Myeloid sarcoma with maxillary gingival swell

BACKGROUND Myeloid sarcoma(MS),also referred to as granulocytic sarcoma or chloroma,is a rare type of extramedullary malignant tumor.MS comprises primitive granulocytic precursor cells that play a key role in the early stages of white blood cell development.Notably,the occurrence of this tumor in the gingiva is rare.CASE SUMMARY The present study reported the case of MS with gingival swelling in the maxillary region,with aleukemic presentation in a 32-year-old male patient.Following two courses of chemotherapy,computed tomography of the region demonstrated complete clearance of the tumor.At the 12-month follow-up appointment,the patient was in a stable condition with the absence of progression.The etiology,clinical features,diagnosis,and relevant treatment of MS are discussed in the present study.CONCLUSION Diagnosis of MS may be confirmed following histological and immunohistochemical examinations.

Diagnostic and therapeutic challenges of myeloid sarcoma in the oral cavity

Myeloid sarcoma(MS)is a rare neoplasm characterized by the proliferation of immature myeloid precursor cells outside the bone marrow.The pathogenesis of MS is complex and not completely understood.Moreover,it develops in any extramedullary site of the body.In this editorial,we discuss the article published by Li et al,which presents a clinical case involving a 32-year-old man who exhibited gingival inflammation in the maxillary region.It was initially diagnosed as periodontal disease.However,clinical evaluation revealed a firm,grayishwhite mass which underscored the need for comprehensive diagnostics to distinguish MS from other oral conditions.This article emphasizes the different clinical presentations of similar case studies in the literature,and highlights the difficulty in diagnosing oral MS due to its rarity and variability in clinical manifestation.The treatment of MS depends on the clinical presentation,tumor location,and the patient's response to conventional therapies.The various therapeutic options currently available are analyzed and discussed.Early intervention and multidisciplinary management are crucial for improving treatment outcomes.Increased awareness and education about the various clinical presentations of MS lead to earlier diagnosis and timely treatment,thereby enhancing patients'survival and quality of life.Continued research is essential for optimizing therapeutic strategies and addressing the challenges presented by this rare neoplasm.

Cervical myeloid sarcoma as an initial clinical manifestation: Four case reports

BACKGROUND Cervical myeloid sarcoma(MS)is a rare hematological malignancy characterized by the formation of extramedullary soft tissue masses in the cervical region.Due to its uncommon presentation in the female reproductive system,cervical MS poses significant diagnostic and therapeutic challenges.Consequently,there is a pressing need for more research and clinical experience to better understand,diagnose,and manage this condition effectively.CASE SUMMARY This report details four cases,the diagnostic process,treatment strategy,and outcomes,discussing cervical MS as an initial clinical manifestation.The disease exhibits varied clinical presentations,such as irregular vaginal bleeding and pa-lpation of cervical masses.The treatment approaches discussed include neoad-juvant chemotherapy,surgery,and postoperative chemotherapy,though mana-ging the disease remains challenging.The report also features a comprehensive literature review that underscores the importance of immunohistochemistry for accurate diagnosis,identifying key markers,including myeloperoxidase,cluster of differentiation(CD)68,and CD43,stressing the need for further research to improve treatment strategies and prognosis.CONCLUSION Immunohistochemical diagnosis and tailored therapeutic strategies are essential.Further research is crucial in improving outcomes and developing effective treatment protocols.

Myeloid sarcoma as the only manifestation in a rare mixed lineage leukemia-fusion-driven acute myeloid leukemia:A case report

BACKGROUND The mixed lineage leukemia(MLL)-eleven-nineteen lysine-rich leukemia(ELL)fusion gene is a rare occurrence among the various MLL fusion genes.We present the first case in which myeloid sarcoma(MS)was the only manifestation of adult MLL-ELL-positive acute myeloid leukemia(AML).CASE SUMMARY We report a case of a 33-year-old male patient who was admitted in June 2022 with a right occipital area mass measuring approximately 7 cm×8 cm.Blood work was normal.The patient underwent right occipital giant subscalp mass excision and incisional flap grafting.Immunohistochemistry was positive for myeloperoxidase,CD43 and CD45 and negative for CD3,CD20,CD34,and CD56.The bone marrow aspirate showed hypercellularity with 20%myeloblasts.Flow cytometry showed that myeloblasts accounted for 27.21%of the nucleated cells,which expressed CD33,CD38,and CD117.The karyotype was 46,XY,t(11,19)(q23;p13.1),-12,+mar/46,XY.Next-generation sequencing showed a fusion of MLL exon 7 to exon 2 of ELL.A diagnosis of MLL-ELL-positive AML(M2 subtype)with subcutaneous MS was made.CONCLUSION MLL-ELL-positive AML with MS is a rare clinical entity.Additional research is needed to elucidate the molecular mechanisms of the pathogenesis of MS.

