J Rhinol.  2018 Nov;25(2):118-122. 10.18787/jr.2018.25.2.118.

A Case of Epithelioid Hemangioendothelioma on the Choana

• Sohn JH
• Cho KR

• KMID: 2431227
• DOI: http://doi.org/10.18787/jr.2018.25.2.118

Abstract

Epithelioid hemangioendothelioma is a rare vascular tumor with intermediate malignity and metastasis risk. It presents epithelioid cells with intracytoplasmic vacuoles and low mitotic activity. Its vascular nature can be confirmed by immunohistochemical studies (vimentin, CD31, CD34, and factor VIII). It is extremely rare in the nasal cavity, with only one case reported on the middle turbinate in Korea. The authors present a case of epithelioid hemangioendothelioma on the choana with a size of 2mm, which easily coult have been misdiagnosed as a blood clot.

Keyword

Epithelioid hemangioendothelioma; Epistaxis; Nasal cavity

MeSH Terms

Epistaxis
Epithelioid Cells
Hemangioendothelioma, Epithelioid*
Korea
Nasal Cavity
Neoplasm Metastasis
Turbinates
Vacuoles

Figure

• 

  Fig. 1 Preoperative endoscopic finding shows large hematoma occupying the choana and the nasopharyngeal space. Right-sided view (A) and left-sided view (B).

• 

  Fig. 2 Axial (A) and coronal (B) CT image shows that ill-defined enhancing soft tissue density fills up the posterior nasal cavity and the nasopharyngeal space (black dotted circle). 1–2 mm sized nodule with enhancement is seen on medial choanal arch (white arrow).

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  Fig. 3 The tumor appeared as a nodular mass composed of abundant vascular channels (H&E, ×10) (A). There are spindle cell proliferation around the vessels (H&E, ×100). The tumor cells are vacuolated with intracytoplasmic erythrocytes (white arrows in yellow box) (B).

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  Fig. 4 The site of the lesion is well recovered and no recurrence is observed on postoperative endoscopic findings in 3 weeks (A) and 21 months (B) after the surgery.

Reference

1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982; 50(5):970–981.

2. Bollinger BK, Laskin WB, Knight CB. Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations. Cancer. 1994; 73(3):610–615.

3. Yang YJ, Kim YB, Kim SB, Sohn JH. A case of hemangioendothelioma on the nasal septum. Korean J Otorhinolaryngol-Head Neck Surg. 2001; 44(11):1220–1223.

4. Kim EH, Yoon MH, Yeo NK. A case of epithelioid hemangioendothelioma of the nasal cavity. Korean J Otorhinolaryngol-Head Neck Surg. 2016; 59(3):242–245.

5. O'Hara CD, Nascimento AG. Endothelial lesions of soft tissues: a review of reactive and neoplastic entities with emphasis on low-grade malignant (“borderline”) vascular tumors. Adv Anat Pathol. 2003; 10(2):69–87.

6. Albuquerque AKAC, Romano SO, Eisenberg ALA. Epithelioid hemangioendothelioma: 15 years at the national cancer institute. literature review. J Bras Patol Med Lab. 2013; 49(2):119–125.

7. Semino L, Pagella F, Delu G, Todeschini A, Luinetti O, Zappoli F, et al. Endoscopic treatment of ethmoidal hemangioendothelioma: case report and review of the literature. Am J Otolaryngol. 2006; 27(4):287–290.

8. Ellis GL, Kratochvil FJ 3rd. Epithelioid hemangioendothelioma of the head and neck: a clinicopathologic report of twelve cases. Oral Surg Oral Med Oral Pathol. 1986; 61(1):61–68.

9. Gordon-Nunez MA, Silva eM, Lopes MF, de Oliveira-Neto SF, Maia AP, Galvao HC. Intraoral epithelioid hemangioendothelioma: a case report and review of the literature. Med Oral Patol Oral Cir Bucal. 2010; 15(2):340–346.

10. Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev. 2014; 8(2):82–91.

11. Patel NR, Salim AA, Sayeed H, Sarabia SF, Hollingsworth F, Warren M, et al. Molecular characterization of epithelioid hemangioendotheliomas identifies novel WWTR1-CAMTA1 fusion variants. Histopathology. 2015; 67(5):699–708.

12. Perkins P, Weiss SW. Spindle cell hemangioendothelioma: an analysis of 78 cases with reassessment of its pathogenesis and biological behavior. Am J Surg Pathol. 1996; 20:1196–1204.

13. Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher CD. Epithelioid hemangioendothelioma of skin and soft tissues: clinico pathologic and immunohistochemical study of 30 cases. Am J Surg Pathol. 1997; 21(4):363–374.

14. Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, et al. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). An analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer. 1983; 51(3):452–464.

15. Girolamo Di, Giacomini PG, Coli A, Castri F, Padova AD, Bigotti G. Epithelioid haemangioendothelioma arising in the nasal cavity. J Laryngol Otol. 2003; 117(1):75–77.

16. Resnik KS, Kantor GR, Spielvogel RL, Ryan E. Cutaneous epithelioid hemangioendothelioma without systemic involvement. Am J Dermatopathol. 1993; 15(3):272–276.

17. Yoon SW, Park MJ, Kim KH, Ko BJ, Kim CK, Sohn JH. Early endoscopic electrocoagulation of the sphenopalatine artery for refractory posterior epistaxis: preliminary study. J Rhinol. 2014; 21(2):112–118.

18. Kim SW, Lee MH, Maeng CH, Yun DG, Kim CD. Clinical analysis of epistaxis: result of the recent 10 years. Korean J Otorhinolaryngol-Head Neck Surg. 2000; 43(11):1216–1221.

19. Zhang X, Wang EW, Wei H, Shi J, Snyderman CH, Gardner PA, et al. Anatomy of the posterior septal artery with surgical implications on the vascularized pedicled nasoseptal flap. Head Neck. 2015; 37(10):1470–1476.