Chondromyxoid Fibroma of the Frontal SinusA Case Report and Review of the Literature

Washington University in St Louis Dept of Neurosurgery Otolaryngology and Oculoplastics

Townsend Melanie MD Chicoine Michael MD Custer Phillip MD Murphy Rory MD Schneider John MD

INTRODUCTION DISCUSSION

RESULTS

Figure 1 2 CT and MRI of the left frontoethmoid sinus lesion

ABSTRACT

METHODS AND MATERIALS

CONCLUSIONS

REFERENCES

Objective Chondromyxoid fibroma (CMF) is a rare cartilaginous tumor that is especially uncommon in the paranasal sinuses and skull base Only 3 cases of CMF with frontal sinus origin are reported including this case Proper diagnosis and management is essential for differentiating these tumors from their malignant counterparts

Methods We present a case of CMF arising in the frontal sinus and eroding intracranially in a healthy 45 year-old female An in depth discussion of the diagnosis and management of sinonasal and skull base CMF is presented along with a review of the literature

ResultsConclusion Twenty eight cases of sinonasal and skull base CMF were identified Complete excision is the treatment of choice for CMF with radiation therapy reserved for recurrences and difficult to reach locations as there have been reports of malignant transformation rate of 1-2 following radiation En bloc excision is not considered necessary at the expense of critical structures such as the eye The radiographic appearance of these tumors can be very similar to chondrosarcoma its malignant counterpart and proper diagnosis via histology is critical

A retrospective review of available literature was

conducted using PubMed Search terms included

ldquochondromyxoid fibromardquo ldquoparanasal sinusrdquo ldquoskull

baserdquo and ldquonasal cavityrdquo Publication dates from

1997 to 2016 were identified and included All

included articles were analyzed for radiologic and

histologic features demographic data symptoms

management and follow up

bull CMF tumors are extremely rare especially in

the paranasal sinuses

bull On imaging they often have malignant

appearing features and biopsy is required for

diagnosis

bull Complete surgical excision is the treatment

of choice but en block resection at the

expenses of critical structures such as the

eye is not recommended

bull Curettage excision is associated with

recurrence in this case series

bull Radiation is recommended only or difficult to

reach tumors or recurrences

Chondromyxoid fibroma (CMF) is a rare

cartilaginous tumor with benign pathologic

features and no metastatic potential About

5 of CMF lesions arise in the head and

neck and are even less common in the

paranasal sinuses1-3 Two cases of CMF

originating in the frontal sinus were identified

in the literature and we present a third It is

imperative that CMF be differentiated from

chondroblastoma and chondrosarcoma

which can have overlapping features but very

different treatment algorithms

CASE REPORT

A 45 year-old female presented with

progressive blurry vision in her left eye and

persistent headache in the left occipital and

frontorbital regions She also had drooping of

her left upper eyelid and edema of her left

eyebrow CT revealed complete opacification

of the left frontal and ethmoid sinuses with

hyperexpansion and erosion of bone Fig 1

3-D image reconstruction revealed complete

erosion of the medial third of the left superior

orbital rim MRI revealed a T1 hypointense

T2 hyperintense enhancing mass centered in

the left frontal sinus with extension into the

left anterior ethmoid air cells Fig 2

Destruction of the anterior and posterior

frontal tables was seen with erosion through

the superiormedial orbital wall and

impingement on the left medial rectus The

mass entered the anterior cranial fossa and

abutted the anteroinferior frontal lobe dura

Concern was for a malignant neoplasm but

biopsy revealed a CMF The patient

underwent a combined endoscopic and open

bicoronal approach for resection Fig 34

Figure 4 5 View of the posterior table after tumor excision Pericranial flap used for reconstruction

Figure 34 Bicoronal approach with osteplastic flap open Largest fragment of excised tumor

