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Frontiers in Pediatrics 2022Myeloid sarcoma (MS) is a rare extramedullary mass with myeloid expression, which is easy to be missed and misdiagnosed, especially in the pediatric population. We...
PURPOSE
Myeloid sarcoma (MS) is a rare extramedullary mass with myeloid expression, which is easy to be missed and misdiagnosed, especially in the pediatric population. We analyze the clinicopathological characteristics, immunophenotypic, cytogenetic, and molecular studies, therapeutic approaches, and outcomes, to optimize the management of such patients.
METHODS
A retrospective, single-center, case series study of eleven children diagnosed with MS by pathology was performed.
RESULTS
The male-to-female ratio was 8:3, and the median age at diagnosis was 7 years. The most commonly involved sites were the skin and orbital region, followed by lymph nodes, central nervous system, and testis. Seven cases (64%) with -MS and four cases (36%) presented as -MS. Immunohistochemically, MPO and CD117 were the most commonly expressed markers, followed by CD33, CD43, CD34, CD68, and lysozyme. Chromosomal abnormalities were detected in 4 patients. Two patients had the presence of deleterious mutations (FLT3, ASXL, KIT, and DHX15) on molecular detection. Ten patients were treated with chemotherapy based on AML regimens. The median follow-up time was 33.5 months in eleven patients. Two patients relapsed, one died, and one lost to follow-up. The 2-year overall survival (OS) rate estimated by Kaplan-Meier curves was 90.9% ± 8.7%, and the event-free survival (EFS) rate was 64.9% ± 16.7%.
CONCLUSIONS
MS diagnosis is usually challenging. Adequate tumor biopsy and expanded immunohistochemistry are necessary for the correct diagnosis of MS. Early and regular systemic chemotherapy promises long-term survival.
PubMed: 36545668
DOI: 10.3389/fped.2022.927894 -
International Journal of Surgery Case... 2016Myeloid Sarcoma (MS) or Granulocytic Sarcoma is an uncommon laryngeal malignancy. It may arise from myelodysplastic syndromes, malignancy or de novo. Presentation in the...
INTRODUCTION
Myeloid Sarcoma (MS) or Granulocytic Sarcoma is an uncommon laryngeal malignancy. It may arise from myelodysplastic syndromes, malignancy or de novo. Presentation in the larynx is rare and some may present with Acute Myeloid Leukaemia (AML) whereby the later may be asymptomatic.
CASE PRESENTATION
A 44-year-old South East Asian lady presented with a six months history of hoarseness, shortness of breath, reduced exercise tolerance, weight loss and laryngeal irritation. Symptoms progressed to coughing with liquids two months prior. On examination, she had a resting biphasic stridor and laryngoscopy revealed right immobile vocal cord with a firm right ventricle mass extending into the right paraglottic space. She was pale and haematology investigations revealed microcytic hypochromic anaemia. Magnetic Resonance Imaging (MRI) of the neck and thorax showed thickening of the right false cord, true cord and aryepiglottic fold. A biopsy taken during endolaryngeal microsurgery (ELMS) confirmed myeloid sarcoma of the right ventricle and para glottic mass. Further investigation revealed a background of AML and she then underwent chemotherapy.
DISCUSSION
MS is a rarity with only nine reported cases between the years of 1954 until 2015. Immunohistochemistry and immunophenotyping are definite for diagnosis confirmation as MS cells often exhibit myeloperoxidase (MPO), lymphocyte common antigen (LCA) and CD117 markers. MS is treated with are chemotherapy (either systemic or intrathecal), radiotherapy, surgical excision or in combination. Systemic chemotherapy has better efficacy and prognosis as compared to localised treatment of radiotherapy or surgical excision. However, there has yet to be a definitive chemotherapy protocol. Prognosis is poor with a 5-year survival rate of 48%.
CONCLUSION
Although laryngeal MS is a rare phenomenon, early recognition is key and patients should always be investigated for an underlying myeloproliferative or dysplastic disease.
PubMed: 26957189
DOI: 10.1016/j.ijscr.2016.02.012 -
South Asian Journal of Cancer Dec 2021Myeloid sarcoma (MS) is a malignant extramedullary tumor consisting of immature cells of myeloid origin. It may precede, present concurrently or follow acute myeloid...
Myeloid sarcoma (MS) is a malignant extramedullary tumor consisting of immature cells of myeloid origin. It may precede, present concurrently or follow acute myeloid leukemia (AML) in de novo case or may also be present and might be the only manifestation of recurrent AML, myelodysplastic syndrome, or chronic myeloid leukemia. It frequently involves skin, orbit, bone, periosteum, lymph nodes, and gastrointestinal tract, soft tissue, central nervous system, and testis. Because of its different localization and symptoms, and the lack of diagnostic algorithm, MS is a real diagnostic challenge particularly in patients without initial bone marrow involvement. The correct diagnosis of MS is important for optimum therapy, which is often delayed because of a high misdiagnosis rate. We reported three cases of MS derived from spine presented with back pain, paraplegia, paraparesis, respectively, and reviewed the relevant literature.
