Symptoma

Atypical Teratoid Rhabdoid Tumor

RTPS

Several months later the patient presented with disseminated intrathecal disease and ultimately died 42 months after the initial resection. [ncbi.nlm.nih.gov]

  • Hunting

    Hunt SJ, Anderson WD: Malignant rhabdoid tumor of the liver. A distinct clinicopathologic entity. Am J Clin Pathol 94: 645–648, 1990 Google Scholar 24. [doi.org]

    Int J Radiat Oncol Biol Phys 61 : 809–816 Zelefsky MJ, Fuks Z, Hunt M, Liebel SA (2002) High dose intensity modulated radiation therapy for prostate cancer: early toxicity and biochemical outcome in 772 patients. [nature.com]

  • Noncompliance

    One patient received RT after the patient was taken off study for noncompliance; this patient ultimately experienced disease recurrence and died. Of the 11 patients who received conformal RT, two patients have experienced relapse. [doi.org]

    [PUBMED Abstract] Million L, Anderson J, Breneman J, et al.: Influence of noncompliance with radiation therapy protocol guidelines and operative bed recurrences for children with rhabdomyosarcoma and microscopic residual disease: a report from the Children's [cancer.gov]

  • Galactorrhea

    She denied galactorrhea, but her menstruation had ceased 2 months earlier. There was no weakness or convulsion. She was on no medication, and had no significant past medical history and family history was unremarkable. [ncbi.nlm.nih.gov]

  • Euthyroid

    She has been on regular follow-up visits at the outpatient clinic for the last 3 years with no clinical or radiological evidence of recurrence after which a good recovery was noted, with a eucortisolemic and euthyroid state on hormonal replacement therapy [ncbi.nlm.nih.gov]

  • Axillary Mass

    Ellison, Camelia Stefanovici and Sherry Krawitz, Intracranial Atypical Teratoid/Rhabdoid Tumor Presenting as an Axillary Mass: A Case Report and Review of Literature, Pediatric and Developmental Pathology, 17, 2, (122), (2014). [doi.org]

  • Recurrent Vomiting

    The most common presenting symptom was recurrent vomiting with a mean duration of 6 weeks. Nine cases (60%) were infratentorial and cerebrospinal fluid was positive in 2 cases (13.3%) at time of diagnosis. [ncbi.nlm.nih.gov]

  • Transient Blindness

    Constantini, S, Umansky, F, Nesher, R, Shalit, M 1987 Transient blindness following intracranial pressure changes in a hydrocephalic child with a V-P shunt Childs Nerv Syst 3 379 381 Google Scholar 14. [doi.org]

  • Urinary Dribbling

    A 30-month-old African American boy with multiple comorbidities presented with the inability to ambulate, encopresis and urinary dribbling. The MRI showed an intradural extramedullary mass extending downwards from the L3-4 level. [ncbi.nlm.nih.gov]

  • Rabies

    Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult With Long Survival: Case Report and Review of the Literature Mussa Hussain Almalki, Ashjan Alrogi, Abdulkarim Al-Rabie, Sadeq Al-Dandan, Abdullah Altwairgi, Yasser Orz Abstract Atypical [dx.doi.org]

  • Stroke

    Phanthumchinda, K, Intragumtornchai, T, Kasantikul, V 1991 Stroke-like syndrome, mineralizing microangiopathy, and neuroaxonal dystrophy following intrathecal methotrexate therapy Neurology 41 1847 1848 Google Scholar 12. [doi.org]

A metastatic workup was negative for disseminated disease. A transoral biopsy procedure revealed an atypical teratoid/rhabdoid tumor on histological examination. [ncbi.nlm.nih.gov]

[…] gene (the hSNF5/INI1 gene), which maps to the 22q11.2 locus and is a sensitive and specific marker.[ 1 7 22 37 40 ] Immunohistochemical study using antibody against the INI1 gene product or FISH to identify loss of the INI1 locus is the current routine workup [surgicalneurologyint.com]

[…] protein leading to a cytoplasmic localization of the protein or a missense mutation leading on to a nonfunctional expression of the protein in the nucleus (retained nuclear staining). [22], [23] This mutation may have to be confirmed by a cytogenetic workup [ccij-online.org]

  • Hyponatremia

    The most frequently reported significant toxicities included bone marrow suppression; febrile neutropenia; infection; GI (anorexia, mucositis, nausea, vomiting, abdominal pain); electrolyte and hepatic disturbances (hypokalemia, hyponatremia, hypomagnesemia [doi.org]

  • Ischemic Changes

    Oshman, DG 1991 Hemiparesis and ischemic changes of the white matter after intrathecal therapy for children with acute lymphocytic leukemia Cancer 67 2058 2061 Google Scholar 11. [doi.org]

Although improving results are reported for AT/RT using intensive treatment regimens, treatment-related morbidity is considerable in this young patient population. [ncbi.nlm.nih.gov]

Maximal surgical resection, intrathecal chemotherapy, and consolidative autologous PBSCT may improve prognosis and avoid radiation. [ncbi.nlm.nih.gov]

These findings lend support to the hypothesis that rhabdoid tumors in all sites have a common genetic etiology. [ncbi.nlm.nih.gov]

These findings are in favor of a common etiology for rhabdoid tumor and a subset of brain tumors developing in infancy. [doi.org]

Although radiotherapy (RT) historically has been delayed in patients aged METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify 144 patients with ATRT from 1973 to 2008. [ncbi.nlm.nih.gov]

Abbreviations: Cum, cumulative; ATRT, atypical teratoid rhabdoid tumor; SEER, Surveillance, Epidemiology, and End Results database. [dovepress.com]

Keywords: Pediatric, intracranial, atypical teratoid/rhabdoid tumors, surgery, chemotherapy and radiotherapy Abstract: Aims and Objectives: To review the clinical features and current understanding of the epidemiology, biology and management of pediatric [oalib.com]

SMARCA4 genes is required for the diagnosis of atypical teratoid / rhabdoid tumor (ATRT) Tumors with similar morphology but lacking this mutation are classified as CNS embryonal tumor with rhabdoid features ICD coding C71 : malignant neoplasm of brain Epidemiology [pathologyoutlines.com]

Epidemiology [ edit ] Estimated 2-3% of primary CNS tumors in Majority of patients present at age A workshop on ATRT ( PMID 12142780 ) reported that pathologic review of a recent CCG study suggests ATRT may represent a significant proportion of brain [en.wikibooks.org]

Our results suggest that TEAD4 plays a role in the pathophysiology of AT/RT, which represents a new insight into the biology of this aggressive tumor. [scholars.northwestern.edu]

Current paper discusses current views on molecular pathophysiology of the tumor. 2010 The Author(s). European Journal of Neurology 2010 EFNS. [ncbi.nlm.nih.gov]

Smith, Shwetal Mehta, Emad Youssef, Jenny Eschbacher, Kathy Tucker, Laughlin Dawes, Neil Lambie, Elizabeth Algar and Elizabeth Hovey, Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology [doi.org]

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This case report highlights that independent prognostic factors are urgently needed for optimizing treatment stratification and preventing overtreatment. [ncbi.nlm.nih.gov]

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