Volume 6, Issue 3 , Pages 153-155, September 2011
Primary paranasal sinus meningioma of a 1.7-year-old boy
Article Outline
Abstract
A 1.7-year-old boy was admitted to our hospital with the first impression of ossified fibroma in the right ethmoid sinus. However, histopathological examination of the surgical specimen turned out to be meningioma. The mass was completely removed endoscopically assisted by image-guided navigation system. This is the youngest ectopic meningioma case reported. The clinic figure and treatment of primary paranasal sinus meningioma is briefly discussed. Considering the young age and small blood volume of this patient, careful preoperative preparation and the cooperation of multi disciplines were of vital importance.
Keywords: Ectopic meningioma, Paranasal sinus neoplasm, Endoscope, Child
1. Introduction
Meningioma of the paranasal sinus is an uncommon tumor. The first description of paranasal sinus meningioma was by Shaheen [1] in 1931. Here we report a 1.7-year-old boy with a primary ectopic meningioma in the right ethmoid sinus. This is the youngest patient of all reported primary meningioma cases of the sinonasal tract [2], [3], [4] ranging from 4 to 88 years old.
Treatment of paranasal sinus meningiomas is similar to other tumors originating in the sinuses and is, most commonly, surgical [5]. The surgical approach is chosen depending on the location, the sinus involved and the extensions of the tumor. Frontal craniotomy, rhinotomy and endoscopic surgery are commonly used. However, the surgery of paranasal sinus meningioma for young children was rarely reported in the literature. This report presents a case that the tumor was successfully removed endoscopically assisted by image-guided navigation system.
2. Case report
A 1.7-year-old boy was admitted to our hospital with a history of persistent nasal obstruction and intermittent epistaxis of the right side for 5 months. Decongestant treatment for rhinitis failed.
On endoscopic examination, there was a pink, hard mass with smooth surface occupying the right nasal cavity. The mass extended to the concha and septum occluding deep structures.
CT scan showed an oval mass lesion of 3.79
cm×2.22cm with a complete bony boundary and mixed density in the right ethmoid sinus (Fig. 1). The mass expanded into the right orbital area, left ethmoid sinus and both nasal cavity. The boundary between the lesion and right lamina papyracea was obscure. The mass was first considered as ossified fibroma.
Fig. 1.
(A) axial CT and (B) coronal preoperative CT image.
Image-guided endoscopic nasal surgery was performed. The mass filling the right ethmoid sinus and fusing the middle turbinate was completely removed endoscopically with its capsule. In order to dissect the tumor thoroughly, it is quite important to identify and cut along the edge. The image-guided navigation system allowed for the identification of the true edge of the tumor as well as adjacent vital structures including lamina papyracea, cribriform plate etc. during the procedure. The posterior one-third of the lamina papyracea fusing with the mass was drilled with diamond bur and then dissected. The periorbital fascia was intact. The biopsy was cellular, spongy bone like lesion with savage bleeding. Three nutrient arteries were found, followed, and identified as main branches of ethmoid artery. About 1500ml bleeding was replenished with 800ml blood transfusion. The middle concha was retracted laterally to support the orbit. Considering the difficulty of redressing for such a young child, bio-degradable fragmentable foam, Nasopore, was dressed.
Histopathological examination of the surgical specimen showed meningioma. Microscopically, the tumor was proliferative diffusely in propria lamina under the lamina epithelialis, which was composed of a mixed population of immature bone trabecula surrounded by abundant polygonal to spindled tumor cells (Fig. 2). Tumor exhibited a meningotheliomatous pattern (Fig. 3), characterized by lobules of cell with whorl formation, indistinct cell borders, and bland nuclei with delicate chromatin. The nuclei were ovoid, with uniform size and shape. Nuclear chromatin was bland with inconspicuous nucleoli. Typical intranuclear pseudoinclusions and psammoma body was absent. Immunohistochemically, the tumor cells expressed vimentin (Fig. 4), and EMA (Fig. 5).
Fig. 2.
The tumor was composed of a mixed population of immature bone trabecula surrounded by abundant polygonal to spindled tumor cells (HE 100×).
Fig. 3.
The tumor exhibited a meningotheliomatous pattern (HE 400×).
Fig. 4.
Immunohistochemical staining for vimentin was positive (IHC 400×).
Fig. 5.
Immunohistochemical staining for EMA was positive (IHC 200×).
The patient was transferred to IUC for 1 day postoperatively. The tampon was removed 2 days after the operation. The postoperative course was uneventful. Before the patient was discharged on the 4th postoperative day, another CT scan was performed. The mass was completely removed (Fig. 6).
Fig. 6.
Postoperative CT scan.
At the first follow up 6 months after the surgery, no sign of recurrence or inflammation was found.