PubMed: 34984205
DOI: 10.1055/s-0041-1742079 -
BMC Cancer Nov 2019Myeloid sarcoma (MS), also known as chloroma, is an extramedullary manifestation of malignant primitive myeloid cells. Previously, only small studies investigated...
BACKGROUND
Myeloid sarcoma (MS), also known as chloroma, is an extramedullary manifestation of malignant primitive myeloid cells. Previously, only small studies investigated clinical and imaging features of MS. The purpose of this study was to elucidate clinical and imaging features of MS based upon a multicenter patient sample.
METHODS
Patient records of radiological databases of 4 German university hospitals were retrospectively screened for MS in the time period 01/2001 and 06/2019. Overall, 151 cases/76 females (50.3%) with a mean age of 55.5 ± 15.1 years and 183 histopathological confirmation or clinically suspicious lesions of MS were included into this study. The underlying hematological disease, localizations, and clinical symptoms as well as imaging features on CT and MRI were investigated.
RESULTS
In 15 patients (9.9% of all 151 cases) the manifestation of MS preceded the systemic hematological disease. In 43 cases (28.4%), first presentation of MS occurred simultaneously with the initial diagnosis of leukemia, and 92 (60.9%) patients presented MS after the initial diagnosis. In 37 patients (24.5%), the diagnosis was made incidentally by imaging. Clinically, cutaneous lesions were detected in 35 of 151 cases (23.2%). Other leading symptoms were pain (n = 28/151, 18.5%), neurological deficit (n = 27/151, 17.9%), swelling (n = 14/151, 9.3%) and dysfunction of the affected organ (n = 10/151, 6.0%). Most commonly, skin was affected (n = 30/151, 16.6%), followed by bone (n = 29/151, 16.0%) and lymphatic tissue (n = 21/151, 11.4%). Other localizations were rare. On CT, most lesions were homogenous. On T2-weighted imaging, most of the lesions were hyperintense. On T1-weighted images, MS was hypointense in n = 22/54 (40.7%) and isointense in n = 30/54 (55.6%). A diffusion restriction was identified in most cases with a mean ADC value of 0.76 ± 0.19 × 10 mm/s.
CONCLUSIONS
The present study shows clinical and imaging features of MS based upon a large patient sample in a multicenter design. MS occurs in most cases meta-chronous to the hematological disease and most commonly affects the cutis. One fourth of cases were identified incidentally on imaging, which needs awareness of the radiologists for possible diagnosis of MS.
Topics: Adult; Aged; Diagnostic Imaging; Female; Germany; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Middle Aged; Proportional Hazards Models; Retrospective Studies; Sarcoma, Myeloid; Symptom Assessment; Tomography, X-Ray Computed
PubMed: 31775680
DOI: 10.1186/s12885-019-6357-y -
World Journal of Clinical Oncology Oct 2021Myeloid sarcoma (MS) is a rare hematologic malignancy defined as an extramedullary tumor of immature granulocytic cells. It can occur as primary or and be associated...
BACKGROUND
Myeloid sarcoma (MS) is a rare hematologic malignancy defined as an extramedullary tumor of immature granulocytic cells. It can occur as primary or and be associated with myelodysplasia or myeloproliferative neoplasms. The most frequent locations are the skin, lymph nodes and bones. The case of a patient with a diagnosis of primary granulocytic gastric MS is reported.
CASE SUMMARY
A 19-year-old female patient with MS, whose abdominal computed tomography showed a bulky tumor of 16.5 cm in the gastric chamber with infiltration in the retroperitoneal, pancreatic and bile duct region; the histological study showed gastric mucosa diffusely infiltrated by mononucleated cells and the immunohistochemistry expressed myeloperoxidase. After receiving induction chemotherapy based on the 3 + 7 regimen (daunorubicin/cytarabine), the patient developed severe hematological toxicity and neutropenic typhlitis which required a prolonged medical treatment. She presented a rapid disease progression. Although she received supportive treatment, the patient died.
CONCLUSION
Gastric primary MS is a rare and aggressive course neoplasm, fostering knowledge is very important to decide its management and to promote more approaches focused on understanding this pathology and its particularities in our population.
PubMed: 34733617
DOI: 10.5306/wjco.v12.i10.960 -
Case Reports in Gastroenterology 2021Myeloid sarcoma (MS) is a rare solid neoplasm that consists of extramedullary myeloid precursor cells. Generally, it is associated with underlying acute myeloid leukemia...
