Chameleonic Chloroma: A Case of Myeloid Sarcoma Presenting as a Pancreatic Head Mass.

We report a case of pancreatic myeloid sarcoma (MS), an extremely rare manifestation of acute myeloid leukemia (AML), in a 35-year-old male who presented with epigastric pain and watery stools. Initial diagnostic testing was inconclusive; however, following an extensive evaluation, endoscopic biopsies suggested AML, which was confirmed by a bone marrow biopsy. Given that few cases are documented in the literature, pancreatic MS without a preexisting hematologic malignancy poses a significant diagnostic challenge.

Acute myeloid leukemia: An unusual manifestation of the trachea.

Objectives: Hematologic malignancy involving the trachea is rare. It is even less common for tracheal involvement to be the initial manifestation of this disease. We present a case report highlighting an unusual diagnosis of acute myeloid leukemia (AML) that first presented with prominent tracheal manifestations. There have been only three other published case reports of extramedullary AML with involvement of the trachea. Methods: We discuss direct laryngoscopy and bronchoscopy findings, including pinkish-white irregular lesions, which were similar to findings described in the available literature for tracheal AML. Results: Laboratory findings from our case are reported, including peripheral smear demonstrating 57% blasts and bone marrow biopsy confirming the diagnosis of AML, and the relevance of these findings is discussed. Conclusion: In patients with unusual airway lesions, laboratory testing and a comprehensive airway evaluation including biopsy are necessary to narrow the differential diagnosis. Level of Evidence: 5.

Myeloid sarcoma presenting as fingertip necrosis with underlying suppurative tenosynovitis: A case report.

We report an unusual case presentation of a patient with necrotic tissue changes of the right second and third fingers, found to have myeloid sarcoma with Staphylococcus-positive tenosynovitis and underlying acute myeloid leukemia, to highlight the importance of comprehensive evaluation in patients with atypical wounds.

Isolated Intracranial Extramedullary Hematopoiesis in an Adult Patient.

This article describes the case of a 56-year-old male patient who presented with a headache and swelling on the left side of his head. Medical examinations, including non-contrast computed tomography (CT) and contrast-enhanced magnetic resonance imaging (MRI), revealed the presence of an expansile soft tissue lesion in the frontoparietal bone structures, causing compression of the brain tissue. Further immunohistochemical analysis confirmed the diagnosis of extramedullary hematopoiesis (EMH) in the bone lesion, with predominant involvement of the myeloid series. EMH is a compensatory mechanism of the body to meet the demand for erythropoiesis. While EMH is commonly associated with thalassemia in children and myelofibrosis in adults, it can occur in various tissues throughout the body, including the intracranial region, although it is rare. The imaging findings of EMH on MRI typically show iso-hypointense signals on T1-weighted images and hypointense signals on T2-weighted images due to the presence of hemosiderin. However, in this case, the lesions appeared hyperintense on T2-weighted images, which posed a challenge in the differential diagnosis. The article highlights the importance of clinical evaluation and imaging findings in diagnosing EMH, as these lesions can resemble other conditions such as metastases, angiomatous meningioma, chloroma, or epidural hematoma. Teaching Point: Isolated cases of intracranial EMH should be considered as a differential diagnosis in adult patients as they may be radiologically confused with malignant conditions such as metastases, angiomatous meningioma, chloroma or epidural hematoma.

Paraplegia as a Rare Clinical Presentation of Relapsed Acute Myeloid Leukemia.

Myeloid sarcoma, also known as granulocytic sarcoma or chloroma, is an extra-medullary accumulation of malignant myeloid blast cells, leading to a solid tumor formation. Herein, we report a rare presentation of a case with acute myeloid leukemia (AML), whose disease relapse was clinically evident as acute flaccid paraplegia with a certain sensory level. On thoracic spine magnetic resonance imaging (MRI), an epidural mass compressing the spinal cord at the level of the thoracic spine segment 4 (T4) was found. The mass histology confirmed the diagnosis of myeloid sarcoma.

