Ann Thorac Surg 2009;88:2019-2021. doi:10.1016/j.athoracsur.2009.05.047
© 2009 The Society of Thoracic Surgeons
Case Reports
A Rare Case of Giant Solitary Fibrous Tumor of the Esophagus
Hui Li, MDa,*,
Bin Hu, MDa,
Tong Li, MDa,
Mulan Jin, MDb,
Jianyu Hao, MDc
a Department of Thoracic Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, People's Republic of China
b Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, People's Republic of China
c Department of Gastroenterology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, People's Republic of China
Accepted for publication May 15, 2009.
* Address correspondence to Dr Li, Department of Thoracic Surgery, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, People's Republic of China (Email: huilee{at}vip.sina.com).
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Abstract
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Giant solitary fibrous tumor of the esophagus is a very rare neoplasm. We herein report a successful surgically treated solitary fibrous tumor of the esophagus. A 49-year-old man presented with a history of difficulty in swallowing and chest pain. The preoperative diagnostic workup, including a computed tomographic chest scan, endoscopy, endoscopic ultrasonography, and barium swallow, demonstrated a giant pedunculated intraluminal mass in the esophagus. The tumor was completely resected through a transthoracic esophagotomy, combined with an intraoperative endoscopy. A microscopic examination and immunohistochemical studies supported the diagnosis of a benign solitary fibrous tumors of the esophagus. The patient remained well with no evidence of recurrence 16 months after surgery.
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Introduction
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Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin that occurs typically in the pleura, but can occur in nonpleural tissue. In extremely unusual cases, SFT may arise from the esophagus. We herein describe this rare case of a giant SFT of the esophagus.
A 49-year-old man with a history of difficulty in swallowing and chest pain for a period of 2 months was referred to our department. A barium swallow showed a distended esophagus with a large and huge mass beginning at the upper esophagus and reaching to the lower part of the esophagus (Fig 1A). A computed tomographic chest scan demonstrated a large intraluminal mass (density, 22 Hounsfield units) that extended throughout the entire esophagus. No direct invasion of adjacent intrathoracic structures was seen (Fig 1B). Endoscopic examination revealed a 15-cm long pedunculated tumor covered by normal esophageal mucosa, originating from the upper third of the esophageal wall and extending to the distal thoracic esophagus (Fig 1C). Endoscopic ultrasonography appeared a hypoechoic intraluminal tumor, with a stalk of 10.9 mm x 17.4 mm that was attached to the esophageal wall. A thin-walled vessel was found in the stalk of the tumor (Fig 1D).
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Fig 1. (A) Barium swallowing image displays the large mass occupying the distended esophageal lumen beginning at the upper esophagus and reaching to the lower part of the esophagus. (B) Computed tomographic image shows a large intraluminally mass extended throughout the entire esophagus. (C) Endoscopic examination shows a 15-cm long pedunculated tumor covered by normal esophageal mucosa. (D) Endoscopic ultrasonography reveals a hypoechoic tumor with a stalk that was attached to the esophageal wall.
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The patient underwent a small right thoracotomy combined with intraoperative endoscopy. The stalk of the tumor was then transected endoscopically by snare electrocoagulation with excellent hemostasis. The tumor was completely removed through vertical esophagotomy. A frozen section suggested a benign mesenchymal tumor.
On gross examination, the mass appeared to be as a well-demarcated, solid tumor (15 cm x 5 cm x 3 cm) with a grey–white surface (Fig 2A). Pathologic examination showed the tumor was lined by squamous epithelium with a highly cellular neoplasm composed of non-atypical spindle or mast cells in collagenous background (Fig 2B). No nuclear polymorphism was noted. Immunohistochemical studies revealed CD34 and vimentin positive. The S-100, desmins, and cytokeratin were negative. The Ki-67 was positive in approximately 1% in tumor cells. On the basis of the previously mentioned results, pathologic evaluation ascertained the presence of a benign SFT of the esophagus.
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Fig 2. (A) Grossly, the mass appeared to be as a well-demarcated, solid tumor (15 cm x 5 cm x 3 cm). (B) Histopathologic examination (hematoxylin and eosin, x200) reveals the characteristic "patternless pattern" with non-atypical spindle or mast cells in collagenous background.
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The patient recovered uneventfully and remained well without evidence of recurrence 16 months after surgery.
