Jessica K.R. Boult1, Upasana Roy1, Carolina Bernauer2, Carol Box1, Louise Howell3, Elise Y. Lepicard1, Yann Jamin1, James P.B. O'Connor1, Janet M. Shipley2, and Simon P. Robinson1
1Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, United Kingdom, 2Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom, 3Core Research Facilities, The Institute of Cancer Research, London, United Kingdom
Synopsis
Hypoxic gene signatures are prevalent
in paediatric rhabdomyosarcomas and are important
in conferring resistance to standard treatments. Oxygen-enhanced MRI (OE-MRI) and
histological assessment of pimonidazole adduct formation show that xenografts derived
from three human rhabdomyosarcoma cell lines exhibit high levels of hypoxia with
differences in vascular perfusion evident between the models.
There is much interest in hypoxia-alleviating
strategies to reduce tumour hypoxia for therapeutic gain, and these will be assessed
in rhabdomyosarcoma models in vivo using
OE-MRI. A reduction in hypoxia in response to atovaquone in spheroids derived
from the same rhabdomyosarcoma cell lines in
vitro has been confirmed.
Introduction
Rhabdomyosarcomas (RMS) are the
most common soft tissue sarcomas in children and adolescents and a major cause of
cancer-related mortality in this age group. A growing body of evidence suggests
that hypoxia is important in
conferring resistance to treatment in RMS1-3.
We have demonstrated the
potential of oxygen-enhanced MRI (OE-MRI) for mapping hypoxia non-invasively in vivo4-6, and it
is important that this method is further evaluated in tumour types in which hypoxia
has been implicated in poor prognosis.
Here we report the use of OE-MRI
and histopathology to characterise the hypoxic phenotype of a panel of human
RMS cell lines propagated in mice or grown as tumour spheroids in vitro.Methods
RH41, RD and RH30 RMS xenografts
were propagated subcutaneously in the flanks of female NCr nude mice. When tumours reached ~350mm3
pimonidazole (60mg/kg i.p.) was administered 30min prior to imaging on a Bruker Biospec 7T system, performed over a 3cm field-of-view using a 4cm volume coil with the tumour surrounded by dental paste to
minimise susceptibility artefacts.
OE-MRI using IR-TrueFISP T1-mapping
and susceptibility-contrast MRI were performed and analysed as previously
described6 with additional acquisition of diffusion-weighted images (EPI
TE=38.1, TR=1500ms, 8 averages, 5 b-values 200-1000s/mm2)
for the estimation of apparent diffusion coefficient (ADC)7, and the
use of the USPIO contrast agent ferumoxytol (150µmolFe/kg)8.
Following MRI, the perfusion
marker Hoechst 33342 (15mg/kg i.v.) was administered for 1min and tumours
rapidly excised and snap frozen. Whole tumour frozen sections cut in the
imaging plane were processed for fluorescent detection of pimonidazole adduct
formation and Hoechst 33342 uptake, and subsequently stained with H&E7.
To assess response to a hypoxia-alleviating
therapy, RH41 and RD spheroids were formed in ultra-low attachment plates and
treated for 24h with 3.75µM-60µM atovaquone, which decreases hypoxia by
reducing oxygen consumption rate9, or DMSO control. Subsequently
they were incubated with 200µM pimonidazole for 2h prior to fixation and immunofluorescent
detection of pimonidazole on paraffin-embedded sections.
One-way ANOVA was performed to assess statistical
significance, assuming a significance level of 5%.Results
T2-weighted images demonstrated
that RH41 tumours were more homogeneous than RD and RH30 tumours (Figures
1&2), which demonstrated cystic or necrotic areas on both MRI and H&E-stained
sections (Figure 2). Median ADC values were higher in RH30 tumours (768±27x10-6mm2/s)
than RH41 and RD (616±27 and 636±24x10-6mm2/s,
respectively, P<0.05, data not shown).
Oxygen-induced ΔR1
maps show some intratumoural heterogeneity but little change was observed
(Figure 1). Binarised maps derived from ΔR1 signifying voxels that
enhanced with (OxyE) or were refractory to (OxyR) hyperoxia show relatively
equal proportions in all three models. Perfused voxels refractory to hyperoxia
(pOxyR) identified using a USPIO-derived perfusion mask were distributed across
the tumours often in small islands interspersed with areas of enhancing voxels
and non-perfused (NP) areas (Figures 1&2).
For all three models, areas of
pimonidazole adduct formation were spatially associated with pOxyR or non-perfused
voxels, while denser areas of Hoechst 33342-stained perfused vasculature
associated with pOxyE voxels (Figure 2).
More intertumoural heterogeneity
in ΔR1 and consequently OxyR and pOxyR values was observed in the
RH41 cohort than RD and RH30 (Figure 3). Mean cohort OxyR was ~50% with pOxyR above
25% in all three models, which corresponded to ~30% pimonidazole adduct
formation in all models. No differences in these parameters were found between
the groups. Hoechst 33342-perfused fraction was higher in RD tumours (8.5±1.0%) than
RH41 and RH30 (5.8±0.5 and 4.7±0.6%, respectively, P<0.05), which
was also reflected in a difference in USPIO-perfused fraction between RD and
RH30 (90±2
and 66±7%, P<0.05).
