No abstract available.
Liver ; Rhabdoid Tumor ; Sarcoma

No abstract available.
Adult* ; Humans ; Liver* ; Rhabdoid Tumor*

Malignant rhabdoid tumor (MRT) is a rare but distinctive neoplasm of unknown histogenesis, occurring primarily in children. It has a characteristic histologic pattern and aggressive clinical behavior, and was originally thought to be a malignant sarcomatous variant of Wilms tumor; numerous cases of MRT arising from extrarenal sites have, however, been reported. We describe the radiologic findings of two cases of malignant extrarenal rhabdoid tumor that arose in the pelvic paravertebral region of two children. Both were confirmed by surgical excision and pathologic examination.
Child ; Humans ; Rhabdoid Tumor* ; Wilms Tumor

In 1978, the malignant rhabdoid tumor was known as a sarcomatous variant of Wilm's tumor because of its histological similarities to rhabdomyosarcoma. Since then, it has been regarded as a clinico-pathologic entity which commonly occurs in childhood and shows aggressive biologic behavior. However, histologically, identical tumors have been reported in various extrarenal organs and tissues, but have turned out to have different characteristics. Therefore, whether the rhabdoid tumor was a phenotype or an entity became controversial. We report herein, a case of a malignant rhabdoid tumor on the face.
Phenotype ; Rhabdoid Tumor* ; Rhabdomyosarcoma ; Wilms Tumor

In 1978, the malignant rhabdoid tumor was known as a sarcomatous variant of Wilm's tumor because of its histological similarities to rhabdomyosarcoma. Since then, it has been regarded as a clinico-pathologic entity which commonly occurs in childhood and shows aggressive biologic behavior. However, histologically, identical tumors have been reported in various extrarenal organs and tissues, but have turned out to have different characteristics. Therefore, whether the rhabdoid tumor was a phenotype or an entity became controversial. We report herein, a case of a malignant rhabdoid tumor on the face.
Phenotype ; Rhabdoid Tumor* ; Rhabdomyosarcoma ; Wilms Tumor

Malignant rhabdoid tumor is a clinically aggressive neoplasm that was initially described as a distinctive renal tumor of childhood. But among the malignant rhabdoid tumors, extrarenal rhabdoid tumor is rare. We report an extrarenal neoplasm histologically and ultrastructurally identical to renal rhabdoid tumor that arose in the retroperitoneum of a 4-month-old boy and presented as a right lower abdominal mass. The tumor had an aggressive clinical course despite multimodal therapeutic regimens, and the patient died with disseminated disease 12 months after diagnosis.
Diagnosis ; Humans ; Infant ; Male ; Rhabdoid Tumor*

Primary CNS rhabdoid tumor is an extremely rare malignant tumor affecting children. Clinical, radiological, and histopathological features of malignant rhabdoid tumor are presented. Following the complete extirpation of a large tumor at the frontoparietal region, a 16-year-old male patient suffered from several local recurrences. The neoplasm is, defined by its histopathological and ultrastructural characteristics and by the pattern of its antigenicity as a malignant rhabdoid tumor(MRT).
Adolescent ; Child ; Humans ; Male ; Recurrence ; Rhabdoid Tumor*

Female ; Humans ; Kidney ; pathology ; Kidney Neoplasms ; diagnosis ; Rhabdoid Tumor ; diagnosis

A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.
Brain Neoplasms/therapy ; Brain Neoplasms/pathology ; Brain Neoplasms/metabolism* ; Case Report ; Child ; Glial Fibrillary Acidic Protein/analysis ; Human ; Immunohistochemistry ; Male ; Rhabdoid Tumor/therapy ; Rhabdoid Tumor/pathology ; Rhabdoid Tumor/metabolism* ; Vimentin/analysis

Malignant rhabdoid tumor (MRT) was first described as a "rhabdomyosarcomatoid" variant of Wilms tumor, but was later identified as a distinct entity. MRTs are extremely rare and highly aggressive neoplasm with poor outcome, occurring mostly in kidneys and central nervous system, less frequently in extrarenal sites. MRTs are referred to as "congenital" when it is diagnosed at or immediately after birth. There are few reports about congenital disseminated MRT at birth. We report a case of congenital malignant rhabdoid tumor presenting as a soft tissue mass in the right arm at birth although the autopsy revealed the systemic disseminated tumors through the whole body with typical microscopic findings.
Arm ; Autopsy ; Central Nervous System ; Humans ; Infant, Newborn ; Kidney ; Parturition ; Rhabdoid Tumor ; Wilms Tumor