Sarcomas and carcinosarcomas are

rare tumors that can be seen using medical microscopes, accounting for approximately 0.1 to 1.5% of all esophageal

tumors. They present with the symptom of dysphagia, which does not differ from the dysphagia associated with the

more common epithelial carcinoma. Tumors located within the cervical or high thoracic esophagus can cause symptoms

of pulmonary aspiration secondary to esophageal obstruction. Large tumors originating at the level of the tracheal

bifurcation can produce symptoms of airway obstruction and syn­cope by direct compression of the tracheobronchial

tree and heart. The duration of dysphagia and age of the patients af­fected with these tumors are similar to those

with carcinoma of the esophagus.

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A barium swallow usually shows a large polypoid

intialuminal esophageal mass, causing partial obstruction and dilatation of the esophagus proximal to the tumor.

The smooth polypoid nature of the lesion, seen through a medical microscope, although not diagnostic, is

distinctive enough to suggest the presence of a sarcoma rather than the more common ulcerating, stenosing

carcinoma.

Esophagoscopy commonly shows an intraluminal necrotic mass. When biopsy is attempted and

the tissue examined under a medical microscope, it is important to remove the necrotic tis­sue until bleeding is

seen on the tumor’s surface. When this is not done, the biopsy specimen will show only tissue necrosis when it is

examined under a microscope. Even when viable tumor is obtained on biopsy, it has been observed that it cannot be

definitively identified as carcinoma, sar­coma, or carcinosarcoma on the basis of the histology of the portion

biopsied. Biopsy results cannot be totally relied on to identify the presence of sarcoma, and it is often the

polypoid nature of the le­sion, which arouses suspicion that it may be something other than

carcinoma.

Polypoid sarcomas of the esophagus, in contrast to infiltrating carcinomas, remain

superficial to the muscularis propria and are less likely to metastasize to regional lymph nodes. In one series of

14 patients, local extension or tumor metastasis would have pre­vented a potentially curative resection in only

five. Thus the pres­ence of a large polypoid tumor should not deter the surgeon from resecting the

lesion.

Sarcomatous lesions of the esophagus can be divided into epi­dermoid carcinomas

with spindle cell features, such as carcinosar­coma, and true sarcomas that arise from mesenchymal tissue, such as

leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma. Based on current histologic criteria for diagnosis,

fibrosarcoma and rhab­domyosarcoma of the esophagus are extremely rare lesions and may not in fact

exist.

Surgical resection of polypoid sarcoma of the esophagus is the treatment of choice,

since radiation therapy has little success and the tumor remain superficial, with local invasion or distant

metastases occurring late in the course of the disease. As with carcinoma, the absence of both wall penetration and

lymph node metastases is nec­essary for curative treatment. Surgical resection is consequently responsible for the

majority of the reported 5-year survivals. Re­section also provides an excellent means of alleviating the

patient’s symptoms. The surgical technique for resection and the subsequent restoration of the gastrointestinal

continuity is similar to that de­scribed for carcinoma.

style="text-align: justify">It was observed that four of the

eight patients with carci­nosarcoma survived for 5 years or longer. Even though this number is small, it suggests

that resection produces better results in ep­ithelial carcinoma with spindle cell features than in squamous cell

carcinoma of the esophagus. Similarly, with leiomyosarcoma of the esophagus, the same scattered reports exist with

little information on survival. Of seven patients with leimyosarcoma, two died from their disease-one in 3 months

and the other 4 years and 7 months after resection. The other five patients were reported to have survived more

than 5 years.

style="font-size: 10pt; font-family: Arial">It is difficult to evaluate the benefits of resection for leiomyo­

blastoma of the esophagus, due to the small number of reported patients with tumors in this location. Most

leiomyoblastomas occur in the stomach, and 38% of these patients succumb to the cancer in 3 years. Fifty-five

percent of patients with extragastric leiomy­oblastoma also die from the disease, within an average of 3 years.

Consequently, leiomyoblastoma should be considered a malignant lesion and apt to behave like a leiomyosarcoma. The

presence of nuclear hyperchromatism, increased mitotic figures, tumor size larger than 10 cm, and clinical symptoms

of longer than 6 months’ duration are associated with a poor prognosis.

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