Author Age

(yr)gender

Symptoms at

presentation

Location Radiographic features Treatment approach Follow up

Azorin4 46M Supracilliary mass

otherwise

asymptomatic

Frontal sinus Destruction of the

posterior table with

dural involvement

Subfrontal approach

superior orbitotomy

en block resection

22mo DF

Baujat7 50F NAO HA Nasal bone extension into

ethmoids and frontal sinus

Invasion of skull base

dural involvement

Frontal osteoplastic

bone flap and dural

excision

18mo DF

Castle8 43F Sinus pressure Ethmoids invading through LP Bony erosion

displacement of

orbital contents

NR NR

Cruz9 10F Progressive

exophthalmos

Ethmoids invading through LP LP erosion and orbital

compression

Bicoronal approach

with en bloc tumor

resection

NR

Frank10 26M Diplopia Petroussphenoid bones

extending into clinoid sella

and cavernous sinus

Calcifications on CT Subtemporal approach

to excision

NR

Hashimoto11 32M Painless forehead

swelling

exophthalmos

Ethmoid sinus with extension

into frontal sinus and orbit

Lobulated mass on CT

with bony erosion

Excision 2yr DF

Isenburg12 34F NAO Ethmoids Calcifications on CT Endoscopic excision 8mo DF

Januszek13 51F NR Nasal septum extension into

sphenoid and maxillary sinus

Bony erosion on CT Excision Recurred at

12mo with re-

excision

Kadom6 14M Decline in school

performance HA

NAO

Frontal bone extension into

ethmoid and frontal sinus

with orbital infiltration

intradural extension

Calcifications on MRI

partially cystic and

inhomogeneous

Excision NR

Keel14 65F HA Clivus with sphenoid and

ethmoid extension

Bony erosion on CT Curettage 26mo DF

Keel14 66F NAO Sphenoid with extension into

ethmoids and hard palate

Bony erosion on CT Curettage recurred at

6mo radiation

given and DF

at 20mo

Keel14 34F Vision changes HA Clivus with nasopharynx

extension

Bony erosion on CT Incomplete excision

via curettage radiation

11mo DF after

radiation

Koay15 57F Painless swelling

over nasal dorsum

Nasal bone with extension

into frontal and ethmoid

sinuses

Erosive features on CT Incomplete excision

reported

NR

McClurg3 49F NAO Nasal septum with extension

into maxilla

Bony expansion and

destruction

Midface degloving 16mo DF

Mendoza16 1moM Respiratory

difficulty since birth

Ethmoid sinuses Homogenous

enhancement on MRI

En block resection 2yr DF

Morris17 52F Vertigo Sphenoid sinus locally destructive with

calcifications on CT

Endoscopic excision 2yr DF

Nazeer18 66F NAO Sphenoid sinus with sellar

and nasopharynx extension

Expansile and

enhancing on T1 MRI

heterogeneous on T2

Curettage Recurred at

1yr repeat

curettage and

DF at 6mo

Nazeer18 20daysM NAO Ethmoid sinuses Bony erosion on CT Excision 12mo DF

Perez-

Fernandez19

60M NAO epistaxis Maxillary sinus with ethmoid

extension

Bony expansion and

erosion

Combined endoscopic

and Caldwell-Luc

resection

5yr DF

Smith20 49F NAO Floor of nasal cavity

extending into hard palate

Bony erosion Excision 30mo DF

Shek37 6F NAO epistaxis

visual impairment

Ethmoids frontal bone maxilla nasal cavity

sella turcica

Heavily calcified Curettage x4 and

radiation following last

excision

Recurred until

radiation

Thomas21 NR Facial pain NAO Nasal cavity and palate NR En bloc excision 2yr DF

Townsend 45F HA visual changes Frontal sinus with extension

into orbit ethmoids and

dural abutment

Bony erosion

T1hypointense

contrast enhancing T2

heterogeneous

Osteoplastic frontal

sinus flap

6mo DF

Veras22 60F Incidental finding Nasal septum Sclerotic and scalloped

edges on CT lobulated

mass

Excision 12mo DF

Vernon23 44M Retroorbital pain Sphenoid sinus with

nasopharynx extension

Bony erosion on CT T2

heterogeneity

Endoscopic removal

with ethmoidectomy

NR

Wang24 60F Incidental finding Nasal septum with ethmoid

extension

Bony erosion on CT Excision 6mo DF

Wolf25 35F Frontal HA Frontal-sphenoid junction

with orbital infiltration

Expansion into orbital

cavity

Craniotomy with

piecemeal removal

NR

Yoo26 2moM Dyspnea nasal

cavity mass

Nasal cavity inferior

turbinate

T1 enhancing T2

heterogeneous

