Chemotherapy Options for Poor Responders to Neoadjuvant Chemotherapy for Orbital Granulocytic Sarcoma.
Granulocytic sarcoma (GS) is a rare manifestation of myeloid proliferation, characterized by formation of a mass comprised of immature cells of myeloid origin. Orbital granulocytic sarcoma is rarer still, with only a small fraction of GS patients having orbital involvement. Given the rarity of orbital GS, no unified therapy plan has been identified, as large prospective trials are not feasible, but it is widely accepted that patients with GS ought to be treated with systemic intensive chemotherapy consistent with standard of care regimens for acute myelogenous leukemia (AML) or chronic myelogenous leukemia (CML). Development of a treatment plan for GS in poor responders involves a systemic leukemia plan as novel therapeutics have not been investigated for treatment GS per se, but used more widely for AML. GS is most commonly associated with AML and thus will be addressed in that context in this review. Patients with GS associated with CML should receive CML-specific therapy. When conventional and traditional cytotoxic GS/AML chemotherapy regimens are insufficient, patients often require a combination of novel therapeutics, stem cell transplantation (SCT), and radiation. Much of the recent advancement in AML therapy, as well as in AML translational research, has been in targeting molecular facets of the disease and enabling more specificity with treatment. The aim of treating patients for whom conventional treatment was unsuccessful with personalized therapy has not yet been realized, but many of the novel therapeutics reviewed below have demonstrated promise and are cause for optimism. In our center, when a GS/AML patient is refractory to frontline therapy, we rely on novel chemotherapy therapeutic options as outlined below.
Division of Pediatric Hematology/Oncology, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, 55455, USA.
Nathan
N
Gossai
Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA.
Rachel
R
Cafferty
Division of Pediatric Hematology/Oncology, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, 55455, USA. weige007@umn.edu.
Brenda
B
Weigel
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor; D003131:Combined Modality Therapy; D019008:Drug Resistance, Neoplasm; D006801:Humans; D058990:Molecular Targeted Therapy; D020360:Neoadjuvant Therapy; D009918:Orbital Neoplasms / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D012008:Recurrence; D019233:Retreatment; D023981:Sarcoma, Myeloid / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D017211:Treatment Failure; D016896:Treatment Outcome
D014408:Biomarkers, Tumor
Acute myeloid leukemia; Bortezomib; Crenolanib; Gemtuzumab ozogamicin; Granulocytic sarcoma; Immunotherapy; Lestaurtinib; Novel therapeutics; Quizartinib; Ruxolitinib; Sorafenib; Vorinostat