Fibromyxoma is a rare benign tumour of mesenchymal origin that mostly involves the posterior part of the mandible. It is a locally aggressive and slowly growing painless tumour that mostly occurs in second and third decades of life. We report a case of 2 years old child with huge mass of the right maxilla. After proper diagnosis mass was completely excised via sublabial approach and reported histopathologically as fibromyxoma. Because of its rarity in the maxilla and in this age, it is being reported here

Agarwal, S P

Kumar, Sunil

Singh, Hitendra Prakash

Verma, Veerendra

English

Atharva Scientific Publications (E-Jounals)

Kumar et al                                              Pediatric Fibromyxoma Maxilla 
Vol 2 Issue 1 Jan – Mar 2016                                                           Indian J Case Reports / 1 
Case Report 
Pediatric Fibromyxoma Maxilla – A rare entity 
Sunil Kumar, Hitendra Prakash Singh, Veerendra Verma, S P Agarwal 
Department of ENT & Head Neck Surgery, King George’s Medical University, Lucknow, Uttar Pradesh, India 
Correspondence to:  Dr Sunil Kumar, Assistant Professor, Department of ENT & Head Neck Surgery, King George’s 
Medical University, Lucknow, Uttar Pradesh, India. Email: drsunil_kumar123@rediffmail.com. 
Received: 11 December 2015    Initial Review: 04 February 2016    Accepted: 28 February 2016    Published Online: 05 March 2016 
ABSTRACT 
Fibromyxoma is a rare benign tumour of mesenchymal origin that mostly involves the posterior part of the mandible. It is a 
locally aggressive and slowly growing painless tumour that mostly occurs in second and third decades of life. We report a case 
of 2 years old child with huge mass of the right maxilla. After proper diagnosis mass was completely excised via sublabial 
approach and reported histopathologically as fibromyxoma. Because of its rarity in the maxilla and in this age, it is being 
reported here. 
KEYWORDS: Odontogenic tumors; Fibromyxoma; Maxilla 
ibromyxoma is a rare slowly growing benign 
tumour, which usually occurs in the 2nd and 3rd 
decades of life. It is rarely seen in children or adults 
above 50 years of age [1]. Average age of its presentation 
is 31 years [2]. Fibromyxoma usually occurs in tooth 
bearing area; thus, is supposed to be arising from the 
mesenchymal portion of a tooth germ most likely of the 
dental papilla [3]. It has the tendency of bony destruction 
and extension into the surrounding structures. Main 
symptom of fibromyxoma is swelling and problems due to 
displacement of the surrounding structures [4]. Surgical 
excision is the mainstay of the treatment. We report this 
case due to the unusual presentation of this rare tumour in 
this age involving maxilla.   
CASE REPORT: 
A 2 year old male child was presented to us in our 
outpatient department as swelling over right side face for 8 
months. Swelling was slowly progressive in size since it 
was first noticed (Fig 1). On examination, there was firm 
swelling with marked facial disfigurement of right side 
face. Swelling was non-tender, non-fluctuant and non-
pulsatile. Right side proptosis with superior and lateral 
deviation without any visual impairment was present. 
Anterior rhinoscopy revealed obliterated right nasal cavity 
with marked septal deviation towards left side. On oral 
cavity and oropharynx examination, there was ipsilateral 
palatal bulge with breach in the mucosa without loosening 
of teeth. There was no numbness and altered sensations 
over face or oral mucosa. There was no cervical 
lymphadenopathy. Rest of the systemic examination was 
normal. 
On investigations, complete blood count and urine 
examination was normal. Computed Tomography (CT) 
scan of para-nasal sinuses revealed a large expansile lesion 
in right maxillary sinus with gross thinning of the bony 
walls and superior compression over the right orbit. There 
was hypoplastic frontal sinus with mucosal thickening in 
the ethmoid, maxillary and sphenoid sinuses more on right 
sides (Fig 2, 3). Previous biopsy from the mass was 
fibroma.  
Child was planned for complete excision of mass under 
general anaesthesia. After complete pre-anaesthetic work-
up, the tumour was approached by means of sublabial 
route with complete enucleation and curettage (Fig 4). 
F 
Kumar et al                                              Pediatric Fibromyxoma Maxilla 
Vol 2 Issue 1 Jan – Mar 2016                                                           Indian J Case Reports / 2 
Grossly, the tumour was solid (9cm×5cm×5cm) in 
consistency (Fig 5). Post-operative period was uneventful. 
Histopathological examination revealed fascicles of benign 
spindle cells with myxoid area with well-defined capsules 
suggestive of fibromyxoma (Fig 6). There was no 
recurrence after two year of follow up. 
 
