Epithelioid Hemangioma of the Nasal Cavity: A Diagnostic Challenge

doi:10.21203/rs.3.rs-3442749/v1

Epithelioid hemangioma also known as angio-lymphoid hyperplasia with eosinophilia is a rare benign vascular neoplasm of unknown etiology. It always poses a diagnostic challenge for clinicians and is usually misdiagnosed as Kimura’s, IgG4-related disease or malignant vascular tumors. The present case report describes an extremely rare presentation of epithelioid hemangioma inside the left nasal cavity causing complete obstruction and nasal septal deviation towards the right side in a young male. The case highlights a non-conventional presentation of epithelioid hemangioma and the importance of histo-morphological features in the diagnosis of this entity.

Epithelioid hemangioma

nasal cavity

Kimura’s disease

histomorphology

Epithelioid hemangioma (EH) also known as angio-lymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular neoplasm of unknown etiology.¹ Well and Whimstar described this entity for the first time in 1961 as a later stage of Kimura’s disease, but later on, it was considered a separate entity.^1,2 EH commonly occurs in the head and neck region with the scalp and forehead being the most common site.³ Usually, it presents as a cutaneous erythematous papule or angiomatoid nodule but very rarely it involves nasal cavity. EH always poses a diagnostic challenge for clinicians and is usually misdiagnosed as Kimura’s, IgG4-related disease or malignant vascular tumors.⁴ It is not known for spontaneous regression, although malignant transformation of EH is also not reported to date. A complete excision of the lesion is a must because local recurrence is seen in one-third of the patients.⁵ The diagnosis is made on careful histopathological evaluation exhibiting a distinctive lobular architecture and well-formed blood vessels lined by plump epithelioid endothelial cells.^3,6 There is only a handful of cases of EH localized to the nose involving the nasal mucosa or vestibule causing an obstruction. Here, we describe an extremely rare presentation of EH inside the left nasal cavity causing complete obstruction and nasal septal deviation towards right side in a young male.

A 25-year-old male presented with complaints of epistaxis and nasal obstruction leading to difficulty in breathing for five months. The lesion increased in size over due course of time causing complete left-sided nasal obstruction. There was no history of trauma and family history was unremarkable. A paranasal Computerized Tomography (CT) was advised which showed a hyperdense soft tissue in the left-anterior and posterior nasal cavities with hyperdense mucosal thickening in the left frontal, ethmoid and maxillary sinuses with a deviated nasal septum towards the right side and a neoplastic etiology was suggested. [Figure 1] A punch biopsy was taken and was sent for histopathology. Hematoxylin and eosin-stained sections depicted a lobular proliferation of small capillary-sized vessels lined by plump epithelioid endothelial cells surrounding central vessels and scattered around them. These epithelioid endothelial cells had abundant eosinophilic to amphophilic cytoplasm and enlarged nuclei with fine chromatin and central nucleoli. A few of the blood vessels also exhibited hobnailing while the stroma was infiltrated with dense inflammatory infiltrate comprising lymphocytes, eosinophils and plasma cells. Based on the morphological features immunohistochemistry for CD34, ERG and CD31 was done. CD34 highlighted the vascular proliferation and focal epithelioid endothelial cells were also positive. CD31 was positive in the blood vessels and in plump endothelial cells as well. ERG showed strong nuclear positivity in neoplastic plump epithelioid endothelial cells. In accordance with these findings, a diagnosis of epithelioid hemangioma was made. [Figure 2]

As the lesion was vascular, a contrast-enhanced CT angiography was also performed which revealed a 5.7 X 4.0 cm heterogeneously enhancing lesion in the left nasal fossa which was communicating with the facial artery. A Digital Subtraction Angiography (DSA) with embolization was planned followed by which the vascularity of the lesion was decreased and the mass was completely excised with negative margins. The histopathological findings were similar to those described above.

The patient is stable with a significant improvement in breathing and had no complications to date and is doing well.

