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A Hidden Cause of Dysphagia
CLINICAL CHALLENGES AND IMAGES IN GI A Hidden Cause of Dysphagia Leila Neshatian and David A. Katzka Division of Gastroenterology and Hepatology, Mayo Clinic Rochester, Rochester, Minnesota
Question: A 78year-old man was referred to our clinic for progressive esophageal dysphagia. He had first noted intermittent difficulty with swallowing solids 6 months before the visit. His symptoms had worsened progressively over the past 2 months to both solids and liquids. He had a 10 pounds loss over this time period. His past medical history was significant for coronary artery disease and history of bypass grafting 15 years before the presentation. He had a remote history of smoking (>30 years ago). There was no pertinent finding in the physical examination. Routine blood tests were unremarkable, except for slightly elevated lactate dehydrogenase at 228. His local evaluation with upper endoscopy revealed a moderate stenosis measuring 6 cm in length 25 cm from the incisors. The mucosa was intact with the inner diameter 1 cm. The mucosal biopsies were normal. The remainder of the esophagus, the entire stomach and duodenum were normal. Barium esophagography (Figure A) showed a 6 cm area of smooth narrowing of the mid esophagus. CT of the chest (Figure B, arrow) showed a circumferential esophageal thickening with soft tissue infiltration involving the esophagus starting at the level of the carina. There were slightly enlarged lymph nodes in the adjacent mediastinum. He underwent endoscopic ultrasonography (EUS), which confirmed the diffuse esophageal wall thickening. Yet again the mucosal biopsies and a lymph node fine needle aspiration (FNA) were negative. At this point, the patient was referred to our institution. What is your diagnosis? Look on page 550 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. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.05.048
Gastroenterology 2015;149:549–550
CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3 (page 549): Primary Esophageal Lymphoma Repeat EUS demonstrated diffuse thickening of the deep mucosa, submucosa, muscularis propria and adventitia with loss of normal wall layer pattern (Figure C). There was no lymphadenopathy. Multiple FNAs from the esophageal wall demonstrated positive immunostaining for CD20, CD10, and PAX-5 and showed scattered CD3þ T cells in the background. PET/CT showed marked increased fluorodeoxyglucose uptake was noted in the mid esophagus wall as well as retrocrural lymph nodes, paraspinal musculature and thoracic spine from T4 to T12. The patient was diagnosed with grade 1-2 follicular lymphoma with diffuse growth patterns, stage IVB. Follicular lymphomas, the most common indolent lymphomas in the western countries, are rare in the gastrointestinal (GI) tract and GI involvement is typically seen in the presence of disseminated disease.1 Esophageal lymphomas comprise <1% of all GI lymphomas. The majority of esophageal lymphomas are diffuse large B-cell and mucosa-associated lymphoid tissue lymphomas and esophageal follicular lymphomas are extremely rare.2 EUS has a crucial role in evaluation of submucosal esophageal pathologies. Standard endoscopic biopsy in submucosal infiltrating tumors has no role and the diagnostic yield of bite-on-bite biopsy is unknown. EUS and EUS-guided FNA should be considered in the evaluation of esophageal stricturing diseases. EUS-guided FNA is a safe and effective method in diagnosis of esophageal and mediastinal pathologies.3 GI lymphomas used to be of diagnostic challenge owing to small size of samples obtained endoscopically. In more recent years, with advances in the immunology and cytogenetic techniques, FNA biopsies have been excellent samples providing a high diagnostic yield in GI lymphomas. Given the increasing incidence of lymphomas including the GI lymphomas over the past few decades,2 it is important for the gastroenterologist to be mindful of GI lymphomas and recognize the different patterns of GI involvement and presentation. Diagnosis of follicular lymphomas is made by positive CD20, CD10, and BCL-6 cells with cytoplasmic overexpression of BCL-2. Chemotherapy for follicular lymphoma is only indicated for symptomatic patients. Addition of rituximab to chemotherapy has improved response rate and overall survival.1 Our patient was started on chemotherapy with bendamustine/rituxan with the intention of completing a total of 6 cycles. His symptoms of dysphagia improved significantly after initiation of chemotherapy. He had a repeat PET scan after completion of second cycle of chemotherapy, which showed near complete interval response to chemotherapy.
References 1. 2. 3.