Fig 1: showing swelling over right side face. Fig 2: CT scan axial section showing large expansile lesion in the right 
maxillery sinus. Fig 3: CT scan coronal section showing large expansile lesion  causing gross compression over the right 
orbit and nasal cavity. 
 
Fig 4: intra-operative photograph. Fig 5: resected specimen. Fig 6: Fascicles of benign spindle cells with myxoid area (H 
& E 100X) 
DISCUSSION 
The term myxoma was first used by Virchow in 1863, but 
the term fibromyxoma was described by Marcove et al in 
1964 who reported extragnathic locations of fibromyxoma. 
In African countries, odontogenic myxoma is the second 
most common tumour after ameloblastoma (frequency of 
occurrence 1-19%), while in Asia, Europe and America, 
frequency lies between 0.5-17%. Overall myxomas 
represent 2.3-17.7% of all odontogenic tumors with 
fibromyxomas comprising only a small number of all such 
tumours [5].  
Fibromyxoma is a rare odontogenic tumour with 
reported incidence rate as low as 0.07 per million 
population. Fibromyxomas are rare in children under 10 
years of age and average age of presentation of this tumour 
is about 31 years. The tumor is slow growing thus the 
average delay in presentation is about 3-5 years.  
It usually arises from the mesenchymal tissue of the 
dental papilla; hence, is also known as odontogenic 
myxoma. The myxoma of the jaw bones is a locally 
aggressive, non-metastasizing tumor. These are usually 
located intraorally most often in the posterior region of the 
mandible, its angle and ramus and rarely extra-orally [6]. 
The anterior region of the mandible and maxilla are rarely 
affected. It runs a benign course causing local symptoms 
leading to loosening of teeth and expansion of alveolus. In 
early stage the lesion often grows without symptoms; later, 
there may be marked bony expansion leading to gross 
facial disfigurement with or without proptosis. Paresthesia, 
Fig 4 Fig 5 Fig 6 
Fig 1 Fig 2 Fig 3 
Kumar et al                                              Pediatric Fibromyxoma Maxilla 
Vol 2 Issue 1 Jan – Mar 2016                                                           Indian J Case Reports / 3 
hyposthesia, anesthesia or pain is relatively uncommon in 
these patients. Patients with posteriorly located tumours 
usually present late and with bigger lesions than anteriorly 
located tumour probably due to more visible disfigurement 
and malalignment of teeth in lesions of anterior area. 
Fibromyxoma of the maxilla is more aggressive in nature 
than that of mandible as is grows through the maxillary 
sinus.  
On gross examination, the tumour appears as a smooth, 
glistening, gelatinous, lobulated mass with soft to firm 
consistency [7]. Microscopically, tumour consists of loose 
stellate cells with long anastomosing cytoplasmic 
processes [8]. It differs from odontogenic fibroma by 
absence of collagen fibrous tissue and lesser numbers of 
odontogenic epithelial rests. A fibromyxoma contains 
essentially both the features. The radiological examination 
is done mostly by Computed Tomography, which play an 
important role in the diagnosis of a fibromyxoma and in 
the differential diagnosis from other pathological 
conditions such as ameloblastoma, fibrous dysplasia, 
aneurysmal bone cyst, giant cell granuloma, intra-osseous 
hemangioma, simple cysts etc. The radiological features of 
fibromyxoma are variable and present as uni or 
multilocular radiolucency with honeycomb and soap 
bubble appearances [9]. The antral walls are usually 
expanded but rarely destroyed and tumour margins may 
differ with the true margins. Fibromyxoma is usually 
diagnosed on the basis of radiological as well as 
histopathological examination.  
These tumours are managed surgically and the choice 
of surgical approach and resection is guided by the 
location and extent of the tumour. Surgery varies from 
enucleation and curettage to complete resection with or 
without peripheral osteotomy. The 
myxomas/fibromyxomas show a recurrence rate up to 25% 
[10]. The absence of capsule and infiltration in 
surrounding tissue is the main cause of recurrence. 
Recurrence can be reduced with more rigorous treatment 
in the form of partial or complete segmental bone 
resection. Patient should be followed up at least for 2 years 
to detect any recurrence of the disease. 
 
 
 
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 How to cite this article: Kumar S, Singh HP, Verma V, 
Agarwal SP. Pediatric Fibromyxoma Maxilla – A rare 
entity. Indian J Case Reports. 2016; 2 (1): 1-3. 
Conflict of interest: None stated, Funding: Nil 


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