A case of a non-anaplastic pleomorphic xanthoastrocytoma (PXA) presenting with leptomeningeal dissemination (LMD) affecting a 9-year-old girl is presented. [ncbi.nlm.nih.gov]

• Pigmented Tumor

  tumor 1: Eosinophilic granular bodies (arrows); 2: Rosenthal fibers; 3: atypical cells with granular or foamy cytoplasm and atypical nuclei with pseudoinclusions (arrowhead) and mitotic figures (arrows); 4: multinuclear giant cells with tumor necrosis [pathologyoutlines.com]

• Subcutaneous Nodule

  The only side effect reported was a transient follicular truncal rash in the first month of administration with fickle subcutaneous nodules, treated with local topical corticosteroids. No ECG changes or/and suspected skin lesions developed. [frontiersin.org]

• Recurrent Headache

  The present case is about a 9-year-old girl who presented with symptoms of recurrent headache, seizures and poor academic performance. [ncbi.nlm.nih.gov]

  A 9-year-old girl presented with the symptoms of recurrent headache, seizures and poor academic performance. [ijpmonline.org]

• Neglect

  Concurrently, the patient developed a severe left hemiparesis with ataxia, hemispatial neglect, and central facial palsy. MRI revealed major disease progression. The patient was subsequently referred to our institution. [n.neurology.org]

• Polyuria

  A 13-year-old Hispanic girl presented with a 1-year history of progressive headaches, polyuria, and generalized fatigue. Findings from the neurological examination were notable only for the presence of papilledema. [ncbi.nlm.nih.gov]

Endocrine workup was unremarkable. [surgicalneurologyint.com]

After beginning vemurafenib at 720 mg twice daily, the patient developed grade 2 diffuse morbilliform eruption with xerosis and follicular prominence at 10 to 14 days, with workup revealing a superficial and deep perivascular lymphocytic infiltrate with [ascopost.com]

Detection of many of the molecular changes that underlie the development of gliomas and other BT can now be done by immunohistochemistry and is getting into the mainstream of the diagnostic workup of BT. [neuropathology-web.org]

• Fibrinoid Necrosis

  Focal fibrinoid necrosis, hyalinization, and a moderate infiltration by lymphocytes and plasma cells were visible in the vessel wall. The tumor cells were in close proximity with adjacent small vessels. [ncbi.nlm.nih.gov]

Treatment[edit] Surgery is often the treatment of choice. Total resection (removal of the tumor) is often possible. [en.wikipedia.org]

We report the second published case of successful treatment with the BRAF inhibitor dabrafenib in a female patient with relapsed anaplastic PXA with a BRAFV600 mutation, and the first published case of dabrafinib treatment following intolerance to vemurafenib [ncbi.nlm.nih.gov]

The Glioma Program also offers families the chance to have their child's tumor molecularly profiled (as long as a biopsy can be taken), which may help identify opportunities for targeted treatment. [childrenshospital.org]

Although a rare and benign tumour type, PXA in the elderly tend to be more malignant, may have the radiological appearance of a malignant tumour and have poor prognosis. [ncbi.nlm.nih.gov]

[…] life (90% are between 0 - 2 years old) Type I may be found in very young children (birth to 2 years of age) Types II and III tend to be found after 2 years of age (Pediatr Dev Pathol 2015;18:504) Sites PPB may arise in the lung, mediastinum and pleura Etiology [pathologyoutlines.com]

Prior studies have shown that TP53 mutations may occur in up to 25% of PXAs, suggesting that PXA may have an etiology similar to diffuse astrocytoma rather than pilocytic astrocytoma. [scinapse.io]

MTS is the most common cause of partial complex epilepsy in adults and is also the most common etiology in young adult patients undergoing surgery. [radiologyassistant.nl]

History/ imaging Most common primary tumors that metastasis to brain: Lung cancer Renal cell carcinoma Breast cancer Melanoma Gastrointestinal tract If there is any uncertainty about etiology, biopsy should be performed ABBREVIATIONS CNS Central nervous [wikidoc.org]

다운로드 (기관인증 필요) 초록보기 Objectives:Preoperative differentiation of the histologic etiology of masses involving the sellar and suprasellar region is very important because it determines the treatment modalities, surgical approaches, and the degree of resection [kiss.kstudy.com]

Abstract To study the clinical characteristics, treatment approach and outcome of pleomorphic xanthoastrocytoma (PXA), patients were identified using the National Cancer Intitute's Surveillance, Epidemiology, and End Results (SEER) database. [ncbi.nlm.nih.gov]

Epidemiology PXA typically develops in children and young adults. It accounts for less than 1% of all astrocytic neoplasms. Localization PXA typically occurs in the superficial cerebral hemispheres, most commonly in the temporal lobe. [prod.wiki.cns.org]

Department of Biostatistics and Epidemiology University of Pennsylvania Philadelphia USA [link.springer.com]

[…] glioproliferative changes associated with focal cortical dysplasia (J Neurooncol 2004;66:17) Lesions with significant mitotic activity (5 mitoses per 10 HPF) or with areas of necrosis may be designated "pleomorphic xanthoastrocytoma with anaplastic features" Epidemiology [pathologyoutlines.com]

It brings together the pathophysiology of disease with detailed clinical guidance on diagnosis and management for the full range of childhood cancers, including aspects important in optimal supportive care. [books.google.com]

Therefore this pathophysiology seems unlikely. Similar to hemorrhage within an arachnoid cyst, trauma can cause a vessel stretched by a tumor mass to tear. Our patient, however, had no history of trauma. [barrowneuro.org]

The discovery of BRAF mutations within a substantial percentage of PXA fosters a clearer understanding of the pathophysiology of these tumors with clear prognostic and therapeutic implications. [read.qxmd.com]

Therefore, we cannot state with certainty whether the imaging parameters represent true pathophysiologic information of the tumor. Thus, further prospective study with strict pathologic validation is recommended. [ajnr.org]

It remains unclear whether adjuvant radiation and chemotherapy are able to prevent progression or dissemination. [ncbi.nlm.nih.gov]

Medication is administered to minimize or prevent seizure activity. Additionally, after surgery, parents should be informed of the risk of seizures, and educated on what to do in the event of a seizure. [en.wikipedia.org]

Knowing what these side effects are can help physicians prepare for and, in some cases, prevent these symptoms from occurring. Progressive or recurrent disease Recurrences can happen with PXAs. [danafarberbostonchildrens.org]