Myeloid sarcoma of the colon as initial presentation in acute promyelocytic leukemia:A case report and review of the literature

BACKGROUND Myeloid sarcoma(MS)rarely occurs in acute promyelocytic leukemia(APL)at onset,but it can develop in relapse cases,especially after APL treated with alltrans retinoic acid(ATRA).Therefore little is known about the clinical features and suitable treatment for APL related MS due to the rarity of the disease,although this may be different from the treatment and prognosis of MS in the relapse stage.To our best knowledge,this is the second case report of APL initial presentation as colon MS.CASE SUMMARY A 77-year-old woman complained of intermittent right lower abdominal pain,black stool,and difficult defecation for 2 mo.Physical examination showed diffuse tenderness during deep palpation and an anemic appearance.Laboratory findings showed positivity for fecal occult blood testing;white blood cell count:3.84×109/L;hemoglobin:105 g/L;platelet count:174×109/L;and negativity for tumor markers.Abdominal enhanced computed tomography showed a space occupying lesion in the colon(1.9 cm).Fibrocolonoscopy revealed a polypoid and ulcerated mass measuring 2.5 cm.The tumor was removed.To our surprise,MS was confirmed by immunohistochemistry.PML/RARαfusion gene was detected in colon specimens by fluorescent in situ hybridization and real-time reverse transcription polymerase chain reaction,which was consistent with the bone marrow.She was diagnosed as having APL related MS.A smooth and unobstructed intestinal wall was found by fibrocolonoscopy,and continuous molecular remission was confirmed in both the bone marrow and colon after four courses of ATRA+arsenic trioxide(ATO).ATRA+ATO showed a favorable therapeutic response for both APL and MS.CONCLUSION Early use of ATRA can benefit APL patients,regardless of whether MS is the first or recurrent manifestation.

Gastric myeloid sarcoma:A case report

BACKGROUND Myeloid sarcoma(MS)is a rare hematologic malignancy defined as an extramedullary tumor of immature granulocytic cells.It can occur as primary or de novo 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 de novo 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 de novo 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.

Different Outcome of Myeloid Sarcoma with Spinal Cord Compression Preceding Acute Myeloid Leukemia:Report of Two Cases and Review of Literatures

Myeloid sarcomas （MS） preceding acute myeloid leukemia （AML） are rare, which presenting as acute spinal cord compression is even rare. Here we report two new cases of myeloid sarcoma patients, whose outcomes were different. Twenty-seven patients with spinal MS preceding AML have been reported to date, including the two cases presented in this article. Surgical decompression was performed in 25 of the 27 patients, and 23 of these received additional anti-AML therapy. Considering our patients and the published cases in the literature we suggest that immunohistochemical study plays an essential role in arriving at a correct diagnosis of MS, and that emergency surgery to resect spinal MS is an available treatment to make neural function recovery, and that the disease must be treated with intensive chemotherapy similar to that used to treat AML as soon as possible after resection or irradiation of the tumor.

Rare myeloid sarcoma/acute myeloid leukemia with adrenal mass after allogeneic mobilization peripheral blood stem cell transplantation

Myeloid sarcoma(MS)is a rare hematological neoplasm that develops either de novo or concurrently with acute myeloid leukemia(AML).This neoplasm can also be an initial manifestation of relapse in a previously treated AML that is in remission.A 44-year-old male patient was diagnosed with testis MS in a local hospital in August 2010.After one month,bone marrow biopsy and aspiration confirmed the diagnosis of AML.Allogeneic mobilization peripheral blood stem cell transplantation was performed,with the sister of the patient as donor,after complete remission(CR)was achieved by chemotherapy.Five months after treatment,an adrenal mass was detected by positron emission tomography-computed tomography(PET-CT).Radiotherapy was performed for the localized mass after a multidisciplinary team(MDT)discussion.The patient is still alive as of May 2013,with no evidence of recurrent MS or leukemia.

Vulvovaginal myeloid sarcoma with massive pelvic floor infiltration: A case report and review of literature

BACKGROUND Myeloid sarcoma(MS),including isolated and leukaemic MS,is an extramedullary myeloid tumour.MS can involve any anatomical site,but MS of the female genital tract is rare,with the ovaries and uterine body and cervix being the most commonly seen sites.Involvement of the vagina and vulva is extremely rare.CASE SUMMARY We report a rare case of MS with involvement of the vulva and vagina and massive infiltration of the pelvic floor.A 26-year-old woman presented with a vulvar mass,irregular vaginal bleeding and night sweats.Magnetic resonance imaging demonstrated an ill-defined,irregular vulvovaginal mass with massive involvement of the paravaginal tissue,urethra,posterior wall of the bladder,and pelvic floor.The signal and enhancement of the huge mass was homogeneous without haemorrhage or necrosis.Positron emission tomography/computed tomography showed high fluorodeoxyglucose uptake by the mass.Peripheral blood count detected blast cells.Vulvovaginal mass and bone marrow biopsies were performed,and immunohistochemistry confirmed the diagnosis of acute myeloid leukaemia(M-2 type,FAB classification)and vulvovaginal MS.The patient was treated with induction chemotherapy followed by allogeneic haematopoietic stem cell transplantation,and achieved complete remission.A systemic review of the literature on vulvovaginal MS was conducted to explore this rare entity’s clinical and radiological features.CONCLUSION Vulvovaginal MS is extremely rare.Diagnosis of vulvovaginal MS can only be confirmed histopathologically.Even though its clinical and imaging presentations are nonspecific,MS should be considered in the differential diagnosis of a newly developed T2-hyperintense,homogeneously enhanced vulvovaginal mass,especially in a patient with suspected haematological malignancy.

