Orbital myeloid sarcoma misdiagnosed for subperiostal hematoma: a case report

Abstract Background Myeloid sarcoma is a solid tumor that consists of immature myeloid cells occurring at an extramedullary site. It can present before, concurrent with, or after the diagnosis of acute myeloid leukemia or other myeloproliferative diseases, and a proportion of patients never develop bone marrow infiltration. Only a few isolated cases of pediatric orbital myeloid sarcoma have been reported, and they are often associated with a high misdiagnosis rate. Case report We report a rare case of pediatric orbital myeloid sarcoma associated with blunt trauma in a 3-year-old Caucasian male patient, which was clinically and radiologically misdiagnosed for orbital subperiostal hematoma. The patient underwent a surgical intervention to drain the hematoma when an orbital mass was found. The microscopic, immunologic, and genetic features of the tumor and the myelogram were in favor of LAM2, and the patient was started with chemotherapy with a favorable evolution within 18 months follow-up. Conclusion Orbital myeloid sarcoma usually exhibits clinical and radiological features that can be easily misleading, especially if it happens de novo or as the first manifestation of acute myeloid leukemia. Only a few isolated cases have reported and proposed trauma as a trigger event of the onset of this type of tumor proliferation, but further investigations and evidence are needed to support this hypothesis.

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Isolated Gastric Myeloid Sarcoma: A Case Report and Review of the Literature

Case Reports in Hematology ◽

10.1155/2014/541807 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 3

Author(s):

Pankit Vachhani ◽

Prithviraj Bose

Keyword(s):

Bone Marrow ◽

Acute Myeloid Leukemia ◽

Case Report ◽

Myeloid Leukemia ◽

Bone Marrow Involvement ◽

Myeloid Sarcoma ◽

Review Of The Literature ◽

Aged Woman ◽

Middle Aged Woman ◽

Acute Myeloid

Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach—an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed.

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Myeloid Sarcoma Involving the Breast

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-32-msitb ◽

2005 ◽

Vol 129 (1) ◽

pp. 32-38 ◽

Cited By ~ 2

Author(s):

Jose R. Valbuena ◽

Joan H. Admirand ◽

Gabriela Gualco ◽

L. Jeffrey Medeiros

Keyword(s):

Acute Myeloid Leukemia ◽

Myeloid Leukemia ◽

Subsequent Development ◽

Clinical Information ◽

Myeloid Sarcoma ◽

T Cell Antigens ◽

Well Differentiated ◽

Immunohistochemical Studies ◽

Acute Myeloid

Abstract Context.—Myeloid sarcoma is a neoplasm of myeloid cells that can arise before, concurrent with, or following acute myeloid leukemia. Rarely, it can also occur as an isolated mass. Objective.—To describe the clinicopathologic features of 6 patients with myeloid sarcoma involving the breast. Design.—Clinical information for all 6 patients was obtained from the medical record. Routine hematoxylin-eosin–stained slides; naphthol AS-D chloroacetate stain; and immunohistochemical stains for myeloid, B-cell, and T-cell antigens were prepared. Results.—There were 6 women with a median age of 52 years (range, 31–73 years). Two patients presented with isolated tumors of the breast, with no history or subsequent development of acute myeloid leukemia. In 3 patients, the breast tumors represented relapse of acute myeloid leukemia. One patient who presented initially with myeloid sarcoma involving the breast, skin, and spleen was lost to follow-up. Histologically, these tumors were classified as well differentiated (n = 3), poorly differentiated (n = 2), and blastic (n = 1). Naphthol AS-D chloroacetate esterase was positive in all 3 cases assessed. Immunohistochemistry showed that myeloperoxidase (n = 5) and CD43 (n = 3) were positive, and CD3 (n = 5) and CD20 (n = 5) were negative in all cases assessed. Lysozyme was positive in 4 (80%) of 5; CD117 was positive in 2 (67%) of 3; and single cases were positive for CD45 (1/3), TdT (1/2), CD79a, and the PAX5 gene product. Conclusions.—Myeloid sarcoma involving the breast is uncommon. In the literature, as in this study, these tumors most often represent relapse or the initial presentation of acute myeloid leukemia. However, 2 of the cases we report presented with isolated masses, without a history or subsequent development of acute myeloid leukemia at last follow-up. Immunohistochemical studies are extremely helpful for recognizing isolated myeloid sarcoma.

