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Case 18687

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Case 18687

Case report published
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Case 18687

An eccentric eye – Imaging


findings in orbital sebaceous gland
carcinoma
Published on 11.09.2024

DOI: 10.35100/eurorad/case.18687
ISSN: 1563-4086
Section: Head & neck imaging
Area of Interest: Eyes
Imaging Technique: CT
Imaging Technique: MR
Imaging Technique: Ultrasound
Case Type: Clinical Case
Authors: Moinuddin Sultan 1, Ajith Varrior 2, Padma
Badhe 2, Shrishail Adke 2
Patient: 75 years, male

Clinical History:

A 75-year-old man presented with gradually progressive painless swelling over the left upper eyelid with ptosis since
eight months (Figure 1). There was no diplopia. It was a 4x3 cm non-tender, firm fungating mass. There was
reduced visual acuity due to cataract. The ocular movements were normal and unrestricted.

Imaging Findings:

On ultrasound, there was a 4.5x3.4 cm heterogeneously hypoechoic lesion with mild central vascularity (Figure 2).
The lens was hyperechoic due to a cataract. There was no intraocular extension. On CECT, it was iso-hyperdense
without fat or calcification. There was heterogeneous enhancement (Figure 3). There was no involvement of the
bones of the orbital wall.

On MRI, it was a heterogeneously enhancing T1 hypointense, T2 hyperintense mass arising from the upper lid
involving the skin, the orbicularis oculi muscle, and the superior orbital septa. The belly of the levator palpebrae
muscle and adjacent loose connective tissue were spared; however, there was loss of fat planes with the tendinous
insertion. The fat planes were maintained with the palpebral lobe of the lacrimal gland. There was diffusion
restriction in the lesion (Figures 4a, 4b, 4c and 4d). The lesion was excised under general anaesthesia with lid
reconstruction. Histopathological examination revealed high-grade malignancy with sebaceous differentiation
suggestive of meibomian gland carcinoma (Figures 5a, 5b and 5c).

Discussion:

Sebaceous glands are holocrine glands consisting of meibomian glands of the tarsus, the glands of Zeis of cilia, and
the glands of the caruncle [1,2]. Sebaceous gland carcinomas (SGCs) are rare but fatal lesions that can be mistaken
for a number of other benign pathologies like chronic blepharoconjunctivitis, chalazion, and meibomianitis [3]. It has
a high mortality rate. Hence, it is of great significance to differentiate these SGCs from other benign conditions [4].

SGCs have a multifocal origin with pagetoid spread, which makes them unique among eyelid malignancies. They
are very slow-growing tumours commonly seen in the elderly population and with female predisposition; however,
they have been reported in patients as young as 3.5 years old [5]. The upper eyelid involvement is very common
due to the presence of larger number of meibomian glands.
Any patient presenting with a recurrent chalazion, especially with loss of eyelashes or with unilateral chronic
blepharitis, should raise suspicion, and biopsy should be advised to rule out SGCs since delay in diagnosis leads to
a poor prognosis [6–8]. The diagnosis is assisted by radiological investigations like MRI and CT scan, which help to
assess the local and distant involvement. CT scan is the best modality to evaluate the presence of calcification and
bony involvement. Ultrasound helps in evaluating the extent of the lesion and viewing the globe, especially the
posterior chamber, in patients with cataracts. MRI paints an accurate picture of the soft tissue involvement due to its
superior contrast resolution. SGCs are hyperintense on T2, and hypointense on T1 with heterogeneous
enhancement [9]. They depict true diffusion restriction, due to the high cellularity.

Histopathology with immunohistochemistry, molecular biology and electron microscopy aid in the diagnosis of SGCs
[6]. Surgical management is generally wide local excision, with wide resection margins due to the diffusely infiltrating
nature of these lesions [10]. Lid reconstruction post-excision is equally important, the commonly used technique
being Mohs micrographic surgery [3,8]. Radiotherapy might be used in patients with tumour size less than 10 mm
and even in patients with T3–T4 stage if surgery is not feasible [11]. Radiotherapy is helpful in cases of involvement
of the bones [3]. Prophylactic neck irradiation reduces the risk of nodal recurrences [11]. Due to delays in diagnosis
and treatment, the mortality rate is 10%, and 25% in the case of metastases.

Poor prognostic factors are upper or both eyelid involvement, tumour size more than 10 mm, symptoms more than
six months, poorly differentiated tumours, infiltration into blood vessels/lymphatics, orbital extension, multi-centric
origin, and pagetoid spread [3,12].

The tumour is staged according to the TNM classification as per the AJCC [13].

