Malignant rhabdoid tumor

Roberts, C.W., Galusha, S.A., McMenamin, M.E., Fletcher, C.D., and Orkin, S.H. (2000) Haploinsufflciency of Snf5 (integrase interactor 1) pre-disposes to malignant rhabdoid tumors in mice. Proc. Natl. Acad. Sci. USA 97, 13796-13800. 

FIGURE 4.33 The histologic image of extrarenal malignant rhabdoid tumor features large cells with eosinophilic cytoplasm, eccentric vesicular nuclei, and prominent nucleoli. 

Tumors that are histologically identical to malignant rhabdoid tumor of the kidney in children may be encountered in a variety of extrarenal sites in adults, including the soft tissues." The hallmarks of these neoplasms are hyaline paranuclear cytoplasmic eosinophilic inclusions eccentric, rounded nuclei with vesicular chromatin prominent nucleoli (Fig. 4.33) and a complex immunophenotype. 

Saito T, Oda Y, Itakura E, et al. Expression of intercellular adhesion molecules in epithelioid sarcoma and malignant rhabdoid tumor. Pathol Int. 2001 51 532-542. 

Shiratsuchi H, Oshiro Y, Saito T, et al. Cytokeratin subunits of inclusion bodies in rhabdoid cells Immunohistochemical and clinicopathological study of malignant rhabdoid tumor and epithelioid sarcoma. Int J Surg Pathol. 2001 9 37-48. 

Argenta PA, Thomas S, Chura JC. Proximal-type epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva A case report, review of the literature, and argument for consolidation. Gynecol Oncol. 2007 107 130-135. 

Perrone T, Swanson PE, Twiggs L, et al. Malignant rhabdoid tumor of the vulva Is distinction from epithelioid sarcoma possible A pathologic and immunohistochemical study. Am J Surg Pathol. 1989 13 848-858. 

Izumi T, Oda Y, Hasegawa T, et al. Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor. Mod Pathol. 2006 19 820-831. 

Malignant rhabdoid tumors of the kidney were described in 1978 by Beckwith and Palmer as highly malignant tumors of infants and children initially thought to represent a variant of Wilms tumor. Similar neoplasms were described in extrarenal sites and in adults. 

Small EJ, Gordon GJ, Dahms BB. Malignant rhabdoid tumor of the heart in an infant. Cancer. 1985 55 2850-2853. 

Balaton AJ, Vaury P, Videgrain M. Paravertebral malignant rhabdoid tumor in an adult A case report of immunocytochemi-cal study. Pathol Res Pract. 1987 182 713-718. 

Dervan PA, Cahalane SF, Kneafsey P, et al. Malignant rhabdoid tumor of soft tissue An ultrastructural and immunohistological study of a pelvic tumour. Histopathol. 1987 11 183-190. 

Parham DM, Peiper S, Robicheaux G, et al. Malignant rhabdoid tumor of the liver Evidence for epithelial differentiation. Arch Pathol Lab Med. 1988 112 61-64. 

Uchida H, Yokoyama S, Nakayama I, et al. An autopsy case of malignant rhabdoid tumor arising from soft parts in the left inguinal region. Acta Pathol Jpn. 1988 38 1087-1096. 

Patron M, Palacious J, Rodriguez-Peralto JL, et al. Malignant rhabdoid tumor of the tongue A case report with immunohistochemical and ultrastructural findings. Oral Surg Oral Med Oral Path. 1988 65 67-70. 

FIGURE 17.26 Malignant rhabdoid tumor consists of sheets of round cells with vesicular nuclei, prominent nucleoli, and variable amounts of eosinophilic cytoplasm with occasional large cytoplasmic eosinophilic globules (hematoxylin-eosin, x200). 

Presence of WTl staining and the EWS-WTl gene fusion distinguish DSRCT from malignant rhabdoid tumor, which is another polyphenotypic small cell malignant neoplasm. 

FIGURE 17.28 Vimentin displays cytoplasmic reactivity in malignant rhabdoid tumor (immunoperoxidase, x400). 

FIGURE 17.33 Absence of staining for INI protein correlates with deletion and mutation of the hSNFS/INII gene in central nervous system, malignant rhabdoid tumor (immunoperoxidase, x400). 

Sigauke E, Rakheja D, Maddox DL, et al. Absence of expression of SMARCBl/INll in malignant rhabdoid tumors of the central nervous system, kidneys and soft tissue an immunohistochemical study with implications for diagnosis. Mod Pathol. 2006 19 717-725. 

Hoot AC, Russo P, Judkins AR, et al. Immunohistochemical analysis of hSNF5dNIl distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol. 2004 28 1485-1491. 

Shiratsuchi H, Saito T, Sakamoto A, et al. Mutation analysis of human cytokeratin 8 gene in malignant rhabdoid tumor a possible association with intracytoplasmic inclusion body formation. Mod Pathol. 2002 15 146-153. 

Uno K, Takita J, Yokomori K, et al. Aberrations of the hSNF5/ INIl gene are restricted to malignant rhabdoid tumors or atypical teratoid/rhabdoid tumors in pediatric solid tumors. Genes Chromosomes Cancer. 2002 34 33-41. 

T-cell lymphoma, ALCL, AML, GIST, breast metaplastic carcinoma, ovarian sex cord stromal tumors, synovial sarcoma, Merkel cell carcinoma, endocrine and neuroendocrine tumors, desmoplastic small round cell tumor, melanoma, nephroblastoma, ependymoma, mesenchymal chondrosarcoma, extrarenal malignant rhabdoid tumor, rhabdomyosarcoma, meningeal... 

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