Volume 6, Issue 3 , Pages 143-145, September 2011
Aggressive psammomatoid ossifying fibroma in a 3-month-old boy—A case report
Article Outline
Abstract
Aggressive psammomatoid ossifying fibroma (APOFs) is a benign mesenchymal tumor of the bone with a large growth potential, occurring usually in young adolescents and children. In this case report we are presenting a 3-month-old boy who was diagnosed with an aggressive psammomatoid ossifying fibroma of the nose and paranasal sinuses. In this age early diagnosis is difficult and may results in misdiagnosis such as chronic rhinitis. Endoscopic approach was chosen to remove the tumor. This case report demonstrates that complete endoscopic removal of large tumor involving nasal cavity and paranasal sinuses is effective, though technically challenging.
Keywords: Aggressive psammomatoid ossifying fibroma, Juvenile ossifying fibroma, Paranasal sinuses
1. Introduction
Ossifying fibroma is a benign neoplasm of the bone with a large growth potential. Locally it may destroy the bone and is capable to recur. One of its forms, described in children and adolescents, is aggressive (juvenile) ossifying fibroma. Most commonly it occurs in children younger than 15 years old and involves paranasal sinuses and orbit. In this case report we are presenting a 3-month-old boy who was diagnosed with an aggressive psammomatoid ossyfing fibroma of the nose and paranasal sinuses.
2. Case report
A 3-month-old boy was admitted to our hospital due to nasal obstruction, sleep apnea episodes and, periodically, nasal bleedings on the left side. Disturbed nasal breathing was observed from the first month of life and intensified since that time. Left side nasal bleedings started in the second month of life and were originally attributed to the mechanical cleaning of the nasal cavities. The child was treated by a pediatrician for a persistently running nose. Pediatrician did not observe any improvement and referred the boy to the otorhinolaryngologist for a consultation in his third month of life.
The specialist examination showed broad root of the nose, left buccal bulging and slight protrusion of the left eye-ball with its limited medial mobility. Left nasal cavity was totally fulfilled with a grayish solid tumor, displacing the nasal septum to the right. Right nasal cavity with a slit-like lumen did not let the thinnest fiberscope through. Computed tomography scanning revealed an abnormal, non-homogenous mass with calcifications, intensifying with contrast administration (Fig. 1) The size of the tumor was 39
mm×26mm×26mm. Anatomically, the mass of the tumor was extending from the level of hard palate up to the anterior skull base. The tumor produced massive bony deformation with destruction of the ethmoidal cells on the left side. The anterior skull base seemed to be missing in the area of 25mm×12mm, but the tumor did not enter cranial cavity through the bony dehiscence. The meninges seemed to be unchanged. Left medial orbital wall was distorted and displaced laterally, markedly decreasing retrobulbar space on this side while nasal septum and right ethmoidal cells were displaced to the right. Both eye-balls and orbital nerves were unchanged in CT scans. Orbital rectus medial muscle was slightly molding in the distorted left orbit.
Fig. 1.
Preoperative CT scan of the tumor.
The child was consulted by a neurologist and oncologist. The neurological examination revealed no changes. Recommended by oncologist, levels of embryoma, neuroblastoma and rhabdomyosarcoma markers were negative. A biopsy for preliminary histopathological examination showed ossifying juvenile fibroma in the specimen. Magnetic resonance imaging was performed in order to examine the skull base thoroughly and exclude any connection between the tumor and the CNS (Fig. 2). No bony dehiscence of the skull base, nor junction with meninges and brain were found.
Fig. 2.
Preoperative MRI of the tumor.
The child was qualified for a total excision of the tumor. Nasal endoscopic techniques were applied with a set of pediatric telescopes 0, 30 and 70 degree (2.7mm) and instrument set for endoscopic surgery of the paranasal sinuses. On removal, it appeared that the mass was originating in the roof of the nose and left ethmoid. The removed tumor was friable, well vascularized, with necrotic lesions and had an irregular surface. Intraoperative reexamination of the postoperative cavity revealed no residuals. It showed total destruction of the lateral nasal wall with opened left maxillary sinus, destruction of the ethmoid and partial destruction of the vomer. The bony anterior skull base was very thin. No signs of CSF leak were observed.
Postoperative period was uneventful and the child was discharged from hospital in 7 days. The final histopathology result was psammomatoid juvenile ossifying fibroma (Fig. 3, Fig. 4).
Fig. 3.
Ossifying fibroma. Giant cells and irregular callus formation among the fusiform cells of the fibromatous stroma.
Fig. 4.
Ossifying fibroma. Callus formation with mineralization.
Postoperative follow-up was carried out in 1, 3, 6 and 12 months. The child's development was normal, with regular weight gain and remission of the nasal obstruction symptoms. In the examination the child's face was growing symmetrically, no exophthalmos was observed, the eye-ball mobility was undisturbed, and the nasal septum was dislocated to the right without influencing nasal breathing. Computed tomography with contrast was performed during the follow-up, 12 months postoperatively (Fig. 5). The scans showed no recurrence in the left nasal cavity, with no pathologic enhancement in the area of the nasopharynx and paranasal sinuses. Nasal fiberscopic examination proved sufficient patency and complete removal of the tumor.
