Idiopathic mast cell activation syndrome and radiation therapy: a case study, literature review, and discussion of mast cell disorders and radiotherapy

Abstract

BackgroundMast Cell Activation Syndrome (MCAS) is classified as an idiopathic mast cell disorder where inconsistent or unknown triggers release inflammatory mediators and cause a constellation of symptoms. Studies demonstrate mast cells increase histamine, tryptase, and inflammatory cytokine expression following ionizing radiation. Additionally, there are cases of cutaneous mastocytosis developing within the initial radiation field suggesting mast cells play a role in local tissue reactions. Literature is sparse on radiation induced toxicity in patients with mast cell disorders.Case presentationA 62 year old female patient with a history of MCAS received breast conservation therapy for invasive lobular carcinoma of the left breast initially AJCC 7th Stage IIB, pT3 pN0 M0. The patient underwent external beam radiotherapy (EBRT) and received 4500 cGy to the left whole breast, followed by a 1000 cGy boost to the lumpectomy cavity. She developed grade 1 radiation dermatitis. Two years later she progressed distantly and received stereotactic body radiation therapy to a lumbar vertebrae lesion to a dose of 2400 cGy in a single fraction. She developed no in-field dermatologic or systemic flare in her MCAS symptoms during radiation therapy.ConclusionsTo our knowledge there are no reported cases in the literature of patients diagnosed with MCAS or other idiopathic mast cell disorders undergoing radiation therapy. Idiopathic mast cell disorders such as MCAS and primary mast cell disorders alike should not be considered a contraindication to treatment with EBRT. This patient population appears to tolerate treatment without systemic flares in symptoms.

Radioprotective potential of whey protein against gamma irradiation-induced lingual damage

“…Furthermore, NF-κB/p65 m-RNA gene expression was upregulated. Following exposure to IR, IgE-mediated mast cell activation triggers the production of histamine and causes increased expression of histamine and inflammatory cytokines ( Landy et al, 2019 ; Tanaka and Furuta, 2021 ). Histamine serves a crucial role in the local tissue inflammatory reaction to IR by increasing NF-κB/p65 activation and inflammatory cytokine production ( Landy et al, 2019 ).…”

“…Following exposure to IR, IgE-mediated mast cell activation triggers the production of histamine and causes increased expression of histamine and inflammatory cytokines ( Landy et al, 2019 ; Tanaka and Furuta, 2021 ). Histamine serves a crucial role in the local tissue inflammatory reaction to IR by increasing NF-κB/p65 activation and inflammatory cytokine production ( Landy et al, 2019 ). The NF-κB/p65 pathway is a redox-regulated transcription factor and is essential for the development of mucositis ( Zhang et al, 2021 ).…”

Diffusely Scattered Macules Following Radiation Therapy

“…Mastocytosis can be divided into 2 groups: CM and SM. 1 The histologic differential diagnosis of CM includes solitary mastocytoma, urticaria pigmentosa, telangiectasia macularis eruptiva perstans, and diffuse mastocytosis. 2 Clinicopathologic correlation is of crucial importance to render the final diagnosis in these disorders.…”

Some cases of hypermobile Ehlers–Danlos syndrome may be rooted in mast cell activation syndrome

“…Over the last decade, as recognition and understanding of MCAS has advanced, the frequency with which hEDS/HSD is found in the MCAS population has become apparent. Several studies have now observed this association, as enumerated in 2017 (Seneviratne, Maitland, & Afrin, 2017) as well as further reported in subsequent case studies and reviews (Arndt, Viswanathan, & Mathur, 2018;Chang & Vadas, 2019;Cohen & Markham, 2017;Daens, Grossin, Hermanns-Lê, Peeters, & Manicourt, 2018;Greiwe, 2018;Hamilton, 2018;Hendrix, Nesheiwat, Towheed, Brar, & Grubb, 2020;Hopping et al, 2020;Huang & Dellon, 2019;Landy et al, 2019;Mu et al, 2019;Pederson & Blettner Brook, 2017;Schofield & Afrin, 2019;Shah, Millan, Alamzaib, & Luu, 2019;Shusterman, Abbas, Patel, & Sussman, 2020;Weinstock, Kaleem, Selby, & Afrin, 2018), though the significant limitations of all of these reports have been noted, too (e.g., all are of small size, most are single-center, etc.) (Vengoechea, 2018).…”