3. Discussion
Although increasing evidence supports the development of ectopic meningiomas from ectopic arachnoid cell clusters, the age of primary sinonasal meningiomas averaged 42.4 years [2]. The reluctance of performing CT examination on young children and slow development of the tumor might partially explain the discrepancy.
Daneshi et al. [6] described three criteria to differentiate paranasal sinus meningiomas from metastatic meningioma or those extending from intracranial tumors: (1) the bony wall of the sinus is intact on radiological imaging or on inspection during surgery. (2) The absence of a simultaneous intracranial meningioma on imaging or at surgery. (3) Expansion of the sinus wall towards the cranium rather than to the opposite direction. The present case met all these criteria.
The radiographic result of ectopic meningioma was less specific, but nasal cavity or paranasal sinus opacification by a mass lesion, accompanied by bony erosion and sclerosis or hyperostosis [7], [8]. The tumor might expand into the base of the skull and/or the orbit. If contrast-enhanced CT scan were performed, there would be intense enhancement of meningiomas in consistent with the abundant blood supply. In the present case, because of the egg shell like expression of the neoplasm (Fig. 1), the preoperative CT is very confusing with osteofibroma. The differential diagnoses of paranasal sinus meningiomas include ossifying fibroma, osteofibrous dysplasia, sinus mucocoele or mucopyelocoele, follicular dendritic cell tumor/sarcoma, osteoblasoma and low-grade osteosarcoma [3], [4]. The diagnose can only be made upon pathology examination.
Like their intracranial counterparts, sinonasal tract meningiomas may exhibit a variety of different histologic patterns [2]. However, most cases were typical meningotheliomatous meningiomas composed of lobules of cells with indistinct borders and possessing generally bland nuclei with delicate chromatin. The immunohistochemical profile of sinonasal tract meningiomas was indistinguishable from intracranial lesions, with all tumors tested expressing epithelial membrane antigen and vimentin immunoreactivity.
The aim of the surgery is total excision. Where this is not possible and for recurrent tumors, radiotherapy can be considered. In the present case, the image-guided navigation system helped to dissect the tumor thoroughly and safely. The image-guided navigation system assisted the identification of the true edge of the tumor as well as adjacent structures including lamina papyracea, cribriform plate etc. As for young patients, thoughtful preoperative preparation and cooperation with multi disciplines were of vital importance. In this case, 800ml packed red blood cell was prepared considering the risk of life threatening blood loss. Pediatric, anesthetic, and ICU specialties were consulted preoperatively. While the difficulty of endoscopic redressing for such a small patient must also be considered, a bio-degradable fragmentable foam, Nasopore, was dressed.
In general, the prognosis of primary meningioma of the sinonasal tract appears to be excellent [9]. There was little difference between the 5-year and 10-year disease-free survival rates, indicating that once the patients survived disease-free for 5 years, they were unlikely to die with tumor [2]. When recurrences developed, they usually arise in the same anatomic site as the primary lesion and probably represent residual disease rather than recurrent tumor. Additional surgery, if clinically feasible, is advisable, because radiation therapy does not always result in a clinical response [10]. If death with tumor does result, it is usually the result of involvement of the vital structures of the midfacial region or complications of the surgery rather than the aggressive nature of the tumor.
4. Conclusion
Although rare, primary paranasal sinus meningioma does occur in very young children. The symptom, sign and CT examination are not specific comparing with other paranasal mass. The diagnose can only be made based on pathology result. Because of the benign behavior of the tumor, the treatment may be difficult but worthy. Thoughtful preoperative preparation is of vital importance especially for the very young children.
References
- . Psammoma in the maxillary antrum. J. Laryngol. Otol. 1931;46:117
- . Primary extracranial meningiomas: an analysis of 146 cases. Head Neck Pathol. 2009;3:116–130
- . Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature. Am. J. Surg. Pathol. 2000;24:640–650
- . Primary meningioma of the paranasal sinus. J. Clin. Neurosci. 2005;12:832–834
- . Ectopic meningioma: a case report with a literature review. Eur. Arch. Otorhinolaryngol. 2006;263:426–429
- . Primary meningioma of the ethmoid sinus: a case report. ENT J. 2003;82:310–311
- . Clinical–radiological aspects of primary extracranial meningioma of the ethmoid sinus in a child. Arq. Neuropsiquiatr. 2008;66:274–275
- . Primary extracranial meningioma of the sinonasal tract. Acta Radiol. 2005;46:415–418
- . Primary meningioma of the nasal cavity and paranasal sinuses. Cancer. 1980;46:1442–1447
- . Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. XIII. Meningiomas. Cancer. 1984;54:1860–1869
PII: S1871-4048(10)00048-1
doi:10.1016/j.pedex.2010.06.003
© 2010 Published by Elsevier Inc.
Volume 6, Issue 3 , Pages 153-155, September 2011
