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Thorascopic Resection of Esophageal Heterotopic Pancreas

2013; Elsevier BV; Volume: 96; Issue: 5 Linguagem: Inglês

10.1016/j.athoracsur.2013.02.061

1552-6259

Debra Lowry, Takman Mack, Brett Partridge, Brian Barbick, Robert M. Marks, Joshua Kindelan,

Gastrointestinal Tumor Research and Treatment

Heterotopic pancreas is normal pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas. Heterotopic pancreatic tissue is a rare congenital anomaly found usually in the stomach, duodenum, or jejunum and is rarely seen in the esophagus. This is a case of heterotopic pancreas found in the esophagus that was removed thorascopically. Heterotopic pancreas is normal pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas. Heterotopic pancreatic tissue is a rare congenital anomaly found usually in the stomach, duodenum, or jejunum and is rarely seen in the esophagus. This is a case of heterotopic pancreas found in the esophagus that was removed thorascopically. Heterotopic pancreas is defined as histologically proven tissue that is not connected to the pancreas. It is most frequently found in the stomach, duodenum, or jejunum. It can has also been found anywhere in the digestive tract, intraabdominally, in the mediastinum, or the thorax [1Temes R.T. Menen M.J. Davis M.S. Pett S.B. Wernly J.A. Heterotopic pancreas of the esophagus masquerading as Boerhaave’s syndrome.Ann Thorac Surg. 2000; 69: 259-261Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. Rarely has it been found in the esophagus, with only 13 cases reported in the literature. Surgical management through a minimally invasive approach has not been previously described, to our knowledge.A 25-year-old man with no significant medical history presented with right upper quadrant and epigastric abdominal pain. Evaluation included a computed tomographic scan that revealed a 4.4-cm mass of unknown cause, closely approximated to the esophagus (Fig 1). Positron emission tomography revealed fluorodeoxyglucose avidity, which aroused concern for a tumor rather than an inflammatory process. An esophagogastroduodenoscopy was performed, which revealed multiple fistulous tracts 3 cm proximal to the gastroesophageal junction (Fig 2), in addition to a nodule in the antrum of the stomach. Biopsy specimens were taken from both areas. The antral biopsy specimen revealed pancreatic heterotopia, and the esophageal biopsy specimen was nondiagnostic. Endoscopic ultrasonography revealed a well-circumscribed mass abutting the aorta and inferior pulmonary vein (Fig 3). Repeated biopsy specimens were obtained and were nondiagnostic. An upper gastrointestinal study showed a submucosal mass in the region of the distal esophagus, demonstrating tiny contrast medium–filled extensions to the mass, without communication into the mass itself or the lung parenchyma. After comprehensive imaging and repeated biopsies, the differential diagnosis remained broad and included both benign and malignant processes.Fig 2Esophagoscopic image demonstrating submucosal mass with presumed fistulae adjacent to the lesion.View Large Image Figure ViewerDownload (PPT)Fig 3Endoscopic ultrasonographic image showing a hypoechoic heterogenous mass abutting the aorta and pleura.View Large Image Figure ViewerDownload (PPT)In the absence of a tissue diagnosis or definitive diagnostic imaging, optimal treatment remained unclear. Our approach was to assume a benign process unless faced with pathologic evidence to the contrary. That said, care was taken to not compromise optimal oncologic surgical principles such as en bloc resection and the use of a specimen-removing bag. To obtain a definitive tissue diagnosis, left video-assisted thoracic surgery (VATS) was performed and revealed a soft, mobile, cystlike structure. Given its benign appearance, and concern for possible intraluminal communication, it was decided to perform a VATS resection rather than incisional or core-needle biopsy. The resection was performed through four 5- to 10-mm incisions. The lesion was low in the chest, so a traction suture in the left hemidiaphragm was necessary for adequate visualization. Dissection of the lesion was somewhat challenging because it was partially embedded within the muscular layer of the esophagus and surrounded by fibrotic tissue. Once the lesion was dissected circumferentially, it was noted to be attached to the esophagus by a broad stalk. There appeared to still be a layer of muscle fibers between the