Myeloid sarcoma presenting as an unusual limbal mass

A 19-year-old woman presented to the outpatient department with occasional ocular pain and redness and a perilimbal mass, which she noticed 5 months ago in her left eye. She had no systemic complaints. Ultrasound biomicroscopy of the mass showed a hypoechoic lesion with uniform reflectivity. The patient underwent an excision biopsy and a histopathological analysis revealed features suggestive of a granulocytic sarcoma/myeloid sarcoma. Further haematopathological evaluation confirmed concurrent acute myeloid (myelomonocytic) leukaemia French American British classification M4. There was complete remission of the ocular surface lesion and leukaemia with systemic chemotherapy. At the last follow-up of 18 months post-treatment the patient is free of disease.

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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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Unusual coexistence of sinonasal myeloid sarcoma and acute fulminant invasive fungal sinusitis: a diagnostic dilemma

The Journal of Laryngology & Otology ◽

10.1017/s0022215113000285 ◽

2013 ◽

Vol 127 (4) ◽

pp. 415-418 ◽

Cited By ~ 7

Author(s):

C-L Kuo ◽

Y-B Yu ◽

W-Y Li ◽

Y-L Lee

Keyword(s):

Acute Myeloid Leukaemia ◽

Myeloid Leukaemia ◽

Myeloid Sarcoma ◽

Surgical Debridement ◽

Comorbid Condition ◽

Histopathological Analysis ◽

Fungal Sinusitis ◽

Diagnostic Dilemma ◽

Invasive Fungal Sinusitis ◽

Acute Myeloid

AbstractObjective:We report a rare case of concurrent myeloid sarcoma and acute fulminant invasive fungal sinusitis in a patient with relapsed acute myeloid leukaemia.Case report:A 73-year-old man was diagnosed with acute myeloid leukaemia and developed relapse one year later. After two courses of azacytidine, he began suffering from a dull pain in the left temporal and orbital regions. Sinus computed tomography showed a localised lesion in the left ethmoid sinus, which rapidly progressed to an extensive intracranial mass within one month. Surgical debridement was performed, and histopathological analysis revealed the coexistence of myeloid sarcoma and acute fulminant invasive fungal sinusitis. The patient responded well to prompt surgical debridement, antifungal medication and radiotherapy.Conclusion:Coexistence of sinonasal myeloid sarcoma and acute fulminant invasive fungal sinusitis poses an urgent diagnostic and management challenge to clinicians. Timely recognition of this rare comorbid condition is warranted as application of appropriate treatment can save lives.

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Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia.

Journal of Clinical Oncology ◽

10.1200/jco.1993.11.4.690 ◽

1993 ◽

Vol 11 (4) ◽

pp. 690-697 ◽

Cited By ~ 154

Author(s):

M S Tallman ◽

D Hakimian ◽

J M Shaw ◽

G S Lissner ◽

E J Russell ◽

...

Keyword(s):

Acute Myeloid Leukemia ◽

Myeloid Leukemia ◽

World Literature ◽

Granulocytic Sarcoma ◽

Signs And Symptoms ◽

Free Survival ◽

A Cell ◽

Karyotype Analyses ◽

French American British ◽

Acute Myeloid

PURPOSE Since the only three cases of granulocytic sarcoma among patients with acute myeloid leukemia (AML) seen at our institution during the last 12 years were each associated with the 8;21 translocation [t(8;21)], we sought to determine if this association is specific and more frequent than previously recognized. PATIENTS AND METHODS We report three patients with AML and t(8;21) who developed granulocytic sarcomas, and review the world literature. RESULTS Between 1980 and 1992, 53 cases of AML French-American-British (FAB) M2 were identified at our institution. Eight (15%) patients had t(8;21). Three of these eight patients (38%) developed granulocytic sarcoma. All three of our patients received conventional intensive antileukemic chemotherapy yet had short relapse-free survival durations. Several series of patients with t(8;21) report that granulocytic sarcomas occur in approximately 18% of this population, which is four times the expected incidence in AML. Thirty-seven cases have been previously reported. Although karyotype analyses were not reported in many cases of granulocytic sarcoma in the literature, the vast majority of abnormal karyotypes in patients with AML involved t(8;21). Recent work with a cell line derived from a patient with t(8;21) indicates that such cells are unusually adherent to culture bottles and are aggregable CONCLUSION Our data suggest that this association is more common than generally recognized and may be specific. Patients with t(8;21) should be observed closely for signs and symptoms of granulocytic sarcoma. These patients may have a less favorable prognosis than other patients with t(8;21). Cooperative oncology groups should retrospectively identify patients with AML and t(8;21) who had a poor outcome to determine if they had a disproportionate incidence of granulocytic sarcoma. If so, aggressive therapy such as bone marrow transplantation may be warranted early in the therapeutic strategy.