Epithelioid hemangioma of the nasal cavity is an extremely rare presentation of an uncommon disease having a tendency to mimic various non-neoplastic and neoplastic epithelioid vascular tumors and Kimura’s disease as well.⁷. EH or ALHE had some common features with Kimura’s disease due to its predilection in head and neck and stromal infiltration of lymphocytes and eosinophils as was seen in this case also with few areas showing eosinophilic microabscess formation.⁸ But few clinical and histopathological clues are helpful in differentiating these entities. The latter has lymphoid follicles with eosinophilic infiltration whereas ALHE shows capillary proliferations with epithelioid morphology. Papules or nodules of EH/ALHE are vascular and frequently bleed whereas Kimura’s disease presents with regional lymphadenopathy.^9,10 The disease commonly affects young males of age group 30-33 years and so far, most of the reported cases are from Asians and Caucasians.¹¹ The role of radiology is only limited to knowing the extension of the lesion and histopathological findings are the only key for definitive diagnosis.

To the best of our knowledge, only four cases have been reported previously of EH localized to the nasal cavity or vestibule.^12-15 The detailed findings of previously reported cases have been compared in Table 1. In the case reported by Ratingen et al., ¹³cutaneous involvement was also noted. In all the cases treatment was achieved by surgical excision as was done in our case also. A recurrence was also observed by Youssef et al.¹² and was further managed by intralesional prednisolone and 0.1% tacrolimus ointment.

EH is a benign vascular proliferation but a high chance of recurrence is seen if complete resection is not done. As a result, the treatment of choice is complete surgical excision with clear margins.¹⁶ Conservative treatment includes intralesional steroid and topical ointment however in many cases clinicians prefer a surgical approach only in view of lesional recurrence. In general, EH involves small vessels of the dermis or subcutis, but in extremely rare conditions large muscular arteries could be involved as was present in this case. The CECT angiography revealed the involvement of the facial artery for which DSA with embolization was successfully performed.

To conclude, the case highlights a non-conventional presentation of EH and the importance of histo-morphological features in the diagnosis of this entity.

This paper has been prepared by the abovementioned authors and reviewed and agreed upon for submission. The requirements for authorship as stated above in this document have been met, and that each author believes that the manuscript represents honest work.

Ethics approval and consent to participate

Not applicable, because this article does not contain any studies with human or animal subjects.

Consent for publication

Informed consent was sought from patient regarding participation and publication.

Competing interests

The author(s) declared no potential conflicts of interest.

Informed consent

Informed patient consent was taken before publication of the article. Any identifying details (such as name, date of birth) of the patient will not be published.

Conflicts of interest

The authors have no conflicts of interest to declare.

Funding

None

Author contribution

Conceptualization: AAK, SA, SZ
Data curation: AAZ, SA
Formal analysis: AAZ, SA, SZ
Funding acquisition:
Investigation:
Methodology:
Project administration:
Resources: SZ
Software:
Supervision: SZ
Validation:
Visualization: SA, SZ
Writing – original draft: AAK
Writing – review & editing: SA, SZ.
Approval of final manuscript: all authors

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Table 1: Comparison of previously reported cases of epithelioid hemangioma of the nasal cavity

Author	Age	Sex	Site	Radiology	Treatment
Chiu et al.¹⁵	38	Male	Left nasal vestibule	-	-
Ratingen et al.¹³	62	Female	Right ala of the nose	Soft tissue swelling on both side of nasal bone without extending to depth	Surgical resection
Kamath et al.¹⁴	12	Male	Left ala of nose extending superiorly to glabella	Hypointense on T1 and T2, swelling in subcutaneous plane.	Surgical excision
Youssef et al.¹²	13	Female	Pruritic intranasal mass with multiple pruritic facial skin lesions	Bilateral soft tissue masses arising from right and left nasal vestibule	Surgical resection and for post-surgical recurrence intralesional prednisolone and topical tacrolimus
Present study	25	Male	Left nasal mucosa and vestibule	Hyperdense soft tissue in the left-anterior and posterior nasal cavities	Digital subtraction angiography with embolization followed by resection

No competing interests reported.

Epithelioid Hemangioma of the Nasal Cavity: A Diagnostic Challenge

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