Prognostic Factors of Treatment Outcomes in Patients with Granulocytic Sarcoma

Lan, Tsung-Yu; Lin, Dong‐Tsamn; Tien, Hwei‐Fang; Yang, Rong‐Sen; Chen, Chihyu; Wu, Karl

doi:10.1159/000253592

Cited by 141 publications

(177 citation statements)

References 55 publications

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“…(5,7,22) Five-year survival in both cohorts compare favorably to other series in which five-year survival rates range between 20-30%. (12,13) While benefits in OS with radiation have not been supported in the literature (12) or by our study, we believe that radiation might contribute to improved PFS in cases of isolated chloroma (13) and in those which there was an inadequate response to chemotherapy. Patients in the pediatric age group had the longest median survival, which might reflect better leukemia outcome in general in children compared to older age groups.…”

Section: Discussionmentioning

confidence: 50%

“…(11) Since that time, only few case series of chloromas have been published but information on radiotherapy and response was insufficient for developing clear treatment recommendations. (12,13) We therefore analyzed the experience at our cancer center to examine if data may provide guidelines for this uncommon leukemia manifestation for leukemia and radiation oncology teams. evaluation, tissue biopsy, imaging and/or death in the absence of pathology or imaging.…”

Section: Introductionmentioning

confidence: 99%

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Radiation Therapy for Chloroma (Granulocytic Sarcoma)

Bakst¹,

Wolden²,

Yahalom³

2011

International Journal of Radiation Oncology*Biology*Physics

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Purpose-Chloroma (granulocytic sarcoma) is a rare, extramedullary tumor of immature myeloid cells related to acute non-lymphocytic leukemia or myelodysplastic syndrome (MDS). Radiation therapy (RT) is often used in the treatment of chloromas; however, modern studies of RT are lacking. We reviewed our experience to analyze treatment response, disease control, and toxicity associated with RT in order to develop treatment algorithm recommendations for patients with chloroma.Materials/Methods-38 patients who underwent treatment for chloromas at our institution from 2/1990-6/2010 were identified and their medical records were reviewed and analyzed.Results-The majority of patients that presented with chloroma at the time of initial leukemia diagnosis (78%) have not received RT as it regressed after initial chemotherapy. Yet most patients that relapsed or remained with chloroma after chemotherapy are in the RT cohort (90%). 33 courses of RT were administered to 22 patients. Radiation sub-site breakdown was: 39% head and neck, 24% extremity, 9% spine, 9% brain, 6% genitourinary, 6% breast, 3% pelvis, and 3% genitourinary. Median dose was 20 (6-36) Gy. Kaplan-Meier estimates of progression free survival (PFS) and overall survival (OS) in the RT cohort were 39% and 43%, respectively at 5 years. At a median follow-up of 11 months since RT, only one patient developed progressive disease at the irradiated site and 4 patients developed chloromas at other sites. RT was well tolerated without significant acute or late effects, and provided symptom relief in 95% of cases. Conclusions-The majority of patients with chloromas were referred for RT when there was extramedullary progression, marrow relapse, or rapid symptom relief required. RT resulted in excellent local disease control and palliation of symptoms without significant toxicity. We recommend irradiating chloromas to at least 20 Gy, and propose 24 Gy in 12 fractions as an appropriate regimen.

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Section: Discussionmentioning

confidence: 50%

Section: Introductionmentioning

confidence: 99%

Radiation Therapy for Chloroma (Granulocytic Sarcoma)

Bakst¹,

Wolden²,

Yahalom³

2011

International Journal of Radiation Oncology*Biology*Physics

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“…Although the presence of EM disease may be associated with a poor prognosis and shorter survival, 23 5-year survival rates for patients with MS range between 20% and 30%, which appear similar to AML in general. 71,72 The prognostic significance of cytogenetic alterations in the presence of MS is not fully understood. Although the presence of translocation t(8;21) is associated with a relatively good prognosis when treated with standard induction and intensive consolidation chemotherapy, it remains unclear whether this favorable prognosis remains in the presence of EM disease because there are conflicting reports.…”