stalk and the mucosa, so division of the stalk with an endo-GIA stapler seemed like a feasible option. The concern in doing this was that if the mucosal tracts did indeed communicate with the mass, then the stapled mucosa that remained would present a risk of leak. Our plan if that were to occur was to ensure satisfactory mucosal closure and to cover the site of resection with an intercostal muscle flap, because a two-layered closure would be compromised by our need to resect a significant portion of the adjacent muscular layer with the specimen. We performed the resection over a lighted 56-Fr bougie to prevent unintentionally entraining mucosa into our staple line and to detect perforation. After resection, intraoperative endoscopy revealed smooth mucosa, with no evidence of disruption or remaining fistulous tracts, making it likely that these findings were actually traction dimples. Pathologic examination demonstrated pancreatic heterotopia with a negative margin of resection at the staple line.The patient had an uneventful recovery. A swallow study performed on postoperative day 3 showed no evidence of leak. He was advanced to a soft diet and discharged home. The patient returned for a 2-month follow-up visit and was tolerating a regular diet with no recurrence of epigastric pain.CommentHeterotopic pancreas is found in 0.6% to 13.6% of autopsies [1Temes R.T. Menen M.J. Davis M.S. Pett S.B. Wernly J.A. Heterotopic pancreas of the esophagus masquerading as Boerhaave’s syndrome.Ann Thorac Surg. 2000; 69: 259-261Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. It is more common in male individuals and has the highest incidence in the fourth to the sixth decades of life. It is uncommon to find heterotopic pancreas in the esophagus. Only 13 cases of esophageal heterotopic pancreas have been reported. Although the condition is mostly asymptomatic, symptoms associated with esophageal heterotopic pancreas include epigastric pain, dysphagia, and upper gastrointestinal bleeding [2Shalaby M. Kochman M.L. Lichtenstein G.R. Heterotopic pancreas presenting as dysphagia.Am J Gastroenterol. 2002; 97: 1046-1049Crossref PubMed Google Scholar]. The most common site is the distal esophagus. It can be associated with other anomalies. Two cases have been associated with malignancy [3Guillou L. Nordback P. Gerber C. Schneider R.P. Ductal adenocarcinoma arising in a heterotopic pancreas situated in a hiatal hernia.Arch Pathol Lab Med. 1994; 118: 568-571PubMed Google Scholar, 4Roshe J. Del Buono E. Domenico D. Colturi T.J. Anaplastic carcinoma arising in ectopic pancreas located in the distal esophagus.J Clin Gastroenterol. 1996; 22: 242-244Crossref PubMed Scopus (32) Google Scholar].Management has varied from observation to more radical surgical procedures such as Ivor-Lewis esophagectomy. Resection by VATS has not previously been described, to our knowledge. Because of the presence of malignancy in previously reported cases, it is prudent to remove the abnormal tissue surgically instead of relying on observation. Diagnosis is often difficult and is usually determined once the specimen is removed in its entirety [5Eisenberger C.F. Gocht A. Knoefel W.T. et al.Heterotopic pancreas: clinical presentation and pathology with review of the literature.Hepatogastroenterology. 2004; 51: 854-858PubMed Google Scholar].In this case, multiple biopsy specimens were nondiagnostic and the imaging studies inconclusive, making preoperative diagnosis impossible. The differential diagnosis included both benign and malignant causes, although the patient’s age argued for a benign cause. Although the lesion looked benign under direct visualization from the left hemithorax, its complete surgical resection was indicated, given the patient’s symptoms, the failure to obtain a tissue diagnosis by less invasive means, and the malignant potential of the lesion. Heterotopic pancreas is defined as histologically proven tissue that is not connected to the pancreas. It is most frequently found in the stomach, duodenum, or jejunum. It can has also been found anywhere in the digestive tract, intraabdominally, in the mediastinum, or the thorax [1Temes R.T. Menen M.J. Davis M.S. Pett S.B. Wernly J.A. Heterotopic pancreas of the esophagus masquerading as Boerhaave’s syndrome.Ann Thorac Surg. 2000; 69: 259-261Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. Rarely has it been found in the esophagus, with only 13 cases reported in the literature. Surgical management through a minimally