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Inicio Acta Otorrinolaringológica Española Epithelioid haemangioendothelioma of the nasal cavity in a woman
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Vol. 68. Núm. 6.
Páginas 369-371 (Noviembre - Diciembre 2017)
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Vol. 68. Núm. 6.
Páginas 369-371 (Noviembre - Diciembre 2017)
Case study
DOI: 10.1016/j.otorri.2016.10.003
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Epithelioid haemangioendothelioma of the nasal cavity in a woman
Hemangioendotelioma epitelioide de la cavidad nasal en una mujer
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Emili Navalón-Ramona,
Autor para correspondencia
emilinavalon@gmail.com

Corresponding author.
, Tomàs Pérez-Garriguesb, Pascual Meseguer-Garciac, Tomàs Pérez-Carbonelld
a Centre de Salut Ontinyent-II, Ontinyent, Valencia, Spain
b Servicio de Otorrinolaringología, Hospital Lluís Alcanyís, Xàtiva, Valencia, Spain
c Servicio de Anatomía Patológica, Hospital Lluís Alcanyís, Xàtiva, Valencia, Spain
d Servicio de Otorrinolaringología, Hospital Clínic Universitari, Valencia, Spain
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Table 1. Features of present case and previously reported cases of epithelioid hemangioendothelioma of the nasal cavity.
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Case report

A 32-year-old woman presented with a 7-month history of intermittent left-sided epistaxis and watery rhinorrhea without seasonal predominance. Her medical history included a septorhinoplasty 3 years before. No bleeding source was identified on repeated nasal endoscopies. There were no signs to clarify the source of bleeding on CT, although a coronal cut between the septum and the middle third of the middle turbinate showed an image that was similar to a drop of liquid (Fig. 1). A more thorough endoscopic exploration under general anesthesia and, if needed, sphenopalatine artery bipolar coagulation was considered. A small tumor was found in the medial side of the vertical portion of the middle turbinate body, facing the septum. An endoscopic resection of the vertical aspect of middle and superior turbinates that included a 1-cm bluish-purple tumor in the septal aspect of the left middle turbinate was performed. The pathology report was “EH 0.9cm in maximum diameter with negative surgical margins. Immunohistochemically, tumor cells are immunoreactive for vimentin, CD31, CD34, α-1-antichymotrypsin, and FVIII” (Figs. 2 and 3). At 18 months follow-up, the patient remained free of symptoms and nasal endoscopy showed no abnormalities.

Figure 1.

Coronal section of unenhanced CT: soft tissue window. Mucosal thickening and slight hypodensity of the septal mucosa of the left middle turbinate (arrow).

(0,1MB).
Figure 2.

Histologic image from resected tumor. Tumor is composed of solid nests of large cells with abundant cytoplasm (epithelioid), and large nuclei with clear nucleolus. Two cells are in mitosis. There are abundant small vascular channels formed by tumor cells and intracytoplasmic lumina containing erythrocytes. Hematoxylin–eosin, original magnification ×400.

(0,19MB).
Figure 3.

Immunohistochemistry from resected tumor. CD31. Endothelium specific antibody. Positive membrane staining in tumor cells. Respiratory epithelium is visible on the surface, which is negative. Original magnification ×40.

