miércoles, 25 de julio de 2018

How pleomorphic xanthoastrocytoma brought us together

Presento un artículo escrito por Mónica G. Saenz sobre un muchacho que está recibiendo tratamiento para una xanthoastrocytoma,,,

https://www.mdanderson.org/publications/cancerwise/2018/07/how-pleomorphic-xanthoastrocytoma-brought-us-together.html


How pleomorphic xanthoastrocytoma brought us together

BY MONICA G. SAENZ

My stepson Roberto Saenz was heading to junior prom in 2009 when his brain tumor symptoms began. As he rode in the limo, he started to have a severe headache and began vomiting. His mother, Debbie, took him to the children’s hospital in Corpus Christi, Texas, where doctors diagnosed him with a severe sinus infection.
Growing up, Roberto had had a history of frequent headaches, but they were becoming more painful and medication wasn’t helping. Finally, an MRI revealed a mass at the back of his brain.
In June 2009, Roberto had brain surgery in Corpus Christi and was diagnosed with a brain tumor. He recovered well and was voted prom king his senior year.
A brain tumor recurrence
The next fall, another MRI revealed a small “spot” near the resection site, where his tumor had been removed. Unable to determine if it was scar tissue or tumor, Roberto’s doctors continued to monitor him closely. His headaches began again, and when the MRI showed progression, Roberto had his second brain surgery in May 2011. This time, the pathology confirmed a devastating diagnosis: grade III pleomorphic xanthoastrocytoma (PXA) with anaplastic features, an extremely rare brain cancer.
Roberto’s doctor and our family decided to seek consultation from MD Anderson. I can still hear our hero, Roberto, saying, “Let’s do this.” As a family, we were heartbroken and very emotional, but our warrior was ready to fight.
United as one team
On June 2, 2011, we began a journey together. I’m married to Roberto’s father. With Roberto’s mother, Debbie, her husband, and all of Roberto’s siblings and stepsiblings, our separate families united as one team. We traveled to MD Anderson and met with the Brain and Spine CenterNeuro-Oncology team. Dr. Marta Penas-Prado was straight-forward and gave no promises, but she was very positive and had a game plan. She recommended 30 rounds of radiation therapy and one year of oral chemotherapy (temozolomide), which Roberto began that month.
By the time we got home from Houston, Debbie’s co-workers had planned a fundraiser, and our first Team Superman shirt was in the making. We began sharing his journey on Facebook, and the support has been overwhelming. We’ve mailed team shirts to supporters across the U.S., from Hawaii to Florida.
Brain tumor recurrences
Roberto’s MRIs were stable for almost three years, until a small enhancement appeared in January 2014. Close surveillance was ordered again.
By that summer, Roberto’s doctors agreed a third surgery was needed. The post-op MRI confirmed the tumor was completely removed, but the pathology results showed the cancer had grown more aggressive.
Roberto and I drove home together that day. I asked if he was scared. “No, this is the path God has chosen for me, and I will not give up,” he said. Roberto continued MRIs every three months and restarted oral chemotherapy.
In October 2014, we learned the brain tumor progressed again, but surgery was not an option. Instead, Roberto enrolled in a Phase II clinical trial at MD Anderson. We learned that his pleomorphic xanthoastrocytoma has a BRAF gene mutation, which can increase the growth and spread of cancer. This same mutation is also seen in melanoma. The clinical trial medicine worked for melanoma and we prayed it would also work for Roberto.
Though the tumor was stable for a while, the pressure and excruciating headaches returned by fall 2016. Roberto had three more brain surgeries back to back and received a second round of radiation between surgeries. After another recurrence in January 2018, Roberto began Avastin, an IV chemotherapy that we hope will answer our prayers.
Team Superman
Since the beginning, our small community of San Diego, Texas, has rallied around Roberto. Family and friends have honored him with fundraisers, prayer gatherings, donations and blessings. Some have even taken pictures in their Team Superman shirts on vacation or worn them when we travel to MD Anderson. Kids see Roberto and say, “You’re Superman!”
We know our son is making a difference in ways he doesn’t even realize. Through Roberto, a whole community has come together in faith to pray.
As caregivers we may cry, scream, pull our hair, and feel overwhelmed and heartbroken. But what matters is that our son was willing to accept this path and fight! So we have to pick up the pieces, mend them together and unite for him. With faith, hope and love, we have the strength to be by his side every step of the way, one day at a time.

Request an appointment at MD Anderson online or by calling 1-855-387-7711.

martes, 17 de julio de 2018

Xanthoastrocytoma: dabrafenib, trametinib, and bevacizumab

To our knowledge this is the first time dabrafenib, trametinib, and bevacizumab have been combined to treat a pediatric high grade glioma. This is also the first report of BRAF inhibition in glial LMD. Our experience suggests that targeted therapy with dabrafenib and trametinib can be safely combined with anti-angiogenic therapy and may improve quality of life and survival in patients with LMD associated with high grade PXA. The growing experience with targeted therapy in rare pediatric gliomas may justify a need for a larger clinical trial.