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Orbital myeloid sarcoma misdiagnosed for subperiostal hematoma: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-021-03025-8 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Bahaa Razem ◽

Mohamed Raiteb ◽

Sanaa El Mrini ◽

Faiçal Slimani

Keyword(s):

Acute Myeloid Leukemia ◽

Case Report ◽

Myeloid Leukemia ◽

De Novo ◽

Myeloid Sarcoma ◽

Orbital Mass ◽

Genetic Features ◽

Immature Myeloid Cells ◽

Acute Myeloid

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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Myeloid Sarcoma: The Mayo Clinic Experience of Ninety Six Case Series

Blood ◽

10.1182/blood.v128.22.2798.2798 ◽

2016 ◽

Vol 128 (22) ◽

pp. 2798-2798

Author(s):

Jennifer Yui ◽

Mythri Mudireddy ◽

Mrinal M Patnaik ◽

Naseema Gangat ◽

Aref Al-Kali ◽

...

Keyword(s):

Bone Marrow ◽

Acute Myeloid Leukemia ◽

Mayo Clinic ◽

Myeloid Leukemia ◽

Research Funding ◽

Bone Marrow Involvement ◽

Myeloid Sarcoma ◽

Secondary Acute Myeloid Leukemia ◽

Acute Myeloid

Abstract Background: Myeloid sarcoma is a tumor mass consisting of myeloid blasts occurring at anatomical site other than the bone marrow (Arber et al. Blood 2016;127(20):2391-2405). It is a subgroup of acute myeloid leukemia, which can be localized or disseminated and may involve multiple organs. It can present with or without a positive bone marrow. It may precede or follow bone marrow involvement. It may be identified at diagnosis or relapse, and is not uncommon after stem cell transplantation (Koc et al. Cancer 1999;85(3):608-615; Yoshihara et al. Biol. Blood Marrow Transplant 2012;18(12):1800-1807). Objective: To describe the clinical characteristics, cytogenetics, prognosis and outcome of patients with myeloid sarcoma with or without bone marrow involvement. Methods: The Mayo Clinic database was interrogated using the ICD-9 codes 205.0, 205.2, 205.3, as well as terms "myeloid sarcoma," "chloroma," and "extramedullary sarcoma" in clinical notes and pathology reports. Patients' follow up information was collected until July 2016. Results: Ninety six patients with a diagnosis myeloid sarcoma were identified. The diagnosis was based on biopsy results and in some cases imaging studies in addition to bone marrow biopsy. The median age was 53 (range 17-83) years, and 64 (67%) patients were males. Myeloid sarcoma with de novo (primary) and secondary acute myeloid leukemia (with antecedent hematologic malignancy and therapy related) accounted for 64% (61) and 36% of the cases respectively. The sites involved based on their frequency of occurrence included integumentary system (skin and soft tissues) in 37 (38%), lymphatic system in 17 (18%), the gastrointestinal and genitourinary system in 14 (15%), the nervous system in 9 (9%), the breast in 3 (3%) and multiple and other single sites in 16 (17 %). Bone marrow cytogenetics findings were documented in 74 (77%) patients; favorable, intermediate, and poor cytogenetic abnormalities account for 7 (9%), 45 (61%), and 22 (30%) cases respectively. After a median follow up of 135 weeks, 57 (59%) patients died. The median survival of primary and secondary acute myeloid leukemia with myeloid sarcoma was 52 and 11.5 months (P<0.0001); and that of favorable, intermediate and unfavorable cytogenetics abnormalities was 169, 52 and 17.5 months (P=0.04) respectively. Twenty six (27%) patients had no bone marrow involvement; and 18 (69%) of them were primary myeloid sarcoma (without antecedent malignancy or therapy). The median (range) age of those with and without bone marrow involvement was 53 (17-83) and 56 (17-81) years (P=0.6). At diagnosis patients with and without bone marrow involvement have a median (range) hemoglobin (gm/dL) (10.3 (6.2-15.4) vs 13.1 (9.9-15.2) P=0.0002), white blood cell count (X109/L) (21.4 (1.1-182.5) vs 5.8 (2.4-23.2) P<0.0001), and platelet count (X109/L) (71 (8-437) vs 250 (17-561) respectively. Aggressive chemotherapy therapy was given to 58 (83%) and 20 (77%) of patients with and without bone marrow involvement (P=0.6). The median survival was 17 and 20 months with and without bone marrow involvement (P=0.4). Of those with bone marrow involvement, 49 (70%) achieved complete remission, and 26 (53%) of those individuals subsequently relapsed. Conclusion: The treatment outcome of patients with myeloid sarcoma with or without bone marrow involvement seems the same. The conventional risk factors, antecedent hematological neoplasms and cytogenetic findings, have significant impact on survival. Disclosures Al-Kali: Celgene: Research Funding; Onconova Therapeutics, Inc.: Research Funding.

