involving orbit with intracranial extradural extension was surgically treated performing anterior craniofacial resection followed by adjuvant intensity-modulated radiation therapy to the tumor bed. Currently, the “gold standard” for ethmoid carcinoma with intracranial extension is the anterior craniofacial approach. It allows wide exposure of the complex anatomical structures at the base of the skull permitting monobloc tumor resection. A multidisplinary team approach involving an otolaryngologist, neurosurgeon, plastic surgeon, pathologist, and radiologist is required for effective management of these tumors.

Keywords: Carcinoma, craniofacial, ethmoid, skull base


How to cite this article:
Angral S, Varshney S, Arora RK, Malhotra M, Tyagi AK, Kumar A. Anterior craniofacial resection for squamous cell carcinoma ethmoid sinus involving anterior skull base. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2021;5:38-41

How to cite this URL:
Angral S, Varshney S, Arora RK, Malhotra M, Tyagi AK, Kumar A. Anterior craniofacial resection for squamous cell carcinoma ethmoid sinus involving anterior skull base. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2021 [cited 2021 Dec 4];5:38-41. Available from: https://www.aiaohns.in/text.asp?2021/5/1/38/320575




  Introduction Top


Sinonasal squamous cell carcinoma (SCC) is a rare form of head-and-neck malignancy, with an incidence of 3%, while SCC of the ethmoid sinus is even more infrequent with relatively few cases cited in the literature.[1] Management of anterior skull base tumors is complex due to the anatomic detail of the region and the variety of tumors that occur in this area. Currently, the “gold standard” for surgery is the anterior craniofacial resection.[2]

Craniofacial tumors are divided into three major groups: (a) primary tumors of the paranasal sinuses (PNS) that expand intraorbitally and intracranially; (b) tumors arising from the skin or appendages (lacrimal gland) and expanding into the orbit and anterior cranial fossa; and (c) primary intracranial tumors that originate within the cranium and expand extracranially into the orbit and PNS.[3] The operative strategy depends on the site of origin, extent, predominant growth direction, and biologic behavior.[3]


  Case Report Top


A 61-year-old male patient with no systemic comorbidity presented to our outpatient department with chief complaints of left orbital swelling for 8 months with the loss of vision for 2 months. There was associated history of left-sided nasal obstruction, anosmia with intermittent episodes of self-resolving epistaxis of 5-month duration. The patient was a chronic smoker with a smoking index of 30 pack-years. There was no prior history of any exposure to radiations. On examination, a huge ulceroproliferative growth was noted over the left eyelid causing downward and lateral displacement of the eyeball with absence of orbital movements and no perception of light (−ve) [Figure 1]. A diagnostic nasal endoscopy was done which showed a fleshy friable mass in the left middle meatus [Figure 2], obliterating the roof of the nasal cavity, and a representative biopsy was sent for histopathological examination (HPE) which revealed features suggestive of SCC of possibly nasoethmoid origin. Central nervous system examination showed cranial nerve I, II, III, IV, and VI involvement on the left side.
Figure 1: (a-c) Preoperative

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Figure 2: Nasal endoscopy

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Contrast-enhanced computed tomography scan of the nose and PNS revealed a 6 cm × 3 cm enhancing mass occupying the left nasal cavity, ethmoid sinus, and orbit with destruction of medial wall of the left maxillary sinus. The tumor also invades the left basifrontal region of the brain, left medial and superior rectus with left proptosis [Figure 3]. A contrast-enhanced magnetic resonance imaging was also done which showed lobulated homogeneous lesion within left orbit causing displacement of the optic nerve and eyeball to the left side, with destruction of lamina papyracea, involving ethmoidal cells bilaterally superiorly extending up to the cribriform plate [Figure 4].
Figure 3: (a) Computed tomography scan axial cut. (b) Computed tomography scan coronal section

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Figure 4: (a and b) Magnetic resonance imaging

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Informed written consent from the patient was obtained for the surgical procedure. Intraoperatively, the extent of the tumor was assessed and the tumor was found to be intracranial extradural. The tumor was resected via anterior craniofacial resection approach using Weber–Ferguson incision and frontal craniotomy. Orbital exenteration with partial maxillectomy and removal of ethmoidal labyrinth followed by reconstruction via pericranial flap for skull base repair, temporalis muscle for orbital and maxillary cavity obliteration, and a paramedian forehead flap for orbital reconstruction was done [Figure 5] and [Figure 6]. The tumor was removed en bloc and sent for HPE [Figure 7]. The final HPE report showed nonkeratinizing SCC and all the resected margins were free of tumor. The tumor was staged as pT4aN0Mx (Stage IV A).
Figure 5: (a-h) Peroperative

