Analysis of PET parameters predicting response to radiotherapy for myeloid sarcoma.

PURPOSE: Positron-emission tomography (PET)-CT has recently been used for diagnostic imaging and radiotherapy for myeloid sarcoma, but there is little research on predicting the response of radiotherapy. The aim of this study was to analyze the association between PET-CT variables and the response to radiotherapy in patients with myeloid sarcoma. MATERIALS AND METHODS: This study was conducted in myeloid sarcoma patients who received radiotherapy and PET-CT before and after radiotherapy. The response to radiotherapy was evaluated based on the European Organization for Research and Treatment of Cancer PET response criteria, and binary regression analysis was performed to assess the factors predicting reductions in the maximum standardized uptake value (SUVmax). RESULTS: Twenty-seven sites in 12 patients were included in the study. Complete metabolic responses were seen in 24 patients after radiotherapy, a partial metabolic response in one, and progressive metabolic disease in two patients. The prescribed dose of more than 3000 cGy10 was significantly greater in the treatment control group (P = 0.024). In binary logistic regression analysis predicting reductions in the SUVmax of more than 70% after radiotherapy, the pretreatment SUVmax (≥ 7.5) and further chemotherapy after radiotherapy showed significant differences in univariate and multivariate analyses. CONCLUSION: Good metabolic responses (complete or partial) to radiotherapy were achieved in 92.6% of the myeloid sarcoma patients. Radiation doses < 3000 cGy10 and increased SUVmax were related to treatment failure and high SUVmax before radiotherapy was a factor influencing SUVmax reduction. Further large-scale studies are needed.

Single Fraction Radiation for Myeloid Sarcoma Is as Effective as Multi-Fraction Regimens for Tumor Regression and Control.

INTRODUCTION/BACKGROUND: Myeloid sarcoma is a rare extramedullary manifestation of immature myeloid/monocyte cells. Radiotherapy (RT) yields good local control, but data on different fractionation schemes are limited. The goal of this retrospective study was to share our institutional experience and assess volumetric regression with differential fractionation. MATERIALS AND METHODS: We evaluated patients treated for myeloid sarcoma between 2000 and 2019 and categorized them into Group A (treated with RT) and Group B (no RT). We assessed local control using cumulative incidence function analysis. Post-treatment imaging sequences were analyzed for volumetric calculations. RESULTS: Forty-four patients with 80 lesions were assessed. Twenty-three patients with 52 lesions received RT (Group A), and 6 lesions received a single fraction of RT. There were 2 instances of local progression in Group A and 8 in Group B, with a cumulative incidence function estimate of local progression in Group A of 2.4% at 1 year and 6.9% at 2 years, significantly reduced compared to 29.7% and 35.5% in Group B, respectively (hazard ratio 0.13 [95% confidence interval 0.030.63], P = .011). No lesion treated with a single fraction of RT developed local progression. Volumetric analysis for 19 chronologically followed lesions (including 3 treated with a single fraction) revealed no difference in regression between single or multi-fraction treatment. CONCLUSION: RT for myeloid sarcoma yields excellent local control and may be as effective in a single fraction as more protracted courses, though this requires validation. For a diagnosis associated with poor survival, a single palliative fraction may be optimal with potential for higher utilization.

18F-Fluorodeoxyglucose PET/CT-Guided Palliative Radiotherapy Provides Durable Responses to Over a Dozen Sites of Disease in Relapsed Myeloid Sarcoma.

Myeloid sarcoma (MS) is a rare entity, and FDG PET/CT is a useful tool for staging at diagnosis and response assessment. We present a case of a 72-year-old woman diagnosed with multifocal extramedullary MS, using FDG PET/CT to guide palliative radiotherapy to 13 sites of disease over 2 separate relapses with complete and durable local responses and minimal toxicity. This case represents the largest reported burden of disease in MS successfully treated with FDG PET/CT-guided radiotherapy.

Acute myeloid leukaemia relapse presenting as cardiac myeloid sarcoma.

A 50-year-old woman previously diagnosed with acute myeloid leukaemia presented with a 3-month history of shortness of breath and a right-sided facial rash. A chest CT revealed an intracardiac mass in the right atrium extending into her superior and inferior vena cava. Surgery was performed to remove the mass and pathology was consistent with myeloid sarcoma. After surgery, adjuvant radiation therapy was directed to the residual disease. The patient eventually relapsed in other sites not including the right atrium and eventually succumbed to her disease.

Use of Radiation in Extramedullary Leukemia/Chloroma: Guidelines From the International Lymphoma Radiation Oncology Group.

Survival times for patients with leukemia generally have improved in recent decades, and this improvement has been attributed to an enhanced understanding of the genetics driving the cause of the disease and improved combinations of chemotherapy and targeted therapy. Durable control of systemic disease in blood and bone marrow has significantly improved survival, but extramedullary relapse can pose therapeutic challenges for which radiation therapy can have an important role. This report discusses the current role of radiation therapy for patients with leukemia, specifically the extramedullary manifestations of leukemia.

