Malignant rhabdoid tumor of the liver: Case report and literature review

Abstract

A case of malignant rhabdoid tumor (MRT) occurring as a primary hepatic neoplasm in a 12-month-old Japanese female infant is presented. The patient had a slight fever for 2 weeks and presented with a palpable mass in her left hypochondrial region. After admission, the hepatic artery was embolized due to intra-abdominal hemorrhage arising from the tumor. The patient received chemotherapy with cisplatin, cyclophosphamide and adriacin. Despite treatment, the patient developed dyspnea, pancytopenia and disseminated intravascular coagulation. Rupture of the tumor resulted in death within 3 weeks. A limited abdominal autopsy revealed that the liver weighed 1240 g and was occupied by multiple hemorrhagic and/or necrotic tumor nodules. Histologically, neoplastic cells had an abundant eosinophilic cytoplasm containing paranuclear inclusions, and vesicular nuclei with a centrally located prominent nucleolus. Ultrastructurally, the cytoplasmic inclusions were composed of whorled filaments measuring 10 nm. Immunohistochemically, almost all of the neoplastic cells were positive for vimentin and cytokeratins (CK) 8 and 18, some were positive for CK 7 and 19, while none were positive for CK 1, 10, 13-17 and 20. The tumor cells did not express desmin, myoglobin, and alpha-fetoprotein. We found 18 cases of MRT of the liver published in English language literature and then, adding the present case, we summarized the 19 cases. Hepatic MRT is an uncommon neoplasm. However, it should be considered in the differential diagnosis of an aggressive liver neoplasm in childhood.

INI1 (BAF 47) Immunohistochemistry is an Essential Diagnostic Tool for Children With Hepatic Tumors and Low Alpha Fetoprotein

“…[1][2][3][4] This entity has a grave prognosis with most patients succumbing to their illness, although long-term survival has been reported. 5 Making an accurate diagnosis of MRT remains a challenge.…”

Malignant rhabdoid tumor of the liver: a case report with US and CT manifestation

“…One case underwent liver transplantation. 4 The prognosis in the known 25 patients was generally poor; 15 (60%), 19 (76%), and 21 (84%) patients died by 3, 6, and 12 months after diagnosis, respectively. The median survival time was 2 months (range, 5 days to >6 years).…”

“…2 MRT of the liver was fi rst reported in 1982, 3 and 26 cases have been reported in the English literature since then. [4][5][6][7][8] The clinical characteristics of the previous 27 cases and our present case (15 boys and 11 girls; for 2, gender was not mentioned) are as follows: the median age at presentation was 8 months (range, 2-107 months). Treatment consisted of chemotherapy, surgery, or both.…”

“…Three patients were still living at the time these articles were published; their survival times were 3, 6, and 6 years, respectively. 3,4 If treatment is effective, long-term survival is possible even in cases of metastasis. One of the cases with lymph node metastasis survived for a long time.…”

Primary Malignant Rhabdoid Tumor of the Stomach, a Rare Case Report and Review of Literature

“…Bekwith and Palmer first described it in kidneys of children in 1978 [1]. After that, similar tumors have rarely been reported at several extrarenal sites including the gastrointestinal tract in esophagus [2], stomach [3], small intestine [1], large intestine [4], liver [5], and pancreas [6], most of which have been adenocarcinoma with rhabdoid features, but true malignant rhabdoid tumor with no foci of adenocarcinoma in the GI tract has been extremely rare and to the best of our knowledge less than 10 cases in the stomach have been reported in the English literature [7].…”