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170: Adult-Onset Of Kaposiform Haemangioendothelioma Of The Oral Cavity: A Case-Report.[AAOM2021]
EP36069
Poster Title: 170: Adult-Onset Of Kaposiform Haemangioendothelioma Of The Oral Cavity: A Case-Report.[AAOM2021]
Submitted on 29 Mar 2021
Author(s): Federica Rocchetti1, Cira Rosaria Tiziana Di Gioia2, Gianluca Tenore1, Gaspare Palaia1, Andrea Cassoni1, Umberto Romeo1
Affiliations: 1: Department of Oral Sciences and Maxillofacial Surgery. Sapienza, University of Rome, Italy; 2: Department of Radiologic, Oncologic and Pathological Anatomy Sciences. Sapienza, University of Rome, Italy
This poster was presented at 2021 American Academy of Oral Medicine Virtual Conference
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Poster Information
Abstract: BACKGROUND:

Kaposiform Haemangioendothelioma (KHE) is a rare vascular neoplasm of intermediate malignant potential derived from endothelial spindle cells. It is locally aggressive and is associated with Kasabach-Meritt Phenomenon (KMP) which is characterized by thrombocytopenia, life-threatening hemorrhage, and lymphangiomatosis. KHE occurs almost exclusively in infancy and early childhood and typically appears on the extremities, trunk and retroperitoneum. KHE also occurs in the head and neck region, however is rare.

The aim of this case-report is to describe an uncommon case of KHE occurring intraoral in adult patient without KMP.

CASE SUMMARY:

A 39-year-old Caucasian woman, with a history of systemic lupus erythematosus and psoriasis, was referred to our Department for the presence of three exophytic and erythematous lesions located on the left retromolar region, occurring since about 5 months and associated with some minor bleeding related to trauma.

Ortopantomograph X-ray and Magnetic Resonance Imaging with and without contrast showed an ill-defined enhancing mass. Multiple reactive lymph nodes were found.

An incisional scalpel biopsy, together with the extraction of the third molar was made. Histological examination reported a partially ulcerated oral mucosa, partly covered by malpighian epithelium with mild hyperparakeratosis, acanthosis, focal spongiosis, papillomatosis, and lymphocytic and neutrophil granulocytic exocytosis. In the corion there were numerous capillary vascular structures coated by spindle endothelial cells, weakly eosinophilic cytoplasm and round nucleus. These vascular structures were organized in a lobular/glomeruloid type growth pattern and were present also in the muscle tissue included in the withdrawal. Immunostains showed positivity to CD31, CD34 and vimentina. Ki-67 proliferative rate was 20%. HHV-8 was negative. Diagnosis of KHE was made.

The patient underwent surgical resection of the mass without any problems or complications. The platelet count was 189,000/mL, suggesting an absence of KMP. Follow-up at one year shows no recurrence.

CONCLUSIONS:

To the best of our knowledge this is the third adult case, of intraoral KHE, reported in the literature. Although the incidence of KHE is low, it can cause mortality in children and adults. Further studies are needed to define the guidelines for the therapies and follow-up in patients with KHE.
Summary: The aim of this case-report is to describe an uncommon case of KHE occurring intraoral in adult patient without KMP.

Ask the author questions about this poster:federicarocchetti@gmail.com
References: 1) DeFatta RJ, Verret DJ, Adelson RT, Gomez A, Myers LL. Kaposiform hemangioendothelioma: case report and literature review. Laryngoscope. 2005 Oct;115(10):1789-92. doi:10.1097/01.mlg.0000176539.94515.75.
2) Vashi P, Abboud E, Bier-Laning C, Gupta D. Adult-onset Kaposiform hemangioendothelioma of the tongue: case report and review of the literature. Curr Oncol. 2016 Oct;23(5):e517-e520. doi: 10.3747/co.23.3239. Epub 2016 Oct 25.
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