Esophageal cancer is the sixth leading cause of cancer-related death and eighth most common cancer, affecting > 450000 people worldwide. Esophageal squamous cell carcinoma is the most common histological type, whereas esophageal adenoid cystic carcinoma (EACC) is rare. The liver is the most common distant metastatic site in esophageal cancer. Anal metastasis is rare and has not been reported in clinical practice before. Here, we report anal metastases in a patient with EACC after regular chemotherapy and surgical resection.

A 61-year-old esophageal cancer patient was found to have lung and brain metastases during standardized treatment. The patient's treatment plan was continuously adjusted according to the latest treatment guidelines. However, the patient subsequently noticed rectal bleeding and itching, and after obtaining pathology results at the local hospital, anal metastasis of esophageal cancer was diagnosed.

Postoperative pathology and immunohistochemistry confirmed EACC with rare anal metastasis. More exploration of EACC diagnosis and treatment is needed.

Esophageal adenoid cystic carcinoma (EACC) is an exceedingly rare malignant tumor of the esophagus, posing significant challenges in the clinic.

This report detailed the case of a 72-year-old male whose diagnosis of EACC was confirmed through postoperative histopathological examination. The patient underwent thoracoscopy-assisted radical resection of the esophageal tumor, coupled with lymph node dissection. Pathological findings revealed an adenoid cystic carcinoma infiltrating the entire layer of the muscularis propria, locally extending into the outer membrane of the esophageal fiber, involving the cardia and exhibiting no lymph node metastasis. The patient's condition was classified as primary EACC, T3N0M0, per the American Joint Committee on Cancer (2017; 8th edition). One month after surgery, the patient received postoperative adjuvant radiation therapy.

In addressing the rarity and high potential for biopsy misdiagnosis of EACC, this study delved into its diagnostic methods and treatment.

The aim of this analysis is to assess radiotherapy's role and technical aspects in an array of rare gastrointestinal (GI) cancers for adult patients. Collection data pertaining to radiotherapy and digestive rare cancers were sourced from Medline, EMBASE, and Cochrane Library. Preoperative chemoradiotherapy improved outcomes for patients with esophageal undifferentiated carcinoma compared with esophageal salivary gland types of carcinomas. For rare gastric epithelial carcinoma, perioperative chemotherapy is the common treatment. Adjuvant chemoradiotherapy showed no benefice compared with adjuvant chemotherapy for duodenal adenocarcinoma. Small bowel sarcomas respond well to radiotherapy. By analogy to anal squamous cell carcinoma, exclusive chemoradiotherapy provided better outcomes for patients with rectal squamous cell carcinoma. For anal adenocarcinoma, neoadjuvant chemoradiotherapy, followed by radical surgery, was the most effective regimen. For pancreatic neuroendocrine tumors, chemoradiotherapy can be a suitable option as postoperative or exclusive for unresectable/borderline disease. The stereotactic body radiotherapy (SBRT) is a promising approach for hepatobiliary malignancy. Radiotherapy is a valuable option in gastrointestinal stromal tumors (GIST) for palliative intent, tyrosine kinase inhibitors (TKIs) resistant disease, and unresectable or residual disease. Involved field (IF) radiotherapy for digestive lymphoma provides good results, especially for gastric extranodal marginal zone lymphoma (MALT). In conclusion, radiotherapy is not an uncommon indication in this context. A multidisciplinary approach is needed for better management of digestive rare cancers.

Adenoid cystic carcinoma (ACC) is a malignant epithelial tumor that predominantly originates in the salivary glands of the head and neck. However, ACC arising in the thoracic cavity is a rare entity. Diagnosis of primary esophageal ACC (EACC) involves use of gastrointestinal endoscopic ultrasound fine-needle aspiration; however a low diagnostic yield has been reported in the literature. We describe an uncommon presentation of EACC debuting with predominant airway obstructive symptoms and diagnosed through endobronchial ultrasound-guided fine-needle aspiration. We propose the use of an endobronchial sonographic approach to diagnose EACC and other esophageal malignant neoplasms with mediastinal involvement and airway proximity.

Esophageal adenoid cystic carcinoma is a rare cancer that is a challenge to treat because the tumor often invades local structures. Complete resection with grossly negative margins is key to disease-free survival. We describe a case in which the esophageal tumor invaded a significant portion of the posterior trachea, making a tracheal resection with primary anastomosis impossible. Therefore, to resect the tumor completely, we performed a laparoscopic esophagectomy and posterior tracheal resection with tracheoplasty using a rotational flap while the patient was on venovenous extracorporeal membrane oxygenation.

