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  • Syringocystadenoma papilliferum in the right lateral canthus

Syringocystadenoma papilliferum in the right lateral canthus
By Kareem Waleed Alsaffarini, Han Xern Khoo and Egle Rostron

2 August 2024 | Kareem Waleed Alsaffarini, Han Xern Khoo, Egle Rostron | EYE - Pathology, EYE - Oncology
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Syringocystadenoma papilliferum (SP) is an exceedingly rare, benign adnexal tumour primarily affecting the skin. It is characterised by the presence of cystic structures and papillary projections. This uncommon dermatological condition is typically found in areas rich in apocrine glands, such as the scalp, face, or neck [1]. Despite its rarity, SP can present a diagnostic challenge due to its clinical variability, often mimicking other more common skin lesions, necessitating meticulous evaluation and precise diagnosis.

Case report

In the context of this diagnostic complexity, we present the case of a 70-year-old patient who was referred to a minor operations list after a routine intraocular pressure assessment by a hospital optometrist. During the examination, a palpable lump was discovered in the right lateral canthus of the patient’s eye. Notably, the patient had no history of glaucoma but had been aware of the presence of the lesion for over a decade. It had recently exhibited signs of gradual enlargement, prompting the referral for further evaluation and management.

 

Direct photo of lesion.

 

Photo of lesion from the side.

 

Upon thorough evaluation, the decision was made to proceed with a complete excision of the lesion. Notably, the procedure was uneventful, with no postoperative complications observed. Histological analysis of the excised tissue confirmed the diagnosis of SP, revealing the characteristic complex cystic structures and papillary projections [2]. Immunohistochemical analysis provided further diagnostic support. The postoperative follow-up for this patient demonstrated stable visual acuity (aided) at 6/7.5 in the right eye and 6/5 in the left eye.

Discussion

This intriguing case of SP underscores the multifaceted nature of this rare benign tumour. Syringocystadenoma papilliferum often manifests itself as an asymptomatic skin lesion, making its discovery purely incidental, as was the case here. Its ability to mimic more common dermatological conditions adds to the diagnostic challenge faced by clinicians [1].

It is crucial to emphasise that while SP is typically benign, it does have the potential for malignant transformation such as towards basal cell carcinoma, syringocystadenocarcinoma, and ductal carcinoma [3,4]. In fact, basal cell carcinoma has been found to be present in as nearly as 10% of patients with SP [5]. Therefore, early recognition and accurate diagnosis are pivotal for proper management. This case exemplifies how complete excision can yield favourable outcomes. The stable visual acuity and absence of recurrence in the results clinic validate the effectiveness of surgical intervention in managing SP. Furthermore, this case highlights the significance of regular follow-up appointments for long-term surveillance, underscoring the importance of thorough evaluation and early diagnosis in managing cutaneous lesions.

 

References

1. Karg E, Korom I, Varga E, et al. Congenital syringocystadenoma papilliferum. Pediatr Dermatol 2008;25(1):132–3.
2. Rammeh-Rommani S, Fezaa B, Chelbi E, et al. Syringocystadenoma papilliferum: report of 8 cases. Pathologica 2006;98(3):178–80.
3. Baino A, Akhdari N, Amal S, et al. Syringocystadénome papillifère [Syringocystadenoma papilliferum]. Ann Dermatol Venereol 2015;142(6–7):460–2.
4. Aggarwal D, Chatterjee D, Keshavamurthy V, et al. Contigous squamous proliferations in syringocystadenoma papilliferum: a retrospective study of 14 cases. Indian J Dermatol Venereol Leprol 2023;89(2):266–73.
5. Sangma MMB, Dasiah SD, Bhat VR. Syringocystadenoma papilliferum of the scalp in an adult male – a case report. J Clin Diagn Res JCDR 2013;7:742–3.

 

Declaration of competing interests: None declared.

 

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Kareem Waleed Alsaffarini
CONTRIBUTOR
Kareem Waleed Alsaffarini

York Hospital, UK.

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CONTRIBUTOR
Han Xern Khoo

St James University Hospitals, Leeds Teaching Hospitals NHS Trust, UK.

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CONTRIBUTOR
Egle Rostron

Eye Correction Centre – UK East.

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Originally Published
EYE NEWS VOLUME 31 ISSUE 2 AUGUST/SEPTEMBER 2024
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