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A Case of Small Polypoid Esophageal Carcinoma With Multidirectional Differentiation, Including Neuroendocrine, Squamous, Ciliated Glandular, and Sarcomatous Components.

Squamous cell carcinoma, adenocarcinoma, and undifferentiated carcinoma, including small cell carcinoma, are the major histologic types of primary esophageal carcinoma. Composite malignant tumors of 2 or more different cell types, however, have been reported with some frequency at this site.[1,2] We describe a case of small polypoid esophageal carcinoma containing carcinomatous and sarcomatous components. The former component included neuroendocrine, squamous, and ciliated glandular differentiation. The coexistence of neuroendocrine carcinoma and spindle cell sarcoma in the esophagus is extremely rare. Only one such case has been reported previously.[3] To the best of our knowledge, no cases of ciliated adenocarcinoma in the esophagus have been reported to date. In this article, we describe the clinicopathologic features of the patient and discuss a possible pathogenic mechanisms for this unusual tumor.

REPORT OF A CASE

A 55-year-old man with a history of increasing dysphagia was admitted to our hospital for clinical evaluation. He had smoked about 20 cigarettes a day and had been a heavy drinker (about half a bottle of whisky every day) for 35 years. A barium swallow examination disclosed a small filling defect in the lower third of the esophagus. An endoscopic examination revealed the presence of a small polypoid tumor. Histologic examination of a biopsy specimen obtained from the polypoid tumor revealed a neuroendocrine carcinoma. The patient underwent subtotal esophagectomy with a lymphadenectomy. Thirty-three months after the surgery, the patient died of a myocardial infarction. Routine follow-up examinations until the patient's death had shown no evidence of recurrence. An autopsy was not performed.

PATHOLOGIC FINDINGS

Macroscopic examination of the resected esophagus revealed a smooth-surfaced polypoid tumor with a superficial extension measuring 1.5 x 1.4 x 1.0 cm. The mucosal lesion surrounding the tumor was clearly unstained after the application of Lugol solution. The invasion of the polypoid tumor was limited to the submucosal layer (Figure 1). Histologic examination of the tumor revealed multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and spindle cell sarcomatous components. Each component was found in 60%, 10%, 5%, and 25% of the tumor, respectively (Figure 2). The neuroendocrine carcinoma component, forming the bulk of the polypoid tumor, consisted of nests of undifferentiated carcinoma cells containing small, hyperchromatic nuclei and a moderate amount of amphophilic cytoplasm (Figure 3). The squamous cell carcinoma exhibited a superficial extension. The adenocarcinoma was located in a small region of the polypoid tumor. Within the tubular structure of the adenocarcinoma, ciliated tumor cells were found on the luminal surface (Figure 4). The sarcomatous component consisted of sheets of malignant spindle cells with no specific mesenchymal differentiation (Figure 3). The spindle tumor cells contained moderate amounts of eosinophilic cytoplasm and elongated, hyperchromatic nuclei with tapered or blunt ends. The sarcomatous component mainly occupied the upper region of the polypoid tumor and also had invaded the submucosal layer. Although the sarcoma abutted on the carcinoma in several locations, the demarcation between the 2 regions remained sharp. Lymph node metastasis was not found.

[Figures 1-4 ILLUSTRATION OMITTED]

Immunohistochemical stainings using antibodies against [Alpha]-smooth muscle actin (Dako Corporation, Carpinteria, Calif; 1:100 dilution), chromogranin (Dako; 1:500 dilution), cytokeratin (AE1/3, Signet Laboratories Inc, Dedham, Mass; 1:50/1:5000 dilution), and p53 protein (Nichirei, Tokyo, Japan; 1:1000 dilution) were performed on formalin-fixed, paraffin-embedded sections using the biotin-peroxidase complex technique. The neuroendocrine carcinoma and ciliated adenocarcinoma cells were strongly positive for chromogranin (Figure 5) and cytokeratin. The squamous cell carcinoma showed a positive reaction for cytokeratin, but was negative for chromogranin. The spindle cells of the sarcomatous component were positive for [Alpha]-smooth muscle actin and negative for all other stains. Each component was negative for p53 protein.

[Figure 5 ILLUSTRATION OMITTED]

COMMENT

The main classifications of esophageal carcinoma are squamous cell carcinoma; adenocarcinoma; and undifferentiated carcinoma, including small cell carcinoma.[4] Composite malignant tumors of the esophagus are rare, but the occurrence of neoplasms, such as carcinosarcoma, adenosquamous carcinoma, and undifferentiated carcinoma with squamous or glandular differentiation, is well-recognized.[5-9] Our present case consisted of a small esophageal carcinoma exhibiting multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and sarcomatous components.

