Juvenile psammomatoid ossifying fibroma of the posterior fossa: a case report and review
© The Author(s) 2016
Received: 6 March 2016
Accepted: 5 July 2016
Published: 15 July 2016
Ossifying fibroma is a rare benign bone tumor that occurs mostly in the jaw, but also affects paranasal sinuses and fronto-ethmoidal complex. Occipital bone is an extremely rare location for these tumors; only two cases have been reported.
We present the first case reported as psammomatoid subtype of ossifying fibroma, according to the 2005 WHO classification. An 18 years old male patient with infratentorial tumor, in the occipital bone, that produces mass effect over the cerebellum.
This case may provide a guide to consider these lesions for a more rapid and precise diagnostic in future cases.
Juvenile psammomatoid ossifying fibroma (JPOF) is a rare benign bone tumor that often affects facial bones, especially the mandible, it commonly occurs in young patients, and the treatment is indicated because of deformity and esthetical reasons mostly, en bloc resection is the treatment of choice. It is as subtype of juvenile ossifying fibroma in the WHO classification of odontogenic tumors (Barnes et al. 2005). JPOF of cranial bones are extremely rare, and, to our knowledge, only two cases affecting the occipital bone have been described; this is the first case reported as a psammomatoid subtype.
JPOF generally occurs in a young age (Johnson et al. 1991; Makek 1983; Margo et al. 1985) with a mean of 17.7 years (Makek 1983), the most affected site are the paranasal sinuses (61.6–70 %), where the commonest are the ethmoidal sinus, followed by maxilla (20 %) and mandible (7–10 %). It is infrequent in the calvaria (10–12 %) (Johnson et al. 1991; Makek 1983), affecting most commonly the parietal bone (4.6 %), temporal bone 3.5 % and frontal bone 3.4 %. (Makek 1983).
The main differential diagnosis is fibrous dysplasia. JPOF usually is monostotic and well demarcated lesion, lacks ground-glass attenuation, and contains areas of mineralization (Makek 1983; Smith and Zabaleta 1952; Wenig et al. 1995; Yamashita et al. 1977). In addition, fibrous dysplasia tends to be more elongated because it is a developmental abnormality rather than a neoplastic process (Chung et al. 2008; Johnson et al. 1991). Histologically, JPOF is a fibroosseous proliferation composed of fibrous stroma, admixed bony spicules, and the presence of psammomatoid ossicles. In contrast to fibrous dysplasia, osteoclasts and osteoblasts typically line the trabeculae, which are composed of entrapped lamellar bone.
Other differential diagnosis is to be made with conventional ossifying fibroma (COF) and with juvenile trabecular ossifying fibroma (JTOF). COF is an odontogenic neoplasm arising from the periodontal ligament and affecting the tooth bearing are as of the mandible, and maxilla, whereas the nasoethmoidal location is rare (Speight and Carlos 2006). COF is well-encapsulated and radiologically presents as an expansile soft-tissue-mass with sharp demarcation from adjacent bone. On the contrary, the juvenile variants, JPOF and JTOF are unencapsulated and arise outside of the tooth bearing areas, either in the maxilla or the craniofacial skeleton and affect a younger group of age where as the juvenile trabecular fibroma appears in patients below 15 years and both with slightly predilection to male patients (El-Mofty 2002). JPOF commonly affects patients older than JTOF. Histologically, the juvenile variants share a similar stroma but JPOF is characterized by innumerable small ossicles resembling psammoma bodies, while JTOF contains trabeculae of fibrillary osteoid and woven bone (El-Mofty 2002; Speight and Carlos 2006).
Cases of JPOF of the occipital bone
Blurred vision, VI nerve palsy
The treatment of choice for JPOF is complete surgical excision. Partial or incomplete resection leads to recurrences and is reported to range from 30 to 56 % (Bertrand et al. 1993; Margo et al. 1985). In our case, a total in bloc resection was performed, with no recurrence after 5 years follow up. Radiotherapy is not indicated because of its radio resistance and post-radiation complications (Jung et al. 1999). Malignant degeneration has not been reported so far.
The JPOF is a benign tumor with aggressive behavior that rarely affects the cranial bones; this is the first reported case within this localization causing neurological deficits. Total resection is the treatment of choice. Prognosis is good, without any case of metastasis being reported.
Study conception and design: CC, AS, MR. Acquisition of data: CC, MR, AS. Analysis and interpretation of data: CC, AP, AS. Drafting of manuscript: CC, MR, LL, AP. Critical revision: CC, AP, LL. All authors read and approved the final manuscript.
We appreciate the patient and his family forgiving us the permission to publish this case.
The authors declare that they have no competing interests.
The patient has consented to the submission of the case report for submission to the journal.
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