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Long-Lasting Remission in De Novo Breast Myeloid Sarcoma Treated with Decitabine and Radiotherapy

Diagnostics ◽

10.3390/diagnostics9030084 ◽

2019 ◽

Vol 9 (3) ◽

pp. 84

Author(s):

Carla Minoia ◽

Vincenza de Fazio ◽

Giovanni Scognamillo ◽

Anna Scattone ◽

Nicola Maggialetti ◽

...

Keyword(s):

Elderly Patient ◽

Acute Myeloid Leukemia ◽

Clinical Outcome ◽

Disease Control ◽

Myeloid Leukemia ◽

De Novo ◽

Myeloid Sarcoma ◽

Treatment Modalities ◽

Adverse Clinical Outcome ◽

Acute Myeloid

Myeloid sarcoma (MS) represents a rare disease with an adverse clinical outcome for patients not candidate to acute myeloid leukemia (AML)-like chemotherapies. Here we present the case of an elderly patient affected by a bilateral breast localization of MS treated with the hypomethylating agent decitabine associated to radiotherapy. The association of the two treatment modalities has allowed an optimal and long-lasting disease control.

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Coagulopathy in HLA-DR Antigen-Negative Acute Myeloid Leukemia.

Blood ◽

10.1182/blood.v104.11.4457.4457 ◽

2004 ◽

Vol 104 (11) ◽

pp. 4457-4457

Author(s):

Hideki Uchiumi ◽

Takafumi Matsushima ◽

Arito Yamane ◽

Hiroshi Handa ◽

Hiroyuki Irisawa ◽

...

Keyword(s):

Acute Myeloid Leukemia ◽

Myeloid Leukemia ◽

De Novo ◽

Myeloid Cell ◽

Leukemic Cell ◽

Leukemic Cells ◽

Hla Dr ◽

Risk Of Mortality ◽

Acute Myeloid

Abstract Background: HLA-DR antigen is present on hematopoietic progenitors and granulocyte/monocyte, erythrocyte and megakaryocytic precursors but absent at the promyelocytic stage during myeloid cell maturation. In accordance with this, majority of promyelocytic leukemia (APL) cells were negative for HLA-DR. Meanwhile, some of non-APL acute myeloid leukemia (AML) cells is found to express HLA-DR. However, the clinical significance of HLA-DR antigen on AML cells is currently unclear. Purpose: We sought to determine the prevalence and clinical characteristics of negativity in HLA-DR expression by retrospectively analyzing 181 consecutive patients with de novo adult AML. Patients and Methods: AML patients examined in the current study (aged 15–86 years) had been diagnosed between August 1995 and July 2004, and categorized to M0 (8 patients), M1 (35), M2 (74), M3 (20), M4 (25), M5 (15), and M6 (4), based on the FAB classification. Median follow-up time was 19.3 months. Phenotypic analyses of leukemic cells were performed using CD45 gating methods. HLA-DR-negative AML was defined as HLA-DR expression less than 20% of cells in the CD45 leukemic cell gate. Results: Among 181 patients, HLA-DR antigens were not detected on AML cells from 46 patients; 20 with APL and 26 with non-APL (non-APL/DR(−)), the latter of which included M0 (2 patients), M1 (15), M2 (7), M4 (2). Leukemic cells from other non-APL patients were HLA-DR-positive (non-APL/DR(+)). None of non-APL/DR(−) patients had t(15;17) nor PML/RARa rearrangement on cytogenetic analysis. Twenty out of 26 patients with non-APL/DR(−) had normal chromosome, and 6 had abnormal karyotypes. In the non-APL/DR(−) group, various degrees of nuclear folding, convolution, or lobulation were observed in 9 patients. Although treatment response and overall survival rate were similar in the three groups (APL, non-APL/DR(−), and non-APL/DR(+)), both FDP levels at diagnosis (57.3 vs 13.2, p<0.05) and maximal FDP levels (232.6 vs 43.8, p<0.01) were significantly higher in non-APL/DR(−) compared with non-APL/DR(+). The maximal FDP levels in the non-APL/DR(−) patients were comparable to those in the APL patients. FDP levels greater than 40 mg/ml were significantly more prevalent in the non-APL/DR(−) than in the the non-APL/DR(−) group. Logistic regression analysis demonstrated that low HLA-DR expression was an independent risk factor for FDP > 40 mg/ml. Conclusion: Our study suggests that AML with negative HLA-DR antigen tend to be associated with abnormality in coagulation and fibrinolysis even if they are genetically non-APL. We propose that more attention should be paied for HLA-DR expression to avoid a devastating coagulopathy which carries a high risk of mortality unless specifically addressed.

