NCCN Guidelines
® Updates: Soft Tissue Sarcoma .
Volume 14 //Number 6 // June 2016 .
The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for
Soft Tissue Sarcoma published in this issue (page 754) include the latest updates.
To assist readers interested in noting how the guidelines were updated, highlights
of major changes pertaining to the abbreviated version published in this issue
are printed below. To view the complete list of updates and full version of the
guidelines, visit the NCCN Web site at NCCN.org.
Updates in Version 2.2016 of the NCCN Guidelines for Soft Tissue Sarcoma
from Version 1.2015 include:
SARC-B (1 of 3)
• The following translocation has been modified:
! Embryonal RMS:
♦ Aberration: Complex alterations; Gene(s) involved: Multiple,
MYOD1 mutation
• Footnote 2 has been modified: “This table is not exhaustive for either
sarcomas with characteristic genetic changes or the genes involved. For
example, additional genetic aberrations found in alveolar RMS including PAX3-
NCOA1, PAX3-NCOA2, and PAX3-INO80D. CIC-DUX4 fusion is
present in primitive round or short spindle cell sarcomas, resulting from
translocation of t(4;19)(q35;q13) or t(10;19)(q26;q13). It is not clear
if this is an entirely new subtype of sarcoma or a new subtype of Ewing
sarcoma. BCOR-CCNB3 fusion is considered Ewing-like sarcoma. NCOA2
gene rearrangements and MyoD mutation have recently been identified in
spindle cell RMS. MIR143-NOTCH fusion has recently been identified
in glomus tumor. Receptor tyrosine kinase/RAS/PIK3CA aberrations are
found in 93% of RMS cases. Loss of TSC1 (9q34) or TSC2 (16p13.3)
(mTOR pathway) or gene fusions of the TFE3 gene (microphthalmia-associated
transcription factor family) have been identified in PEComa. MPNST is
associated with loss of SUZ12/EED and alteration of NF1 and CDKN2A.
Consultation with a pathologist who has expertise in sarcoma diagnosis
and molecular diagnostic techniques should be obtained prior to testing.”
SARC-D (1 of 4)
• “These guidelines are intended to treat the adult population. For adolescent and
young adult patients, refer to the NCCN Guidelines for Adolescent and Young
Adult (AYA) Oncology” is a new footnote.
SARC-E (1 of 6)
• Trabectedin was added as a single agent for soft tissue sarcoma subtypes with
non-specific histologies and rhabdomyosarcoma.
• “For Soft Tissue Ewings, see NCCN Guidelines for Bone Cancer” is new to
the page.
• Footnote “f”: “Recommended only for palliative therapy” has been added
to the following:
! Gemcitabine and vinorelbine .
! Vinorelbine and low-dose cyclophosphamide .
! Trabectedin .
SARC-E (2 of 6)
• Palbociclib was added for Well-differentiated/Dedifferentiated liposarcoma
(WD-DDLS) for retroperitoneal sarcomas .
CHICAGO // by Sam Kailes // Contributing Writer, MedPage Today.
In a preliminary trial, the novel agent Plitidepsin -- derived from the sea squirt Aplidium albicans -- showed activity among relapsed/recurrent multiple myeloma patients, researchers reported here.
Among the 18 patients treated with Plitidepsin plus Bortezomib (Velcade) and dexamethasone, 10 of them -- or 55% of the group -- responded to the treatment, said Maria-Victoria Mateos, MD, PhD, consultant hematologist at the University Hospital Salamanca, Spain.
"Initial efficacy results show promising activity in heavily pretreated patients with relapsed or recurrent multiple myeloma, even in bortezomib and lenalidomide (Revlimid)-refractory patients," Mateos said in her oral presentation at the annual meeting of the American Society of Clinical Oncology. "These results support the continued development of plitidepsin in patients with multiple myeloma."
Progression-free survival was 8.3 months, she reported. She said that two of the patients had achieved a stringent complete response; one complete response; four very good partial responses and three partial responses. She said that three patients achieved minor responses and one patient had stable disease. Four of the 18 patients progressed on treatment. The median duration of response has not yet been reached; some patients have been treated for 18 months.
Mateos said that plitidepsin appears to work by inducing oxidative stress and causing cell death through apoptosis.
...
AGUSTÍ SALA / BARCELONA // 9 DE JUNIO DEL 2016 .
Endor Nanotechnologies ha desarrollado un tratamiento innovador para el cáncer de páncreas y colon capaz de superar la resistencia del tumor. La compañía, con sede en el Parc Científic de Barcelona (PCB), está a un paso de iniciar los ensayos clínicos fase II (se realiza en una muestra de pacientes que padecen la enfermedad), para lo que busca entre dos y tres millones de euros. De hecho ya ha llegado a un acuerdo con un hospital belga para hacer pruebas.
Si no lograran los recursos necesarios la compañía retrasaría los ensayos al año que viene, cuando podrá financiarlos con los ingresos obtenidos con la actividad comercial que mantiene con productos dermatológicos, según el fundador y consejero delegado de la empresa, Joaquín Querol.
La empresa, fundada en el 2005, lanzó el año pasado al mercado su primer producto basado en nanopartículas contra los efectos de la edad en la cara. Con esta actividad financia hasta ahora su investigación contra el cáncer. Lo curioso es que este negocio surgió como derivada de los trabajos que realizan en investigación oncológica.
La nueva terapia de la compañía ha sido reconocida con el premio 'Producto biotecnológico del año 2016 en el Congreso Europeo de Biotecnología celebrado en Riga, Letonia, celebrado el mes pasado.
"Las terapias oncológicas actuales tienen un problema crítico que limita su eficacia: la respuesta terapéutica inicial es transitoria y finalmente derrotada por la capacidad del tumor de hacerse resistente al tratamiento. Hemos desarrollado una tecnología que supera esta limitación fundamental", explica Querol.
CRECIMIENTO DEL TUMOR
El descubrimiento de que los tumores no crecen de forma caótica sino siguiendo pautas muy concretas ha permitido el desarrollo de nuevas terapias, Endor está entre ellas con un tratamaiento capaz de bloquear estas pautas, con lo que se logra que el tumor deje de crecer y entre en un estado de muerte celular. En resumen, la terapia no actúa sobre las componentes bioquímicos del tumor sino sobre la forma en la que crece. Es por esos que no genera resistencia.
En la actualidad, más de 100.000 personas en el mundo están afectadas por cáncer de páncreas. La esperanza de vida no supera los cinco meses de media. En cuanto al de colon, los afectados superan 1.500.000.