Myeloid Sarcoma as a Presentation of Acute Myeloid Leukemia and Blastic Phase in the Course of Chronic Myeloid Leukemia: A Case Report and Literature Review.

BACKGROUND: Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare disease entity characterized by the emergence of an extramedullary tumor, which may be antecedent, coexisting, or manifest secondarily to an ongoing malignancy of lymphoid origin. Owing to its low prevalence, scientific reports addressing this matter comprise mainly retrospective studies with a limited number of participants, rather low-quality research, and only few case reports. Despite MS's rarity, the need for enhancing their diagnostic tools and refinement of their therapeutic regimens is broadly recognized among physicians. CASE SUMMARY: In this case series, we present the clinical histories of two patients diagnosed with MS. The former (Case 1) exhibited MS of the sternum alongside chronic myeloid leukemia (CML), while in case of the latter (Case 2) MS presented as the initial manifestation of a current acute myeloid leukemia (AML). Treatment for both patients included chemotherapy (CHTH) and radiation (RT); however, patient 1 with CML died due to cardiorespiratory insufficiency secondary to an infection, while patient 2 is in clinical remission (CR) for 16 months since their MS diagnosis. Furthermore, a comprehensive analysis of previously reported cases was conducted which incorporated MS in patients with AML and CML. CONCLUSION: The objective of this report was to emphasize the heterogeneity among the clinical manifestations of MS, to underline the relevance of the histopathological and molecular diagnostic tools in opting for the appropriate therapy, and that, in spite of it occurring rather uncommonly, physicians should think of MS in the presence of pathological masses in patients under risk of hematological malignancies.

Mediastinal Mass as an Initial Presentation of Acute Myeloid Leukemia in a Young Man.

A 29-year-old male, hemodynamically stable, presented with chest pain radiating to the interscapular region, with no fever, cough, dyspnea, or other constitutional symptoms. He had right cervical lymphadenopathy on physical examination. Investigations revealed a 3.1 cm anterior mediastinal nodular mass, peripheral immature blood cells, and thrombocytopenia. Bone marrow core biopsy findings were consistent with acute myeloid leukemia (AML). The mediastinal mass was resected via robotic-assisted thoracoscopic surgery. Histopathology revealed involvement of the mediastinal adipose tissue with myeloid sarcoma. Molecular testing showed TP53 mutation, signifying a poor prognosis. The patient failed several lines of therapy and expired. This case demonstrates an atypical presentation of AML and emphasizes the criticality of early detection in individuals who do not exhibit the usual symptoms associated with the disease. The presence of immature cell lines in peripheral blood should prompt an investigation to determine bone marrow involvement in an otherwise healthy young adult.

Myeloid sarcoma: more and less than a distinct entity.

Myeloid sarcoma (MS) is a distinct entity among myeloid neoplasms defined as a tumour mass of myeloid blasts occurring at an anatomical site other than the bone marrow, in most cases concomitant with acute myeloid leukaemia (AML), rarely without bone marrow involvement. MS may also represent the blast phase of chronic myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS). However, the clinical and molecular heterogeneity of AML, as highlighted by the 2022 World Health Organization (WHO) and International Consensus (ICC) classifications, indirectly define MS more as a set of heterogeneous and proteiform diseases, rather than a homogeneous single entity. Diagnosis is challenging and relies mainly on histopathology, immunohistochemistry, and imaging. Molecular and cytogenetic analysis of MS tissue, particularly in isolated cases, should be performed to refine the diagnosis, and thus assign prognosis guiding treatment decisions. If feasible, systemic therapies used in AML remission induction should be employed, even in isolated MS. Role and type of consolidation therapy are not univocally acknowledged, and systemic therapies, radiotherapy, or allogeneic hematopoietic stem cell transplantation (allo-HSCT) should be considered. In the present review, we discuss recent information on MS, focusing on diagnosis, molecular findings, and treatments also considering targetable mutations by recently approved AML drugs.