Myeloid sarcoma (MS) is a rare solid neoplasm that consists of extramedullary myeloid precursor cells. Generally, it is associated with underlying acute myeloid leukemia (AML) or AML yet to manifest clinically. It can present as isolated, also known as primary MS without evidence of AML or other myeloproliferative neoplasms. We present the case of a previously healthy 36-year-old male, who was admitted to hospital with new-onset painful obstructive jaundice and final diagnosis of isolated MS was made after through investigations. We are pleased to report that he had favorable response to the treatment and remains well.
PubMed: 34594168
DOI: 10.1159/000514528 -
Autopsy & Case Reports 2021Myeloid sarcoma (MS) is a rare extramedullary neoplasm of myeloid cells, which can arise before, concurrently with, or following hematolymphoid malignancies. We report... (Review)
Review
Myeloid sarcoma (MS) is a rare extramedullary neoplasm of myeloid cells, which can arise before, concurrently with, or following hematolymphoid malignancies. We report 04 such cases of MS, diagnosed in this institute over a period of 6 years, during various phases of their respective myeloid neoplasms/leukemias. These cases include MS occurring as a relapse of AML (Case 1), MS occurring as an initial presentation of CML (Case 2), MS occurring during ongoing chemotherapy in APML (Case 3), and MS presenting as a progression of MDS to AML (Case 4). In the absence of relevant clinical history and unemployment of appropriate immunohistochemical (IHC) studies, these cases have a high risk of being frequently misdiagnosed either as Non-Hodgkin's Lymphoma (NHL) or small round cell tumors or undifferentiated carcinomas, which may further delay their management, making an already bad prognosis worse. This case series has been designed to throw light on the varied presentation of MS and the lineage differentiation of its neoplastic cells through the application of relevant IHC markers along with their clinical correlation.
PubMed: 34805008
DOI: 10.4322/acr.2021.339 -
Radiology. Cardiothoracic Imaging Feb 2021Supplemental material is available for this article.
Supplemental material is available for this article.
PubMed: 33778664
DOI: 10.1148/ryct.2021200540 -
Head and Neck Pathology Mar 2023Oral ulcers represent a full thickness loss of the mucosal epithelium leading to exposure of the submucosal connective tissue. These are common and usually... (Review)
Review
BACKGROUND
Oral ulcers represent a full thickness loss of the mucosal epithelium leading to exposure of the submucosal connective tissue. These are common and usually self-limited lesions, although they may sometimes result from neoplasms, most commonly squamous cell carcinoma. Lymphoproliferative disorders may be difficult to diagnose in apthous ulcers since they mimic reactive inflammation.
METHODS
This review presents ten rare oral lymphoid proliferations which should not be missed when assessing oral ulcer biopsies.
RESULTS
The ten lesions include several with diagnostic cells which look similar to the histiocytes of a reactive inflammatory ulcer, including Rosai-Dorfman disease, reticulohistiocytoma, Langerhans cell histiocytosis, and traumatic ulcerative granuloma. Other lesions, such as EBV-positive mucocutaneous ulcer, extranodal marginal zone lymphoma of mucosal-associated lymphoid tissue, and plasmablastic lymphoma have lymphoid and/or plasma cell differentiation that mimic the reactive lymphocytes and plasma cells found in reactive ulcers. Two dendritic cell lesions, follicular dendritic cell sarcoma and blastic plasmacytoid dendritic cell neoplasm, both have distinct phenotypes which are required to make an accurate diagnosis.
CONCLUSION
Each of these lesions are diagnosed by evaluating their histology, along with their phenotypic profile, which is sometimes enhanced by pertinent molecular findings.
Topics: Humans; Oral Ulcer; Biopsy; Lymphoproliferative Disorders; Histiocytes
PubMed: 36928739
DOI: 10.1007/s12105-023-01532-2 -
Journal of Clinical Medicine May 2015Myeloid sarcoma (MS) of the central nervous system (CNS) is a rare presentation of leukemic mass infiltration outside of the bone marrow. It may involve the... (Review)
Review
Myeloid sarcoma (MS) of the central nervous system (CNS) is a rare presentation of leukemic mass infiltration outside of the bone marrow. It may involve the subperiosteum and dura mater and, on rare occasions, can also invade the brain parenchyma. The disease is most commonly seen in children or young adults; however, it has been described in multiple age groups. MS can be seen in patients with acute myeloid leukemia (AML), chronic myeloid leukemia and other myeloproliferative disorders. This entity has the potential to be underdiagnosed if the MS appearance precedes the first diagnosis of leukemia. The main reason is that their appearance on CT and MRI has a broad differential diagnosis, and proper diagnosis of MS can only be made if the imaging findings are correlated with the clinical history and laboratory findings. Herein, we describe the intracranial CNS manifestations of MS in patients with AML on CT and MRI involving the brain and/or meninges. This study is based on a systematic review of the literature. In addition, three case reports from the author's institution with AML and intracranial involvement of MS are included. Our aim is to enhance the awareness of this entity among both clinicians and radiologists.
PubMed: 26239467
DOI: 10.3390/jcm4051102