Combination of

debridement and bloc

resection

2yr DF

Yahgi35 38F NAO serous

rhinorrhea

Clivus with extension into the

sphenoid

Expansile lesion on CT Transmaxillary

excision incomplete

excision

Persistent

tumor re-

excised and DF

at 45yr

Twenty eight cases were identified including 6

in patients less than 18 years old Table 1 A

review by McClurg et al identified 20 of these

cases There were several reports of dural

involvement orbital infiltration and erosion of

bone into the cavernous sinus The nasal

septum and ethmoid sinuses were the most

frequently reported origin Presentation

commonly included nasal airway obstruction

visual disturbances and headache On CT

CMF appear as a soft tissue density with

frequent expansion of or frank erosion of bone

Gross calcifications are seen roughly 15 of the

time MRI features include low signal intensity

on T1 images contrast enhancement and

heterogeneous enhancement on T2 sequences

similar to other cartilaginous tumors Histologic

features include well circumscribed lobulated

tumors with both myxoid and chrondroid

elements Mitosis are rare and areas of necrosis

are infrequently seen but occasional mild to

moderate nuclear atypia is seen and can

contribute to the difficulty in correct diagnosis

Treatment is primarily surgical Tumor

recurrence was most common after curettage

resection and re-excision or external beam

radiation were subsequently used for control A

small 1-2 rate of malignant transformation of

CMF after radiation was reported

Table 1

HL Jaffe L Lichtenstein Chondromyxoid fibroma of bone distinctive benign tumor likely to be mistaken especially for cho1 ndrosarcoma Arch Pathol 45 (1948) 541ndash551

Hammad HM Hammond HL Kurago ZB Frank JA Chondromyxoid fibroma of the jaws Case report and review of the literature Ora2 l Surg Oral Med Oral Pathol Oral Radiol

Endod 1998 Mar85(3)293-300

McClurg S Leon M Teknos T Iwenofu H Chondromyxoid fibroma of the nasal septum Case report and review of literature 3 Head Neck 2013 Jan35(1)E1-5

Azor4 ın A Gil G Saacutenchez-Aniceto C Ballestın F-J Martınez-Tello Chondromyxoid fibroma of the frontal sinus British Journal of Oral and Maxillofacial Surgery Volume 41 Issue

6 December 2003 418-420

Ostrowski ML Spjut HJ Bridge JA 5 2002Chondromyxoid fibromas In Fletcher CDM Unni KK Mertens F editors World Health Organization Classification of Tumors Pathology

and genetics of tumours of soft tissue and bone Lyon IARC Press 2002 p 243ndash245

Kadom N Rushin EJ Yuan A Santi M Chondromyxoid fibroma of the frontal bone in a teenager 6 Pediatr Radiol 2009 Jan39(1)53-6

Baujat B Attal P Racy E et al Chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinus7 es report of one case and a review of the literature

Am J Otol 200122150ndash153

Castle J Kernig M Chondromyxoid fibroma of the ethmoid sinus Head Neck Pathol 8 2011 2011 Sep5(3)261-4

Cruz AA Mesquita IM Becker AN Chahud F Orbital invasion by chondromyxoid fibroma of the ethmoid sinus Ophthal Plast Reco9 nstr Surg 200723427ndash428

Frank E Deruaz JP de Tribolet N Chondromyxoid fibroma of the petrous10 -sphenoid junction Surg Neurol 198727182ndash186

Hashimoto M Izumi J Sakuma I Iwama T Watarai J Chondromyxoid fibroma of the ethmoid sinus Neuroradiology 11 1998 40577ndash579

Isenberg SF Endoscopic removal of chondromyxoid fibroma of the ethmoid sinus Am J Otolaryngol 12 199516205ndash208

Januszek G Niemczyk K Gornicka B Gotlib T Chondromyxoid fibroma of the nasal septum Otolaryngol Pol 13 2010 6488ndash92

Keel SB14 Bhan AK Liebsch NJ Rosenberg AE Chondromyxoid fibroma of the skull base a tumor which may be confused with chordoma and chondrosarcoma A report of three

cases and review of the literature The American Journal of Surgical Pathology Volume 21(5) May 1997 pp 577-582

Koay CB Freeland AP Athanasou NA Chondromyxoid fibroma of the nasal bone with extension in the frontal and ethmoid sinuses15 J Laryngol Otol 1995109258ndash261

Mendoza M Gonzalez I Aperribay M Hermosa JR Nogues A Congenital chondromyxoid fibroma of the ethmoid case report Pedia16 tr Radiol 199828339ndash341