Section: Does the Presence Of Em Involvement Confer A Worse Prognosismentioning

confidence: 99%

How I treat extramedullary acute myeloid leukemia

Bakst¹,

Tallman

Douer

et al. 2011

Blood

415

586

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IntroductionAcute leukemia may present in a variety of extramedullary (EM) tissues with or without bone marrow disease. EM involvement by acute leukemia is a relatively rare, but clinically significant, phenomenon that often poses therapeutic dilemmas. Myeloid sarcoma (MS) and leukemia cutis (LC) represent 2 well-known EM manifestations. MS (also known as granulocytic sarcoma or chloroma) is a rare EM tumor of immature myeloid cells. It was first described in 1811 1 and later named "chloroma" by King 2 in 1853 because of its green color caused by the presence of myeloperoxidase (MPO). 3 Five decades later, the relationship of MS to acute leukemia was identified. 4 The term "granulocytic sarcoma" was introduced later by Rappaport to describe only tumors of granulocytic origin 5 ; however, the term is now often applied to any tumor related to acute leukemia or myelodysplastic syndrome (MDS).LC is the infiltration of the epidermis, dermis, or subcutis by neoplastic leukocytes (leukemia cells) resulting in clinically identifiable cutaneous lesions. LC commonly results in subcutaneous nodules and can be confusingly referred to as cutaneous granulocytic sarcoma. 6,7 For the purposes of this article, LC will refer to cutaneous involvement only. LC has been described mostly in acute myeloid leukemia (AML), but also in the accelerated phase of chronic myeloid leukemia, MDS, and rarely in acute lymphocytic leukemias. 7 In this article, we describe an approach and therapeutic strategies for patients with EM manifestations of leukemia by addressing a series of questions commonly raised by the practicing clinician. How common are MS and LC, respectively?MS is reported in 2.5%-9.1% 8-10 of patients with AML and occurs concomitantly, following, or, rarely, antedating the onset of systemic bone marrow leukemia. 11 Isolated MS, defined by the absence of a history of leukemia, MDS, or myeloproliferative neoplasm and a negative bone morrow biopsy, has been described in limited case reports. 12 Many of these patients are often misdiagnosed with lymphoma. 13,14 Certain known AML cytogenetic abnormalities, in particular t(8,21), have been associated with a higher incidence. 15 MS can also develop at relapse with or without marrow involvement. The incidence of patients developing MS after allogeneic hematopoietic cell transplantation (HCT) has been reported to be 0.2%-1.3% with poor overall survival. 16,17 LC occurs in ϳ 3% of patients with AML 18 and less frequently in chronic leukemias. 19 The reported incidence may be overestimated if biopsy is not performed because skin lesions similar to LC have a wide range of inflammatory, neoplastic, and infectious etiologies. 19 Certain subtypes of AML are more commonly associated with skin infiltration. The most frequent association occurs with acute myelomonocytic and monocytic differentiation with involvement in up to 50% of patients. 18,20,21 The incidence of LC may be higher in children, particularly infants with myeloid leukemia. 22 How do MS and LC most often present clinically?MS most...

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“…Patients treated with chemotherapy showed longer survival than untreated patients. [35] Although the mortality rate of acute leukemia patients has been reduced with the emergence of new therapies, many patients still suffer from refractory disease or relapse, and EMI is one of the main causes of poor prognosis in these patients. [8] EXTRAMEDULLARY NICHE IN AML During development, HSCs are initially present in AGM and then migrate into the fetal liver and embryonic bone, which remains the only active site of hematopoiesis in adult life.…”

Section: Genetic and Molecular Features Of Extramedullary Infiltratiomentioning

confidence: 99%

Molecular and cellular aspects of extramedullary manifestations of acute myeloid leukemia

Mohammadi-Asl¹,

Khosravi²,

Shahjahani³

et al. 2015

J Cancer Metastasis Treat

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The myeloid extramedullary tumor is a solid tumor formed by infiltration of immature myeloid cells in various tissues of the body. This tumor is also identified as chloroma or myeloid sarcoma (MS). MS is a manifestation of acute myeloid leukemia (AML) occurring at presentation or during treatment or relapse. MS is associated with multiple chromosomal abnormalities and molecular mutations since patients with these disorders bear a high potential for MS manifestation. There is a high incidence of extramedullary infiltration (EMI) in AML. AML patients with EMI have a worse prognosis than patients without it. Hematopoietic stem cells and leukemic stem cells reside in a special bone marrow microenvironment called niche, which is essential for their normal functions. Cancers are exploited dysfunctional cell-cell and matrix-cell interactions, which convert a normal niche into a neoplastic niche. This study summarizes the current knowledge on the molecular and cellular characteristics of AML with EMI and extramedullary niches in AML patients.

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Prognostic Factors of Treatment Outcomes in Patients with Granulocytic Sarcoma

Cited by 141 publications

References 55 publications

Radiation Therapy for Chloroma (Granulocytic Sarcoma)

Radiation Therapy for Chloroma (Granulocytic Sarcoma)

How I treat extramedullary acute myeloid leukemia

Molecular and cellular aspects of extramedullary manifestations of acute myeloid leukemia

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