Malignant rhabdoid tumor of the colon: Report of a case

Abstract

A malignant rhabdoid tumor of the colon is very rare and only three cases have been previously described. A 76-year-old man was admitted to the hospital complaining of epigastralgia. An elastic mass was palpable in the right upper abdomen. A barium enema and endoscopic examination showed a giant gyrate tumor arising from the cecum. Abdominal ultrasonography and a computed tomography scan revealed the tumor to be located in the colon associated with multiple liver metastases and gallbladder stones. A right colectomy and cholecystectomy were thus performed. The tumor was histologically composed of sheets of large round and polygonal nuclei with vesicular chromatin, and abundant acidophilic cytoplasm, often containing hyalin-like inclusion. The cytoplasm was positive for vimentin and neuron-specific enolase, and hyaline globules of the rhabdoid tumor cells stained positive for cytokeratin in some cells. Transmission electron microscopy showed characteristic rhabdoid cells with an aggregation of intermediate filaments. A histologic diagnosis of malignant rhabdoid tumor of the colon was made. The tumor demonstrated several unusual findings for malignant rhabdoid tumors including diploidy by a flow cytometric analysis, and positive nuclear immunohistochemical staining for p53 protein and Ki-67 antigen. We report herein the third known case of a pure colonic rhabdoid tumor.

Rhabdoid Colorectal Carcinomas: Reports of Two Cases

“…Although the histogenesis of rhabdoid tumors is unknown, all reported cases share similar histological, immunohistochemical, and ultrastructural findings 3. The ultrastructural findings of the reported cases showed that the rhabdoid cells had eccentric nuclei containing prominent nucleoli and paranuclear inclusions composed of intermediate filaments of the mesenchymal cytoskeleton 8,9,12,13. Although the percentage of tumor cells with a rhabdoid morphology that are needed in a carcinoma to constitute a composite rhabdoid tumor is still up for debate, the percentage of rhabdoid tumor cells in previously reported cases have been greater than 60% 10,17.…”

“…There is a possibility that these two MSI-high tumors might not be associated with rhabdoid features but instead are associated with their right side colonic locations. The overexpression of the p53 protein, suggesting mutation of the p53 gene, was noted in four out of the 10 reported cases 7,8,11,13. In the remaining 6 cases, p53 protein results were not described.…”

Primary Rhabdoid Tumor of the Gallbladder: Report of a Case

“…In two cases of colonic MRT immunohistochemical p53 staining has been observed. 10,11 On ultrastructural examination, the cytoplasm of rhabdoid cells showed whorls of filaments in paranuclear areas, thus corresponding to the glassy hyaline masses visible under a light microscope. 3,12 Recently, these neoplasms have been shown to consistently demonstrate monosomy of chromosome 22 either with or without a partial deletion of the remaining companion chromosome.…”

“…6,9 In some studies Ki-67 and/or proliferating cell nuclear antigen immunoreactivity is shown to be strongly positive in tumor cells, thus indicating a high proliferative activity. 8,10 The presence of p53 mutation in malignant renal rhabdoid tumor (MRRT) and MERT has yet to be elucidated. In two cases of colonic MRT immunohistochemical p53 staining has been observed.…”

Soft-Tissue Extrarenal Rhabdoid Tumor with a Unique Long-Term Survival

“…Extrarenal rhabdoid tumors have now been reported in many organs, including the brain [9^11], eye [12], heart [13], liver [14], colon [15], tongue [16], bladder [17], prostate [18], vulva [19], uterus [20], skin [21], and soft-tissue from many sites such as the extremities [22], neck, mediastinum, retroperitoneum, pelvis, and paraspinal regions [23]. Some reported cases of extrarenal rhabdoid tumor have the same clinical pattern as their renal counterparts, including young age, early dissemination, and lethal outcome [5].…”