Endoscopic submucosal dissection in a rare case of carcinoma cuniculatum of the esophagus initially misdiagnosed as benign squamous papilloma

Abstract

Endoscopic submucosal dissection in a rare case of carcinoma cuniculatum of the esophagus initially misdiagnosed as benign squamous papilloma A 65-year-old woman presented for evaluation of a large esophageal mass, diagnosed on biopsy as squamous cell papilloma. Computed tomography (CT) showed no lymphadenopathy or distant metastases. The patient refused surgery. Endoscopic mucosal resection (EMR) was attempted at a tertiary referral center but could not be carried out because of difficulty in lifting the lesion. Thus, she was referred to our center for endoscopic submucosal dissection (ESD). Esophagogastroduodenoscopy (EGD) revealed a large, circumferential, partially obstructive esophageal mass extending from 22 to 30 cm from the incisors (• " Fig. 1 a, b). Successful circumferential ESD was carried out on the proximal 6 cm of the lesion (• " Fig. 2 a, b), however, the distal 2 cm could not be resected owing to severe submucosal fibrosis. Histopathological analysis of the ESD specimen revealed an unusual histomorphologic pattern including hyperkeratosis, acanthosis, dyskeratosis, intraepithelial neutrophils, keratin-filled furrows, and koilocyte-like cells consistent with carcinoma cuniculatum (• " Fig. 3). Invasion in the submucosa was present. Laparoscopy-assisted esophageal resection was performed, and the resected specimen was staged pathologically as T1bN0. The patient had an uneventful recovery. Carcinoma cuniculatum of the esophagus is a very rare and extremely well differentiated variant of squamous cell carcinoma, first described in 2005 [1]. Since then, it has been reported in less than 15 cases [2,3]. The etiology is unknown but there have been reported associations with chronic esophageal irritation, achalasia, diverticulum, and prior caustic injury [1,4]. Carcinoma cuniculatum of the esophagus has traditionally been managed with esophagectomy [3]. This is the first reported case of carcinoma cuniculatum in which ESD was attempted with partial success. The success was limited owing to submucosal invasion but adequate staging was still achieved: the ESD specimen provided an accurate histopathologic diagnosis and staging to guide further therapy. Thus, ESD could be used not only as a treatment option but also as a staging tool to assess the depth of invasion. Endoscopy_UCTN_Code_TTT_1AO_2ACCompeting interests: None Fig. 3 Histopathological analysis of the ESD specimen from an esophageal mass in a 65-year-old woman. Low power view (2 ×) showing well differentiated squamous epithelium with extensive hyperkeratosis, acanthosis, dyskeratosis, intraepithelial neutrophils, and koilocyte-like cells. Cases and Techniques Library (CTL) E531Coman Roxana M et al. ESD for carcinoma cuniculatum misdiagnosed as benign squamous papilloma … Endoscopy 2014; 46: E531-E532This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

Esophageal Carcinoma Cuniculatum Associated with Non-Necrotizing Granulomatous Inflammation and Lymphadenopathy: Clinicopathologic Features and Diagnostic Challenges

“…Since 1954, CC has been subsequently identified in various non-cutaneous mucosal sites such as the oral cavity and larynx. The 2 earliest esophageal CC cases were only recently recognized in the literature in 2005 [ 3 ], with merely 13 additional cases published to date [ 2 , 4 – 6 ]. In this report, we present the case of a 52-year-old man with esophageal CC with extensive lymphoplasmacytic and non-necrotizing granulomatous inflammation and regional lymph nodes revealing non-necrotizing granulomatous lymphadenopathy, which has never been previously described.…”

Esophageal Carcinoma Cuniculatum Diagnosed on Mucosal Biopsies Using a Semiquantitative Histologic Schema: Report of Two Esophagectomy-Confirmed Cases

“…Carcinoma cuniculatum, a rare variant of squamous cell carcinoma, has been increasingly recognized by clinicians and pathologists, and about two dozen of cases have been reported in the literature primarily in esophagectomy specimens [3][4][5]. A few cases have also been reported in endoscopically resected (EMR and ESD) specimens [7,8]. Preoperative diagnosis on mucosal biopsies obtained by EGD is extremely challenging due to its bland cytology and presence of inflammation [3,6,7].…”

“…Preoperative diagnosis on mucosal biopsies obtained by EGD is extremely challenging due to its bland cytology and presence of inflammation [3,6,7]. Hence, it can often be misdiagnosed as active esophagitis, Candida esophagitis, papilloma, or is inconclusive [3,4,8].…”

“…Preoperative diagnosis of esophageal carcinoma cuniculatum is extremely difficult and in most cases, the preoperative biopsies are often interpreted as active esophagitis, Candida esophagitis, papilloma, or inconclusive. Most carcinoma cuniculatum of the esophagus reported in the literature were only diagnosed on esophagectomy [3][4][5][6], endoscopic mucosal resection (EMR) [7], or endoscopic submucosal dissection (ESD) specimens [8]. Chen et al retrospectively examined 35 preoperative esophageal mass biopsies obtained from 25 endoscopic procedures in 11 patients with a resection-proven diagnosis of carcinoma cuniculatum [7].…”

Histomorphology and Molecular Profiling of Well-Differentiated Squamous Cell Carcinoma of the Esophagus Including its Unusual Variants

“…Carcinoma cuniculatum was first described in the skin as a variant of squamous cell carcinoma peculiar to the foot. 63 Subsequently, non-cutaneous carcinoma cuniculatum cases have been reported in many different organ systems, including the esophagus, 53,[64][65][66][67][68] head, neck, [69][70][71][72][73] cervix, 74 and penis. 75 Carcinoma cuniculatum was initially considered to be a special type of VC.…”