• acute myeloid leukaemia
• diagnostic approaches
• myeloid sarcoma

• acute myeloid leukemia (AML)
• allogeneic hematopoietic stem cell transplantation (HSCT)
• diagnosis
• extramedullary AML
• immunophenotype
• induction chemotherapy
• isolated MS
• molecular background
• myeloid sarcoma (MS)
• radiotherapy
• synchronous MS
• treatment

• acute myeloid leukemia
• extramedullary blast
• genetic
• mutation
• myeloid sarcoma

• Acute myeloid leukemia
• Immunohistochemistry
• Myeloid sarcoma

• Humans
• Sarcoma, Myeloid/diagnosis
• Sarcoma, Myeloid/pathology
• Leukemia, Myeloid, Acute/diagnosis
• Diagnosis, Differential
• Biopsy
• Immunophenotyping

• SET-CAN/NUP214 fusion gene
• T-cell acute lymphoblastic leukemia (T-ALL)
• acute myeloid leukemia (AML)
• leukemia
• molecular anomaly
• prognosis
• treatment

• Chloroma
• Extramedullary leukaemia
• Granulocytic sarcoma
• Myeloid neoplasms
• Myeloid sarcoma

• Mutations
• Myelofibrosis
• Myeloid neoplasm
• Progression
• Transformation

• Humans
• Granulocyte Precursor Cells/pathology
• Myeloproliferative Disorders/diagnosis
• Myeloproliferative Disorders/genetics
• Myeloproliferative Disorders/pathology
• Myelodysplastic Syndromes/diagnosis
• Myelodysplastic Syndromes/genetics
• Myelodysplastic Syndromes/pathology
• Mutation
• Myelodysplastic-Myeloproliferative Diseases/genetics
• Myelodysplastic-Myeloproliferative Diseases/pathology
• Leukemia, Myeloid, Acute/diagnosis
• Leukemia, Myeloid, Acute/genetics

• Acute myeloid leukemia
• Chemotherapy
• Genetics
• Immunophenotype
• Myeloid sarcoma
• Prognosis
• Radiotherapy

• chloroma
• genetic
• granulocytic sarcoma
• myeloid sarcoma
• prognosis

• Adult
• Humans
• Male
• Child
• Female
• Sarcoma, Myeloid/diagnosis
• Sarcoma, Myeloid/genetics
• Retrospective Studies
• Leukemia, Myeloid, Acute
• Prognosis
• Bone Marrow/pathology

• RNA sequencing
• myeloid sarcoma
• risk stratification

• BCL-2 inhibitors
• FLT3 inhibitors
• Genetic mutations
• IDH inhibitors
• Myeloid sarcoma

Myeloid sarcoma (MS) is relatively rare, occurring mainly in the skin and lymph nodes, and MS invasion of the ulnar nerve is particularly unusual. The main aim of this article is to present a case of MS invading the brachial plexus, causing ulnar nerve entrapment syndrome, and to further clinical understanding of the possibility of MS invasion of peripheral nerves.

We present the case of a 46-year-old man with a 13-year history of well-treated acute nonlymphocytic leukaemia who was admitted to the hospital after presenting with numbness and pain in his left little finger. The initial diagnosis was considered a simple case of nerve entrapment disease, with magnetic resonance imaging showing slightly abnormal left brachial plexus nerve alignment with local thickening, entrapment, and high signal on compression lipid images. Due to the severity of the ulnar nerve compression, we surgically investigated and cleared the entrapment and nerve tissue hyperplasia; however, subsequent pathological biopsy results revealed evidence of MS. The patient had significant relief from his neurological symptoms, with no postoperative complications, and was referred to the haemato-oncology department for further consultation about the primary disease. This is the first report of safe treatment of ulnar nerve entrapment from MS. It is intended to inform hand surgeons that nerve entrapment may be associated with extramedullary MS, as a rare presenting feature of the disease.

MS invasion of the brachial plexus and surrounding tissues of the upper arm, resulting in ulnar nerve entrapment and degeneration with significant neurological pain and numbness in the little finger, is uncommon. Surgical treatment significantly relieved the patient’s nerve entrapment symptoms and prevented further neurological impairment. This case is reported to highlight the rare presenting features of MS.