Fusarium solani infection as an initial manifestation of AML transformation in myelodysplastic syndrome: A case report.

Severely immunocompromised patients are at increased risk for uncommon infectious diseases with atypical presentations. Fusarium sp., has been reported in patients with hematological malignancies and prompt diagnosis is necessary due to high mortality. We report a myelodysplastic syndrome (MDS) patient who presented Fusarium solani infection associated with granulocytic sarcoma as an initial presentation of acute myeloid leukemia (AML) transformation. We performed histological examination, immunohistochemistry analysis, culture of the biopsy tissue and DNA sequencing to make a conclusive diagnosis of F. solani and granulocytic sarcoma, reinforcing the necessity of performing complete evaluation of skin lesions in immunocompromised patients.

Isolated Central Nervous System Chloroma as a Presenting Sign of Relapsed Pediatric Acute Lymphoblastic Leukemia.

Central nervous system (CNS) chloromas are an exceedingly rare presentation of CNS relapse in acute lymphoblastic leukemia (ALL). We report a relapsed ALL patient who presented with 2 separate chloromas and cerebrospinal fluid lymphoblastocytosis, and outline a treatment plan of systemic chemotherapy and CNS-directed radiation therapy. A review of the literature indicates that multiagent chemotherapy combined with CNS radiotherapy is effective, with hematopoietic stem cell transplantation used in half of reported cases. We conclude that intensive systemic multiagent chemotherapy with CNS-directed radiation therapy can be successfully used to treat relapsed pediatric ALL with CNS lymphoblastic chloroma.

Radiotherapy for extramedullary leukaemic manifestation (Chloroma).

PURPOSE: Extramedullary leukaemic disease (EMD, synonym chloroma) is a rare solid manifestation of myeloid leukaemia for which the value of radiotherapy (RT) as a treatment strategy remains controversial. The aim of this study is to analyse the effectiveness of various RT doses for EMD in the modern treatment era. MATERIALS AND METHODS: Between January 2000 and June 2016, 20 patients with total of 45 lesions underwent RT for EMD at our institution. RESULTS: With a median radiation dose of 26 Gy (range 4-42 Gy), local remission could be achieved in 91% of patients (complete remission rate: 71%). The median duration of local control (DOLC) was 17 months (95% confidence interval [CI] 0.5-33) and the median overall survival (OS) after chloroma onset was 24 months (95% CI 11-38). No noticeable difference between high- and low-dose regimens has been observed (74% versus 68%; P = 0.5). In the multivariate analysis, only Eastern Cooperative Oncology Group (ECOG) score and bone marrow state during RT have proven to be determinant for durable local control and OS. CONCLUSIONS: Low-dose RT (≤26 Gy) achieves good local control compared to high-dose regimes. Bone marrow state during RT and ECOG score during RT may play a crucial role, influencing both DOLC and OS.

[Radiotherapy of chloroma or granulocytic sarcoma: A literature review]. / Radiothérapie des chloromes ou sarcomes granulocytiques: revue de la littérature.

Granulocytic sarcoma, or chloroma, is a rare clinical entity, usually associated with a blood disease, including acute myeloid leukemia. Management strategies are based on the combination of systemic therapy and local therapy (surgery or radiation). Data for radiotherapy dose are derived from retrospective studies and case reports. We conducted a literature review using the Pubmed search engine to clarify the terms and indications for radiotherapy of chloromas.

[Primary granulocytic sarcoma of lacrimal gland]. / Pervichnaia mieloidnaia sarkoma sleznoi zhelezy.

The article presents clinical description and instrumental findings (ultrasound examination and multi-slice computed tomography of the orbits) of a rare lacrimal gland tumor, which precedes or concurs with acute myeloblastic leukemia. Due to the low incidence of myeloid (granulocytic) sarcoma, it is not possible to develop a proper algorithm for its diagnosis and treatment. Few descriptions that are available in the literature neither give an idea of the variety of manifestations and the order of organ involvement, nor allow any vital prognosis. Verification of the diagnosis can only be based on immunohistochemical findings of the primary tumor and bone marrow biopsy material. The authors emphasize the importance of combination treatment (radiation therapy of the orbits and chemotherapy) in the prevention of leukemia.

Aggressive Myeloid Sarcoma Causing Recurrent Spinal Cord Compression.

OBJECTIVE: Myeloid sarcoma is a rare extramedullary solid tumor comprised of immature myeloid precursor cells, most commonly associated with acute myelogenous leukemia (AML). We present the case of a patient with a history of Shwachman-Diamond syndrome and AML who presented with myeloid sarcoma causing acute spinal cord compression. CASE DESCRIPTION: The patient was a 20-year-old man who presented with acute onset weakness and numbness in his lower extremities. Magnetic resonance imaging revealed a thoracic dorsal epidural mass. Despite the history of AML, we elected to forego image-guided biopsy and up-front radiation due to the rapidly progressive nature of his myelopathy. Immediate surgical decompression was performed, but the patient had recurrence of tumor leading to further compression 13 days postoperatively. Subsequently, emergent radiation was performed, leading to resolution of cord compression and local disease control. CONCLUSIONS: To our knowledge, there are no randomized controlled trials examining the appropriate timing for postoperative radiation. Because most typical neuro-oncologic cases have no need for immediate postoperative radiation, our practice has been to wait 14 days to initiate postoperative radiation to ensure wound healing. One unique feature of our case was the rapid recurrence of symptoms due to tumor progression. Given this observation, we believe that radiation therapy should be considered as soon as possible after confirmatory pathology diagnosis for patients presenting with neurological compromise due to myeloid sarcoma of the spine.