Differential Diagnosis List: Basal cell carcinoma, Melanoma, Sebaceous gland carcinoma, Sebaceous cell
carcinoma, Squamous cell carcinoma

Final Diagnosis: Sebaceous gland carcinoma

References:

Ni C, Kuan KP (1979) Meibomian gland carcinoma: a clinicopathological study of 156 cases with long-period follow
up of 100 cases. Jpn J Ophthalmol 23:388-401
Buitrago W, Joseph AK (2008) Sebaceous carcinoma: the great masquerader: emgerging concepts in diagnosis and
treatment. Dermatol Ther 21(6):459-66. doi: 10.1111/j.1529-8019.2008.00247.x. (PMID: 19076624)
Wali UK, Al-Mujaini A (2010) Sebaceous gland carcinoma of the eyelid. Oman J Ophthalmol 3(3):117-21. doi:
10.4103/0974-620X.71885. (PMID: 21120046)
Zürcher M, Hintschich CR, Garner A, Bunce C, Collin JR (1998) Sebaceous carcinoma of the eyelid: a
clinicopathological study. Br J Ophthalmol 82(9):1049-55. doi: 10.1136/bjo.82.9.1049. (PMID: 9893597)
Straatsma BR (1956) Meibomian gland tumors. AMA Arch Ophthalmol 56(1):71-93. doi:
10.1001/archopht.1956.00930040077010. (PMID: 13338927)
Foster CS, Allansmith MR (1978) Chronic unilateral blepharoconjunctivitis caused by sebaceous carcinoma. Am J
Ophthalmol 86(2):218-20. doi: 10.1016/s0002-9394(14)76815-1. (PMID: 686123)
Sweebe EC, Cogan DG (1959) Adenocarcinoma of the Meibomian gland; a pseudochalazion entity. AMA Arch
Ophthalmol 61(2):282-90. doi: 10.1001/archopht.1959.00940090284015. (PMID: 13616795)
Doxanas MT, Green WR (1984) Sebaceous gland carcinoma. Review of 40 cases. Arch Ophthalmol 102(2):245-9.
doi: 10.1001/archopht.1984.01040030195025. (PMID: 6696670)
Kawaguchi M, Kato H, Noda Y, Kobayashi K, Miyazaki T, Hyodo F, Matsuo M (2022) Imaging findings of malignant
skin tumors: radiological-pathological correlation. Insights Imaging 13(1):52. doi: 10.1186/s13244-022-01205-8.
(PMID: 35316414)
Wolfe JT 3rd, Yeatts RP, Wick MR, Campbell RJ, Waller RR (1984) Sebaceous carcinoma of the eyelid. Errors in
clinical and pathologic diagnosis. Am J Surg Pathol 8(8):597-606. doi: 10.1097/00000478-198408000-00003.
(PMID: 6465419)
Takagawa Y, Tamaki W, Suzuki S, Inaba K, Murakami N, Takahashi K, Igaki H, Nakayama Y, Shigematsu N, Itami J
(2019) Radiotherapy for localized sebaceous carcinoma of the eyelid: a retrospective analysis of 83 patients. J
Radiat Res 60(5):622-9. doi: 10.1093/jrr/rrz046. (PMID: 31271440)
Devi B, Das RK, Medhi S (2017) Case report of a rare case of meibomian gland carcinoma of the lower eyelid. Int J
Res Med Sci 4(8):3629-31. doi: 10.18203/2320-6012.ijrms20162343
Watanabe A, Sun MT, Pirbhai A, Ueda K, Katori N, Selva D (2013) Sebaceous carcinoma in Japanese patients:
clinical presentation, staging and outcomes. Br J Ophthalmol 97(11):1459-63. doi: 10.1136/bjophthalmol-2013-
303758. (PMID: 24037608)
Figure 1
a

Description: Clinical examination demonstrates a large fungating exophytic lesion (red arrow) arising
from the undersurface of the upper eyelid (white star). Origin: © Department of Radiology, Seth GS
Medical College and KEM Hospital, Mumbai, Maharashtra, India
Figure 2
a

Description: Ultrasound shows a well-defined heterogeneously hypoechoic lesion (red arrow) with well-
defined margins and mild central vascularity on colour Doppler. The lens was hyperechoic due to a
cataract (not shown). Origin: © Department of Radiology, Seth GS Medical College and KEM Hospital,
Mumbai, Maharashtra, India
Figure 3
a

Description: Unenhanced and contrast-enhanced CT shows a well-defined iso-hyperdense lesion (red


arrow) with heterogeneous enhancement. There was no fat or calcification within. There was no
involvement of the bones of the orbital wall. Origin: © Department of Radiology, Seth GS Medical
College and KEM Hospital, Mumbai, Maharashtra, India
Figure 4
a

Description: T1 non-fat saturated coronal image shows a large well-defined hypointense exophytic
lesion (red arrow). Origin: © Department of Radiology, Seth GS Medical College and KEM Hospital,
Mumbai, Maharashtra, India
b

Description: Fat-saturated T1 contrast axial image shows heterogeneous enhancement. Origin:


© Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
c

Description: Sagittal T2 weighted image shows the hyperintense lesion (red star) involving the
overlying skin (green arrow), orbicularis oculi muscle (orange arrow), superior orbital septa (blue arrow)
and abutting the eyeball (white star). Origin: © Department of Radiology, Seth GS Medical College and
KEM Hospital, Mumbai, Maharashtra, India
d

Description: There was diffusion restriction with a drop on ADC (red arrow). Origin: © Department of
Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
Figure 5
a

Description: Gross surgical specimen of excised left upper eyelid mass lesion. Origin: © Department
of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
b

Description: H&E slide at 45x (5b) shows tumour cells arranged in sheets and lobules, and 100x (5c)
shows cells with a high N:C ratio and hyperchromatic nuclei with eosinophilic cytoplasm.Origin:
© Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
c

Description: H&E slide at 45x (5b) shows tumour cells arranged in sheets and lobules, and 100x (5c)
shows cells with a high N:C ratio and hyperchromatic nuclei with eosinophilic cytoplasm.Origin:
© Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

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