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Figure 6: (a-e) Reconstruction of defect

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Figure 7: (a and b) Excised specimen with orbital exenteration

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The patient had a smooth postoperative course with no significant complications [Figure 8],[Figure 9],[Figure 10]. Adjuvant intensity-modulated radiation therapy (IMRT) was administered to the patient 4 weeks postoperatively. A total dose of 60 Gy in 30# was given. The patient has been on regular follow-up for 6 months and there has been no radiological or clinical evidence of disease recurrence.
Figure 8: (a and b) Postoperative

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Figure 9: Postoperative (6 months)

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Figure 10: Postoperative computed tomography scan

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  Discussion Top


In 1963, Ketcham et al.[4] first reported the effectiveness of advanced continuous flow reactor (ACFR) for the removal of intracranial organs by en bloc resection of a paranasal tumor extending from the nasal cavity to the skull base plate. The oncologic principles of anterior craniofacial resection involve an en bloc resection of tumor, including the ethmoid sinuses, superior nasal septum, and floor of the anterior cranial fossa, corresponding to the interorbital area (i.e., anterior craniofacial resection) or extended laterally to include part of the bony orbit or its soft-tissue contents (anterolateral craniofacial resection).[2]

Sinonasal SCC is a rare form of head-and-neck malignancy, with an incidence of 3%, while SCC of the ethmoid sinus is even more infrequent with relatively few cases cited in the literature.[1] Since it is difficult to diagnose ethmoid cancer in the early stage of disease, most patients are diagnosed with locally advanced SCC of the ethmoid sinus (LASCC-ES). Ethmoid cancer easily extends into the anatomical structures around the ethmoid sinus, including the skull base plate, intracranial organs, facial skin, and orbital content. The general treatment for LASCC-ES is multidisciplinary consisting of surgery, radiotherapy (RT), or chemoradiotherapy (CRT).

Some LASCC-ES patients reject the option of ACFR because of the significant extent of anatomical damage that is often accompanied by facial deformation and/or visual impairment. Therefore, LASCC-ES patients who reject ACFR and require that organ anatomy and function should be preserved undergo alternating CRT or concomitant RT and intra-arterial cisplatin (Radiotherapy and concomitant intra-arterial Cisplatin) as first reported by Robbins et al.[5] in 1992.

Adding concomitant chemotherapy to adjuvant radiation therapy, i.e., high-dose cisplatinum, as a radiosensitizer and to mitigate the occurrence of distant metastasis, might be beneficial, in light of this tumor's significant potential for local and distant recurrence.[6]


  Conclusion Top


Due to the rarity of sinonasal SCC involving the anterior skull base, the standard of care has not been established, but aggressive management using craniofacial resection followed by adjuvant IMRT with or without chemotherapy seems to be an acceptable option. A multidisplinary team approach involving an otolaryngologist, neurosurgeon, plastic surgeon, pathologist, and radiologist is required for effective management of these tumors.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sisson GA, Bytell DE, Becker SP, Ruge D. Carcinoma of the paranasal sinuses and cranial-facial resection. J Laryngol Otol 1976;90:59-68.  Back to cited text no. 1
    
2.
Varshney S, Bist SS, Gupta N, Singh RK, Bhagat S. Anterior craniofacial resection-for paranasal sinus tumors involving anterior skull base. Indian J Otolaryngol Head Neck Surg 2010;62:103-7.  Back to cited text no. 2
    
3.
Ermiş I, Arinci A, Elmaci I, Hayirlioğlu T, Oral Z. The treatment of craniofacial meningioma – A case report. J Psychiatry Neurolog Sci 1995;8:61-4.  Back to cited text no. 3
    
4.
Ketchem AS, Wilkins RH, Van Buren J M, Smith RR. A combined intra-cranial facial approach to the para-nasal sinuses. The American J Surg 1963:106;698-703.  Back to cited text no. 4
    
5.
Robbins KT, Storniolo AM, Kerber C, Seagren S, Berson A, Howell SB. Rapid superselective high-dose cisplatin infusion for advanced head and neck malignancies. Head Neck 1992;14:364-71.  Back to cited text no. 5
    
6.
Al Feghali KA, Traboulsi H, Youssef B. Basaloid squamous cell carcinoma of the ethmoid sinus with invasion into the skull base treated with craniofacial resection and adjuvant intensity-modulated radiation therapy: A case report. Cureus 2015;7:e421.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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