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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 204-205

Unicorn - Malignant haemangioendothelioma of nose & paranasal sinuses


1 Department of ENT, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry 605 006, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry 605 006, India

Date of Submission20-Nov-2019
Date of Web Publication25-May-2021

Correspondence Address:
Pampa Ch Toi
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_2327_19

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How to cite this article:
Ravichandran SP, Toi PC. Unicorn - Malignant haemangioendothelioma of nose & paranasal sinuses. Indian J Med Res 2020;152, Suppl S1:204-5

How to cite this URL:
Ravichandran SP, Toi PC. Unicorn - Malignant haemangioendothelioma of nose & paranasal sinuses. Indian J Med Res [serial online] 2020 [cited 2021 Aug 4];152, Suppl S1:204-5. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/204/316826

Patient's consent obtained to publish clinical information and images.


A 21 yr old male presented to the ENT department, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry, India, in October 2016, with recurrent swelling in the inner aspect of the right eye along with watering and a forehead swelling for six months. Previous excision of a similar mass was alone in April 2014. Examination showed a well-defined swelling of 4 × 3 cm in the right medial canthus and a midline forehead swelling of 16 × 15 cm starting from the root of the nose.

Fine-needle aspiration cytology showed malignant haemangioendothelioma. Imaging showed a mass in the right nose, maxillary sinus and ethmoidal sinus extending into the right orbit with frontal bone erosion and minimal intracranial extension [Figure 1]A. Craniofacial resection and external fronto-ethmoidectomy [Figure 1]B and [Figure 1]F, Video) was done along with radiotherapy in view of mitotic figures [Figure 1]C, nuclear pleomorphism and CD31 positivity [Figure 1]D. He was followed up for three years with no recurrence as per imaging [Figure 1]E.
Figure 1: (A) Magnetic resonance imaging showing a heterogeneous mass in the right nasal cavity, maxillary sinus and orbit with frontal bone erosion (white arrow). (B) A flap elevated from the underlying forehead swelling (white arrow). (C) Photomicrograph showing sheets of round-to-oval tumour cells with eoinophilic cytoplasm and intracytoplasmic vacuoles (green arrow), Red blood cells within the vascular lumina (red arrow), mild-to-moderate nuclear atypia (yellow arrow) (H and E, ×40) Inset showing positive immunohistochemistry with CD31 positivity in tumour cells (×20). (D) Post-tumour resection showing roof of the orbit and supraorbital cell (yellow arrow). (E) Titanium plate reconstruction post-tumour excision. (F) Post-operative computed tomography image showing no residual tumour.

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[Additional file 1]

Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour with an incidence of 1 in 1,000,000 cases. Its occurrence in paranasal sinuses is rare, and it poses a histologic enigma due to its similarity to other tumours.

Acknowledgment: Authors acknowledge Dr Arun Alexander for performing the surgery and providing the surgical images.

Conflicts of Interest: None.


    Figures

  [Figure 1]



 

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