Chloroma - A Rare Case of Metastatic Tumor to Dorsal Spine in Patient of Chronic Myeloid Leukemia Causing Acute Paraparesis.

Introduction: Chloromas are defined as metastatic granulocytic solid tumors of myeloid origin occurring at an extramedullary site. In this case report, we present an uncommon case of chronic myeloid leukemia (CML) presenting with metastatic sarcoma to the dorsal spine causing acute paraparesis. Case Report: A 36-year-old male presented in the OPD with complaints of progressive upper back pain and acute paraparesis since a 1 week. The patient is a previously diagnosed case of CML on treatment for the same. MRI of the dorsal spine indicated extradural soft-tissue lesions in D5-D9 extending to the right side of the spinal canal displacing the cord to the left. Considering the acute paraparesis that the patient developed, he was taken for an emergency decompression of the tumor. Microscopy showed infiltration of fibrocartilaginous tissue of polymorphous origin mixed with atypical myeloid precursor cells. Immunohistochemistry reports show atypical cells diffusely expressing myeloperoxidase, focally expressing CD34 and Cd117. Conclusion: Rare case reports like this are the only literature available on remission in CML cases with sarcomas. The acute paraparesis in our patient was prevented from increasing to a paraplegia by surgical means. Immediate decompression of the spinal cord in patients with paraparesis and associated radiotherapy and chemotherapy should be considered in all patients with myeloid sarcomas of CML origin. While examining patients of CML, the possibility of a granulocytic sarcoma should always be kept in mind.

Bilateral adnexal masses: A case report of acute myeloid leukemia presenting with myeloid sarcoma of the ovary and review of literature.

•We report a case of a patient with acute myeloid leukemia (AML) presenting as myeloid sarcoma.•This patient with bilateral adnexal masses was managed via total robotic hysterectomy with bilateral salpingo-oophorectomy.•There are a limited number of reports of bilateral ovarian occurrences that exist in the literature.•Myeloid sarcoma of the ovaries may present with vaginal bleeding to dysmenorrhea, dysuria, and palpable abdominal mass.

Myeloid sarcoma of the thoracic spine: A case report.

Background: Myeloid sarcoma is an uncommon malignant neoplasm that typically arises at extramedullary sites and is associated with a diagnosis of acute myeloid leukemia. While myeloid sarcoma can involve any organ, central nervous system involvement is rare, particularly in the adult population. Case Description: An 87-year-old female presented with progressive paraparesis of 5 days' duration. The magnetic resonance imaging (MRI) revealed an epidural tumor from T4 to T7 with cord compression. When she underwent a laminectomy for tumor resection, the pathology revealed a myeloid sarcoma with monocytic differentiation. Although she improved postoperatively, she elected to pursue hospice care and expired 4 months later. Conclusion: Myeloid sarcoma is an uncommon malignant spinal neoplasm rarely seen in adults. For this 87-year-old female, MRI-documented cord compression warranted decompressive surgery. Although this patient did not opt for adjuvant therapy, other patients with such lesions may undergo additional chemotherapy or radiation therapy. Nevertheless, optimal management for such malignant tumor is still undefined.

Pediatric Myeloid Sarcoma, More than Just a Chloroma: A Review of Clinical Presentations, Significance, and Biology.

Myeloid sarcomas (MS), commonly referred to as chloromas, are extramedullary tumors of acute myeloid leukemia (AML) with varying incidence and influence on outcomes. Pediatric MS has both a higher incidence and unique clinical presentation, cytogenetic profile, and set of risk factors compared to adult patients. Optimal treatment remains undefined, yet allogeneic hematopoietic stem cell transplantation (allo-HSCT) and epigenetic reprogramming in children are potential therapies. Importantly, the biology of MS development is poorly understood; however, cell-cell interactions, epigenetic dysregulation, cytokine signaling, and angiogenesis all appear to play key roles. This review describes pediatric-specific MS literature and the current state of knowledge about the biological determinants that drive MS development. While the significance of MS remains controversial, the pediatric experience provides an opportunity to investigate mechanisms of disease development to improve patient outcomes. This brings the hope of better understanding MS as a distinct disease entity deserving directed therapeutic approaches.