Presentation and outcomes among patients with isolated myeloid sarcoma: a Surveillance, Epidemiology, and End Results database analysis.

Isolated myeloid sarcoma (MS) is a rare extramedullary presentation of acute myeloid leukemia (AML). Little is known about MS outcomes due to its rarity. A population-based analysis of MS using the Survival, Epidemiology, and End Results (SEER) database was performed. We identified 345 patients, aged 15 or older, diagnosed with isolated MS between 1973 and 2010. Overall survival (OS) was calculated and compared between MS and non-MS AML using the log-rank test. Survival was also evaluated based upon the primary site of disease presentation. The 3-year survival rate for MS (0.319; 95% confidence interval [CI]: 0.267-0.371) was greater than for non-MS AML (0.172; 95% CI: 0.168-0.175). There was variation in survival based on the site of involvement. The survival rates for isolated MS involving the pelvis/genitourinary organs, eyes/gonads and gastrointestinal mucosa appeared to be slightly improved when compared to primary sites of soft tissues, lymphatic/hematopoietic tissues or nervous system.

Sarcoma mieloide orbitario en el adulto: a propósito de un caso / Orbital meyeloid sacoma in the adult: report of a case

Caso clínico. Varón de 50 años con antecedentes de leucemia mieloide aguda atendido por una masa orbitaria izquierda. Los estudios histológicos del tumor biopsiado revelan un sarcoma mieloide. Tras tratamiento radioterápico y quimioterapia, el paciente permanece estable. Conclusiones. El sarcoma mieloide orbitario es un tumor raro en el paciente adulto, pero que debido a sus implicaciones sistémicas, ha de tenerse en cuenta en el diagnóstico de una masa orbitaria, en especial en pacientes hematológicos (AU)

Clinical case: 50 years old male patient with history of acute myeloid leukemia who is assisted because of a mass in his left orbit. Histological studies of the biopsied tumor reveal a myeloid sarcoma. After radiotherapy and chemotherapy, the patient remains stable. Conclusions. Orbital myeloid sarcoma is a rare tumor among adult patients, but because of its systemic implications, it should be considered in the diagnosis of an orbital mass, specially in the case of hematological patients (AU)

Clinicopathologic features and responses to radiotherapy of myeloid sarcoma.

BACKGROUND: To evaluate clinicopathological features, radiotherapeutic parameters, and their associations with responses to radiotherapy (RT) in patients with myeloid sarcoma (MS). METHODS: We reviewed 20 patients receiving RT for MS lesions (in 43 RT courses) and analyzed the patients' clinicopathologic features and radiotherapeutic parameters, and their associations with complete responses (CR) to RT using Fisher's exact test and univariate logistic regression analysis. Generalized Estimating Equation was used to analyze all 43 irradiated lesions and account for the correlations in RT responses among lesions from the same patient. RESULTS: We found that the underlying hematological diseases of the evaluated patients were acute myeloid leukemia (AML) in 14 patients (70%), chronic myeloid leukemia in 4 patients (20%), myelodysplastic syndrome with AML transformation in one patient (5%), and de novo MS in one patient (5%). Most patients (55%) received RT for MS at the time of relapse following bone marrow transplantation (BMT). The most common cytogenetic abnormality was t(8;21)(q22;q22). The median RT dose of 20 Gy (range 6-35 Gy), administered in 1.5-3.5 Gy fractions, provided a 63% CR rate. RT dose, sex, cytogenetics, and bone marrow status at the time of RT had no significant effect on CR. Younger age (<50 y, P = 0.06), BMT prior to RT (P = 0.05), and underlying AML (P = 0.05) were marginally associated with higher CR to RT. CONCLUSIONS: Our results indicate that a modest RT dose (20-30 Gy) achieves good local control of MS. Age, previous BMT, and underlying hematologic disease can affect RT response.

Orbital myeloid sarcoma in an adult with acute myeloid leukemia, FAB M1, and 12p-deletion.

A 49-year-old woman with acute myeloid leukemia, FAB M1 subtype, and 12p deletion, presented with progressive right proptosis and diplopia for 1 week. Orbital CT revealed a homogenously enhancing, orbital mass engulfing the inferior rectus muscle. Histopathology revealed myeloid sarcoma for which she underwent external beam radiotherapy. Subsequently, there was no sign of local recurrence, but she succumbed to leukemia involving the central nervous system. This is the first case, to the authors' knowledge, of an orbital sarcoma of FAB M1 myeloblasts bearing a 12p deletion.