We present a case of a 16-year-old girl diagnosed with a left frontal anaplastic PXA with BRAF V600E mutations and high grade features of necrosis. Following subtotal resection, cranial radiation, and temozolomide chemotherapy her tumor recurred with bulky, nodular LMD throughout the cervical, thoracic, and lumbar spine. She received palliative radiation to the thoracic spine and then started targeted therapy with dabrafenib with a partial radiographic response and then trametinib was added to dabrafenib with sustained response for 5 months. When the leptomeningeal tumor progressed, bevacizumab was added to the dabrafenib and trametinib therapy, and the patient remained stable for an additional 4 months. The combined therapy was very well tolerated

https://academic.oup.com/neuro-oncology/article-abstract/19/suppl_6/vi216/4591299?redirectedFrom=fulltext


viernes, 13 de julio de 2018

Unidad de Terapias avanzadas para cáncer infantil del hospital de La Paz en Madrid

Nos hacemos eco de esta estupenda noticia que significará un mejor tratamiento para la curación del cáncer pediátrico en nuestro país.
La Unidad de Terapias avanzadas está lista para comenzar su trabajo que será un referente en España en la lucha y curación del cáncer.
En septiembre se inaugurará de forma oficial.
En estos enlaces podéis encontrar más información sobre el proyecto:

file:///C:/Users/Francisco%20Javier/Downloads/UNIDAD%20CRIS%20CANCER%20INFANTIL%20LA%20PAZ%20Fundacion%20CRIS%20_Empresas.pdf

http://www.madrid.org/cs/Satellite?cid=1354681944494&language=es&pageid=1354688271439&pagename=HospitalLaPaz%2FCM_Actualidad_FA%2FHPAZ_actualidad




martes, 10 de julio de 2018

Peritumoral Edema Affects the Prognosis in Adult Pleomorphic Xanthoastrocytoma: Retrospective Analysis of 25 Patients

Peritumoral Edema Affects the Prognosis in Adult Pleomorphic Xanthoastrocytoma: Retrospective Analysis of 25 Patients

Highlights

PXA is a very rare glial neoplasm, and the clinical and radiologic features of PXA are not uniform.
Tumor size and peritumoral edema are possible prognostic factors of adult PXA.
Peritumoral edema may be associated with recurrence in PXA WHO grade II.
DWI, PWI, and PET findings failed to predict the clinical behavior and histologic grade of PXA.
PXA showed a high recurrence rate, and thus, close follow-up is needed.

Background

Pleomorphic xanthoastrocytoma (PXA) is a very rare glial neoplasm. The clinical behavior of PXA is not uniform, and the purpose of this study was to investigate the clinical characteristics of adult PXA and identify prognostic factors.

Methods

Twenty-five patients aged 18 years or older who were diagnosed with PXA between 2002 and 2016 were analyzed.

Results

Twenty-one patients (84%) had PXA World Health Organization (WHO) grade II and 4 patients (16%) had anaplastic PXA (WHO grade III). The median overall survival of PXA WHO grade II and III was 97 and 56 months, respectively. The 3-year progression-free survival of PXA WHO grade II and III was 65.1% and 50%, respectively (P = 0.37 and 0.21). Diffusion-weighted imaging, perfusion-weighted imaging, and positron emission tomographyfindings failed to predict the histologic grade of PXA and recurrence of PXA WHO grade II. Tumor size >40 mm and presence of evident peritumoral edema (ePTE) were prognostic factors for poor progression-free survival (hazard ratios, 4.4 and 15.2, respectively; P = 0.03 and 0.01) according to a univariate analysis. In the recurrent and silent PXA grade II groups, ePTE was present in 87.5% and 23.1% of cases, respectively (P <0.01).

Conclusions

The clinical and radiologic features of PXA are not uniform. Tumor size and ePTE are possible prognostic factors for adult PXA. Also, peritumoral edema may be associated with recurrence in PXA WHO grade II. PXA showed a high recurrence rate, and thus close follow-up is needed.
Key words
Peritumoral edema
Pleomorphic xanthoastrocytoma
Recurrence

Abbreviations and Acronyms

ADC
Apparent diffusion coefficient
CI
Confidence interval
DWI
Diffusion-weighted imaging
EOR
Extent of resection
ePTE
Evident peritumoral edema
GIC
Glioblastoma initiating cell
GTR
Gross total resection
HPF
High-power field
HR
Hazard ratio
MRI
Magnetic resonance imaging
OS
Overall survival
PET
Positron emission tomography
PFS
Progression-free survival
PTE
Peritumoral edema
PWI
Perfusion-weighted imaging
PXA
Pleomorphic xanthoastrocytoma
RT
Radiotherapy
STR
Subtotal resection
WHO
World Health Organization