Esophageal Carcinoma Cuniculatum

Abstract

Carcinoma cuniculatum, a unique variant of well-differentiated squamous cell carcinoma, has been only rarely reported in the esophagus. We report 9 cases of esophageal carcinoma cuniculatum diagnosed on esophagectomy specimens in 7 men and 2 women during a 20-year period. All but 1 of the patients presented with persistent or progressive dysphagia. All patients had an esophageal mass or lesion on endoscopic examination. In 8 cases (88.8%), the tumor was located in the distal esophagus. Burrowing was noted on the tumor surface in 2 recent cases on macroscopic examination. All carcinomas were invasive either at the mucosa (n=2), submucosa (n=1), muscularis propria (n=4), adventitia (n=3), or adventitia and lung (n=1). All carcinomas demonstrated a common histologic pattern characterized by hyperkeratosis, acanthosis, dyskeratosis, abnormal keratinization, keratin-filled cyst/furrows, koilocyte-like cells, intraepithelial neutrophils, and focal cytologic atypia. In situ hybridization for human papillomavirus subtypes was negative in all 10 tumors tested. None of the cases showed lymph node metastasis. Two patients died postoperatively due to complications. The remaining patients were followed up for a median duration of 84 months (48 to 214 mo). During the follow-up period, 3 patients died 49, 66, and 214 months after esophagectomy at the ages of 66, 68, and 91 years, respectively; death in these 3 cases was not related to recurrence/metastases of esophageal cancer. Four patients were alive without disease at 48, 49, 84, and 87 months after curative resection. Our report identifies a common histomorphologic pattern of esophageal carcinoma cuniculatum and supports the fact that surgical resection of the tumor by esophagectomy provides long-term survival even in patients with T3 tumor.

Carcinoma Cuniculatum of the Esophagus and Tongue: Report of Two Cases, Including TP53 Mutational Analysis

“…In both our cases, there was no significant expression of p16 on immunohistochemistry, arguing against high risk HPV as an etiological factor. This is in keeping with other reports of CC in extracutaneous sites that have not demonstrated any association with HPV [3]. In the study by Landau et al [3], analysis of all their esophageal CCs by chromogenic in situ hybridization HPV tested negative for both low and high-risk HPV subtypes.…”

“…CC most often occurs in the skin, but have also been described in other sites, including esophagus [2,3], oral cavity [4,5], gingiva [6], tongue [7], penis [8], larynx [9] and nail [10]. In its pure form this neoplasm has only capacity for local infiltrative growth, but not metastasis [3,8].…”

“…Rare examples of cutaneous CC have been described in association with long standing neuropathic ulcers secondary to leprosy [13], plantar keratoderma [14] and necrobiosis lipoidica [15]. Etiopathogenetic possibilities that have been discussed by various investigators include smoking, alcohol, reflux and chronic ulcers [2,3,16]. A link to human papillomavirus (HPV) type-11 has also been suggested [17].…”

“…The very well differentiated light microscopic features of this tumor led the authors to label it as ''epithelioma cuniculatum''. The most commonly involved site for CC is the skin with a predilection for the sole, but noncutaneous sites, such as esophagus [2,3], oral cavity [4,5], gingiva [6], tongue [7], penis [8], larynx [9] and nail [10] are also on record. CC is histologically typified by deeply invaginating, keratin filled, sinus-like structures which have been likened to rabbit burrows (lat.…”

“…There may or may not be an associated exophytic component [2]. The former has led many investigators to view CC and verrucous carcinoma (VC) as two ends of a spectrum of extremely well differentiated SCC, which when pure, only have the capability for local infiltration and lacks metastatic potential [3,8]. Herein, we present the clinicopathological features of two cases of CC; one lingual and one esophageal case with a molecular genetic study regarding the mutational status of the TP53 gene of the esophageal case.…”