Isolated intracranial myeloid sarcoma: report of a case and review of the literature.

Myeloid sarcoma is a rare malignant tumor of primitive myeloid cell origin often associated with hematologic disorders. The central nervous system is rarely involved and differentiating between myeloid sarcoma and other tumors is not possible on imaging. Here we present the rare case of an isolated intracranial myeloid sarcoma, initially misdiagnosed radiologically as a meningioma, treated with surgical total resection and subsequent chemotherapy, with no signs of any hematological disorder at follow up. Differential diagnosis and management strategies, as well as follow-up implications are discussed along with literature review, which pointed out that only five cases with no further signs of hematological disorders at follow up have been described in the literature so far and this case has the longest follow up of them at 9 years.

Clinicopathological characteristics, genetics and prognosis of patients with myeloid sarcoma: a single-center study.

AIM: Myeloid sarcoma (MS) is a rare tumour comprising myeloid blasts occurring at an anatomical site other than the bone marrow. We sought to investigate both paediatric and adult patients with MS diagnosed at our institution and determine possible correlations among their clinicopathological, phenotypic, molecular and prognostic features. METHODS: This study retrospectively evaluated the data of 45 patients diagnosed with MS at Ege University Faculty of Medicine Hospital, Turkey, over a 17-year period. RESULTS: The male-to-female ratio was 1.5:1, and the median age was 39.12 years. The most commonly involved sites were the skin, lymph nodes, soft tissues and bone. Immunohistochemically, CD68-KP1 was the most commonly expressed marker, followed by CD33, myeloperoxidase, CD117, lysozyme, CD68-PGM1 and CD34. Of the patients, 26 (57.7%) presented with de novo MS, 7 (15.5%) had simultaneous acute myeloid leukaemia and 12 (26.8%) had a previous history of haematological disorders. Kaplan-Meier survival analysis revealed that the 2-year and 5-year overall survival (OS) rates were 46.4% and 39.8%, respectively; the median OS duration was 11 months. Increasing age had a negative prognostic relationship with survival (p = 0.04). Chromosomal abnormalities were detected in approximately 6/10 (60%) of paediatric patients and 6/9 (66.7%) of adult patients. t(8;21)(q22;q22) translocation was identified in 20% of paediatric patients. CONCLUSIONS: MS diagnosis is usually challenging; an expanded immunohistochemical panel should be used for an accurate diagnosis. Although MS generally has a poor prognosis, increasing age appears to be associated with a worse outcome.

Myeloid Sarcoma of the Breast: A Diagnostic Dilemma With Pathologic Correlation.

Myeloid sarcoma (MS) is a rare extramedullary solid tumor arising most often in patients with current or subsequent acute myeloid leukemia (AML). Patients of all ages may present with involvement of the skin, lymph nodes, intestinal tract, bone, and/or central nervous system. Isolated involvement of the breast is rare, and only a small number of cases have been described in the literature. Breast MS may present as a palpable mass on clinical evaluation. In this broad literature review from 2010 to 2022, the most common findings on mammography are either solitary or multiple masses, followed by architectural distortion and, less commonly, no discrete findings. Sonography may demonstrate hypoechoic or mixed echogenicity mass(es) with circumscribed or indistinct, not discrete margins. Myeloid sarcoma may present as an enhancing mass or nonmass enhancement on breast MRI and is typically moderately radiotracer avid on 18F-fluorodeoxyglucose-PET. At histopathology, MS is characterized by myeloid blasts in varying stages of granulocytic or neutrophilic maturation; diagnosis typically requires immunophenotyping. There is no consensus for treatment of MS, although systemic chemotherapy for AML is often used as MS is considered the tissue equivalent of AML. This article will discuss and illustrate imaging and pathology findings when the breast is involved by MS.

