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Nasoseptal chondrosarcoma resection requiring anterior fossa floor repair: a case report.

Abstract

We present a case in which a 23-year-old male patient diagnosed with nasoseptal chondrosarcoma with anterior cranial fossa extension underwent combined surgical approaches to optimize complete resection of the tumor. Surgery was followed by a 6-month course of proton-beam radiation therapy. Extensive tumors of this type with significant intracranial extension require surgical repair of the anterior fossa floor. We employed a repair technique not previously associated with the resection of these tumors, using a split-calvaria osteoplastic rotational flap. This method provided structural integrity while avoiding the risks associated with the use of free bone grafts and metallic meshes.

Introduction

Nasoseptal chondrosarcoma is described as a rare, malignant tumor with approximately 50 cases reported thus far in the English and Japanese literature. (1,2) It is characterized by tumor cells that form a cartilaginous matrix. Less than 10% of all chondrosarcomas occur in the craniofacial region, and nasoseptal occurrence is rare. (3-6) Extension of these tumors may lead into the anterior cranial fossa through the cribriform plate. With a reported recurrence rate as high as 65% with positive margins, complete resection with negative margins and adjuvant radiation therapy are the accepted therapies for this cancer. (1,4)

In patients with intracranial tumor extension, reconstruction of the defect in the anterior cranial fossa floor following surgical resection presents a challenge in providing a durable seal between the nasal and cranial compartments. Inadequate reconstruction has been shown to lead to serious complications. (1,7) Published reports of nasoseptal chrondrosarcomas with intracranial extension have not addressed these problems.

We present a new case of nasoseptal chondrosarcoma with anterior cranial fossa extension in a 23-year-old man in whom a durable repair of the osseous defect was successfully implemented with a technique not well known in the otolaryngology literature and not previously associated with the repair of defects caused by these tumors. Following combined surgical approaches for complete resection of the tumor, reconstruction of the osseous defect in the anterior fossa floor was achieved with a split-calvaria osteoplastic rotational flap.

Case report

A 23-year-old otherwise healthy male patient presented after approximately 1 year of intermittent nasal congestion, hyposmia, and decreased taste, with no improvement after conservative medical therapy. Physical examination revealed left ophthalmic (V1) and maxillary (V2) distribution hypoesthesias. Endoscopic examination revealed that a bilateral nasal cavity mass involved the posterior nasal septum and middle meatus and extended into the nasopharynx. The mass was covered by red mucosa and superficial telangiectasias. The findings of a transnasal biopsy were consistent with chondrosarcoma.

Computed tomography (CT) of the head and sinuses revealed near-complete opacification of the sphenoid, ethmoid, and frontal sinuses, with extensive bony destruction in the high posterior nasal cavity and dehiscence of the anterior-mid skull base, suggesting intracranial extension. Magnetic resonance imaging (MRI) of the head and sinuses revealed a large, midline, posterior mass filling the posterior nasal cavity (figure 1, A) and extending posteriorly into the sphenoid and ethmoid sinuses and 16 mm superior to the floor of the anterior cranial fossa (figure 1, B and C).

[FIGURE 1 OMITTED]

The tumor extended deep toward the clivus, leaving a thin but intact posterior clival wall. The brain parenchyma appeared uninvolved. The mass appeared markedly heterogeneous, nearly isointense to fat on the T2-weighted sequences, and isointense to muscle on the T1-weighted sequences.

A combined otolaryngologic and neurosurgical approach was planned. Surgical excision of the tumor initially involved transnasal endoscopic removal of the tumor, which was converted to an open lateral rhinotomy approach because of the extent of the tumor resection required and difficulty in assessing margins. Bifrontal craniotomy with osteoplastic temporalis flaps was then performed to further expose the tumor overlying the anterior cranial fossa. The remaining tumor was grossly removed using a combined transnasal and transcranial approach.

[FIGURE 2 OMITTED]

Cranial reconstruction following the tumor excision involved a split-calvaria osteoplastic rotational flap to close the defect in the anterior fossa floor in a manner previously described for defects of this nature. (7) A small island of split calvarium was harvested from the posterior midfrontal bone and remained attached to the frontal pericranium (figure 2, A). This combination of pericranial flap and bone was mobilized and shaped to conform and fill the anterior fossa osseous defect (figure 2, B). A lumbar drain was placed for postoperative drainage.

[FIGURE 3 OMITTED]

This patient's postoperative course was complicated by temporary mental-status depression following over-drainage of cerebrospinal fluid. With prolonged use of a ventriculostomy and lumbar drain catheter, the patient also developed a brief episode of meningitis, which responded well to antibiotics.

Permanent histopathologic analysis revealed a high-grade chondrosarcoma (figure 3). The patient completed a 6-month course of proton irradiation therapy. Repeat nasal mucosa biopsy was negative for malignancy. Follow-up imaging and endoscopic surveillance have revealed no recurrence of tumor and have shown good integrity of the anterior fossa floor reconstruction (figure 4) and clivus. At 36 months postsurgery, the patient is doing well but continues to have complete anosmia and significant nasal crusting. Persistent postoperative epiphora was corrected with dacryocystorhinotomy (DCR).

Discussion

Chondrosarcoma is a malignant tumor characterized by tumor cells that form a cartilaginous matrix. It is the third most common primary malignant tumor of bone, after multiple myeloma and osteosarcoma. Chondrosarcomas may arise de novo (primary), or superimposed (secondary) upon preexisting cartilaginous lesions, such as enchondromas or osteochondromas. Most tumors occur in patients in their thirties, with a slight male predilection (1.5:1).

