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A Rare Cause of Dysphagia in an Adult Patient
Clinical Challenges and Images in GI, continued
A Rare Cause of Dysphagia in an Adult Patient Javier Molina-Infante, Gema Vinagre-Rodriguez, and Elisa Martín-Noguerol Unit of Gastroenterology, Hospital San Pedro de Alcantara, Caceres, Spain
Question: A 52-year-old woman was admitted for dysphagia to solids of 3 months’ duration. The patient referred to similar episodes weekly for the last 5 years, which resolved through fasting. Her medical history included profound mental retardation, congenital polysyndactyly, and neck enlargement. On examination, a large, soft, nontender neck mass with a right predominance was palpable; the swelling moved on swallowing. Radiographic examination of the neck revealed a large mass with an air–fluid level behind the trachea, which was initially thought to be a giant Zenker’s diverticulum (Figure A). However, a barium swallow showed an external compression of the upper esophagus from the right-sided mass, besides an outpouching of the right pyriform apex (Figure B, arrow). Computed tomography confirmed the presence of a large right cystic lesion measuring 9 ⫻ 7 ⫻ 6 cm with an air–fluid level, displacing the esophagus in close proximity with it (Figure C). Upper endoscopy was performed subsequently. What is the most likely diagnosis? What is the proper management? Look on page 1534 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2011 by the AGA Institute 0016-5085/$36.00 doi:10.1053/j.gastro.2010.07.061
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GASTROENTEROLOGY Vol. 141, No. 4
Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 1160): Mucinous Cystadenoma of the Mesocolon With High-Grade Dysplasia Microscopic examination showed that the cyst was lined by a tall mucinous columnar epithelium (Figure C) with chronic inflammation and fibrosis of the underlying wall. Stratification of these cells was present in some areas with complex glandular arrangements. Atypia, hyperchromatic nuclei and enlarged nucleoli were also seen (Figure D), but no definite invasion of the wall could be demonstrated in multiple sections. There was no typical gut lining, underlying cellular mesenchymal stroma, or muscle. The colon and the appendix were unremarkable. Mucinous cystadenomas are known to occur in many different organs, such as the ovaries, appendix, pancreas, and retroperitoneum. Cases occurring in the mesocolon are unusual with an uncertain histogenesis.1 Their origin from coelomic metaplasia/ neoplasia in preexisting mesothelial cysts seems to be the most likely explanation.2 Presentation is usually with abdominal distension or a palpable abdominal mass; however, pain, vomiting, diarrhea, and symptoms of gastrointestinal and urinary obstruction have all been reported.3 There is no definitive diagnostic test. However, imaging studies may be of assistance in determining the source and relationship of the cyst. They are characterized by the formation of a large multilocular or, less commonly, unilocular cysts, lined by a single layer of mucin-producing columnar epithelium. On rare occasions, a borderline or invasive component may be present.2 Although extremely rare, mucinous cystadenomas should be considered in the differential diagnosis of intraabdominal cysts. The treatment of choice is the excision “en bloc” to eliminate the risk of infection, recurrence, and malignancy. References 1. Shaco-Levy R, Tsodikov V, Levy I. Mucinous cystadenoma of the ascending colon: a novel presentation. Scand J Gastroenterol 2003;11:1193–1195. 2. Banerjee R, Gough J. Cystic mucinous tumors of the mesentery and retroperitoneum: report of three cases. Histopathology 1988;12:527–532. 3. McEvoy AW, Cahill CJ, Jameson C. Mucinous cystadenoma of the sigmoid mesocolon: a previously unreported abdominal tumour. Eur J Surg Oncol 1997;23:88 –90. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 4 (page 1161): Congenital Fourth Branchial Arch Cyst
Upper endoscopy showed an extrinsic compression 20 cm from the incisors, which had a fistulous gap measuring 1 cm, surrounded by small, multiple, polypoid sessile lesions (Figure D). The endoscope was introduced through this false lumen giving access to a complex multiloculated cavity, fully covered by identical mucosal lesions and containing a whitish, dense fluid (Figure E). Histology of all of these lesions disclosed typical features of papillomas. The diagnosis is congenital fourth branchial arch cyst, fistulized to the esophagus, with papillomatous mucosal transformation. Cysts of the fourth branchial cleft are extremely rare congenital malformations that arise from the apex of the piriform sinus and course through the inferior neck. The case reported has unique features. yet these cysts have a left predominance (94%) and usually present in childhood as recurrent neck abscess or acute suppurative thyroiditis.1 As such, noninfected cysts have been anecdotally reported in adults as cold thyroid nodules or cervical noninfectious masses.2 Imaging techniques may show a thin-walled cyst with fluid inside, beside an outpouching of the pyriform apex.1 Surgical excision is the treatment of choice. The cyst should be traced to its origin in the aerodigestive system and removed. Partial thyroidectomy may be necessary to gain adequate exposure of the pyriform sinus.1,2 Cysts are usually covered with squamous epithelium (75%), although respiratory, thyroid, thymic, and parathyroid tissues have been rarely reported. Papillomatosis, which is characterized by benign, nonkeratinizing hypertrophy of all squamous
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layers, has been related to chemical and mechanical irritation.3 Therefore, it is conceivable to speculate that this incidental finding during endoscopic examination of the cyst was likely associated with chronic passage of food through the esophageal fistula. References 1. Nicoucar K, Giger R, Pope HG Jr, et al. Management of congenital fourth branchial arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009;44:1432–1439. 2. Shrime M, Kacker A, Bent J, et al. Fourth branchial complex anomalies: a case series. Int J Pediatr Otorhinolaryngol 2003;67:1227–1333. 3. Neumann H, Kuester D, Mönkemüller K. Atypical esophagitis. Gastroenterology 2009;137:790, 1188. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
