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At the Focal Point

Andrew B. Nguyen, MD, Department of Surgery, New Yorkospital, Queens, New York, Farooq Mirza, MD, Division of

Thoracic Surgery, Department of Cardiothoracic Surgery, NewYork Hospital, Queens, New York, New York–Presbyterian Hos-pital, Weill Cornell Medical College, New York, New York,Kaumudi Somnay, MD, Division of Gastrointestinal Medicine,

Primary gastric amyloidosis presenti

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by Rokitansky in 1842 and is now known to result from the de

186 GASTROINTESTINAL ENDOSCOPY Volume 76, No. 1 : 2012

aul C. Lee, MD, Subroto Paul, MD, Division of Thoracic Sur-ery, Department of Cardiothoracic Surgery, New York Hospitalueens, New York–Presbyterian Hospital, Weill Cornell Medicalollege, New York, New York, USA

Department of Medicine, New York Hospital, Queens, New York, http://dx.doi.org/doi:10.1016/j.gie.2012.02.019

CommentaryThe process of embryologic development is indeed complicated. It is a miracle to me that more things don’t go wrong. Herewe have a consequence of faulty development of the posterior division of the primitive foregut, which occurs around thefourth week of gestation and resulted in an esophageal cyst. Esophageal cysts are either lined with epithelium (simple cyst) orcomposed of esophageal muscle and submucosa without epithelial duplication (esophageal duplication). Cysts comprise upto 20% of benign esophageal lesions, and most are diagnosed in childhood. Symptoms are caused by external compression ofadjacent structures. Sixty percent of esophageal cysts occur in the lower third of the esophagus, producing dysphagia as thepredominant symptom, and, if the cyst is posterior in location, cardiac arrhythmias may result; 20% occur in the upper third ofthe esophagus, where compression of the tracheobronchial tree causes dyspnea, and 20% occur in the middle third of theesophagus and usually present with retrosternal chest pain and dysphagia. Diagnosis today is by imaging and endoscopy:barium esophagography and endoscopy reveal extrinsic compression with an intact mucosa; a CT scan shows a fluid-filledcystic structure, and EUS demonstrates a 3- to 5-layer cystic, fluid-filled structure. Because most patients with esophageal cystseventually become symptomatic, cysts should be resected when they are diagnosed. This case nicely illustrates how endo-scopic management may replace more invasive treatment such as thoracotomy. Because esophageal cysts do not communi-cate with the lumen, and these cysts were relatively small, rubber band ligation was appropriate and effective. Khalil Gibransaid “Progress lies not in enhancing what is, but in advancing toward what will be.” In this case, we see elements of both.

Lawrence J. Brandt, MD

Associate Editor for Focal Points

ng as an isolated gastric mass

A 67-year-old man was admitted with melena and anemia(hemoglobin 11 g/dL). His medical history was notable forhypertrophic obstructive cardiomyopathy that had been treated5 years previously with myomectomy and placement of a bio-prosthetic aortic valve. EGD revealed a 2.5- to 3-cm round massat the gastric cardia that was oozing blood from superficialerosions (A). Gastric adenocarcinoma was suspected, but biop-sies revealed amyloidosis (B). CT scan confirmed a mass in theardia with several bilateral pulmonary nodules suggestive ofetastasis (C). Because of our persistent concern about malig-ancy, repeated EGD and biopsies followed by EUS-FNA wereerformed, which confirmed amyloidosis (D). Surgical and he-atologic consultations were obtained. Bone marrow biopsy

howed negative results for amyloidosis or myeloproliferativeiseases, and review of the prior myomectomy biopsy speci-en was again negative for amyloid. Repeated CT of the chest

howed resolution of the lung nodules. The patient declined

spirin and while using acid-suppressive therapy (hemoglobin4 g/dL). However, after undergoing surveillance biopsies 1ear later, the patient experienced bleeding, which could not beontrolled endoscopically (hemoglobin 11 g/dL). Angiographyevealed extravasation of the left gastric artery, which stoppedith embolization. Bleeding resumed the next day, and theatient underwent surgery with local excision of the mass.athologic examination showed amyloid.

ISCLOSUREAll authors disclosed no financial relationships relevant

o this publication.

ene Rivera, MD, Vivek Kaul, MD, FACG, Arthur DeCross,D, AGAF, Department of Gastroenterology & Hepatology,

hrista Whitney-Miller, MD, Department of Pathology, Uni-ersity of Rochester Medical Center, Rochester, New York, USA

urgery and did not have recurrent bleeding while not taking http://dx.doi.org/10.1016/j.gie.2012.02.059

CommentaryI love the amyloid story and retell it here for my own enjoyment and for those who do not know it. Amyloid was first described

position of insoluble proteinaceous material produced in

www.giejournal.org

At the Focal Point

association with a variety of diseases. In the GI tract, amyloid may appear as ulcers, nodules, or polypoid masses, and it mayhave ischemic, malabsorptive, hemorrhagic, or dysmotility presentations. Nodular amyloid is unusual in the GI tract and mostoften is confined to the skin, where it usually involves acral areas, and the lung. Its varied presentations allow amyloid to joinsyphilis, cytomegalovirus, and lupus as another “great imitator.” Amyloid fibrillar protein can be seen on biopsy, but it is Congored staining viewed under polarized light that gives amyloid its characteristic apple-green birefringence. The term “Congored” was introduced at the 1885 Berlin West Africa Conference as the name for the first direct textile dye. At that time, theCongo River basin was an exotic new geopolitical and potentially profitable area; hence the name had marketing cachet, justlike another textile dye with an African name used in medicine, Sudan black. The inventor, Paul Bottiger, subsequently sold hispatent rights for Congo red to a major German textile dyestuff company (AGFA) that ultimately merged with others to form theultimately infamous I.G. Farben Company. Congo red rarely is used today as a textile dye because it stains the fabrics of othergarments when they are washed together. Unfortunately, there is no specific therapy for the disease, and median survival isonly about a year. The take-home message here is to be aware of amyloid as a possibility, even when you see what seems to bea familiar disease pattern, and especially when a patient has associated chronic inflammatory disease, multiple myeloma, ormonoclonal gammopathy or is receiving hemodialysis. We often think of amyloid whenever a large tongue prevents theendoscope from being easily advanced into the posterior pharynx. However, each time we traverse the posterior we shouldremind ourselves to consider Rokitansky’s amyloid.

Lawrence J. Brandt, MD

Associate Editor for Focal Points

www.giejournal.org Volume 76, No. 1 : 2012 GASTROINTESTINAL ENDOSCOPY 187