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Mediastinal Myeloid Sarcoma with TP53 Mutation Preceding Acute Myeloid Leukemia with a PICALM-MLLT10 Fusion Gene

Acta Haematologica ◽

10.1159/000491596 ◽

2018 ◽

Vol 140 (2) ◽

pp. 97-104 ◽

Cited By ~ 3

Author(s):

Leslie Naesens ◽

Helena Devos ◽

Friedel Nollet ◽

Lucienne Michaux ◽

Dominik Selleslag

Keyword(s):

Acute Myeloid Leukemia ◽

Case Report ◽

Myeloid Leukemia ◽

Tp53 Mutation ◽

Metabolic Response ◽

Fusion Gene ◽

Granulocytic Sarcoma ◽

Myeloid Sarcoma ◽

Tumor Mass ◽

Acute Myeloid

Introduction: Myeloid sarcoma (MS), previously known as granulocytic sarcoma or chloroma, is a rare neoplastic condition defined as a tumor mass consisting of myeloblasts or immature myeloid cells occurring at an extramedullary site. Clinical presentation is diverse and determined by a tumor mass effect or local organ dysfunction. Case Report: We report the case of a 25-year-old previously healthy male with rapidly progressive shortness of breath. A chest CT scan demonstrated a heterogenous anterosuperior mediastinal mass with pleural and pericardial invasion. A diagnosis of MS with both myeloid and lymphoid characteristics was made by pathologic, morphologic, and immunophenotypic investigation. Next generation analysis revealed a pathogenic TP53 mutation (c.1035_1036insCT, p.Glu346Leufs*25). After 4 cycles of chemotherapy only a partial metabolic response and tumor size reduction was obtained. A pretransplant bone marrow biopsy revealed the progression of disease to acute myeloid leukemia. Cytogenetic analysis demonstrated a t(10; 11)(p12;q21). Fluorescence in situ hybridization confirmed the presence of a PICALM-MLLT10 fusion gene. Conclusion: MS with a mediastinal localization is rare and often misdiagnosed as malignant lymphoma. Acute leukemia harboring a PICALM-MLLT10 fusion gene is characterized by a mixed T cell and myeloid phenotype. The rearrangement is a rare recurrent translocation associated with specific clinical features, as illustrated in this case report.

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Sacral Myeloid Sarcoma Manifesting as Radiculopathy in a Pediatric Patient: An Unusual Form of Myeloid Leukemia Relapse

Case Reports in Radiology ◽

10.1155/2018/4257012 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Joana Ruivo Rodrigues ◽

Manuel João Brito ◽

Rui Pedro Faria Pais ◽

Sílvia Carvalho

Keyword(s):

Emergency Department ◽

Bone Marrow ◽

Acute Myeloid Leukemia ◽

Myelodysplastic Syndrome ◽

Pediatric Patient ◽

Myeloid Leukemia ◽

Granulocytic Sarcoma ◽

Myeloid Sarcoma ◽

Leukemia Relapse ◽

Acute Myeloid

Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms.