(0,23MB).
Discussion

EH was first described by Weiss and Enzinger in 1982 as a tumor that affects soft tissue.1 It is an angiocentric vascular tumor with endothelial component. Both by clinical behavior as by histological characteristics, EH is considered a tumor of intermediate malignancy because, although it has potential to recur or metastasize, it does so with a frequency and speed much lower than those of conventional angiosarcoma. It appears at any age and is often closely associated to a blood vessel, usually a medium-sized or large vein.2 It can occur in several locations: soft tissue, bone, liver, lung, skin, oral cavity, gastrointestinal tract, peritoneum, lymph nodes, meninges, mediastinum, pleura, heart, spleen, thyroid, or parotid gland.3 Only six cases of this tumor in the nasal cavity have been found up to 2016 in the review of literature in English carried out (Table 1).3–8 The tumor consists of endothelial cells with epithelioid and histiocytoid appearance. It usually appears as a single mass in soft tissue at any anatomic site, but when it does so in the lung, liver, and bone tends to be multifocal. Tumor cells have eosinophilic cytoplasm and rounded nucleus, within a myxoid stroma. Characteristically, the cytoplasm of these cells is vacuolated, which helps establish the diagnosis. It is also typical that these vacuoles harbor erythrocytes and converge to form intracytoplasmic lumina, which could represent aborted vascular channels. Nuclear pleomorphism is minimal and no mitoses are usually observed. Histologically EH may be mistaken for an angiosarcoma, but the latter usually has more cellular atypia, mitoses, and necrosis. The neoplastic cells have immunoreactivity for endothelial markers such as factor VIII-related antigen, Ulex europaeus agglutinin 1 lectin, CD31, CD34, vimentin, and FLI1. Wide local excision of tumor with surgical margin is the appropriate treatment, though it requires a close clinical follow-up because of its potential to recur and metastasize.

Table 1.

Features of present case and previously reported cases of epithelioid hemangioendothelioma of the nasal cavity.

n  Sex  Age  Site  Clinic  Treatment  Follow-up  Year  Author 
23  Middle meatus  Intermittent epistaxis  Surgery  12m NED  2003  Di Girolamo3 
25  Middle turbinate  Intermittent epistaxis  Surgery  36m NED  2005  Tseng4 
Nasal cavity  NA  Surgery  2 recurrences at 3 and 5 y. Now 12 y NED  2008  Naqvi5 
40  Septum  Intermittent epistaxis  Surgery  9m NED  2010  Patnayak6 
54  Nasal cavity  Nasal obstruction  Surgery  24m NED  2012  Hao7 
27  Nasal cavity  Intermittent epistaxis and facial pain  Surgery  26m NED  2016  Ogita8 
32  Middle turbinate  Intermittent epistaxis  Surgery  18m NED  2016  Current 

Abbreviations: M, male; F, female; m, months; y, years; NA, not available; NED, no evidence of disease.

There are few cases of EH in the head and neck, and the localization of this tumor in the nasal cavity is exceptional. Only six cases have been reported, one of them in a woman. We present the second epithelioid hemangioendothelioma case in the nasal cavity of a woman.

Funding

There has been no funding.

Conflict of interest

The authors declare that they have no conflict of interest.

References
[1]
S.W. Weiss, F.M. Enzinger.
Epithelioid hemangioendothelioma. A vascular tumour often mistaken for carcinoma.
Cancer, 50 (1982), pp. 970-981
[2]
V. Ravi, S. Patel.
Vascular sarcomas.
Curr Oncol Rep, 15 (2013), pp. 347-355
[3]
A. Di Girolamo, P.G. Giacomini, A. Coli, F. Castri, A. de Padova, G. Bigotti.
Epithelioid hemangioendothelioma arising in the nasal cavity.
J Laryngol Otol, 117 (2003), pp. 75-77
[4]
C.C. Tseng, S.H. Tsay, T.L. Tsai, C.H. Shu.
Epithelioid hemangioendothelioma of the nasal cavity.
J Chin Med Assoc, 68 (2005), pp. 45-48
[5]
J. Naqvi, N.G. Ordonez, M.A. Luna, M.D. Williams, R.S. Weber, A.K. El-Naggar.
Epithelioid hemangioendothelioma of the head and neck: role of podoplanin in the differential diagnosis.
Head Neck Pathol, 2 (2008), pp. 25-30
[6]
R. Patnayak, A. Jena, M.K. Reddy, A.K. Chowhan, L.M.C. Rao, N. Rukhamangadha.
Epithelioid hemangioendothelioma of nasal cavity.
J Lab Phys, 2 (2010), pp. 111-113
[7]
K.F. Hao, G.Q. Liu, Y.F. Song.
Epithelioid hemangioendothelioma of the nasal cavity: report of a case.
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi, 47 (2012), pp. 1041-1042
[8]
S. Ogita, T. Endo, K. Nomura, T. Ogawa, M. Watanabe, K. Higashi, et al.
Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base.
Surg Neurol Int, 7 (2016), pp. 53
Copyright © 2017. Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello
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