The Fifth edition of the World Health Organization classification of digestive system and American Joint Committee on Cancer staging manual contain substantial refinements of information for esophageal tumors. The epithelial tumors of esophagus are classified as benign, dysplasia, and malignant groups. Dysplasia is divided into Barrett dysplasia and squamous dysplasia and graded into either low-grade or high-grade. Malignant esophageal tumors are often adenocarcinoma or squamous cell carcinoma. The main update in cancer staging in esophageal tumors is the subdivision of the prognostic staging into 3 groups; squamous cell carcinoma, adenocarcinoma, and carcinoma after adjuvant therapy. HER-2 amplification is recognized as a molecular target for therapy of esophagogastric adenocarcinoma. The other esophageal tumors are adenoid cystic carcinoma, mucoepidermoid/adenosquamous carcinoma, undifferentiated carcinoma and neuroendocrine neoplasms. Overall, the incorporation of new data and definitions on histopathology, prognostic factors, and genetics are important for personalized management of patients with esophageal tumors.

Oesophageal and gastro-oesophageal junction (GOJ) neoplasms, and their predisposing conditions, may be encountered by the practicing pathologist both as biopsy samples and as surgical specimens in daily practice. Changes in incidence of oesophageal squamous cell carcinomas (such as a decrease in western countries) and in oesophageal and GOJ adenocarcinomas (such as a sharp increase in western countries) are being reported globally. New modes of treatment have changed our histologic reports as specific aspects must be detailed such as in post endoscopic resections or with regards to post neo-adjuvant therapy tumour regression grades. The main aim of this overview is therefore to provide an up-to-date, easily available and clear diagnostic approach to neoplastic and pre-neoplastic conditions of the oesophagus and GOJ, based on the most recent available guidelines and literature.

The landscape of neoplastic pathology of the oesophagus is dominated by malignancies of epithelial origin, in particular by oesophageal adenocarcinoma and oesophageal squamous cell carcinoma. However, several other histopathological variants can be distinguished, some associated with peculiar histopathological profiles and prognostic behaviours and frequently underrecognized in clinical practice. The aim of this review is to provide a comprehensive characterization of the main morphological and clinical features of these rare variants of oesophageal neoplastic lesions.

Submucosal glands (SMGs) present throughout human esophagus with clusters at either the upper third or lower third of the organ. SMGs tend to atrophy with age, and neoplasms arising in these glands are rare. In order to bring convenience to diagnosis, we summarize the histopathologic characteristics of all esophageal submucosal gland tumors (SGTs). Due to the morphological similarity, the nomenclature of salivary tumors is adopted for SGTs. However, there is great confusion about the definition and histogenesis of these tumors, especially the malignant subtypes. In the literature, esophageal mucoepidermoid carcinoma and adenoid cystic carcinoma usually adjoin the surface squamous epithelium and coexist with intraepithelial neoplasia or invasive squamous cell carcinoma (SCC). In addition, the typical gene alterations of salivary tumors have not been reported in these SGTs. Therefore, we propose to apply stringent diagnostic criteria to esophageal SGTs so as to exclude mimickers that are SCCs with various degree of SMG differentiation.

Adenoid cystic carcinoma (ACC) rarely occurs in the digestive tract, particularly in the gastroesophageal junction.

A 44-year-old male vomiting blood was admitted to our hospital. Endoscopic ultrasound showed a 2.2 × 3.0 cm submucosal tumor in the gastroesophageal junction.

According to the histopathological examination, the tumor was composed predominantly of ductal epithelial and myoepithelial cells. Immunohistochemical staining revealed that the tumor expressed cytokeratin, cluster of differentiation 117, p63, and calponin. Based on these findings, ACC was diagnosed.

Endoscopic submucosal dissection (ESD) was performed to remove the tumor. As the margins of the ESD specimen were positive, the patient underwent total gastrectomy with D2 lymphadenectomy. Finally, neither residual tumor nor lymphatic metastasis was detected in the surgical specimens.

No sign of recurrence has been detected during 36 months of follow-up as of October 2018.

ESD may be an alternative treatment for cardial ACC invading the submucosa.

In the World Health Organization (WHO) classification, adenocarcinoma of esophagus comprises preinvasive type (dysplasia), adenocarcinoma, adenoid cystic carcinoma, adenosquamous carcinoma, and mucoepidermoid carcinoma. For adenocarcinoma, it is important to determine the grading of the cancer and histological variants such as signet ring adenocarcinoma. In the current day management of esophageal adenocarcinoma by neoadjuvant therapy, the histology of the cancer and the lymph nodal status may change after the therapy. Tumor regression grading systems could be used to assess the response to the neoadjuvant therapy in esophageal adenocarcinoma.