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Revista Española de Enfermedades Digestivas

versión impresa ISSN 1130-0108

Rev. esp. enferm. dig. vol.104 no.8 Madrid ago. 2012

http://dx.doi.org/10.4321/S1130-01082012000800015 

LETTERS TO THE EDITOR

 

Rare esophageal tumors of mesenchymal origen

Tumor esofágico de origen mesenquimal poco frecuente

 

 


Key words: Sarcoma. Mesenchimal tumor. Esophagus.

Palabras clave: Sarcoma. Tumor mesenquimal. Esófago.


 

 

Dear Editor,

The esophagus is one of the organs of the digestive tract where tumors are very aggressive. They occur in smokers and/or drinkers with clinical dysphagia especially for solids. Most are malignant epithelial adenocarcinoma being the most frequent if located in the distal esophagus and squamous cell carcinoma when they are built in proximal region. Less frequent are tumors of mesenchymal origin.

 

Case report

We present a 77-year-old male, smoker of 50 packs/year, with chronic obstructive pulmonary disease (COPD) and atrial fibrillation. He was admitted to the emergency because of progressive dysphagia for solids of three months duration and, in the last month, with liquid weight loss and anorexia.

Endoscopy detected an exophytic neoformation in the middle third of the esophagus causing six centimeters partial stenosis of the lumen. The scan showed an esophageal tumor without local or distant spread. Bronchoscopy was negative for malignancy, thus ruling invasion of the respiratory tree.

The microscopic study showed a mesenchymal spindle cell proliferation arranged in bundles, with increased vascularity and high mitotic index with abundant necrosis (Fig. 1 A and B).

 

Immunohistochemical analysis was negative for desmin, S-100 protein, c-kit and cytokeratin, and C 117 for cytokeratins AE1/AE3, CAM5, 2 and 5/6.

Therefore, we concluded that our patient had a malignant mesenchymal tumor consistent with a poorly differentiated sarcoma. Was assessed for surgical and oncology, dismissing them for impairment of general condition, with stent placement.

 

Discussion

Sarcomas of the esophagus are very rare, less than 2% of malignant mesenchymal tumors in this location, being leiomyosarcoma and stromal tumors the most frequent (1,2).

Patients are usually males and present with dysphagia. Sometimes, patients complain of respiratory symptoms by invasion of the tracheobronchial tree (3).

Differential diagnosis must be made with B-cell lymphomas, sarcomas (leiomyosarcomas or carcinosarcomas) and metastatic breast carcinoma or melanoma, as well as gastrointestinal stromal tumors, leiomyomas, hamartomas, hemangiomas and granular cell tumors or schwannomas (4,5) (Table I).

 

 

The present case was negative for all immunohistoche-mical markers and fits in the diagnosis of poorly differentiated sarcoma.

Four factors must be taken into account when planning treatment: the clinic, the behavior of the lesion, the patient's condition, and the risk that the surgery is performed.

The treatment of choice is surgery once excluded the presence of local and distant invasion (6-8). However, because few studies have been published and little casuistry has been reported no treatment is protocolized. Attempts have been done to treat advanced disease with chemotherapy and radiotherapy, but with little success. Therefore, most patients benefit from the placement of a palliative esophageal stent for dysphagia as in our case.

 

Cristina Pisabarros-Blanco, Begoña Álvarez-Cuenllas, María García-Alvarado,
Luis Vaquero-Ayala, Santiago Vivas-Alegre and Pedro Linares-Torres

Department of Digestive Diseases. Complejo Asistencial Universitario de León. León, Spain

 

References

1. Raza MA, Mazzara PF. Sarcomatoid carcinoma of esophagus. Arch Pathol Lab Med 2011;135:945-8.         [ Links ]

2. Tomizawa Y, Taniguchi M, Mori M. An unusual case of intraluminally growing esophageal tumor. Diagnosis: Carcinosarcoma of the esophagus. Gastroenterology 2011;141:10-1.         [ Links ]

3. Lokesh V, Naveen T, Pawar YS. Spindle cell sarcoma of esophagus: a rare case presentation. J Cancer Res Ther 2010;6:100-1.         [ Links ]

4. Rubin BP, Fletcher JA, Fletcher CD. Molecular insights into the histogenesis and pathogenesis of gastrointestinal stromal tumors. Int J Surg Pathol 2000;8:5-10.         [ Links ]

5. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD117 (KIT). Mod Pathol 2000;13:1134-42.         [ Links ]

6. Eizaguirre Zarza B, Burgos Bretones JJ. GIST tumors. Rev Esp Patol 2006;39:209-18.         [ Links ]

7. Gaur DS, Kishore S, Saini S, Pathak VP. Carcinosarcoma of the oesophagus. Singapore Med J 2008;49:283-5.         [ Links ]

8. Akagi I, Miyashita M, Makino H, Nomura T, Ohkawa K, Tajiri T. So-called carcinosarcoma of the esophagus: report of a case. J Nihon Med Sch 2008;75:171-4.         [ Links ]

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