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Cytogenetic Diagnostics and Outcome in a Series of Thirty Three Greek Pediatric Acute Myeloid Leukemia Patients

Blood ◽

10.1182/blood.v112.11.4889.4889 ◽

2008 ◽

Vol 112 (11) ◽

pp. 4889-4889

Author(s):

Kalliopi N Manola ◽

Agapi Parcharidou ◽

Vassilios Papadakis ◽

Maria Kalntremtziou ◽

Chryssa Stavropoulou ◽

...

Keyword(s):

Acute Myeloid Leukemia ◽

Myeloid Leukemia ◽

De Novo ◽

Bone Marrow Cells ◽

Normal Karyotype ◽

Acute Leukemias ◽

Early Treatment Response ◽

Pediatric Aml ◽

Acute Myeloid

Abstract Acute myeloid leukemia (AML) accounting for approximately 17% of all childhood acute leukemias, arises either de novo or from a backround of myelodysplasia or previous chemotherapy. Cytogenetics is considered one of the most valuable prognostic determinants in AML while current risk–group classification in the limited cases of pediatric AML, is mainly based on cytogenetics and early treatment response. We reviewed the clinical and cytogenetic characteristics and the outcomes of 33 cases of childhood AML between 1997 and 2007 in order to investigate the incidence of the main FAB subtypes, the incidence of primary AML compared to secondary AML (s-AML) and the correlation between specific chromosome abnormalities and outcome in greek pediatric AML patients. Chromosome studies were performed on unstimulated bone marrow cells, derived from 33 pediatric AML patients, who were <18 years of age at the time of diagnosis. Eighteen patients were male and 15 were female. According to FAB classification one patient was classified as M0 (3%), 13 patients as M2 (39.4%), 4 as M3 (12.12%), 4 as M5 (12.12%), 2 as M6 (6.1%) and 4 as M7 (12.12%). No patient was classified as M4 while 5 patients with s-AML (15.15%) could not be classified. The median follow-up of all patients was 57.95 months (0.03–132.47). Overal survival and event free survival were 66,7% and 75,8% respectively. Eight patients with s-AML and 25 patients with primary AML were identified. The median age of patients with s-AML at diagnosis was 9.15 years while the median age of patients with primary AML was 7.2 years. Six out of 8 patients with s-AML died at a median follow up of 11.03 months. Nineteen out of 25 patients with primary AML are alive in complete remission (CR). Cytogenetic analysis was performed at diagnosis in 32 patients and results were obtained in 30 of them. The karyotype was abnormal in 21 out of 30 patients (70%). Normal karyotype was found in 9 patients, t(8;21)(q22;q22) in 5, t(15;17)(q22;q21) in 3, t(9;11)(p22;q23) in 3, −7/del(7q) in 5, del(9q) in 3, and complex karyotype in 4 patients. Three out of 4 patients with M3 are alive in CR with a median follow-up of 98.6 months while one with s-AML-M3 died 13 days post diagnosis. Three out of five patients with M2 and t(8;21), including 1 patient with s-AML, died at a median follow-up of 4.35 months. Three out of 5 patients with −7/del(7q) had s-AML and died in less than 4 years, while the two others are alive for more than 5 years, in CR. Although all patients with M7 had complex karyotypes, they are alive after a median follow-up of 96.73 months, 3 of them in CR and 1 in relapse. These results indicate that in greek patients, the main FAB subtypes show a distribution similar to that reported in the literature with the exception of M4 which is absent in our study but with a reported incidence of 20%. Pediatric patients with s-AML are older and their outcome is poor and is related to a higher probability of poor cytogenetic features compared to primary AML patients. Interestingly all patients with M7 had a good clinical course although they exhibited complex karyotypes.

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