Surgical Treatment of Congenital Rhabdoid Tumor in a 10-Day-Old Newborn (original) (raw)
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Oral Oncology, 2002
Rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992–2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5–8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.
Infantile Extracranial Rhabdoid Tumor of the Scalp
Case Reports in Medicine, 2021
Extracranial rhabdoid tumor is a rare tumor that can originate in multiple organs, and it is most commonly seen in the kidneys. This tumor has a grave prognosis. We report to the best of our knowledge the first case of infantile scalp extracranial rhabdoid tumor in a 6-month-old male baby who presented with a right parietal scalp mass since the age of 1 month. This mass was initially diagnosed as scalp hemangioma based on clinical and imaging findings. However, this mass was growing fast which necessitated excision. Pathologic examination after excision showed a malignant tumor composed of sheets of rhabdoid cells. Immunohistochemically, this tumor tested positive for vimentin, CD 99, glypican-3, synaptopysin, WT-1, CK, and EMA. INI-1 immunostain was lost in the tumor cells. Subsequently, this tumor was pathologically diagnosed as extracranial scalp rhabdoid tumor. After tumor excision, the patient was referred to pediatric oncology to receive chemotherapy. Experience with scalp ext...
Extrarenal rhabdoid tumours outside the central nervous system in infancy
Pediatric Radiology, 2009
Background Malignant rhabdoid tumours (RT) are increasingly recognized in young children, probably as a consequence of advances in accurate histological diagnosis rather than a true increase in frequency. Although typically presenting as renal tumours in infancy, extrarenal tumours outside the central nervous system (CNS) in children less than 12 months of age are now well recognized, but previous literature on their imaging features is very limited. Objective To demonstrate the imaging features of extrarenal RTs outside the CNS. Materials and methods A retrospective database review was made from 1989 to 2007 of patients diagnosed with extrarenal RT in infancy, i.e. below 12 months of age. Results There were nine patients (six boys and three girls). The age at presentation varied from 1 to 11 months (average 6 months). Tumours were located in the thorax/ mediastinum (n=3), liver (n=3), neck (n=1), shoulder (n=1) and axilla (n=1). The imaging modalities used included US (n=8), CT (n=7) and MRI (n=6). Bone scan was positive in one patient, while metastases at the time of diagnosis occurred in four patients. On MRI the tumours tended to show nonspecific hypointensity on T1-W images and heterogeneous hyperintensity on T2-W images, with heterogeneous enhancement. Conclusion This is the largest radiological series of extrarenal RTs outside the CNS in infancy. In our series no imaging features were found specific to the diagnosis. A tendency towards large size and mediastinal/paravertebral location were noted. A hypodense solid component on CT and a heterogeneous hyperintensity on T2-W MR images suggest that this tumour should be considered in the routine differential diagnosis of soft-tissue tumours in infancy, in addition to rhabdomyosarcoma.
Cancers
Introduction: Malignant rhabdoid tumors (MRT) predominantly affect infants and young children. Patients below six months of age represent a particularly therapeutically challenging group. Toxicity to developing organ sites limits intensity of treatment. Information on prognostic factors, genetics, toxicity of treatment and long-term outcomes is sparse. Methods: Clinical, genetic, and treatment data of 100 patients (aged below 6 months at diagnosis) from 13 European countries were analyzed (2005–2020). Tumors and matching blood samples were examined for SMARCB1 mutations using FISH, MLPA and Sanger sequencing. DNA methylation subgroups (ATRT-TYR, ATRT-SHH, and ATRT-MYC) were determined using 450 k / 850 k-profiling. Results: A total of 45 patients presented with ATRT, 29 with extracranial, extrarenal (eMRT) and 9 with renal rhabdoid tumors (RTK). Seventeen patients demonstrated synchronous tumors (SYN). Metastases (M+) were present in 27% (26/97) at diagnosis. A germline mutation (GL...
The Neuroradiology Journal, 1998
PURPOSE: To describe the imaging and patho logic findings of malignant rhabdo id tumor (MRT), a rare primary brain neoplasm affecting children. METHODS: The CT and/o r MR features , pathologic find ings, and c linical records of three c hi ldren with primary MRT of the brain were retrospectively reviewed. RESULTS: The tumors, large, left-sided cerebral masses , were intraventric ular in two cases. MR images in one patient showed T1-and T 2-weighted signal in tens ity isointense with gray matter. Multiple necrotic/cystic foci were present in all cases, with two showing a patchy pattern of enhancement o n CT and MR. The diagnosis of MRT was documented by ultrastructural and immunohistochemical studies. A ll patients had normal abdom inal CT scans, excluding the possibility of primary rena l rhabdoid tumor metastatic to the bra in. The disease progressed rapidl y in each case, despite surgery, chemotherapy, and cran iospinal irradiation , with serial imaging ev idence of tumor regrowth at the primary site and the development of metastatic satellite lesions. CONCLUSIONS: The diagnosis of primary MRT of the brain can be made only pathologica ll y; however, the nonspecific imaging findings in these cases suggest that MRT should be considered in the differential diagnosis of large chi ldhood intracranial neoplasms. Index terms: Brain neopla sms, in infants and chi ldren; Sa rcoma; Pediatric neuroradiology AJNR 14:107-1 15, Jan/ Feb 1993 Malignant rhabdoid tumor (MRT) is an uncommon childhood neoplasm that typically arises within the kidney. It is characterized by an aggressive clinical course. Since its recognition in 1978 (1), six cases of primary intracranial MRT have been reported (2-7). Only two of these included a description of the imaging findings (6, 7). This report presents three additional