Successful Treatment of Pediatric Acute Myeloid Leukemia Presenting with Hyperbilirubinemia Secondary to Myeloid Sarcoma: A Case Report.

Myeloid sarcoma (MS), a tumor consisting of myeloid blasts with or without maturation, occurs at anatomical sites other than the bone marrow. MS of the gastrointestinal tract presenting with jaundice in children is rare. We report the case of a 4-year-old boy with a 6-week history of symptoms of obstructive jaundice due to a peripancreatic mass compressing the common bile duct. Six weeks later, blasts were found in a peripheral smear prior to surgical biopsy; bone marrow evaluation and flow cytometry results led to a diagnosis of acute myeloid leukemia (AML) with MS. No further invasive testing or temporary drainage was performed. He was started on induction therapy with full therapeutic doses of cytarabine, dose reductions of etoposide, and escalating doses of daunorubicin. His liver enzymes normalized, and he completed subsequent cycles of chemotherapy with full doses. The abdominal ultrasound showed resolution of the mass after the second cycle of chemotherapy. He is currently in remission three years after completing therapy. AML-directed chemotherapy in patients with obstructive jaundice secondary to MS may be beneficial without requiring invasive testing or temporary drainage procedures. Daily follow-up is crucial for chemotherapy dose modifications. Management plans should be individualized according to the patient's clinical condition.

Bilateral Orbital Myeloid Sarcomas: A Unique Presentation of Acute Myeloid Leukemia.

Myeloid sarcomas (MS) are solid manifestations of acute myeloid leukemia (AML) and are commonly present in children. These tumors can arise in many tissues including bone, soft tissue, or skin, and are commonly seen in the orbit. As practically all MS will, if left untreated, eventually present as AML, early diagnosis and initiation of treatment are imperative. We highlighted a case of bilateral orbital MS in a pediatric patient that presented concurrently with AML and the steps taken to diagnose and initiate treatment. Our case highlights the potentially occult presentation of AML as well as myeloid sarcoma and, therefore, the importance of swift workup and diagnosis. Epidemiology, radiographic features, diagnosis, and treatment for myeloid sarcoma and AML were discussed.

Myeloid Sarcoma Mimicking Dental Abscess in a Patient with Chronic Myeloid Leukemia: Diagnostic and Therapeutic Dilemma.

Chronic myeloid leukemia (CML) is a hematologic malignancy that has significant improvement in its prognosis after the introduction of tyrosine kinase inhibitors. Transformation to accelerated phase or blast phase can happen. Myeloid sarcoma or chloroma is an uncommon extramedullary disease. It is very unusual for patients with CML to develop myeloid sarcoma. We report a young man with CML in the chronic phase who developed myeloid sarcoma. There were many difficulties in the diagnosis of myeloid sarcoma due to the simulation of other more common conditions like infections and other malignancies. In addition, there are treatment challenges because of lack of standardized treatment. The case shed light on this rare complication, the challenging diagnosis, and its implication in patients with CML.

Early Ophthalmological Manifestations of Acute Myeloid Leukemia: Current Perspectives.

Acute myeloid leukemia (AML) is a hematological malignancy affecting different organ systems including the eye. The purpose of this review is to present and evaluate the medical literature regarding the early ophthalmological manifestations of acute myeloid leukemia. AML affects the ocular system through direct infiltration of tissues, secondary to hematological abnormalities, or in the form of chloroma or myeloid sarcoma in the brain or orbit consequently leading to a variety of manifestations depending on the ocular tissue involved. It is imperative for ophthalmologists to be aware of the early ophthalmological manifestations of AML which will allow for earlier diagnosis and treatment of this life-threatening disease.