Control RD and RH41 spheroids >300µm in diameter
demonstrated pimonidazole adduct-positive hypoxic cores (Figure 4). RD
spheroids treated with 60µM and 30µM atovaquone, and
RH41 spheroids treated with 60µM atovaquone, no longer presented a hypoxic core;
this was confirmed by a reduction in mean fluorescence intensity compared to
DMSO controls. Atovaquone treatment did not affect the diameter of the
spheroids over 24h (data not shown).Discussion
RMS xenografts derived from RH41, RD
and RH30 cells present with high levels of hypoxia, as assessed by OE-MRI and
pimonidazole adduct formation, which also corresponded well spatially. The
levels of OxyR, pOxyR and pimonidazole adduct staining observed were higher
than those previously reported in range of other subcutaneously-grown
xenografts4-6,10.
Numerous strategies have been investigated to reduce tumour hypoxia for
therapeutic gain11. Remarkably, the majority of these clinical
trials failed to identify and select patients with hypoxic tumours prior to enrolment12,13.
Non-invasive
methods, such as OE-MRI, that are able to quantify the extent and heterogeneity
of hypoxia within an individual tumour would clearly offer clinical benefit in
personalised treatment planning.
RH41 and RD spheroids presented with hypoxic
cores when grown to 300µm diameter in
vitro. As a prelude to studies investigating hypoxia-alleviating
therapies in vivo, spheroids
were treated with the repurposed anti-malarial agent atovaquone. Hypoxia was
reduced in a dose-dependent manner, with a significant reduction in
pimonidazole staining observed at 30µM in RD and 60µM in RH41 spheroids.
These data support
subsequent in vivo studies to assess
the response of RMS xenografts to atovaquone or other hypoxia-modifying
therapeutics using OE-MRI, ahead of potential combination studies to evaluate
the role of hypoxia-alleviation in improving sensitivity to standard-of-care
therapeutics in RMS.Acknowledgements
We acknowledge support from CR-UK
programme grant C16412/A27725, The Oracle Cancer Trust, and Children with
Cancer UK.References
- Romualdi C, De Pitta C, Tombolan L, et al.
Defining the gene expression signature of rhabdomyosarcoma by meta-analysis.
BMC Genomics 2006;7:287.
- Zhang L, Marrano, P, Wu B, et al. Combined
antitumor therapy with metronomic topotecan and hypoxia-activated prodrug,
evofosfamide, in neuroblastoma and rhabdomyosarcoma preclinical models. Clin
Cancer Res 2016;22:2697-2708.
- Nordsmark M, Asner J, Keller J, et al. Hypoxia
in human soft tissue sarcomas: Adverse impact on survival and no association
with p53 mutations. Br J Cancer 2001;84:1070-1075.
- O'Connor JPB, Boult JKR, Jamin Y, et al.
Oxygen-enhanced MRI accurately identifies, quantifies, and maps tumor hypoxia
in preclinical cancer models. Cancer Res 2016;76:787-795.
- Little RA, Jamin Y, Boult JKR, et al. Mapping
hypoxia in renal carcinoma with oxygen-enhanced MRI: Comparison with intrinsic
susceptibility MRI and pathology. Radiology 2018;288:739-747.
- Lepicard EY, Boult JKR, Jamin Y, et al. 0276
Imaging hypoxia in head and neck cancer xenografts with oxygen-enhanced MRI.
Proc Intl Soc Mag Reson Med 29 2021.
- Reeves EL, Li J, Boult JKR, et al. 0143 Breast
tumour response to PEGPH20-induced stromal modulation assessed by
multiparametric MRI. Proc Intl Soc Mag Reson Med 28 2020.
- Boult JKR, El-Shemerly M, Bachmann F, et al.
0933 Tumour vascular response to the FGFR inhibitor derazantinib assessed using
susceptibility contrast MRI with ferumoxytol. Proc Intl Soc Mag Reson Med 29
2021.
- Ashton TM, Fokas E, Kunz-Schughart LA, et al.
The anti-malarial atovaquone increases radiosensitivity by alleviating tumour
hypoxia. Nature Commun. 2016;7:12308.
- Salem A, Little RA, Latif A, et al.
Oxygen-enhanced MRI is feasible, repeatable, and detects radiotherapy-induced
change in hypoxia in xenograft models and in patients with non-small cell lung
cancer. Clin Cancer Res 2019;25:3818-3829.
- Dewhirst MW, Birer SR. Oxygen-enhanced MRI is
a major advance in tumor hypoxia imaging. Cancer Res 2016;76:769-772.
- Lee ATJ, Pollack SM, Huang P, et al. Phase III
soft tissue sarcoma trials: success or failure? Curr Treat Options Oncol
2017;18:19.
- Lindner LH. Hypoxia-activated prodrug: an
appealing preclinical concept yet lost in clinical translation. Lancet Oncol
2017;18:991-993.