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Comment
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Solitary fibrous tumors of the pleura were first described in 1931 by Klemperer and Rabin [1]. Since then, approximately 800 cases of SFTs were described; in 85% to 90% of cases they arose from the visceral pleura on a stalk and project into the pleural cavity in a pedunculated manner, but have also been described in other locations in the body [2–5]. The tumor with an entirely esophageal location is extremely rare [6].
Due to the rarity and nonspecific clinical presentation, this entity is diagnostically challenging. Imaging studies including computed tomographic scan, magnetic resonance imaging, endoscopic ultrasound, and endoscopy, are not specific and can not confirm benignity or malignancy. Preoperative cytology may be inconclusive or misleading. Immunohistochemistry is an important part of the workup in precisely diagnosing SFTs, particularly if the tumor is in a rare location. Immunohistochemically, solitary fibrous tumors typically stain positive for vimentin, and stain negative for cytoplasmic keratins, which are found in mesothelioma. The CD34 antigen, which exists in normal and neoplastic endothelial cells, is present in nearly all occurrences of these tumors, whether they are benign or malignant. These characteristics differentiate it from other esophageal mesenchymal tumors, such as gastrointestinal stromal tumor (CD34-positive, CD117-positive), leiomyomas (CD34-negative, CD117-negative, desmin-positive, SMA-positive), and leiomyosarcoma (CD34-positive, CD117-negative, desmin-positive, SMA-positive). Their clinical presentations are very similar, but their treatments are different. Distinguishing gastrointestinal stromal tumors from esophageal leiomyomas is diagnostically important, because the gastrointestinal stromal tumors have a high risk of malignant behavior. The pathologic features described for the presented case are in keeping with those of a benign SFT of the esophagus. This was confirmed by the favorable course of the patient who was alive and disease-free 16 months after surgery.
Surgery is the mainstay of treatment for pedunculated tumors of the esophagus. Although an SFT is not primarily malignant disease, it seems to be of utmost importance that removal of the tumor with clear margins be achieved so that locoregional recurrence is prevented. Surgical removal through cervical esophagotomy or endoscopic approach was recommended if the base of the tumor is located in the cervical portion of the esophagus [7]. However, these methods are only suitable for smaller, pedunculated tumors. Thoracotomy or video-assisted thoracoscopic surgery may be the preferred mode removal of these unusual tumors if the location and size of the tumor are not feasible for endoscopic or cervical approach. If a malignant or benign extensive intramural tumor had been identified, an esophagectomy is the choice of the treatment. In the present case, we successfully removed the giant pedunculated intraluminal SFT through a small thoracotomy combined with an endoscopic approach. It is so difficult to resect and pull out the tumor through the endoscopic approach because of the larger tumor size and location of the stalk.
In conclusion, SFTs of the esophagus are an extremely rare neoplasm in adult patients. Precise diagnosis is solely based on the correct interpretation of unique pathologic and immunohistochemical features. Aggressive surgical management remains the treatment of choice.
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References
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- Klemperer P, Rabin CB. Primary neoplasm of the pleura: a report of five cases Arch Pathol 1931;11:385-412.
- Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumours in extrathoracic locations: evidence of their comparability to intra-thoracic tumors Am J Surg Pathol 1998;22:1501-1511.[Medline]
- Suster S, Nascimento AG, Miettinen M, Sickel JZ, Moran CA. Solitary fibrous tumors of soft tissue Am J Surg Pathol 1995;19:1257-1266.[Medline]
- Mentzel T, Bainbridge TC, Katenkamp D. Solitary fibrous tumour: clinicopathological, immunohistochemical, and ultrastructural analysis of 12 cases arising in soft tissues, nasal cavity and nasopharynx, urinary bladder and prostate Virchows Arch 1997;430:445-453.[Medline]
- Changku J, Shaohua S, Zhicheng Z, Shusen Z. Solitary fibrous tumor of the liver: retrospective study of reported cases Cancer Invest 2006;24:132-135.[Medline]
- Makino H, Miyashita M, Nomura T, et al. Solitary fibrous tumor of the cervical esophagus Dig Dis Sci 2007;52:2195-2200.[Medline]
- Pham AM, Rees CJ, Belafsky PC. Endoscopic removal of a giant fibrovascular polyp of the esophagus Ann Otol Rhinol Laryngol 2008;117:587-590.[Medline]
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Abstract
Introduction
