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Giant cavernous hemangioma of the distal esophagus treated with esophagectomy

2007; Elsevier BV; Volume: 133; Issue: 6 Linguagem: Inglês

10.1016/j.jtcvs.2007.02.004

1097-685X

Anthony W. Kim, Robert J. Korst, Jeffrey L. Port, Nasser K. Altorki, Paul C. Lee,

Vascular Malformations and Hemangiomas

Giant cavernous hemangiomas are rare tumors of the esophagus. In 1976, Feist, Talley, and Hunt1Feist K. Talley N.A. Hunt D.R. Hemangioma of the esophagus: an unusual cause of upper gastrointestinal bleeding.JAMA. 1976; 235: 1146-1147Crossref PubMed Scopus (6) Google Scholar described an upper esophageal hemangioma as “giant”; it was preoperatively 4 × 7 cm and pathologically 12.0 × 5.0 × 2.5 cm in size. We present the case of an asymptomatic 68-year-old woman who had a giant hemangioma of the distal esophagus. She underwent an esophagectomy with a gastric pull-up via a left thoracotomy. Her recovery was unremarkable and she is doing well at 4 months’ follow-up. A 68-year-old asymptomatic woman was found to have an abnormality on a chest radiograph before cataract surgery. A follow-up computed tomographic (CT) scan demonstrated a large lobulated distal esophageal mass with multiple small calcifications (Figure 1). Endoscopy was negative for mucosal lesion. A positron emission tomographic (PET) scan revealed that the mass had a standardized uptake value of 2.0. A transthoracic biopsy of the lesion demonstrated only blood and fibrinated material. Magnetic resonance imaging (MRI) reidentified the lobulated soft tissue mass in the distal esophagus and described it as a fusiform lesion involving the esophageal wall, causing narrowing of the lumen. Neither mediastinal invasion nor lymphadenopathy was noted. On the basis of the soft tissue signal and the smooth appearance, the lesion was thought to be a large leiomyoma. The patient was brought to the operating room where she underwent a left posterolateral thoracotomy through the sixth intercostal space. The mass was soft, boggy, and very vascular, and it extended from the inferior pulmonary vein to the gastroesophageal junction (Figure 2). There was concern of malignancy owing to the macroscopic appearance of pericardial invasion. The esophagus was mobilized from below the aortic arch to the gastroesophageal junction. Included in this dissection was an en bloc portion of the pericardium that appeared to be invaded. The diaphragm was subsequently opened circumferentially and the stomach was fully mobilized. The stomach was delivered through the hiatus and divided along the lesser curvature. The esophagus was then divided below the aortic arch. The stomach was then tubularized and a handewn anastomosis was then performed with 3-0 polydioxanone sutures (PDS; Ethicon, Inc, Somerville, NJ). The diaphragmatic defect was then reapproximated and the left thoracotomy was closed after placement of a 28F chest tube. Final pathologic examination revealed an 8.5 × 6.5 × 4.0-cm cavernous hemangioma with calcified thrombi, involving the esophagus and proximal stomach. Postoperatively, the patient’s course was unremarkable. On postoperative day 5, she had a barium esophagogram that was negative for an anastomotic leak. She was subsequently advanced to a soft mechanical diet. She was discharged to her home on postoperative day 8. At 4 months’ follow-up the patient was doing well. Generally, patients with cavernous hemangiomas of the esophagus have presented with hematemesis that ranges from self-limiting to life-threatening or with varying degrees of dysphagia.1Feist K. Talley N.A. Hunt D.R. Hemangioma of the esophagus: an unusual cause of upper gastrointestinal bleeding.JAMA. 1976; 235: 1146-1147Crossref PubMed Scopus (6) Google Scholar, 2Ramo O.J. Salo J.A. Bardini R. Nemlander A.T. Farkkila M. Mattila S.P. Treatment of submucosal hemangioma of the esophagus using a simultaneous video-assisted thoracoscopy and esophagoscopy: description of a new minimally invasive technique.Endoscopy. 1997; 29: S27-S28Crossref PubMed Scopus (13) Google Scholar, 3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar, 4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar, 5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar An asymptomatic presentation, however, is not uncommon.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar, 4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar Endoscopically, hemangiomas usually are described as submucosal lesions that appear anywhere along the esophagus, are bluish, compressible, and protruding.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar, 4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar They can also have atypical appearances including a reddish discoloration, a normal-appearing mucosa, or an ulcerated appearance.1Feist K. Talley N.A. Hunt D.R. Hemangioma of the esophagus: an unusual cause of upper gastrointestinal bleeding.JAMA. 1976; 235: 1146-1147Crossref PubMed Scopus (6) Google Scholar Barium esophagogram may initially identify a filling or luminal defect, but CT scans may better delineate the lesion itself. The intact mucosa often associated with this submucosal lesion may be missed on endoscopy even in the presence of a large intraesophageal tumor.1Feist K. Talley N.A. Hunt D.R. Hemangioma of the esophagus: an unusual cause of upper gastrointestinal bleeding.JAMA. 1976; 235: 1146-1147Crossref PubMed Scopus (6) Google Scholar On CT scans, these tumors appear homogeneous with small calcifications or phleboliths.4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar Although endoscopic ultrasound was not