• Myeloid sarcoma
• Ulnar nerve entrapment syndrome
• Acute nonlymphocytic leukaemia
• Sarcoma
• Upper limb surgery
• Case report

• Hoxa10
• Hoxa11
• KRAS mutation
• MLL translocation
• Migration
• Myeloid sarcoma

• Animals
• Cell Movement/genetics
• Homeodomain Proteins/genetics
• Homeodomain Proteins/metabolism
• Humans
• Leukemia, Myeloid, Acute
• Mice
• Mutation
• Oncogene Proteins, Fusion/genetics
• Proto-Oncogene Proteins p21(ras)/genetics
• Proto-Oncogene Proteins p21(ras)/metabolism
• Sarcoma, Myeloid
• Transcription Factors/genetics

extramedullary acute myeloid leukemia (eAML) is characterized by extramedullary tumor formation infiltrated by myeloid blasts, with or without maturation and effaced architecture. The clinical, genetic and molecular aspects and overall outcomes are well defined worldwide, but not well characterized in our region.

This is a retrospective single center cohort study on 32 patients, who were identified over 10 years to study the clinical, pathologic and genetic-molecular aspects, and survival outcomes.

eAML is rare (1%), occurs at a younger age with male predominance. Central nervous system (CNS) with facial bone invasion is most commonly identified (34.4%). 45.5% were positive for conventional myeloid markers (MPO), CD33, CD117, and 36% positive for CD34 and CD68. 54% with normal karyotype had deleterious mutations on further testing. NGS revealed pathogenic mutations in 76%(N-9/17) and none tested positive for P53, IDH1 or IDH2. At a median follow up time of 43mo (range, 8.6–80mo); 37.5%(N-12) were in complete remission, 62.5%(N-20) relapsed. 28% of relapses were after allotransplant. 31%(N-10) alive and continued in complete remission(CR), and 69%(N-22) of patients have died.

Median overall survival (OS) is 18.4 and relapse free survival (RFS) 18.7 months. OS and RFS were significantly better in patients, who attained CR after induction (IC 11.9 mo vs zero; P = 0.0001; IC 12mo vs zero; P = 0.0001) compared to patients with relapsed disease; and in patients who received allo-transplant consolidation with median OS and RFS 42 vs 8.5mo (P = 0.002) and 42months vs 10 mo (P = 0.006). Thus allotransplant may be considered for all eligible patients in first CR.

achievement of complete remission after induction therapy is associated with improved outcomes in eAML. Allotransplant in first complete remission may be the most effective modality for achieving long-term remissions.

•

eAML is a rare entity and occurs at a younger in Jordan.

• AML
• Extramedullary disease
• Leukemia
• Myeloid sarcoma

Myeloid sarcoma (MS) is a tumor secondary to myeloid leukemia that consists of immature granulocytes with or without mature granulocytes and is a rare extramedullary manifestation of acute myeloid leukemia (AML).

We report a case of a 34-year-old woman diagnosed with AML-M4 who achieved remission after chemotherapy and received allogeneic stem cell transplantation (allo-SCT) for consolidation. Her past medical history showed that she received bilateral breast implants 7 years ago. This patient underwent ultrasound examination of the breast and multiple bilateral breast nodules were revealed that were not considered by clinicians to be concerning. Several months later, the patient’s bilateral nodules rapidly progressed to large palpable masses. Ultrasound-guided biopsy revealed diffuse infiltration of undifferentiated tumor cells and immunohistochemistry (IHC) indicated that the tumor was positive for myeloperoxidase (MPO), cluster of differentiation (CD) 34, CD43, CD68, CD117, and Ki67. The pathological diagnosis was extramedullary recurrence of AML as MS of breast. After the diagnosis, the patient received systemic chemotherapy and drugs containing cytarabine, azacitidine, and methotrexate. However, 1 year after achieving partial remission, the patient died from intracranial invasion of leukemia, brain herniation, and respiratory failure.

It is necessary for the specialist to have a high suspicion index by careful inquiry of the patient’s medical history if a patient presents at the breast clinic with a breast tumor as the chief complaint. Combining information from the patient’s medical history with a tumor biopsy is critical for obtaining the correct diagnosis of the disease.