[FIGURE 4 OMITTED]

Less than 10% of all cases ofchondrosarcoma occurin the craniofacial region. Craniofacial chondrosarcomas frequently arise in the extragnathic bones but may involve any bony or soft-tissue structure. Although our patient presented with a high-grade tumor, generally these are low-grade tumors that respond well to treatment. Chondrosarcomas are divided into three histopathologic grades based on degree of cellularity, nuclear size and atypia, mitotic activity, and surrounding matrix composition, with grade I tumors closely mimicking benign lesions and grade III tumors exhibiting the highest degree of mitosis and cellular atypia. Disease-specific 5- and 10-year survival rates have been reported as 87.2% and 70.6%, respectively. (8)

Because of the nature of nasoseptal chondrosarcoma and its high recurrence rate, aggressive treatment is recommended. This involves wide surgical resection and adjuvant radiation therapy. (1-6) Local recurrence after surgery alone may be as high as 65% if positive margins are found. (4) It has also been reported that nearly 80% of patients who die of this disease have uncontrolled local disease. (1) Therefore, multiple surgical approaches have been described in the literature, with the goal of complete resection and clear margins. These approaches have included the Weber-Ferguson approach, sublabial-transnasal approach, lateral rhinotomy approach, Le Fort I down-fracture, and an anterior craniofacial approach), (1,3-6) More recently, endoscopic approaches have been described for resection of nasoseptal chondrosarcomas limited to the nasal cavity without evidence of intracranial or skull base extension. (4,5,9)

For nasoseptal chondrosarcomas that extend superiorly into the anterior cranial fossa, a combined craniofacial anterior skull base approach, as was used in this case, has been shown to provide excellent exposure for wide local excision. (1) This requires resection of the anterior fossa floor in order to complete the wide local excision of the tumor. Resection of the anterior fossa floor has also been described for malignant growths such as meningiomas, esthesioneuroblastoma, orbital tumors, and other paranasal tumors. (7) However, resection of the anterior fossa floor establishes a communication between the nasal cavity and the intracranial compartment. Surgical reconstruction of the anterior fossa floor osseous defect is necessary to close this communication and may present a challenge. An inadequate seal separating the nasal and cranial compartments has been shown to lead to complications such as CSF rhinorrhea, meningitis, and meningoencephalocele formation. (1,7) Also, failure to adequately reconstruct this defect could lead to injury of intracranial contents during subsequent intranasal procedures. (7)

Significant effort has been taken in designing repairs for defects of this type, to include soft-tissue flaps (including pericranium, galea aponeurotica, and frontalis or temporalis muscle) or to perform flee-tissue transfer. (7) However, the lack of rigidity of these reconstruction techniques has shown them to be less than ideal. Various techniques, including free bone grafts or the use of metallic mesh, have been used as a more durable repair of the osseous defect of the anterior fossa floor. Unfortunately, these methods achieve a less-than-ideal cranioplasty because of the presence of a nonsterile field, subjecting the reconstruction to osteomyelitis, resorption, bacterial colonization, and soft-tissue erosion. (7)

In our case, reconstruction was successfully achieved with a split-calvaria osteoplastic rotational flap for repair of the anterior fossa osseous defect. This technique obviated the above issues, more than adequately repaired the floor, and has thus far maintained its structural integrity.

References

(1.) Kaufman JK, Pritz MB, Righi PD, Bizal JC. Craniofacial resection of a nasoseptal chondrosarcoma: Case report and review of the literature. Surg Neuro 1999;52(3):265-8; discussion 268-9.

(2.) Yamamoto S, Motoori K, Takano H, et al. Chondrosarcoma of the nasal septum. Skeletal Radio1 2002;31(9):543-6.

(3.) Chen CC, Hsu L, Hecht JL, Janecka I. Bimaxillary chondrosarcoma: Clinical, radiologic, and histologic correlation. AJNR Am J Neuroradiol 2002;23(4):667-70.

(4.) Coppit GL, Eusterman VD, Bartels J, Downey TJ. Endoscopic resection of chondrosarcomas of the nasal septum: A report of 2 cases. Otolaryngol Head Neck Surg 2002;127(6):569-71.

(5.) Downey TJ, Clark SK, Moore DW. Chondrosarcoma of the nasal septum. Otolaryngol Head Neck Surg 2001;125(1):98-100.

(6.) Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD. Primary chondrosarcoma of the head and neck in pediatric patients: A clinicopathologic study of 14 cases with a review of the literature. Cancer 2000;88(9):2181-8.

(7.) Kantrowitz AB, Hall C, Moser F, et al. Split-calvaria osteoplastic rotational flap for anterior fossa floor repair after tumor excision. J Neurosurg 1993;79(5):782-6.

(8.) Koch BB, Karnell LH, Hoffman HT, et al. National cancer database report on chondrosarcoma of the head and neck. Head Neck 2000;22(4):408-25.

(9.) Matthews B, Whang C, Smith S. Endoscopic resection of a nasal septal chondrosarcoma: First report of a case. Ear Nose Throat J 2002;81(5):327-9.

Samuel Spear, MD; John Iskandar, MD; Derek Seaquist, MD; Douglas Sorensen, MD

From the Division of Otolaryngology (Dr. Spear and Dr. Sorensen), the Division of Neurosurgery (Dr. Iskandar), and the Department of Pathology (Dr. Seaquist), Madigan Army Medical Center, Tacoma, Wash.

Corresponding author: Capt. Samuel A. Spear, MC, USAF, Madigan Army Medical Center, ATTN: MCHJ-SET, Tacoma,WA 98431-5000. Phone: (253) 968-1420; fax: (253) 968-3154; e-mail: samspear@ gmail.com
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Title Annotation:ORIGINAL ARTICLE
Author:Spear, Samuel; Iskandar, John; Seaquist, Derek; Sorensen, Douglas
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Dec 1, 2008
Words:1757
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