The cantankerous pancreas.

The case reported by Qualia and colleagues is a valuable contribution to the sparse esophageal heterotopic pancreas (HP) literature.1 This entity arises from disturbances in normal embryologic development that create deposits of pancreas throughout the gastrointestinal tract. Consequently, esophageal HP is frequently associated with other foregut abnormalities, including esophageal atresia, tracheoesophageal fistula, duplication cyst, diverticulum, and sequestration. The tumors are submucosal and contain exocrine tissue, endocrine tissue, or a combination of both histologic types. Although nonesophageal HP is found in 0.2–0.5% of patients who have abdominal operations, esophageal HP is extremely rare, with only approximately one dozen cases reported.2-8 In addition to the sites discussed by Qualia and associates, HP has also been found in the mediastinum, lung, liver, gallbladder, bile duct, spleen, fallopian tube, large bowel, mesentery, omentum, umbilicus, tongue, lymph node, and other sites. Although only approximately one third of patients with nonesophageal HP have symptoms, all patients with esophageal HP have been symptomatic.8-11 The presence of symptoms in non-esophageal HP has been related to tumor size, location, and mucosal involvement.9 Diagnostic evaluation traditionally includes both radiographic and endoscopic studies. Computed tomo-graphic scans of gastric HP have not been useful.12-14 Contrast radiographs reveal an intramural lesion with central umbilication in most cases.15 Branching of the central duct within the lesion is sometimes seen. The characteristic endoscopic appearance of HP is a submucosal mass with an intact mucosa and a ductal orifice. In some patients, biopsies are diagnostic. However, the submucosal location often hampers routine biopsy techniques. More recently, esophageal ultrasound (EUS) has been helpful, especially when combined with needle biopsy.4,16-19 This approach allows both anatomic and histologic delineation of the mass and could become the diagnostic modality of choice in the future. Qualia and coworkers describe many possible complications that can occasionally occur with HP. The most important of these is the risk of neoplastic transformation. Adenocarcinoma, solid and papillary tumors, anaplastic cancer, islet-cell adenomas, cystadenocarcinomas, and other cancers have been associated with nonesophageal HP. There have been reports of cancers at most non-esophageal sites, but the frequency of these malignancies is quite low.7,11,20-22 In contrast, the sparse available reports suggest that the risk of malignancy in esophageal HP is approximately 15–20%.3,5,7,8 Although esophageal HP has been found in all age groups, cancers have only occurred in adults. The management of nonesophageal HP is hampered by low clinical suspicion and inaccurate diagno-sis.10,13,23,24 EUS with needle biopsy in elective settings should improve the chances of correct diagnosis and treatment. Minimally invasive resection techniques allow simultaneous diagnosis and management of nonesopha-geal HP.11,18,19,25-28 Therefore, symptomatic patients and patients without a definite diagnosis should undergo limited resection using endoscopic or minimally invasive surgical techniques when possible.11,22,25,27,28 Asymptomatic patients with incidentally found, definitively diagnosed, benign, nonesophageal HP can sometimes be observed.11 In emergent situations where diagnosis is not known, operation should be guided by frozen section to avoid unnecessarily radical resections.11,15 To date, all patients with esophageal HP have been symptomatic. Based upon the limited available data, adult patients with esophageal HP may have a significant risk for malignancy. Consequently, surgical resection should be considered in adults with esophageal HP. If observation is chosen, surveillance endoscopy and endoscopic ultrasound should be undertaken frequently to evaluate for progression. Serial biopsy or EUS needle aspirations should be performed to enhance imaging and facilitate early identification of malignant degeneration. Whether future applications of minimally invasive endoscopic or surgical techniques will be beneficial in esophageal HP remains to be determined.