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Clinical images of bony tumor of medial canthus. A: Initial presentation, 2014. B: 1-month status post partial resection, 2014. C: Relapse, 2015, D: Relapse with malignant transformation 2019.
Source publication
Purpose
Osteosarcoma is an aggressive malignant osteogenic tumor that commonly arises in long bones of pediatric populations. Primary osteosarcomas of the head and neck are rare, comprising less than 0.5% of malignancies in this region, usually affecting the mandible or maxilla. Here we present an extraordinary case of a rare benign osteochondroma...
Context in source publication
Context 1
... on presentation in 2014 showed 3 mm right axial globe proptosis, 2 mm right hypoglobus, complete right nasolacrimal duct obstruction, and a palpable bony prominence extending from right medial orbit over the nasal root to the left anterior medial orbit (Fig. 1 hypertropia and exotropia; intraocular pressure was 21 mm Hg on the right and 22 mm Hg on the left. Computed tomography (CT) imaging confirmed a 20 × 36 mm lobular ossified mass extending from the right ethmoid sinus and medial orbital wall to the left nasal bone (Fig. 2a). Uncomplicated partial resection in October 2014 restored the ...
Citations
... The distinctive characteristics setting periosteal chondroma apart from other chondromas include the absence of systemic diseases such as Ollier disease, Maffucci syndrome, and hereditary multiple exostoses, as well as the lack of malignant transformation in periosteal chondroma, a contrast to some other chondromas that may manifest these features. [20][21][22][23] Consequently, the decision was made to pursue observation instead of opting for an aggressive second surgery. ...
BACKGROUND
Intracranial chondroma is an extremely rare type of tumor composed of mature hyaline cartilaginous tissues. No previous cases of skull base periosteal chondroma have been presented.
OBSERVATIONS
A 31-year-old male had progressive memory loss and diminished motivation for the previous 1.5 years. Magnetic resonance imaging revealed a giant tumor with partial calcification arising from the upper clivus and extending to the prepontine cistern. Compression of the brainstem and hypothalamus was significant. Surgery was performed and intentionally limited to an intracapsular resection with endoscopic endonasal surgery (EES), and the brainstem and hypothalamus were successfully decompressed. Pathological examination findings showed a composition of hyaline cartilage with chondrocyte clusters. Genetic testing with next-generation sequencing indicated alternations in IDH1 R132C, KDR Q472H, IDH2 I142L, and TP53 P72R . On the basis of these findings, a diagnosis of periosteal chondroma was made. Postoperatively, complete relief from all symptoms was noted, and MRI one year later showed no evidence of tumor regrowth.
LESSONS
This is the first known report of skull base periosteal chondroma. Genetic testing was useful for confirming the diagnosis, and EES was effective for treatment. Should such a tumor show adhesion to an important structure, an intracapsular excision can be beneficial for avoiding complications.