invasive approach has not been previously described, to our knowledge. A 25-year-old man with no significant medical history presented with right upper quadrant and epigastric abdominal pain. Evaluation included a computed tomographic scan that revealed a 4.4-cm mass of unknown cause, closely approximated to the esophagus (Fig 1). Positron emission tomography revealed fluorodeoxyglucose avidity, which aroused concern for a tumor rather than an inflammatory process. An esophagogastroduodenoscopy was performed, which revealed multiple fistulous tracts 3 cm proximal to the gastroesophageal junction (Fig 2), in addition to a nodule in the antrum of the stomach. Biopsy specimens were taken from both areas. The antral biopsy specimen revealed pancreatic heterotopia, and the esophageal biopsy specimen was nondiagnostic. Endoscopic ultrasonography revealed a well-circumscribed mass abutting the aorta and inferior pulmonary vein (Fig 3). Repeated biopsy specimens were obtained and were nondiagnostic. An upper gastrointestinal study showed a submucosal mass in the region of the distal esophagus, demonstrating tiny contrast medium–filled extensions to the mass, without communication into the mass itself or the lung parenchyma. After comprehensive imaging and repeated biopsies, the differential diagnosis remained broad and included both benign and malignant processes. In the absence of a tissue diagnosis or definitive diagnostic imaging, optimal treatment remained unclear. Our approach was to assume a benign process unless faced with pathologic evidence to the contrary. That said, care was taken to not compromise optimal oncologic surgical principles such as en bloc resection and the use of a specimen-removing bag. To obtain a definitive tissue diagnosis, left video-assisted thoracic surgery (VATS) was performed and revealed a soft, mobile, cystlike structure. Given its benign appearance, and concern for possible intraluminal communication, it was decided to perform a VATS resection rather than incisional or core-needle biopsy. The resection was performed through four 5- to 10-mm incisions. The lesion was low in the chest, so a traction suture in the left hemidiaphragm was necessary for adequate visualization. Dissection of the lesion was somewhat challenging because it was partially embedded within the muscular layer of the esophagus and surrounded by fibrotic tissue. Once the lesion was dissected circumferentially, it was noted to be attached to the esophagus by a broad stalk. There appeared to still be a layer of muscle fibers between the stalk and the mucosa, so division of the stalk with an endo-GIA stapler seemed like a feasible option. The concern in doing this was that if the mucosal tracts did indeed communicate with the mass, then the stapled mucosa that remained would present a risk of leak. Our plan if that were to occur was to ensure satisfactory mucosal closure and to cover the site of resection with an intercostal muscle flap, because a two-layered closure would be compromised by our need to resect a significant portion of the adjacent muscular layer with the specimen. We performed the resection over a lighted 56-Fr bougie to prevent unintentionally entraining mucosa into our staple line and to detect perforation. After resection, intraoperative endoscopy revealed smooth mucosa, with no evidence of disruption or remaining fistulous tracts, making it likely that these findings were actually traction dimples. Pathologic examination demonstrated pancreatic heterotopia with a negative margin of resection at the staple line. The patient had an uneventful recovery. A swallow study performed on postoperative day 3 showed no evidence of leak. He was advanced to a soft diet and discharged home. The patient returned for a 2-month follow-up visit and was tolerating a regular diet with no recurrence of epigastric pain. CommentHeterotopic pancreas is found in 0.6% to 13.6% of autopsies [1Temes R.T. Menen M.J. Davis M.S. Pett S.B. Wernly J.A. Heterotopic pancreas of the esophagus masquerading as Boerhaave’s syndrome.Ann Thorac Surg. 2000; 69: 259-261Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. It is more common in male individuals and has the highest incidence in the fourth to the sixth decades of life. It is uncommon to find heterotopic pancreas in the esophagus. Only 13 cases of esophageal heterotopic pancreas have been reported. Although the condition is mostly asymptomatic, symptoms associated with esophageal heterotopic pancreas include epigastric pain, dysphagia, and upper gastrointestinal bleeding [2Shalaby M. Kochman M.L. Lichtenstein G.R. Heterotopic pancreas presenting as dysphagia.Am J Gastroenterol. 