• Acute myeloid leukemia
• Breast myeloid sarcoma
• Case report
• Extramedullary manifestation
• Stem cell transplantation

• chemotherapy
• extramedullary
• leukaemia
• prognosis
• survival

• primary granulocytic sarcoma
• oral tumor
• clinical characteristics

• Antibiotics, Antineoplastic/therapeutic use
• Antimetabolites, Antineoplastic/therapeutic use
• Cytarabine/therapeutic use
• Female
• Humans
• Idarubicin/therapeutic use
• Middle Aged
• Mouth/pathology
• Mouth Neoplasms/diagnosis
• Mouth Neoplasms/drug therapy
• Mouth Neoplasms/pathology
• Remission Induction
• Sarcoma, Myeloid/diagnosis
• Sarcoma, Myeloid/drug therapy
• Sarcoma, Myeloid/pathology

Isolated intracranial myeloid sarcoma (MS) is an unusual variant tumor with few cases reported so far in the medical literature. A 29-year-old woman was admitted to our hospital presenting progressive visual loss in the right eye and weight loss (20 kg) without a previous history of hematological disease (HD). Radiologic evaluation showed the evidence of intracranial mass. Histologically, the resected tumor was composed of a uniform population of primitive cells and primarily misdiagnosed as a T-cell non-Hodgkin’s lymphoma (NHL). Chemotherapy with cyclophosphamide, doxorubicin, vinblastine, and prednisone (CHOP) was ineffective. A biopsy and histopathological evaluation were repeated, and immunohistochemical staining revealed the positivity of immature cells to an extensive panel of myeloid markers. These findings were consistent with a diagnosis of MS and bone marrow infiltration. Literature reviews of previous cases were also undertaken.

• intracranial
• isolated
• misdiagnosis
• myeloid sarcoma
• non-Hodgkin’s lymphoma

• acute myeloid leukemia
• allogenic bone marrow transplant
• chemotherapy
• extramedullary leukemia
• granulocytic sarcoma
• myeloid sarcoma
• pancreatic sarcoma

• FNAC
• non-Hodgkin's lymphoma
• primary adrenal lymphoma

Myeloid sarcoma (MS) is a rare, extramedullary tumor consisting of immature white blood cells of myeloid lineage. MS is usually associated with the concurrent diagnosis of acute myeloid leukemia (AML) but can also present in the absence of bone marrow disease or at relapse of AML. MS of the gynecologic tract is exceedingly rare; however, it is hypothesized that it is likely more prevalent than previously understood given postmortem findings and persistence in preserved ovarian tissue. There is minimal literature surrounding MS and extramedullary relapse with no clear guidelines. This is a case report of a 48-year-old woman with MS involving the uterine corpus, fallopian tubes, and left ovary followed by a literature review. The overall aim is to review data regarding leukemic immune evasion and sanctuary sites in order to raise awareness as this represents an important and underrecognized hematologic malignancy in an often misdiagnosed, underrecognized site.

Myeloid sarcoma (MS) is a rarely encountered extramedullary localized tumor that is composed of immature myeloid cells. We reported an extremely rare case of MS with concurrent bone marrow (BM) involvement that invaded into a preexisting sebaceous lymphadenoma in the parotid gland and neck lymph nodes. Prompted by this case, we also present a literature review of MS invasion into salivary glands. A 62-year-old man was initially diagnosed with carcinoma that arose in a sebaceous lymphadenoma in the parotid gland, through a total parotidectomy with neck dissection. After an extensive histopathological review that included immunohistochemistry, a pathologic diagnosis of MS with infiltration into the sebaceous lymphadenoma with concurrent BM involvement was confirmed. MS is difficult to diagnose accurately; herein, we analyzed the clinical presentations and effectiveness of the various diagnostic methods with a review of the literature. There are 17 cases, including our case, reported in 13 studies. Of the cases in which the salivary glands were affected, 10 involved the parotid gland, six involved the submandibular gland, and one involved both. Isolated invasion of the salivary gland was found in one case of parotid gland invasion and three cases of submandibular gland invasion. In 13 cases, the salivary glands were affected by various other lesions. Although there were no incidences of isolated MS, six patients were diagnosed with secondary MS and eight patients with MS with BM involvement, including this case. The diagnosis of MS is difficult given its rarity, and a high index of suspicion and integrated radiologic and careful histopathologic evaluation are required. Most cases of MS infiltrating the salivary gland might be indicated by the possibility of BM involvement. MS with BM involvement predicts poor prognosis and the need for intensive systemic treatment.