J Korean Assoc Oral Maxillofac Surg.  2016 Oct;42(5):307-314. 10.5125/jkaoms.2016.42.5.307.

Retiform hemangioendothelioma in the infratemporal fossa and buccal area: a case report and literature review

• Kim IK
• Cho HY
• Jung BS
• Pae SP
• Cho HW
• Seo JH
• Park SH

• KMID: 2356259
• DOI: http://doi.org/10.5125/jkaoms.2016.42.5.307

Abstract

We report a case of retiform hemangioendothelioma (RH) located in the infratemporal fossa and buccal area in a 13-year-old Korean boy. The tumor originated from the sphenoid bone of the infratemporal fossa area and spread into the cavernous sinus, orbital apex, and retro-nasal area with bone destruction of the pterygoid process. Tumor resection was conducted via Le Fort I osteotomy and partial maxillectomy to approach the infratemporal fossa and retro-nasal area. The diagnosis of RH was confirmed after surgery. In the presented patient, surgical excision was incomplete, and close follow-up was performed. There was no evidence of expansion or metastasis of the residual tumor in the 8 years after surgery. In cases of residual RH with low likelihood of expansion and metastasis, even though RH is an intermediate malignancy, close follow-up can be the appropriate treatment choice over additional aggressive therapy. To date, 29 papers and 48 RH cases have been reported, including this case. This case is the second reported RH case presenting as primary bone tumor and the first case originating in the oromaxillofacial area.

Keyword

Retiform hemangioendothelioma; Infratemporal fossa; Le Fort osteotomy; Residual neoplasm

MeSH Terms

Adolescent
Cavernous Sinus
Diagnosis
Follow-Up Studies
Hemangioendothelioma*
Humans
Male
Neoplasm Metastasis
Neoplasm, Residual
Orbit
Osteotomy
Osteotomy, Le Fort
Sphenoid Bone

Figure

• 

  Fig. 1 Preoperative clinical photograph. A. Extraoral image of buccal cheek swelling. B. Intraoral image of buccal cheek swelling.

• 

  Fig. 2 Preoperation magnetic resonance imaging. A. Axial T2-weighted image of low signal intensity in posterior maxillar and retro-nasal area with bony destruction of pterygoid process (black and white arrows). B. Axial T2-weighted image of homogeneous low signal intensity in buccal area. C. Coronal T2-weighted image of heterogeneous low signal intensity in retro-nasal cavity, infratemporal fossa, and cavernous sinus through foramen rotundum (arrows). D. Sagittal T1-weighted image of homogeneous low signal intensity in infratemporal fossa, and orbital apex through infraorbital fissure (arrows).

• 

  Fig. 3 Intraoperative photograph. A. Le Fort I osteotomy reveals buccal, infratemporal fossa, maxillary sinus and nasal cavity. B. Main mass of buccal, infratemporal and retro-nasal area. C. Segmentally resected masses of inferior orbital fissure area.

• 

  Fig. 4 Microscopic findings of retiform hemangioendothelioma. A. Characteristic elongated and narrow arborizing vascular channels and focal lymphocytic infiltrate (H&E staining, ×40). B. Monomorphic endothelial cells with hobnail appearance simulate the architecture of rete testis (H&E staining, ×200). C. Vacuolated cells and focal papillae with hyaline core (H&E staining, ×200). D. Neoplastic cells are immunohistochemically reactive for vascular marker, CD31 (immunohistochemistry staining, ×200).

• 

  Fig. 5 Seven-month (A, B) and four-year (C, D) follow-up magnetic resonance imaging demonstrating residual tumor segments on infratemporal fossa area with no significant interval changes. A, C. Aixial T2-weighted images of low signal intensity in infratemporal fossa area and inferior orbital fissure (black and white arrows). B, D. Coronal T2-weighted images of low signal intensity in cavernous sinus and pterygoid plate (black and white arrows).

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