Elsevier

World Neurosurgery

Volume 125, May 2019, Pages e843-e848
World Neurosurgery

Original Article
Skull Base Juvenile Psammomatoid Ossifying Fibroma: Clinical Characteristics, Treatment, and Prognosis

https://doi.org/10.1016/j.wneu.2019.01.197Get rights and content

Objective

The diagnosis and management for juvenile psammomatoid ossifying fibroma (JPOF) of the skull base are challenging, and clinical data are limited.

Methods

A retrospective review of JPOF was performed, and the clinical characteristics, treatment strategy, and prognosis were analyzed.

Results

There were 23 patients pathologically confirmed with JPOF, most with JPOF located in the skull base area (19/23, 82.6%). Of those tumors, 43.5% presented with dura matter breakthrough. Most of the chief complaints were headache (n = 8, 34.8%) and visual impairment (n = 5, 21.7%). Most of the tumors were solid tumors with spherical appearance, frequently accompanied by cysts of various size (n = 14, 60.9%). Craniotomy, mostly via the frontal approach, was the most common approach in the present series, comprising 73.6% (17/23) of all cases. The endoscopic endonasal approach was performed in 6 cases (26.1%). In total, 62.5% of patients (15/23) underwent gross total resection, 8.7% of patients (2/23) underwent subtotal resection, and 26.1% of patients (6/23) underwent partial resection. After a mean follow up of 66.1 ± 36.1 months (range, 3–124), 3 patients (13.6%) suffered from tumor recurrence with a mean recurrence time of 13 months.

Conclusions

The present series of skull base JPOFs showed that radical surgery combined with skull base reconstruction contributed to overall good prognosis. Further studies are needed to evaluate the long-term outcomes and to characterize its pathologic characteristics.

Introduction

Ossifying fibroma (OF) is a benign tumor characterized by the replacement of normal bone by a fibrous cellular stroma containing varying amounts of the foci of mineralization or ossification.1, 2 It mainly located in the mandibular and maxillary bones, with a female predominance. Three types of OFs have been classified according to histopathologic characteristics, clinical behaviors, and image features: cemento-ossifying fibroma (COF), juvenile psammomatoid ossifying fibroma (JPOF), and juvenile trabecular ossifying fibroma (JTOF).1, 3 JPOF features aggressive infiltration and the potential for recurrence. Unlike the other 2 types of OF, JPOF is the dominant type that is located in the extragnathic craniofacial bones, leading to a relatively long latent clinical period and larger tumor size; otherwise, the tumor is diagnosed by symptoms of cranial nerve deficiencies.2, 4

Because of its rarity, most of the clinical data pertaining to JPOF are from case reports or small case series. The clinical characteristics, treatment strategy, and prognosis of skull base JPOF have been poorly elucidated. This study includes a series of 23 cases from a single institute, aiming to illustrate the clinical pathologic behavior of JPOF and its clinical diagnosis, treatment strategies, and outcomes.

Section snippets

Materials and Methods

A retrospective study of JPOF was performed. From January 2008 to March 2018, 23 cases of patients with JPOF were surgically treated in our department of neurosurgery. All patients underwent radiologic evaluations, including magnetic resonance–enhanced images, computed tomography (CT) bone window images, or both, prior to surgery. The surgical approach was individually tailored according to the location of the tumor and the erosion of the bone. Occasionally, neurophysiologic monitoring was used

Results

All patients were surgically treated and pathologically confirmed. The male-to-female rate was 1.56 (14/9), with a mean age of 30.5 ± 15.3 years (range, 3–57 years). There were 3 recurrent JPOF cases enrolled, all of whom received surgical treatment before referral to our department. Most of the chief complaints were headache (n = 8, 34.8%), followed by visual impairment (n = 5, 21.7%) and anosmia (n = 2, 8.7%). Notably, there were 4 patients with no symptoms who had been diagnosed incidentally

Discussion

JPOF, as officially named in 2005 by the World Health Organization, is characterized by the presence of multiple round, uniform small masses of osteoid with calcifications in a concentric pattern, imparting a psammoma body-like appearance.1, 2 The descriptive psammomatoid characteristic differentiates JPOF from JTOF and conventional OF.2 Studies have shown that JPOFs are seen in children and young adults, with the mean age of 16–33 years of age and with a slight male predominance.3, 5 In a

Conclusions

JPOF is rare and is often located in the sinonasal area with involvement of the anterior skull base. The most common chief complaints are headache and visual impairment. Skull base JPOF is characterized by its ground-glass opacity on CT scan and cystic formation, in most cases. The present series of skull base JPOFs, together with those reported in the literatures, has shown that radical surgery with skull base reconstruction contributed to overall good prognosis. Further studies are needed to

Acknowledgments

The authors thank the study subjects for participating in the research and all those in Beijing Tian Tan Hospital who contributed to the study.

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Conflict of interest statement: This work was supported in part by the Natural Science Foundation of China (814742370 to Dr. J-T. Zhang and 81672506 to Dr. Wu) and the Special Fund for The Talents of Beijing (2016000021469G212 to Dr. Wang).

Ke Wang and Xiu-Jian Ma are co–first authors.

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