A 53-year-old man presented to Ruijin Hospital, having been followed up for 9 months
with an esophageal lesion that had initially been detected during a routine esophagogastroduodenoscopy
(EGD) and had been diagnosed histopathologically as a high grade intraepithelial neoplasia
(HGIN).
The inpatient EGD revealed a flat (0-IIb) and slightly reddish lesion spanning from
25 to 38
cm of the middle-to-lower esophagus ([Fig. 1]). Magnifying endoscopy with blue-laser imaging (ME-BLI) identified an intrapapillary
capillary loop (IPCL) pattern consistent with type B1 ([Fig. 1]
d–f). Notably, a 5 × 5-mm slightly elevated area with mild
congestion was observed within the lesion at 26 cm ([Fig. 1]
a). The lesion remained unstained after the application of
Lugolʼs solution and exhibited a partial pink-color sign from 30 to 33 cm of the esophagus,
without any signs of deep invasion ([Fig. 1]
g–i). Pathology and immunohistology of the biopsy specimen
revealed CK7+, CK19+, P53(missense mutation), P40(little+), P63(little+), CK5/6(partial+),
P16(little+), Ki67(70%+), AE1/AE3+, CAM5.2+, CK20(−), SOX-10(−), villin(−), HER2(0),
SATB2(−),
GCDFP-15(−) ([Fig. 2]), suggesting (i) extramammary Paget disease; (ii) invasive adenocarcinoma with Paget
dissemination (M1 for the biopsy, more tissue would be needed for the evidence of
invasive
adenocarcinoma) [1]
[2]
[3]
[4].
Fig. 1 Endoscopic features of the lesion before radiofrequency ablation on: a–c white-light endoscopy; d–f magnifying endoscopy with blue-laser imaging; g–i after staining with Lugol’s solution.
Fig. 2 Microscopic appearance of the of biopsy specimen on: a, b
hematoxylin and eosin (H&E) staining; c–l immunohistochemical
staining with: c CK7(+); d CK19(+); e P53(missense mutation); f P63(little+);
g P40(little+); h CK20(−); i Ki67(70%+); j AE1/AE3(+); k CK5/6(partial+); l P16(−).
Given the size of the lesion and the patientʼs refusal to undergo surgery, we performed
radiofrequency ablation therapy ([Fig. 3]). A follow-up EGD at 2 months post-treatment revealed persistent faintly red, rough
mucosa extending from 26 to 38 cm of the esophagus ([Fig. 4]). Notably, brownish areas were observed within the lesion under ME with narrow-band
imaging (ME-NBI), with the majority of type B1 IPCL with partial type R vessels ([Fig. 4]
b, e). Subsequent Lugolʼs solution staining delineated an
irregularly geographically distributed lesion with partial circumferential involvement
([Video 1]). A biopsy taken at 30 cm demonstrated a pink-color sign ([Fig. 4]
f), confirming the previous diagnosis.
Fig. 3 Endoscopic images during radiofrequency ablation therapy.
Fig. 4 Endoscopic images during follow-up esophagogastroduodenoscopy 2 months after treatment
on: a, d white-light imaging; b, e
magnifying endoscopy with narrow-band imaging; c, f after staining
with Lugol’s solution.
Features of a rare primary Paget’s disease of the esophagus under white-light endoscopy
and magnifying endoscopy with blue-laser imaging/narrow-band imaging before and after
radiofrequency ablation treatment.Video 1
The patient currently continues on regular EGD follow-up every 3 months at his local
hospital. If the lesion were to worsen during follow-up, chemoradiotherapy would be
considered.
Endoscopy_UCTN_Code_CCL_1AB_2AC
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