A Rare Cause of Dysphagia in an Adult Patient Javier Molina-Infante, Gema Vinagre-Rodriguez, and Elisa Martín-Noguerol Unit of Gastroenterology, Hospital San Pedro de Alcantara, Caceres, Spain
Question: A 52-year-old woman was admitted for dysphagia to solids of 3 months’ duration. The patient referred to similar episodes weekly for the last 5 years, which resolved through fasting. Her medical history included profound mental retardation, congenital polysyndactyly, and neck enlargement. On examination, a large, soft, nontender neck mass with a right predominance was palpable; the swelling moved on swallowing. Radiographic examination of the neck revealed a large mass with an air–fluid level behind the trachea, which was initially thought to be a giant Zenker’s diverticulum (Figure A). However, a barium swallow showed an external compression of the upper esophagus from the right-sided mass, besides an outpouching of the right pyriform apex (Figure B, arrow). Computed tomography confirmed the presence of a large right cystic lesion measuring 9 ⫻ 7 ⫻ 6 cm with an air–fluid level, displacing the esophagus in close proximity with it (Figure C). Upper endoscopy was performed subsequently. What is the most likely diagnosis? What is the proper management? Look on page 1534 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2011 by the AGA Institute 0016-5085/$36.00 doi:10.1053/j.gastro.2010.07.061
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GASTROENTEROLOGY Vol. 141, No. 4
Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 1160): Mucinous Cystadenoma of the Mesocolon With High-Grade Dysplasia Microscopic examination showed that the cyst was lined by a tall mucinous columnar epithelium (Figure C) with chronic inflammation and fibrosis of the underlying wall. Stratification of these cells was present in some areas with complex glandular arrangements. Atypia, hyperchromatic nuclei and enlarged nucleoli were also seen (Figure D), but no definite invasion of the wall could be demonstrated in multiple sections. There was no typical gut lining, underlying cellular mesenchymal stroma, or muscle. The colon and the appendix were unremarkable. Mucinous cystadenomas are known to occur in many different organs, such as the ovaries, appendix, pancreas, and retroperitoneum. Cases occurring in the mesocolon are unusual with an uncertain histogenesis.1 Their origin from coelomic metaplasia/ neoplasia in preexisting mesothelial cysts seems to be the most likely explanation.2 Presentation is usually with abdominal distension or a palpable abdominal mass; however, pain, vomiting, diarrhea, and symptoms of gastrointestinal and urinary obstruction have all been reported.3 There is no definitive diagnostic test. However, imaging studies may be of assistance in determining the source and relationship of the cyst. They are characterized by the formation of a large multilocular or, less commonly, unilocular cysts, lined by a single layer of mucin-producing columnar epithelium. On rare occasions, a borderline or invasive component may be present.2 Although extremely rare, mucinous cystadenomas should be considered in the differential diagnosis of intraabdominal cysts. The treatment of choice is the excision “en bloc” to eliminate the risk of infection, recurrence, and malignancy. References 1. Shaco-Levy R, Tsodikov V, Levy I. Mucinous cystadenoma of the ascending colon: a novel presentation. Scand J Gastroenterol 2003;11:1193–1195. 2. Banerjee R, Gough J. Cystic mucinous tumors of the mesentery and retroperitoneum: report of three cases. Histopathology 1988;12:527–532. 3. McEvoy AW, Cahill CJ, Jameson C. Mucinous cystadenoma of the sigmoid mesocolon: a previously unreported abdominal tumour. Eur J Surg Oncol 1997;23:88 –90. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 4 (page 1161): Congenital Fourth Branchial Arch Cyst
Upper endoscopy showed an extrinsic compression 20 cm from the incisors, which had a fistulous gap measuring 1 cm, surrounded by small, multiple, polypoid sessile lesions (Figure D). The endoscope was introduced through this false lumen giving access to a complex multiloculated cavity, fully covered by identical mucosal lesions and containing a whitish, dense fluid (Figure E). Histology of all of these lesions disclosed typical features of papillomas. The diagnosis is congenital fourth branchial arch cyst, fistulized to the esophagus, with papillomatous mucosal transformation. Cysts of the fourth branchial cleft are extremely rare congenital malformations that arise from the apex of the piriform sinus and course through the inferior neck. The case reported has unique features. yet these cysts have a left predominance (94%) and usually present in childhood as recurrent neck abscess or acute suppurative thyroiditis.1 As such, noninfected cysts have been anecdotally reported in adults as cold thyroid nodules or cervical noninfectious masses.2 Imaging techniques may show a thin-walled cyst with fluid inside, beside an outpouching of the pyriform apex.1 Surgical excision is the treatment of choice. The cyst should be traced to its origin in the aerodigestive system and removed. Partial thyroidectomy may be necessary to gain adequate exposure of the pyriform sinus.1,2 Cysts are usually covered with squamous epithelium (75%), although respiratory, thyroid, thymic, and parathyroid tissues have been rarely reported. Papillomatosis, which is characterized by benign, nonkeratinizing hypertrophy of all squamous
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layers, has been related to chemical and mechanical irritation.3 Therefore, it is conceivable to speculate that this incidental finding during endoscopic examination of the cyst was likely associated with chronic passage of food through the esophageal fistula. References 1. Nicoucar K, Giger R, Pope HG Jr, et al. Management of congenital fourth branchial arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009;44:1432–1439. 2. Shrime M, Kacker A, Bent J, et al. Fourth branchial complex anomalies: a case series. Int J Pediatr Otorhinolaryngol 2003;67:1227–1333. 3. Neumann H, Kuester D, Mönkemüller K. Atypical esophagitis. Gastroenterology 2009;137:790, 1188. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).