Fig. 5.
Postoperative CT scan—12 month later.
3. Discussion
Aggressive psammomatoid ossifying fibroma (APOFs) is a benign mesenchymal tumor, occurring usually in young adolescents and children, and very rarely in elderly patients [2]. Fibroosseous lesions are uncommon changes among the diseases of the nose and paranasal sinuses and their terminology and classification is still disputed [1], [5]. In medical literature, the term “juvenile ossifying fibroma” is being used for two microscopically distinct fibroosseous lesions of the facial skeleton. Some of them are characterized by the presence of small uniform spherical ossicles imitating psammoma bodies (psammomatoid juvenile ossifying fibroma). The other ones are differentiated by trabeculae of fibrillary osteoid and woven bone (trabecular juvenile ossifying fibroma) [3], [7]. Psammomatoid juvenile ossifying fibroma is diagnosed more frequently than trabecular juvenile ossifying fibroma. Clinically it has been observed that psammomatoid ossifying fibroma usually affects nasal bones, bony skeleton of the paranasal sinuses and of the orbit, while trabecular juvenile ossifying fibroma predominates in the mandible. Both of them are histologically benign, but in some cases locally aggressive growth was observed [4], [6]. Such a possibility is described in the presented case report, where due to expanding growth of the tumor, destruction of the lateral nasal wall and ethmoid with dislocation of the orbital medial wall occurred. Intraoperatively, thinning of the anterior skull base was observed. These changes underline locally invasive character of the tumor, with impending ophthalmological and neurological complications. The size of the tumor in such a young child, in the area hardly accessible for complete surgical excision, is one of the reasons to present this case report.
Diagnostic difficulties in the course of the disease should also be emphasized. Persistently running nose, inflammatory changes in the paranasal sinuses and nasal obstruction are alarming symptoms, particularly in infants and should not be neglected. Nasal fiberoscopy was impossible to conduct preoperatively, due to the total obstruction of the nasal cavity by the tumor and nasal septum displacement to the other side. This problem shows that complete rhinologic examination of the infant with a nasal mass may be challenging and it became one more reason that brought us to present this case. Radiological examination usually involves CT scanning and/or MRI. In the presented case CT examination was insufficient in the evaluation of the anterior skull base and did not answer the question whether the tumor expands into the cranial cavity. Thus, MRI was performed.
Endoscopic approach, with a pediatric instrument set for endoscopic surgery of the paranasal sinuses, was chosen to remove the tumor. In the available literature many procedures of removal are being described but none refers to endoscopic excision of the psammomatoid juvenile ossifying fibroma involving nasal cavity, ethmoid and maxillary sinuses in a 3-month-old child. Endoscopic excision as a method of treatment may not always be possible and depends on tumor localization and expansion [1]. Incomplete removal of the tumor leads to recurrence. In the presented case both fiberoscopic and radiologic postoperative examination, with the absence of any symptoms, provide the evidence for radical endoscopic removal of the tumor.
Psammomatoid juvenile ossifying fibroma is a benign tumor described mainly in young people. The reported case proves that it may be diagnosed in infants as young as 3 months. In this age, early diagnostics is difficult and may result in misdiagnosis such as chronic rhinitis. This case report demonstrates that complete endoscopic removal of large tumors involving nasal cavities and paranasal sinuses is effective, though technically challenging.
References
- . Benign fibro-osseous lesions: a review of current concepts. Adv. Anat. Pathol. 2001;8(3):126–143
- . Aggressive psammomatoid ossyfing fibroma of the interior turbinate and lateral nasal wall. Acta Otorhinolaryngol. Belg. 2003;57(1):87–89
- . Psammomatoid and trabecular juvenile ossyfing fibroma of the craniofacial skeleton: two distinct clinicopathologic entities. Oral Surg. Oral Med. Oral Pathol. Oral Radio Endod. 2002;93(3):296–304
- . An aggressive psammomatoid ossyfing fibroma of the sinonasal tract: report of a case. Ear Nose Throat J. 2007;86(7):400–401
- . Atypical fibro-osseous lesions: diagnostic challenges and treatment concepts. Int. J. Oral Maxillofac. Surg. 1995;24(2):162–169
- . Psammomatoid ossifying fibroma with secondary aneurysmal bone cyst of frontal sinus. Childs Nerv. Syst. 2009;25:1513–1516
- . Psammomatoid and trabecular variants of juvenile ossifying fibroma—two case reports. Indian J. Radiol. Imaging. 2009;19(2):116–119
PII: S1871-4048(10)00045-6
doi:10.1016/j.pedex.2010.05.011
© 2010 Elsevier Ireland Ltd. All rights reserved.
Volume 6, Issue 3 , Pages 143-145, September 2011
