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INVESTIGATIVE REPORT
Angiogenesis in Ocular and Extraocular Sebaceous Carcinoma
Ferdinand TOBERER 1 , Holger A. HAENSSLE 1 , Arno RÜTTEN 2 , Dmitry KAZAKOV 3 , Liubov KASTNEROVA 3 , Alexander ENK 1 ,
Wolfgang HARTSCHUH 1 , Ines BERTLICH 1 , Julia HARTMANN 1 , Martin LAIMER 4 , Wolfgang WEYERS 5 , Peter HELMBOLD 1 and
Heinz KUTZNER 2
Department of Dermatology, Venereology and Allergology, University Medical Center, Ruprecht-Karls-University Heidelberg, Heidelberg,
Dermatopathology, Bodensee, Friedrichshafen, Germany, 3 Sikl’s Department of Pathology, Medical Faculty in Pilsen, Charles University in
Prague, Pilsen, Czech Republic, 4 Department of Dermatology, University Hospital of the Paracelsus Medical University Salzburg, Salzburg,
Austria, and 5 Center for Dermatopathology Freiburg, Freiburg, Germany
1
2
To shed more light on the pathogenesis of sebaceous
carcinoma, we analysed the expression of proteins re
lated to angiogenesis in 18 ocular and 22 extraocular
sebaceous carcinomas using a broad panel of immu
nohistochemical markers. To quantify the expression
of D2-40, vascular endothelial growth factor, vascular
endothelial growth factor receptor-2 and -3, we cal
culated a quantification score by considering the per
centage of positive tumour cells (0=0%, 1=up to 1%,
2=2–10%, 3=11–50%, and 4=>50%) in relation to
the staining intensity (0=negative, 1=low, 2=medium,
and 3=strong). Additionally, lymphatic microvessel
density in the D2-40 stained sections was counted.
Vascular endothelial growth factor receptor-3 (quanti
fication score 9.42 ± 2.94) was significantly more stron
gly expressed than vascular endothelial growth factor
receptor-2 (quantification score 2.15 ± 2.42, p < 0.001).
Furthermore, epidermal vascular endothelial growth
factor expression was negatively correlated with the
intratumoural lymphatic vessel density, and the ratio of
small lymphatics to large lymphatics was much higher
in intratumoural tissue than in paratumoural tissue
and in intraindividual control tissue, suggesting a
lymphangiogenetic potential of sebaceous carcinoma.
Key words: angiogenesis; lymphatic vessels; sebaceous carci-
noma.
Accepted Oct 14, 2019; E-published Oct 14, 2019
Acta Derm Venereol 2019; 99: 1270–1274.
Corr: Ferdinand Toberer, Department of Dermatology, Venereology and
Allergology, University Medical Center, Ruprecht-Karls-University, Im
Neuenheimer Feld 440, DE-69120 Heidelberg, Germany. E-mail: ferdi-
[email protected]
S
ebaceous carcinoma (SC) represents a rare cuta-
neous adnexal neoplasm that may arise as ocular
or extraocular SC (1). Extraocular SC is rare (25% of
reported cases) and mostly affects the head and neck (2).
According to a review including 1,349 cases of SC, the
median age at diagnosis was 73 years, and a slight male
predominance (54% men and 46% women) was noted
(3). Both ocular and extraocular SC have the capability
for regional and distant metastases (4). The tendency
for regional metastases is increased in ocular SC. Ne-
vertheless, the cancer-specific mortality rates are similar
among all anatomic locations (4). Although surgery is
doi: 10.2340/00015555-3342
Acta Derm Venereol 2019; 99: 1270–1274
SIGNIFICANCE
Sebaceous carcinoma represents a rare cutaneous tumour
that may arise ocular and extraocular. We analysed the ex-
pression of proteins related to angiogenesis in 18 ocular and
22 extraocular sebaceous carcinomas using a broad panel
of immunohistochemical markers including D2-40, vascular
endothelial growth factor (VEGF), VEGFR-2 and VEGFR-3.
We found an increased intratumoural ratio of small lympha-
tics to large lymphatics and a negative correlation between
epidermal VEGF expression and the intratumoural density
of lymphatics underlining the hypothesis that sebaceous
carcinoma may induce new lymphatic vessels. Additionally,
VEGFR-3 expression was significantly higher than VEGFR-2
expression in both ocular and extraocular tumours.
the treatment of choice for local disease, radiation and
systemic chemotherapy are options for recurrent and
metastatic disease (4).
SC may arise in the context of Muir-Torre syndrome
(MTS) or in association with prior irradiation, immuno
suppression and familial retinoblastoma (4). Nonetheless,
the pathogenesis of SC is not yet completely understood.
Mutational inactivation of p53 may be pathogenetically
relevant in MTS-associated SC and sporadic cases (5, 6).
Immunohistochemical analyses have demonstrated that
proteins involved in angiogenesis, cell proliferation and
inflammation, such as epidermal growth factor receptor
(7) and vascular endothelial growth factor receptor
(VEGFR) (8), are overexpressed in SC and may play a
role in its pathogenesis.
To the best of our knowledge, this is the first study
analysing the expression of proteins related to angioge-
nesis in ocular and extraocular SC using a broad panel
of immunohistochemical markers, including D2-40 (po-
doplanin), vascular endothelial growth factor (VEGF),
VEGFR-2 (KDR) and VEGFR-3 (FLT4). These proteins
may not only contribute to the pathogenesis of SC but
also act as potential therapeutic targets.
MATERIALS AND METHODS
Specimens
A total of 40 SC cases, including 18 ocular (female/male: 9/9)
and 22 extraocular (female/male: 14/8) cases, were analysed
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.