Leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review.

Abstract

Abstract. Leiomyosarcoma constitutes approximately 0.5% of the malignant neoplasias of the esophagus and its association with megaesophagus has not been described. We report on a case of a woman with dysphagia that was slowly progressive from the age of 19 due to chagasic megaesophagus. The woman was subjected to cardiomyotomy at the age of 49. She presented a rapid worsening of the dysphagia due to leiomyosarcoma at the age of 61, and was subjected to subtotal esophagectomy with cervical esophagogastroplasty. She developed pulmonary and hepatic metastases 14 months after surgery and died six months later.Chagasic megaesophagus, like idiopathic achalasia or megaesophagus, is related to an increased risk of epidermoid carcinoma of the esophagus. 1-5 However, we are unaware of any studies showing an association between megaesophagus and leiomyosarcoma. We report herein a case of chagasic megaesophagus who developed leiomyosarcoma of the esophagus. CASE REPORTThe subject was a 61-year-old woman (housewife) who had been living in rural conditions in central Brazil. She stated that she did not smoke or drink alcoholic beverages. She had a history of dysphagia from the age of 19 that became accentuated with time and was referred to as chagasic. After a diagnosis of chagasic megaesophagus was made, she was subjected to cardiomyotomy at the age of 49 and showed a distinct improvement. She maintained an unchanged state until two months before her last hospitalization, a time when the dysphagia became aggravated to a point where she had difficulty even in ingesting liquid alimentation, which was accompanied by significant weight loss and anemia. Results of serologic examinations for Chagas' disease were positive using three different techniques (complement fixation, immunofluorescence, and hemagglutination). Endoscopic examination revealed a vegetating lesion obstructing the passageway through the distal third of the esophagus, which showed dilation. A diagnosis of malignant neoplasia was confirmed by biopsy. Thoraco-abdominal tomography and abdominal ultrasound did not reveal metastases. She was subjected to subtotal esophagectomy with esophagogastroplasty and anastomosis in the lower cervical region.Macroscopic examination of the surgical specimen showed a polypoid tumor occupying the distal third of the esophagus. It measured 9 ϫ 5 ϫ 4.5 cm, was covered by mucosa with dispersed erosions, had an elastic, off-white, striated appearance at cut surfaces, and infiltrated the wall to a depth of up to 0

Association of uterine leiomyoma and Chagas' disease.

“…3 This disease predominantly affects inhabitants of rural areas and generally those of low socioeconomic and cultural level, independent of race, sex, or color. 6 The FMTM Teaching Hospital is a tertiary referral hospital with free public attendance. The individuals served there are normally of low socioeconomic and cultural level; the majority originate from the Triâ ngulo Mineiro (western extremity of the State of Minas Gerais), situated in central Brazil, a region where Chagas' disease is endemic.…”

“…[1][2][3][4][5][6] It has been associated with a greater frequency of malignant neoplasias in Chagas' disease patients than in non-Chagas' disease patients; in a comparison of the tumor sites, a significantly greater frequency of esophagus cancer and uterine cervix in the Chagas group was observed. 3 A revision of necropsies showed that there was greater frequency of carcinomas of the esophagus among Chagas' disease patients with megaesophagus compared with non-Chagas' disease patients.…”

Parasite Infection, Carcinogenesis and Human Malignancy

“…A case report described a patient with chagasic megaesophagus who had developed esophageal leiomyosarcoma (Adad et al, 1999). A case-control study has shown that 27% of women with uterine leiomyoma were serologically positive for CD compared to 16% of controls with other benign gynecological alterations (Murta et al, 2002).…”

Rare Cause of Stricture Esophagus—Sarcoma: A Case Report and Review of the literature

“…Leiomyosarcoma constitutes about 0.5% of the malignant neoplasms of the oesophagus [12]. Oesophageal leiomyosarcoma possibly arises from the muscularis mucosa [3].…”