Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease
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CASE REPORT
Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease Gintaras Januzis1, Dovydas Sakalys1* , Martynas Mantas Krukis1 and Dmitrij Seinin2
Abstract Background: Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. Case presentation: We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion’s clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. Conclusions: With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment. Keywords: Epithelioid hemangioendothelioma, Oral cancer, Vascular tumor, Endothelial cells, Periodontitis, Gingival pathologies, Immmunohistochemical markers, CD31, CD34, ERG Background The term hemangioendothelioma (HE) was first proposed by Borrmann in 1899 as a low-grade malignancy vascular tumor [1]. HEs can relapse but very rarely metastasize, significantly less often than angiosarcomas do [1]. According to their histopathological *Correspondence: [email protected] 1 Department of Maxillofacial Surgery, Faculty of Odontology, Lithuanian University of Health Sciences, A. Ramanausko – Vanago g. 8‑4, Kaunas, Lithuania Full list of author information is available at the end of the article
characteristics, hemangioendotheliomas are classified as kaposiform, Dabskos, or epithelioid. The kaposiform type is usually found in infants and in surface soft tissues, and is associated withcoagulopathy and thrombocytopenia (Kasabach–Merritt syndrome) [1, 2]. The Dabskos type is usually found in young people and mostly in limbs [1]. Epithelioid hemangioendothelioma (EHE) is
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