Primary Malignant Non-Epithelial Tumours of the Thoracic Oesophagus and Cardia in a 25-Year Surgical Material

Abstract

During 25 years, 708 patients with primary malignant tumours of the thoracic oesophagus (n = 376) or cardia (n = 332) were referred to our department. Two hundred and forty-nine patients had squamous cell carcinoma and 425 adenocarcinoma. The other 34 tumours, which were primarily classified as undifferentiated carcinoma, malignant carcinoid or non-epithelial, were re-evaluated by means of a second microscopic histologic examination and immunohistologic investigation. This showed primary malignant non-epithelial tumours in seven patients (0.99%): two malignant melanomas, one leiomyosarcoma, one malignant fibrous histiocytoma in the oesophagus (1.06%), two malignant lymphomas, and one malignant melanoma in the cardia (0.90%). All but two of the patients with non-epithelial malignant tumours were 67 years of age or older, and oesophagogastrectomy was performed in all. All tumours were 5 cm or more in diameter (median, 8 cm). Distant metastases were found in three cases. Five died of postoperative complications, one of cancer recurrence 7 months after the operation, and one of an unrelated cause without cancer recurrence 16 months postoperatively. Except for two of the melanomas, the diagnosis was not established until histologic examination of the surgical specimen was performed and, for the third melanoma and the malignant fibrous histiocytoma, not until the present re-evaluation. The characteristics of these seven tumours are discussed, and the importance of obtaining a correct diagnosis from endoscopic biopsy specimens is emphasized.

Giant tumor of malignant fibrous histiocytoma (undifferentiated pleomorphic sarcoma) in the cervical esophagus: a case report

“…It was fi rst reported in 1983 [3], and since then only seven cases, including our case, have been reported in the international literature (seven men; age range, 46-78 years; mean age, 60 years) [16][17][18][19][20]. Mean diameter of the tumor was 9.0 cm ( Table 1).…”

Esophageal sarcomas

“…Of these, 13 were women and 11 were polypoid, supporting the observation by Rainer and Brus that women with polypoid tumors may have a better prognosis [15,21]. Further search of the literature uncovered an additional 37 cases, 29 of which were reported in a 1980 review of the Japanese experience that noted a 22% 5-year survival, including four patients alive and well 9-13 years after resection [lo, [16][17][18][19]. Radical esophagectomy has been the treatment of choice, but Choh et al note that simple resection of well-localized lesions can be as effective as radical excision [ 151.…”

Primary malignant melanoma of the esophagus successfully treated by an esophagectomy followed by systemic chemotherapy

“…The combination of an extended radical surgery and DAV (DTIC, ACNU, VCR) therapy has been reported to be associated with a more favorable prognosis than in past reports of PMME [10,22,23]. In some reports, combination therapy included platinum analogues and tamoxifen [22].…”

“…Futhermore, Ohashi et al suggested that an increased number of melanocytes, which is frequently observed in areas with a hyperplastic epithelium and chronic esophagitis, may play an important role as a precursor lesion for malignant melanoma of the esophagus [7]. PMME accounts for 0.1%-0.2% of all the esophageal tumors and about 250 cases have so far been described in the literature [8][9][10]. PMME shows a relatively uniform clinical pattern: patients complain of dysphagia (79.8%), weight loss (37.5%), and substernal or epigastric pain (33.1%).…”