performed in this patient, early reports have detailed the usefulness of this procedure in further characterizing this lesion. Sonographically, hemangiomas range from anechoic to hypoechoic to isoechoic and involve the mucosal and submucosal layers with an intact muscularis propria.4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar, 5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar Endoscopic ultrasound can also confirm the absence of continuity with major blood vessels.4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar, 5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar In the patient presented in this report, magnetic resonance imaging and angiography (MRI/MRA) were used preoperatively to help distinguish this lesion. Characteristic MRI findings have included an isointense signal from the mass on T1-weighted images and a high signal on T2-weighted images.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar, 4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar Other reports have described atypical features such as a high-intensity signal from the mass on both T1- and T2-weighted images.4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar In this specific patient, the T1-weighted images demonstrated a predominantly soft tissue type signal. The T2-weighted images demonstrated the soft tissue signal type with areas of small hyperintense signals and small areas of hypointense signals. MRA demonstrated the absence of any dominant vessels extending into or toward the mass. Despite recent advances, arriving at the definitive diagnosis of a hemangioma preoperatively can be elusive when using noninvasive modalities. Endoscopic biopsy of the intraesophageal tumor is commonly used to obtain a preoperative histologic diagnosis. Often a concern of hemorrhage is the reason cited for not performing an endoscopic biopsy, but in reality this is a rare occurrence.5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar In lieu of the more frequently described endoscopic biopsy, a transthoracic biopsy was performed in the patient in this current report. Unfortunately, the tissue obtained in this case yielded an inconclusive result. A review of the current literature does not describe the routine use of transthoracic needle biopsy in the diagnosis of this type of tumor. When preoperative studies are inconclusive, surgical resection is often indicated both for diagnosis and to rule out malignant disease.5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar In this case report, the tumor was noted to be large on preoperative imaging studies and locally invasive on initial exploration. These features suggested a malignant process and therefore a resection was indicated. Additionally, the potential risk of bleeding from the vascular tumor, if left unresected, warranted resection.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar, 5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar The modern literature suggests that endoscopic resection is adequate in the treatment of this tumor. Submucosal esophageal cavernous hemangiomas have been successfully resected by the endoscopic polypectomy or mucosal resection techniques.4Tominaga K. Arakawa T. Ando K. Umeda S. Shiba M. Suzuki N. et al.Oesophageal cavernous haemangioma diagnosed histologically, not by endoscopic procedures.J Gastroenterol Hepatol. 2000; 15: 215-219Crossref PubMed Scopus (14) Google Scholar, 5Cantero D. Yoshida T. Ito M. Suzumi M. Tada M. Okita K. Esophageal hemangioma: endoscopic diagnosis and treatment.Endoscopy. 1994; 26: 250-253Crossref PubMed Scopus (24) Google Scholar Alternatively, other ablative techniques, such as laser therapy, also have been described in the treatment of cavernous hemangiomas.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar These modalities, however, are used in the treatment of lesions that are smaller than the one described in the present patient. Before modern endoscopic instrumentation, limited local resection in the form of a polypectomy or enucleation was the accepted or even favored method of treatment. Approaches to a limited resection have classically been through a thoracotomy.2Ramo O.J. Salo J.A. Bardini R. Nemlander A.T. Farkkila M. Mattila S.P. Treatment of submucosal hemangioma of the esophagus using a simultaneous video-assisted thoracoscopy and esophagoscopy: description of a new minimally invasive technique.Endoscopy. 1997; 29: S27-S28Crossref PubMed Scopus (13) Google Scholar Recently, thoracoscopic removal has also been described in the treatment of these lesions with the simultaneous use of a flexible endoscope.2Ramo O.J. Salo J.A. Bardini R. Nemlander A.T. Farkkila M. Mattila S.P. Treatment of submucosal hemangioma of the esophagus using a simultaneous video-assisted thoracoscopy and esophagoscopy: description of a new minimally invasive technique.Endoscopy. 1997; 29: S27-S28Crossref PubMed Scopus (13) Google Scholar This obviously is possible only if the lesion is small enough for this type of resection. Esophagectomy has rarely been described in the treatment of this lesion, but this is because, in general, the majority of cavernous hemangiomas of the esophagus reported are on the order of 2 to 3 cm.3Shigemitsu K. Naomoto Y. Yamatsuji T. Ono K. Aoiki H. Haisa M. et al.Esophageal hemangioma successfully treated by fulguration using potassium titanyl phosphate/yttrium aluminum garnet (KTP/YAG) laser: a case report.Dis Esoph. 2000; 13: 161-164Crossref PubMed Scopus (21) Google Scholar The hemangioma described in this report was giant, and concerns of malignancy and potential hemorrhage warranted an esophagectomy.