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| PHOTO ESSAY |
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| Year : 2021 | Volume
: 1
| Issue : 1 | Page : 28-29 |
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Tufted angioma of the eyelid – A rare entity
Debi Kundu1, Subramanian Krishnakumar2, Md Shahid Alam1
1 Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Aditya Birla Sankara Nethralaya, Kolkata (A Unit of Medical Research Foundation, Chennai), West Bengal, India
2 Larson and Turbo Department of Ocular Pathology, Sankara Nethralaya, Vision Research Foundation, Chennai, Tamil Nadu, India
| Date of Submission | 19-May-2020 |
| Date of Acceptance | 15-Sep-2020 |
| Date of Web Publication | 31-Dec-2020 |
Correspondence Address:
Dr. Md Shahid Alam
Orbit Oculoplasty Reconstructive and Aesthetic Services, Aditya Birla Sankara Nethralaya, (A Unit of Medical Research Foundation, Chennai), Kolkata, West Bengal
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ijo.IJO_1571_20
Keywords: Eyelid, Kasabach–Merritt syndrome, tufted angioma
How to cite this article:
Kundu D, Krishnakumar S, Alam MS. Tufted angioma of the eyelid – A rare entity. Indian J Ophthalmol Case Rep 2021;1:28-9 |
Tufted angioma or Angioblastoma of Nagakawa was first recognized in Japanese literature.[1] It is a rare benign tumor of skin and subcutaneous tissue and extremely uncommon in the periocular region. To the best of authors' knowledge, only three cases of tufted angioma involving the eyelid have been reported to date.
A 13- year-old female presented with swelling of the right upper lid for the past 4 months. Examination revealed a firm, nontender nodule over the right upper lid without skin discoloration and abnormal blood vessels [Figure 1]a. On eversion palpebral-conjunctiva showed an impression of the swelling with a violaceous hue. A provisional diagnosis of chalazion was considered and the patient was taken up for incision and curettage. Intraoperatively soft tissue lesion with blood vessels was noted. The lesion was removed in piecemeal achieving a near-total excision and the specimen was sent for histopathological examination. | Figure 1: (a): External photograph of the patient showing a nodular swelling in the right upper eyelid. (b): Microphotograph (Hematoxylin and Eosin 20×) showing a lobule of tumor cells comprising of blood less capillaries lined by plump endothelial cells and surrounded by spindle cells (black arrows), with intervening slits without mitotic figures. (c): Microphotograph (Hematoxylin and Eosin 40×) shows poorly canalized capillaries in the lobule in the dermis. There is a crescent-shaped space surrounding the capillary lobule (blue arrows)
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Histopathology showed vascular tumor consisting of lobules of bloodless capillaries lined by endothelial cells surrounded by spindle cells without atypia [Figure 1]b and [Figure 1]c. A diagnosis of tufted angioma of the eyelid was made and the patient was referred to a hematologist to rule out Kasabach–Merritt syndrome. All the blood investigations were within normal limits. No recurrence was noted at 1 year of follow-up.
| Discussion | |  |
Tufted angioma, first recognized in Japanese literature as “Angioblastoma of Nagakawa”, is rare benign tumor of skin and subcutaneous tissue.[1] It presents as reddish to violaceous papule, plaque or nodule over neck, upper back, and limbs.[2],[3] Histopathologically it is characterized by discrete lobules of relatively bloodless capillaries lined by plump endothelial cells, surrounded by spindle cells with intervening slits without mitotic figures. Lymphatic like vascular channels may be present. Rarely it may be associated with consumptive coagulopathy and Kasabach–Merritt syndrome.[4] In the largest case series of 13 cases by Osio et al., five cases had Kasabach–Meritt syndrome.
The tumor is extremely rare in the periocular region and to the best of authors' knowledge only 3 such cases have been reported to date.[2],[3] One of the cases reported by Mittal et al. presented as epidermoid cyst.[2] We noted a similar presentation in our case too where the provisional diagnosis was chalazion.
Though Tufted angiomas are more of histopathological interest they should be differentiated from common capillary hemangioma, kaposiform hemangioendothelioma, and giant cell angioblastoma in children and from Kaposi's sarcoma and low-grade angiosarcoma in adults.[2] Spontaneous regression was noted in 5 out of 13 cases reported by Osio et al.[4]
Tufted angioma is a rare benign vascular tumor generally affecting neck, limbs, and trunk. It is extremely rare in the periocular region. It can be associated with consumptive coagulopathy hence all patients should undergo a complete hematological workup.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Alessi E, Bertani E, Sala F. Acquired tufted angioma. Am J Dermatopathol 1986;8:426-9.  |
| 2. | Mittal R, Tripathy D. Tufted angioma (Angioblastoma) of eyelid in adults-report of two cases. Diagn Pathol 2013;8:153. |
| 3. | Verma J, Singh A, Misra V, Singh SP. Tufted angioma of eyelid in an adult-A case report. J Clin Diagn Res 2016;10:ED12-3. |
| 4. | Osio A, Fraitag S, Hadj-Rabia S, Bodemer C, de Prost Y, Hamel-Teillac D. Clinical spectrum of tufted angiomas in childhood: A report of 13 cases and a review of the literature. Arch Dermatol 2010;146:758-63. |
[Figure 1]
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