Diffuse pagetoid squamous cell carcinoma of the esophagus combined with choriocarcinoma and mucoepidermoid carcinoma: An autopsy case report

Abstract

Esophageal squamous cell carcinoma in situ (SCCIS) with diffuse pagetoid features is a recently recognized rare variant of squamous cell carcinoma. A histopathological study of a specimen from a 70-year-old male Japanese patient is reported. The patient died of respiratory failure due to rapidly progressing metastatic pulmonary tumors of unknown origin 73 days after the onset of hemosputum. Autopsy disclosed widespread metastasis of choriocarcinoma in the absence of tumors of the testes or other common sites of germ cell tumors. Elevation of human chorionic gonadotropin (hCG-beta) levels was later detected in the stored serum. Serial histological evaluation of the entire esophagus revealed a small primary site of choriocarcinoma in a background of diffuse SCCIS, mainly of pagetoid type, accompanied by several small foci of submucosally invasive squamous cell carcinoma and primary mucoepidermoid carcinoma. These stimulated nodal metastasis independently of the choriocarcinoma. The SCCIS did not alter the gross mucosal appearance. This is the first reported case of diffuse pagetoid SCCIS combined with choriocarcinoma. Morphological findings and previous studies suggest that the extensive SCCIS of the esophagus resulted from pagetoid spread of tumor cells. The invasive squamous cell carcinoma, mucoepidermoid carcinoma and choriocarcinoma are suggested to have originated from the overlying SCCIS.

Surgically resected primary esophageal choriocarcinoma accompanied with Barrett’s adenocarcinoma: a case report

“…Ten cases of primary esophageal choriocarcinomas including the previously reported nine cases and our case are shown in Table 2 [5][6][7][8][9][10][11][12][13]. The average age was 57.0 (40-80) years and the age of onset was slightly lower than that of patients diagnosed with an esophageal SCC.…”

“…A primary choriocarcinoma of the gastrointestinal tract is rare and even the most frequent primary gastric choriocarcinomas have been reported to arise in approximately 0.08% of stomach malignant tumors [2][3][4]. Primary esophageal choriocarcinomas are extremely rare and only nine cases worldwide have been reported to have occurred after 1970 [5][6][7][8][9][10][11][12][13].…”

“…Choriocarcinomas often occur in the gonads, uterus, mediastinum and retroperitoneum [1]. Primary choriocarcinomas in the gastrointestinal tract are rare, especially primary esophageal choriocarcinomas, which are extremely rare [2][3][4][5][6][7][8][9][10][11][12][13]. We report the world's first resected case of a primary esophageal choriocarcinoma combined with squamous cell carcinoma (SCC)-like components in association with Barrett's adenocarcinoma.…”

Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report

“…Esophageal Paget’s disease is quite rare, with only a few cases having been reported [ 9 – 13 ]. Yates and Koss [ 9 ] described esophageal Paget’s disease associated with a poorly differentiated squamous cell carcinoma of the distal esophagus, whereas Norihisa et al [ 10 ] reported a case of adenosquamous carcinoma of the esophagus with pagetoid extension of the adenocarcinoma component.…”

“…On the other hand, Nonomura et al [ 11 ] and Matsukuma et al [ 12 ] reported esophageal Paget’s disease associated with an early underlying carcinoma, one being an intraepithelial carcinoma and the other, a minimally invasive adenocarcinoma of the esophagus. Ishihara et al [ 13 ] also reported a case of an early invasive carcinoma, which consisted of pagetoid squamous cell carcinoma in situ combined with early invasive components and choriocarcinoma at the metastatic site. Abraham et al [ 5 ] reported a close relationship between Paget cells in the esophagus and an underlying poorly differentiated adenocarcinoma in the esophagus or esophagogastric junction.…”