ABSTRACT
Retiform hemangioendothelioma is a rare vascular neoplasm of intermediate grade, the diagnosis of which can be challenging. We report a case of 35-year-old man with swelling in the postauricular region. He had undergone FNAC which had revealed blood only. Microscopic examination showed narrow, arborizing, vascular channels resembling normal rete testis. Evidence of mitoses or cytological atypia were lacking. Immunohistochemistry showed diffuse and strong staining for CD34 along with CD31 positivity. Immunostains for D240 and GLUT1 were negative. A diagnosis of retiform hemangioendothelioma was made. Histologically, it should be distinguished from Kaposiform hemangioendothelioma, Dabska tumor, epithelioid hemangioendothelioma, and angiosarcoma.
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.
Subject(s)
Adolescent , Humans , Male , Cavernous Sinus , Diagnosis , Follow-Up Studies , Hemangioendothelioma , Neoplasm Metastasis , Neoplasm, Residual , Orbit , Osteotomy , Osteotomy, Le Fort , Sphenoid BoneABSTRACT
Retiform hemangioendothelioma (RH) is a distinct entity in the spectrum of vascular tumors with a high local recurrence rate. It is considered a low‑grade, well‑differentiated cutaneous angiosarcoma with low metastatic potential. We report here for the first time a case of medial canthus recurrent RH. It may be helpful in our practice to include RH as a differential diagnosis of eyelid lesions. It is noteworthy that the progressive course and recurrence tendency of RH might be misdiagnosed as angiosarcoma or basal cell carcinoma (BCC), if not expected and carefully evaluated by the pathologist.