Abstract

BackgroundUndifferentiated carcinoma of the esophagus with rhabdoid features is a very rare histologic finding that is occasionally associated with the loss of SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1); however, until now, few survey reports of this type of tumor have been published. In this study, we describe a case of esophageal carcinoma with undifferentiated components and rhabdoid features that was exclusively positive for vimentin and SMARCB1 in a patient with prolonged survival.Case presentationA 67-year-old man complained of a stomachache and loss of appetite persisting for 1 month. He was then admitted to the hospital. Diagnostic imaging studies revealed a transdiaphragmatic circular ulcerative tumor of the esophagogastric region. Biopsy specimens showed undifferentiated round cell carcinoma. The patient underwent lower esophageal resection and total gastrectomy with lymph node dissection. Microscopic analysis revealed that most of the primary tumor consisted of large undifferentiated round cells and scattered rhabdoid cells. The tumor invaded the muscular layer in the esophagus and the subserosal layer in the stomach, and metastasis was noted in only one lymph node. Immunohistochemical analysis revealed that the round and rhabdoid cells found in the primary tumor were diffusely positive for SMARCB1 and vimentin. The tumor displayed focal positivity for the anti-pan-cytokeratin antibody AE1/AE3. In the positive lymph node, round undifferentiated carcinoma cells were admixed with squamous carcinoma cells that were positive for cytokeratin 5/6 and 34βE12. The MIB-1 index was 19.7% and 0.5% for the round cells from the primary tumor and epithelial cells from the metastatic lymph node lesion, respectively, and 70.1% for the round cells from the metastatic lymph node lesion. The patient has been alive for 10 years after surgery without tumor recurrence.ConclusionsWe reported a rare case of esophageal carcinoma with undifferentiated components, rhabdoid features, and a good prognosis.

Highlights

  • Undifferentiated carcinoma of the esophagus with rhabdoid features is a very rare histologic finding that is occasionally associated with the loss of SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1); until now, few survey reports of this type of tumor have been published

  • We reported a rare case of esophageal carcinoma with undifferentiated components, rhabdoid features, and a good prognosis

  • Round cell subtypes of undifferentiated carcinoma were differentially subclassified; one of such subclass with neuroendocrine granules has been identified as neuroendocrine carcinoma [6, 7], and lymphocyte-rich undifferentiated carcinoma is referred to as lymphoepithelial carcinoma [8, 9] and is associated with Epstein-Barr virus (EBV) infection [10]

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Summary

Background

Rare undifferentiated carcinoma of the esophagus, which is an aggressive neoplasm that is associated with a high incidence of recurrence and/or metastases and a dismal prognosis [1], is characterized by polypoid or sheet-like growth of undifferentiated tumor cells [2]. Whether there are undifferentiated round cell carcinomas without distinct immunohistochemical features other than diffuse vimentin positivity is unknown. We describe a case of esophageal carcinoma with undifferentiated components and rhabdoid features that was exclusively positive for vimentin and SMARCB1 and associated with prolonged survival. The immunohistochemical analysis revealed that the round cells of the primary lesion were diffusely positive for vimentin, SMARCB1, and CD34 (Fig. 3e–g). A few cells in the primary lesion were positive for antibodies against a broad spectrum of cytokeratins including AE1/AE3 (Fig. 3d) and CAM5.2. The MIB-1 index was 19.7% and 0.5% for the round cells from the primary tumor and epithelial cells from the metastatic lymph node lesion, respectively, and 70.1% for the round cells in the metastatic lymph node lesion

Discussion
Conclusions

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