Morris LG Rihani J Lebowitz RA Wang BY Chondromyxoid fibroma of sphenoid sinus with unusual calcifications case report w17 ith literature review Head Neck Pathol

20093169ndash173

Nazeer T Ro JY Varma DG de la Hermosa JR Ayala AG 18 Chondromyxoid fibroma of paranasal sinuses report of two cases presenting with nasal obstruction Skeletal Radiol

1996 25 (8) 779-782

Perez19 -Fernandez CA Armengot-Carceller M Lozano de Arnilla CG Valles AP Basterra-Alegria J Chondromyxoid fibroma of left maxillary and ethmoid sinuses Acta

Otorrinolaringol Esp 20096070ndash72

Smith CA20 Magenis RE Himoe E Smith C Mansoor A Chondromyxoid fibroma of the nasal cavity with an interstitial insertion between chromosomes 6 and 19 Cancer Genet

Cytogenet 2006 Dec171(2)97-100

Thomas B21 Black C Maddox T Venkatraman G Chondromyxoid fibroma of the nasal cavity and palate Ear Nose Throat J 2011 Oct90(10)E17-9

Veras EFT Santamaria IB Luna MA Sinonasal chondromyxoid fibroma Ann Diagn Pathol 22 20091341ndash46

Vernon SE Casiano RR Sphenoid sinus chondromyxoid fibroma mimicking a mucocele Am J Otolaryngol 23 200627406ndash408

Wang C Morrow T Friedman P Lara JF Chondromyxoid fibroma of the nasal septum a case report emphasizing clinical correlat24 ion Am J Rhinol 20001445ndash49

Wolf DA Chaljub G Maggio W Gelman BB Intracranial chondromyxoid fibroma Report of a case and review of the literature A25 rch Pathol Lab Med 1997121626ndash630

Yoo YT26 1 Park JH Sunwoo WS Rhee CS A huge chondromyxoid fibroma of the nasal cavity in a newborn baby Auris Nasus Larynx 2012 Aug39(4)422-4

Wu CT Inwards CY O27 rsquoLaughlin S Rock MG Beabout JW Unni KK Chondromyxoid fibroma of bone A clinicopathologic review of 278 cases Hum Pathol 199829438ndash446

Sreedharanunni S Rajwanshi A Vaiphei K Gupta N Bansal S Fine needle aspiration cytology in two cases of chondromyxoid f28 ibroma of bone and review of the literature

Diagnostic Cytopathology Vol 41 No 10 904-908

De Las Casas LE29 Singh HK Halliday BE Xu F Strausbauch PH Silverman JF Myxoid chondrosarcoma of the sphenoid sinus and chondromyxoid fibroma of the iliac bone

cytomorphologic findings of two distinct and uncommon myxoid lesions Diagn Cytopathol 2000 Jun22(6)383-9

Nasir30 -Ud-Din Ahmed A Pervez S Ahmed R Kayani N Chondroblastoma a clinico-pathological analysis J Coll Physicians Surg Pak 2014 Dec24(12)898-901

Shek TW Peh WC Leung G Chondromyxoid fibroma of skull base a tumour prone to local recurrence J Laryngol Otol 31 1999 113380ndash385

Zillmer DA Dortman HD Chondromyxoid fibroma of the bone 32 36 cases with clinicopathologic correlation Hum Pathol 1989 20 932ndash964

Haroon S33 Nasir-Uddin Pervez S Kayani N Ahmed R Hafeez K Umer M Chondromyxoid fibroma experience of 36 cases of an intriguing entity J Pak Med Assoc 2014

Dec64(12 Suppl 2)S175-9

Hersh DS Firempong AO Chesler D Castellani RJ Woodworth GF Chondromyxoid fibroma invasion of the transverse34 -sigmoid sinus junction causing posterior fossa hemorrhage

Journal of Clinical Neuroscience Volume 24 February 2016 Pages 149-150

Yaghi N DeMonte F Chondromyxoid Fibroma of the Skull Base and Calvarium Surgical Management and Literature Review J N35 eurol Surg Rep 2016 Mar 77(1) 023ndash034

Shek TW Peh WC Leung G 36 Chondromyxoid fibroma of skull base a tumour prone to local recurrence J Laryngol Otol 1999 113 (4) 380-385