2002; 97: 1046-1049Crossref PubMed Google Scholar]. The most common site is the distal esophagus. It can be associated with other anomalies. Two cases have been associated with malignancy [3Guillou L. Nordback P. Gerber C. Schneider R.P. Ductal adenocarcinoma arising in a heterotopic pancreas situated in a hiatal hernia.Arch Pathol Lab Med. 1994; 118: 568-571PubMed Google Scholar, 4Roshe J. Del Buono E. Domenico D. Colturi T.J. Anaplastic carcinoma arising in ectopic pancreas located in the distal esophagus.J Clin Gastroenterol. 1996; 22: 242-244Crossref PubMed Scopus (32) Google Scholar].Management has varied from observation to more radical surgical procedures such as Ivor-Lewis esophagectomy. Resection by VATS has not previously been described, to our knowledge. Because of the presence of malignancy in previously reported cases, it is prudent to remove the abnormal tissue surgically instead of relying on observation. Diagnosis is often difficult and is usually determined once the specimen is removed in its entirety [5Eisenberger C.F. Gocht A. Knoefel W.T. et al.Heterotopic pancreas: clinical presentation and pathology with review of the literature.Hepatogastroenterology. 2004; 51: 854-858PubMed Google Scholar].In this case, multiple biopsy specimens were nondiagnostic and the imaging studies inconclusive, making preoperative diagnosis impossible. The differential diagnosis included both benign and malignant causes, although the patient’s age argued for a benign cause. Although the lesion looked benign under direct visualization from the left hemithorax, its complete surgical resection was indicated, given the patient’s symptoms, the failure to obtain a tissue diagnosis by less invasive means, and the malignant potential of the lesion. Heterotopic pancreas is found in 0.6% to 13.6% of autopsies [1Temes R.T. Menen M.J. Davis M.S. Pett S.B. Wernly J.A. Heterotopic pancreas of the esophagus masquerading as Boerhaave’s syndrome.Ann Thorac Surg. 2000; 69: 259-261Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. It is more common in male individuals and has the highest incidence in the fourth to the sixth decades of life. It is uncommon to find heterotopic pancreas in the esophagus. Only 13 cases of esophageal heterotopic pancreas have been reported. Although the condition is mostly asymptomatic, symptoms associated with esophageal heterotopic pancreas include epigastric pain, dysphagia, and upper gastrointestinal bleeding [2Shalaby M. Kochman M.L. Lichtenstein G.R. Heterotopic pancreas presenting as dysphagia.Am J Gastroenterol. 2002; 97: 1046-1049Crossref PubMed Google Scholar]. The most common site is the distal esophagus. It can be associated with other anomalies. Two cases have been associated with malignancy [3Guillou L. Nordback P. Gerber C. Schneider R.P. Ductal adenocarcinoma arising in a heterotopic pancreas situated in a hiatal hernia.Arch Pathol Lab Med. 1994; 118: 568-571PubMed Google Scholar, 4Roshe J. Del Buono E. Domenico D. Colturi T.J. Anaplastic carcinoma arising in ectopic pancreas located in the distal esophagus.J Clin Gastroenterol. 1996; 22: 242-244Crossref PubMed Scopus (32) Google Scholar]. Management has varied from observation to more radical surgical procedures such as Ivor-Lewis esophagectomy. Resection by VATS has not previously been described, to our knowledge. Because of the presence of malignancy in previously reported cases, it is prudent to remove the abnormal tissue surgically instead of relying on observation. Diagnosis is often difficult and is usually determined once the specimen is removed in its entirety [5Eisenberger C.F. Gocht A. Knoefel W.T. et al.Heterotopic pancreas: clinical presentation and pathology with review of the literature.Hepatogastroenterology. 2004; 51: 854-858PubMed Google Scholar]. In this case, multiple biopsy specimens were nondiagnostic and the imaging studies inconclusive, making preoperative diagnosis impossible. The differential diagnosis included both benign and malignant causes, although the patient’s age argued for a benign cause. Although the lesion looked benign under direct visualization from the left hemithorax, its complete surgical resection was indicated, given the patient’s symptoms, the failure to obtain a tissue diagnosis by less invasive means, and the malignant potential of the lesion.