An 83-year-old woman presented with a history of dysphagia and a 10-kg weight loss
over a 6-month period. On endoscopy, multiple glassy-looking polyps were seen in the
upper and middle thirds of the esophagus (Figure [1 a]). No Barrett’s metaplasia was observed in the distal esophagus. Biopsy specimens
taken from the polyps revealed multifocal adenocarcinoma originating from the ducts
of esophageal mucus glands. The lesions showed extensive mucus production and were
located mainly in the submucosal layer, undermining non-neoplastic squamous epithelium
(Figure [1 b]). There were micropapillary in-situ formations as well as foci of overt, infiltrating,
poorly differentiated cancer (Figure [1 c]). The patient refused further evaluation and therapy.
Figure 1 a Endoscopy showed a glassy polypoid tumor in the middle third of the esophagus with
mucus drainage from the surface. b The corresponding biopsy specimen revealed a well-differentiated adenocarcinoma with
extensive mucus production. c Small tumor foci were shown to be poorly differentiated adenocarcinoma with single-cell
invasion of the adjacent stroma. d Ten months later, an exophytic, friable esophageal tumor mass causing almost complete
luminal obstruction was seen on endoscopy.
Ten months later she presented again with dysphagia. The tumor had progressed to cause
almost complete luminal obstruction, with coalescing, exophytic, friable masses throughout
the entire esophagus, with infiltration of the gastric cardia (Figure [1 d]). Computed tomography showed that there were multiple metastases to regional lymph
nodes, the lungs, and bone. The patient’s general condition worsened rapidly and she
died 11 months after the primary diagnosis.
The vast majority of adenocarcinomas of the esophagus arise in the lower third, within
a segment of Barrett’s mucosa. Salivary-type adenocarcinomas originating from submucosal
esophageal glands are extremely rare [1]. It has been argued, however, that the true incidence of these tumors is higher
than has been reported because of missed diagnoses due to endoscopic tissue sampling
[2]. Because of the rarity of the disease, the impact of treatment on survival is difficult
to assess. Local ablative therapy and radiotherapy might offer palliation of dysphagia
in patients with advanced disease [3].
Most esophageal salivary-type malignancies are adenoid-cystic and mucoepidermoid carcinomas
[1]. Our case did not fit into either category and had to be classified as ”adenocarcinoma
of salivary duct origin, not otherwise specified (NOS)”. The multifocal growth along
the entire organ in this case is a tumor feature that has not been described before.
Endoscopy_UCTN_Code_CCL_1AB_2AC_3AB
