background: rahbdoid tumor of kidney (rtk) is a childhood rare neoplasm, previously thought as a sarcoma variant of wilms’ tumor, but now is recognized as a distinct pathologic entity. method : we report a pathologically proved case of rtk, associated with brain mass in a 4.5-month-old girl that has referred for gross hematuria. shortly after initiating chemotherapy, because of retractile vomit...

Melanoma with rhabdoid features is an uncommon variant of malignant melanoma. Here, we describe a rare case of primary rhabdoid malignant melanoma. A 54-year-old man presented with a black tumor measuring 3×4 cm on the right forearm. Histologic sections showed a tumor mass with rhabdoid features composed entirely of polygonal neoplastic cells with eccentric nuclei, prominent nucleoli, and large...

We examined 18 atypical teratoid and rhabdoid tumors of the brain and 7 renal and 4 extrarenal rhabdoid tumors for mutations in the candidate rhabdoid tumor suppressor gene, INI1. Fifteen tumors had homozygous deletions of one or more exons of the INI1 gene, and the other 14 tumors demonstrated mutations. Germ-line mutations of INI1 were identified in four children, one with an atypical teratoi...

central nervous system atypical teratoid/rhabdoid tumor during infancy is a rare, highly aggressive tumor most commonly seen in the cerebellar area. herein we describe the case of a 4-month-old baby who presented with convulsions. pathologic examination of her cerebellar mass showed an atypical teratoid/rhabdoid tumor. the patient died 5 days after surgery despite complete excision of the mass ...

SMARCB1 is deleted in rhabdoid tumor, an aggressive paediatric malignancy affecting the kidney and CNS. We hypothesized that the oncogenic pathway in rhabdoid tumors involved epigenetic silencing of key cell cycle regulators as a consequence of altered chromatin-remodelling, attributable to loss of SMARCB1, and that this hypothesis if proven could provide a biological rationale for testing epig...

Malignant rhabdoid tumor (MRT) is one of the most aggressive and lethal malignancies in pediatric oncology. Malignant rhabdoid tumor was initially described in 1978 as a rhabdomyosarcomatoid variant of a Wilms tumor because of its occurrence in the kidney and because of the resemblance of its cells to rhabdomyoblasts. The absence of muscular differentiation led Haas and colleagues to coin the t...

extra renal extra cranial malignant rhobdoid tumors (mrt) are rare, frequently lethal and affect mostly children. no definite treatment has been made and the role of radiation therapy is poorly defined. this report explains a 14 year old girl with mrt in her neck soft tissue. she is alive with no evidence of disease 18 months after diagnosis. through the experience with this case, we suggested ...

Malignant rhabdoid tumor was first discovered in the kidney, and rhabdoid tumor of the lung was first reported in 1995. These were included as the variants of large-cell carcinoma, according to the 1999 World Health Organization classification of lung tumors. The rhabdoid tumor of the lung exhibits aggressive biological behavior and has a poor prognosis, and only a few reports of this tumor exi...

Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm in infants and young children. We report a 6 year-old girl with atypical teratoid/rhabdoid tumor. Based on cerebrospinal fluid examination MRI scan and family history of tuberculosis; we diagnosed tuberculous meningitis. There was inadequate response to the antituberculosis therapy; so we performed ste...