Composite malignant tumors containing both carcinomatous and sarcomatous components are known as carcinosarcomas. In previously reported cases of esophageal carcinosarcoma, the sarcomatous component formed the bulk of the tumor, while the epithelial component was mostly squamous cell carcinoma or occasionally adenocarcinoma.[7-9] The coexistence of neuroendocrine carcinoma and spindle cell sarcoma in the esophagus is extremely rare. To our knowledge, only one other case of a polypoid tumor (measuring 2.0 x 1.5 cm) containing neuroendocrine, squamous, glandular carcinomatous, and spindle cell sarcomatous components has been reported.[3] In this previous case, the neuroendocrine component formed the bulk of the tumor, and the spindle cell sarcomatous component showed no specific mesenchymal differentiation. These findings are very similar to those of the present case. However, ciliation in the adenocarcinoma was not described in the previous report, whereas ciliation in the tubular structure of the adenocarcinoma was evident in the present case. Ciliated adenocarcinoma is very rare, but when it does occur it is usually associated with pulmonary[10] or ovarian[11,12] cancer. Chan et al[13] recently reported 5 cases of primary gastric adenocarcinoma with ciliation. They argued that the ciliation in the adenocarcinoma may have occurred as a result of tumor cell metaplasia, although this remains to be verified. To the best of our knowledge, this is fire first report of ciliated adenocarcinoma in the esophagus.

The examination of small or early-stage tumors is especially important when speculating on the histogenesis of a tumor. The present case therefore provides an interesting opportunity to consider the histogenesis of this unusual tumor. The origin of sarcomatous elements in carcinoma has been quite controversial. Recent electron microscopic[14] and immunohistochemical studies[15,16] support the metaplastic concept. The present tumor showed multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and spindle cell sarcomatous features. The independent and simultaneous malignant transformation of 4 different stem cells is difficult to imagine in such a small tumor. Therefore, the tumor in the present case is thought to have derived from a single totipotential stem cell and seems to support a metaplastic growth process.

References

[1.] Takubo K, Sasajima K, Yamashita K, et al. Morphological heterogeneity of esophageal carcinoma. Acta Pathol Jpn. 1989;39:180-189.

[2.] Newman J, Antonakopoulos GN, Darnton J, et al. The ultrastructure of oesophageal carcinomas: multidirectional differentiation: a transmission electron microscopic study of 43 cases. J Pathol. 1992;167:193-198.

[3.] Robertson N, Rahamim J, Smith M. Carcinosarcoma of the oesophagus showing neuroendocrine, squamous and glandular differentiation. Histopathology. 1997;31:263-266.

[4.] Watanabe H, Jass JR, Sobin LH. Histological Typing of Oesophageal and Gastric Tumors. 2nd ed. Berlin, Germany: Springer-Verlag; 1990.

[5.] Attar BM, Levendoglu H, Rhee H. Small cell carcinoma of the esophagus: report of three cases and review of the literature. Dig Dis Sci. 1990;35:145-152.

[6.] Suzuki H, Nagayo T. Primary tumors of the esophagus other than squamous cell carcinoma: histologic classification and statistics in the surgical and autopsied material in Japan. Int Adv Surg Oncol. 1980;3:73-109.

[7.] Wang Z, Itabashi M, Hirota T, et al. Immunohistochemical study of the histogenesis of esophageal carcinosarcoma. Jpn J Clin Oncol. 1992;22:377-386.

[8.] Du Boulay CEH, Isaacson P. Carcinoma of the esophagus with spindle cell features. Histopathology. 1980;3:403-414.

[9.] Orsatti G, Kawahara E, Okada Y, et al. Polypoid adenosquamous carcinoma of the esophagus with prominent spindle cells: report of a case with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med. 1993;117:544-547.

[10.] Spencer H. Pathology of the Lung. Vol 2, 3rd ed. Philadelphia, Pa: WB Saunders; 1977:826, 828.

[11.] Blaustein A. Papillary serous tumors of the ovary: an electron microscopic study. Gynecol Oncol. 1976;4:314-323.

[12.] Fenoglio CM. Overview article: ultrastructural features of common epithelial tumors of the ovary. Ultrastruct Pathol. 1980;1:419-444.

[13.] Chan W, Hui P, Leung K, et al. Gastric adenocarcinoma with ciliated tumor cells. Hum Pathol. 1993;24:1107-1113.

[14.] Balercia G, Bhan AK, Dickersin GR. Sarcomatoid carcinoma: an ultrastructural study with light microscopic and immunohistochemical correlation of 10 cases from various anatomic sites. Ultrastruct Pathol. 1995;19:249-263.

[15.] Eusebi V, Cattani MG, Ceccarelli C, et al. Sarcomatoid carcinomas of the breast: an immunohistochemical study of 14 cases. Prog Surg Pathol. 1989;9:83-99.

[16.] Wick MR, Swanson PE. Carcinosarcoma: current perspective and a historical review of nosological concepts. Semin Diagn Pathol. 1993;10:118-127.

Accepted for publication March 17, 2000.

From the Clinical Laboratory (Drs Kanamoto and Shimoda) and Pathology (Drs Nakanishi and Ochiai) Divisions, the Departments of Internal Medicine (Dr Yamaguchi) and Surgery (Drs Tachimori, Kato, and Watanabe), National Cancer Center Hospital and Research Institute, Tokyo, Japan.

Reprints: Y. Nakanishi, MD, Pathology Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
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Author:Kanamoto, Akira; Nakanishi, Yukihiro; Ochiai, Atsushi; Shimoda, Tadakazu; Yamaguchi, Hajime; Tachimo
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:9JAPA
Date:Nov 1, 2000
Words:1474
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