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.

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.

[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.

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.

Total skin electron beam radiation in a pediatric patient with leukemia cutis: a case report.

Leukemia cutis, a form of extramedullary leukemia involving the skin, is a poor prognostic marker and indicator of imminent hematologic relapse and chemotherapeutic resistance in leukemia patients. Although total skin electron beam radiotherapy is commonly recommended for patients with leukemia cutis, its role and effectiveness remains unclear. Here we describe a 19-month-old infant with acute myelogenous leukemia first diagnosed at 15 months of age. It relapsed in her skin at 18 months of age, associated with raised hyperpigmented lesions on her chest and scalp. Radiation treatment to the total skin surface with radiation therapy resulted in relief of her symptoms before relapse in the bone marrow.

Primary intracerebral myeloid sarcoma.

BACKGROUND: Myeloid sarcoma rarely presents in the absence of systemic myeloid disease. CASE REPORT: In this study, we present a case of intracerebral myeloid sarcoma with no diagnosis of any hematological disease in a 22-year-old male patient in whom brain magnetic resonance image revealed a meningioma. However, biopsy showed myeloid sarcoma. No myeloid disease was determined. The mass disappeared following 8 cycles of chemotherapy. In the literature, we determined only 8 similar cases cited between 1970 and 2011. CONCLUSION: Intracerebral myeloid sarcoma has currently no standard treatment and may be confused with a primary brain disease. Chemotherapy and/or radiotherapy are the most viable and widely used treatment modalities. Potential occurrence of hematological disease should also be closely followed due to conversion risks.

Wolf in sheep's clothing: acute chloroma disguised as a subdural hematoma.

BACKGROUND: Subdural hematomas are not infrequent among patients with hematologic disorders as they are prone to thrombocytopenia from their disease and chemotherapy. However, rarely these patients can also have leukemic involvement of the subdural space. METHODS: Case Report with CT scan and intraoperative photographs. RESULTS: A 45-year-old woman with acute myeloid leukemia presented with progressive headache, somnolence, and hemiparesis. She was noted to be thrombocytopenic. CT scan revealed a heterodense extra-axial lesion consistent with an acute to subacute subdural hematoma. There was no antecedent trauma. After platelet transfusion, she was taken for burr hole evacuation and an opalescent pearly white mass was encountered. Pathology revealed myeloid sarcoma. CONCLUSIONS: Myeloid sarcoma can mimic subdural hematoma both clinically and radiologically. It should be considered when a patient with a prior leukemia and no antecedent trauma presents with an extra-axial lesion.

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.

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.

Myeloid sarcoma of the vagina: a report of 2 cases.

OBJECTIVE: To describe 2 cases of myeloid sarcoma of the vagina, in a patient without a history of acute myeloid leukemia (AML) and in another whose condition was previously diagnosed with AML. MATERIALS AND METHODS: The clinical histories of 2 patients whose conditions were diagnosed with myeloid sarcoma of the vagina were obtained from their medical records. RESULTS: Case 1: A 77-year-old woman with no systemic illnesses presented with a vaginal lump. Clinically, there was a 6-cm periurethral mass that was examined by biopsy. The histopathologic specimen was evaluated on routine and immunohistochemical stains, and myeloid sarcoma was diagnosed after extensive immunohistochemical analysis. The patient was treated with pelvic radiation. She developed extensive myeloid sarcoma of the skin and AML 4.5 months later; she died 2 weeks later, 5 months after the initial presentation. Case 2: A 36-year-old woman with a known history of AML who has had multiple leukemic and extramedullary recurrences presented with a pelvic mass. Physical findings revealed large masses in the vagina and rectovaginal septum, which were confirmed as myeloid sarcoma after biopsy and histologic examination. The patient was treated with pelvic/vaginal radiation. Five months later, she had another leukemic relapse and died within 1 day of palliative chemotherapy. CONCLUSIONS: Myeloid sarcoma of the vagina is extremely rare. Most patients have a poor prognosis and either have a history of or will subsequently develop AML.

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.

Intracardiac chloroma.

Chloromas are not frequently seen in patients with acute myelogenous leukemia and chloromas involving cardiac structures have only been rarely reported in the literature. We report a complete radiographic response to low-dose fractionated radiotherapy in a patient with an intracardiac chloroma.

[Primary myeloid sarcoma of uterine cervix and prevention of fertility. Report of a case]. / Traitement d'un sarcome myéloïde isolé du col utérin et préservation de la fertilité : à propos d'un cas.

The isolated myeloid sarcomas of the cervix are tumors whose forecast was dark for a long time. However, an effective but invasive treatment associating chemotherapy and radiotherapy allows for local remission. This treatment generally causes an ovarian failure and especially infertility. We report the case of a 29-year-old woman suffering from a myeloid sarcoma isolated from the uterine cervix and wishing a pregnancy. A prevention of the deficit ovarian was carried out. This patient presented normal menstrual cycles and a biochemical pregnancy three years after the beginning of the treatment.

Granulocytic sarcoma of the right humerus in a non-leukaemia patient.

Granulocytic sarcoma (GS), an uncommon solid extramedullary tumour, should be considered even in the absence of leukaemia, as delay in diagnosis and treatment worsens the prognosis. We present a GS (single humeral bone lesion) in a non-leukaemia patient, treated with intensive AML (Acute Myeloid Leukaemia) chemotherapy and sequential radiotherapy, in complete response 26 months after diagnosis, confirmed by histopathology and without leukaemia progression.

[Granulocytic sarcoma of the prostate presenting with urinary obstruction which progressed to acute myeloid leukemia].

A 71-year-old man presented with progressive dysuria. Several imaging examinations indicated possibility of prostate tumor, therefore he underwent prostate biopsy. This resulted in a diagnosis of granulocytic sarcoma of the prostate. Since bone marrow appeared normal on aspiration biopsy, he was treated with local irradiation. Prostate swelling then markedly diminished and his symptoms disappeared. However, four months later he progressed to AML. He then received systemic chemotherapy and achieved complete remission. We discuss the management of granulocytic sarcoma when findings do not indicate overt leukemia at the time of diagnosis.

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.

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.