Freedman A. Follicular lymphoma: 2014 update on diagnosis and management. Am J Hematol 2014;89:429–436. Howell JM, Auer-Grzesiak I, Zhang J, et al. Increasing incidence rates, distribution and histological characteristics of primary gastrointestinal non-Hodgkin lymphoma in a North American population. Can J Gastroenterol 2012;26:452–456. Dumonceau JM, Polkowski M, Larghi A, et al. Indications, results, and clinical impact of endoscopic ultrasound (EUS)guided sampling in gastroenterology: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline. Endoscopy 2011;43:897–912.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
550
Question: A 78year-old man was referred to our clinic for progressive esophageal dysphagia. He had first noted intermittent difficulty with swallowing solids 6 months before the visit. His symptoms had worsened progressively over the past 2 months to both solids and liquids. He had a 10 pounds loss over this time period. His past medical history was significant for coronary artery disease and history of bypass grafting 15 years before the presentation. He had a remote history of smoking (>30 years ago). There was no pertinent finding in the physical examination. Routine blood tests were unremarkable, except for slightly elevated lactate dehydrogenase at 228. His local evaluation with upper endoscopy revealed a moderate stenosis measuring 6 cm in length 25 cm from the incisors. The mucosa was intact with the inner diameter 1 cm. The mucosal biopsies were normal. The remainder of the esophagus, the entire stomach and duodenum were normal. Barium esophagography (Figure A) showed a 6 cm area of smooth narrowing of the mid esophagus. CT of the chest (Figure B, arrow) showed a circumferential esophageal thickening with soft tissue infiltration involving the esophagus starting at the level of the carina. There were slightly enlarged lymph nodes in the adjacent mediastinum. He underwent endoscopic ultrasonography (EUS), which confirmed the diffuse esophageal wall thickening. Yet again the mucosal biopsies and a lymph node fine needle aspiration (FNA) were negative. At this point, the patient was referred to our institution. What is your diagnosis? Look on page 550 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. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.05.048
Gastroenterology 2015;149:549–550
CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3 (page 549): Primary Esophageal Lymphoma Repeat EUS demonstrated diffuse thickening of the deep mucosa, submucosa, muscularis propria and adventitia with loss of normal wall layer pattern (Figure C). There was no lymphadenopathy. Multiple FNAs from the esophageal wall demonstrated positive immunostaining for CD20, CD10, and PAX-5 and showed scattered CD3þ T cells in the background. PET/CT showed marked increased fluorodeoxyglucose uptake was noted in the mid esophagus wall as well as retrocrural lymph nodes, paraspinal musculature and thoracic spine from T4 to T12. The patient was diagnosed with grade 1-2 follicular lymphoma with diffuse growth patterns, stage IVB. Follicular lymphomas, the most common indolent lymphomas in the western countries, are rare in the gastrointestinal (GI) tract and GI involvement is typically seen in the presence of disseminated disease.1 Esophageal lymphomas comprise <1% of all GI lymphomas. The majority of esophageal lymphomas are diffuse large B-cell and mucosa-associated lymphoid tissue lymphomas and esophageal follicular lymphomas are extremely rare.2 EUS has a crucial role in evaluation of submucosal esophageal pathologies. Standard endoscopic biopsy in submucosal infiltrating tumors has no role and the diagnostic yield of bite-on-bite biopsy is unknown. EUS and EUS-guided FNA should be considered in the evaluation of esophageal stricturing diseases. EUS-guided FNA is a safe and effective method in diagnosis of esophageal and mediastinal pathologies.3 GI lymphomas used to be of diagnostic challenge owing to small size of samples obtained endoscopically. In more recent years, with advances in the immunology and cytogenetic techniques, FNA biopsies have been excellent samples providing a high diagnostic yield in GI lymphomas. Given the increasing incidence of lymphomas including the GI lymphomas over the past few decades,2 it is important for the gastroenterologist to be mindful of GI lymphomas and recognize the different patterns of GI involvement and presentation. Diagnosis of follicular lymphomas is made by positive CD20, CD10, and BCL-6 cells with cytoplasmic overexpression of BCL-2. Chemotherapy for follicular lymphoma is only indicated for symptomatic patients. Addition of rituximab to chemotherapy has improved response rate and overall survival.1 Our patient was started on chemotherapy with bendamustine/rituxan with the intention of completing a total of 6 cycles. His symptoms of dysphagia improved significantly after initiation of chemotherapy. He had a repeat PET scan after completion of second cycle of chemotherapy, which showed near complete interval response to chemotherapy.
References 1. 2. 3.
Freedman A. Follicular lymphoma: 2014 update on diagnosis and management. Am J Hematol 2014;89:429–436. Howell JM, Auer-Grzesiak I, Zhang J, et al. Increasing incidence rates, distribution and histological characteristics of primary gastrointestinal non-Hodgkin lymphoma in a North American population. Can J Gastroenterol 2012;26:452–456. Dumonceau JM, Polkowski M, Larghi A, et al. Indications, results, and clinical impact of endoscopic ultrasound (EUS)guided sampling in gastroenterology: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline. Endoscopy 2011;43:897–912.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
550