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DIGITAL FIBROMYXOMA: A CASE REPORT

Journal of Musculoskeletal Research ◽

10.1142/s0218957721720015 ◽

2021 ◽

pp. 2172001

Author(s):

Mahmood Ajawi ◽

Irfan Abdulkareem ◽

Abdullah H. Darwish ◽

Iftikhar A. Mukhtar ◽

Noora Iftikhar

Keyword(s):

Case Report ◽

Tissue Sample ◽

Ring Finger ◽

High Rate ◽

Excision Biopsy ◽

Histopathological Analysis ◽

Complete Excision ◽

The Right ◽

Hands And Feet

Digital fibromyxoma is a rare soft tissue tumor with a propensity to grow in the digits of the hands and feet. While mostly asymptomatic, the tumors can grow and cause discomfort and eventually pain. It is a benign tumor, with treatment revolving around complete excision to prevent recurrence. In this case report, we discuss the case of a 11-year old boy presented with a swelling in the right ring finger that had been increasing in size for over a year. Despite the lack of pain or limitation of movement in the finger, the swelling caused concern to the patient and family due to cosmetic appearance. An excision biopsy with histopathological analysis of the tissue sample suggested the diagnosis of digital fibromyxoma. The high rate of recurrence is due to incomplete excision, and therefore regular follow up is recommended to monitor for recurrence.

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A New Era of Organ-Preserving Treatment in Pediatric Intraocular Retinoblastoma in Russia: A Multicenter Cohort Study

Oncopediatrics ◽

10.15690/onco.v5i1.1866 ◽

2018 ◽

Vol 5 (1) ◽

pp. 51-69 ◽

Cited By ~ 2

Author(s):

Тatiana L. Ushakova ◽

Igor A. Тrofimov ◽

Оlga V. Gorovtsova ◽

Аndrey A. Yarovoy ◽

Svetlana V. Saakyan ◽

...

Keyword(s):

Gamma Knife ◽

Systemic Chemotherapy ◽

Focal Therapy ◽

Primary Treatment ◽

Local Chemotherapy ◽

Multicenter Cohort Study ◽

Cancer Disease ◽

Tumor Spread ◽

Group D

Background.Retinoblastoma (RB) is a life threatening cancer disease. A breakthrough in the treatment of children with RB is associated with the improvement of conservative treatment that was administered in at least one of the two tumor-affected eyes in most bilateral cases, that was chemotherapy both systemic and local (selective intra-arterial and intravitreal) in most cases combined with laser therapy, cryotherapy, or brachytherapy. The development of such techniques as local chemotherapy is focused on preservation of visual functions, reducing the number of enucleations and radiotherapy (RT) course. The success of the healing of RB is closely associated with a multidisciplinary approach to diagnosis and treatment, as well as specialized longterm follow-up clinical examination.Objective.eye and vision preservation against large intraocular tumors with different growth types and localization without the course of remote radiation therapy was the main purpose.Methods.In the period from September 2012 to January 2016, the study enrolled 45 patients with RB when at least one eye had intraocular tumor spread corresponding to the group C or D. According to the ABC international classification, patients have a relatively good prognosis for organ-preserving treatment. 4 of 18 children with bilateral RB had undergone primary enucleation of worse eye the worst eye, group E; 49 (77.8%) of the 63 affected eyes had features for groups C and D. In this study, no patient received local chemotherapy initially, only after prior systemic chemotherapy. Selective intra-arterial chemotherapy (SIAC) was applied to 41 patients (45 eyes; mean course number was 2), and 32 patients (34 eyes) had undergone intravitreal chemo therapy (IViC) (mean course number was 2). Focal therapy and local chemotherapy were the main methods of treatment for progression (new lesions on the retina) in 8 (16.3%) of 49 eyes with tumors of group C (n=1) and D (n=7); the relapse in 14 of 49 (new lesions on the retina) in eyes with tumors of group C (n=5) and D (n=6) and (new lesions on the retina and the vitreous) in eyes with tumors of group D (n=3) (28.5%), and stabilization of disease n=23 (46.9%). We should note that 2 patients underwent repeated course of in case of systemic chemotherapy, 1 patient — a Gamma Knife procedure due to registered disease stabilization, progression or relapse.Results.10 (20.4%) of 49 eyes saved due to the combined chemotherapy. In 45 patients diseasefree survival rate was 56.1±8.9 % (with mean follow-up period 26.9±2.5 months). 1 of 45 patients died from leukemia. 44 of 45 patients are alive without metastasis. The mean follow-up was 20 months (3 to 43 months). Eye salvage rate in group C — 14 (93.3%) of 15, in group D — 31 (91.2%) of 34.Conclusion.These methods: second line of systemic chemotherapy, RT, and a Gamma Knife procedure should be considered as a failure of primary treatment. Our study demonstrated a high efficacy of local chemotherapy with promissing techniques of conservation therapy, which safety increases due to experience.

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