Myeloid sarcoma with ulnar nerve entrapment:A case report

BACKGROUND 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.CASE SUMMARY 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.CONCLUSION 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.

Simultaneous Diagnosis of Myeloid Sarcoma of the Jaw and Mycobacterium tuberculosis Infection

Granulocytic or myeloid sarcoma (MS) is a rare neoplastic condition consisting of a tumor mass of myeloid blasts with or without maturation occurring at an anatomical site other than the bone marrow the association between tuberculosis and MS is extremely rare. A 21-year-old female patient presented cough, sore throat and a suppurative swollen gum for 10 days prior to hospital admission. Physical examination revealed moderate pallor and swollen inferior gum. CBC revealed Hb6.5 g/dL, hematocrit 18.4% MCV 97 fL MCH 34 pg, WBC 18.5 ′ 109/μL (1 My/3 Bt/69 Sg/1 Eo/0 Ba/20 Ly/6 Mo), Platelets 43 ′ 109/μL. The peripheral blood smear presented with 3% blast cells (type 1) and granulocytic dysplasia. Bone marrow biopsy showed 100% cellularity. 50% of cells were from granulocytic precursors, diagnosis of granulocytic sarcoma. The diagnosis of AML was established: granulocytic sarcoma with massive gum infiltration (immature granulocytic cells) and 10% of blasts in bone marrow. The patient received induction chemotherapy (3 + 7 daunorubicin 90 mg/m2), and gum tissue culture was positive for Mycobacterium tuberculosis. Simultaneously, a qRT- PCR test confirmed the same bacteria in the gum tissue. Patient treated with isoniazid, rifampicin, pyrazinamide and ethambutol ciprofloxacin and amikacin). Remission was achieved and the patient was submitted for consolidation/ intensification (HiDAC x3) schema and referred to allogeneic HSCT. After induction and full hematological recovery there was no further evidence or recurrence of fever and lytic lesions. Currently patient is under CR and ling follow up (48 months) did not show recurrence of either AML or tuberculosis.

Gastric myeloid sarcoma without acute myeloblastic leukemia

Myeloid sarcomas(MS)involve extramedullary blast proliferation from one or more myeloid lineages thatreplace the original tissue architecture,and these neoplasias are called granulocytic sarcomas,chloromas or extramedullary myeloid tumors.Such tumors develop in lymphoid organs,bones(e.g.,skulls and orbits),skin,soft tissue,various mucosae,organs,and the central nervous system.Gastrointestinal(GI)involvement is rare,while the occurrence of myeloid sarcomas in patients without leukemia is even rare.Here,we report a case of a 38-year-old man who presented with epigastric pain and progressive jaundice.An upper GI endoscopy had shown extensive multifocal hyperemic fold thickening and the spread of nodular lesions in the body of the stomach.Biopsies from the gastric lesions indicated myeloid sarcoma of the stomach.However,concurrent peripheral blood and bone marrow examinations showed no evidence of acute myeloid leukemia.For diagnosis,the immunohistochemical markers must be checked when evaluating a suspected myeloid sarcoma case.Accurate MS diagnosis determines the appropriate therapy and prognosis.

Isolated myeloid sarcoma in the pancreas and orbit:A case report and review of literature

Myeloid sarcoma(MS) is a type of extramedullary solid haematological tumour. Myeloid sarcoma is classified into two types based on whether onset of the disease is complicated by haematologic diseases: extramedullary infiltration of leukaemia(leukaemic MS) and isolated myeloid sarcoma. The incidence of isolated myeloid sarcoma is low. In particular, isolated myeloid sarcoma involving the pancreas is extremely rare and prone to misdiagnosis. This case report describes the long and eventful diagnostic process of a case of myeloid sarcoma involving the pancreas and orbit. Due to a lack of typical clinical manifestations and imaging characteristics, the patient underwent several rounds of treatment without a confirmed diagnosis. Eventually, the final diagnosis was pathologically confirmed using several types of biopsies and immunohistochemical detection. To date, this type of disease has not been reported in the literature. This case report describes the detailed diagnostic process and discusses the strategies used for diagnosis, which will facilitate the diagnosis of such diseases in the future.

Myeloid sarcoma presenting as a colon polyp and harbinger of chronic myelogenous leukemia

Myeloid sarcoma, also known as granulocytic sarcoma or chloroma is an unusual accumulation of malignant myeloid precursor cells in an extramedullary site, which disrupts the normal architecture of the involved tissue. It is known to occur more commonly in patients with acute myelogenous leukemia and less commonly in those with myelodysplastic syndrome and myeloproliferative neoplasm, such as chronic myelogenous leukemia. The most common sites of involvement include bone, skin and lymph nodes. However, rare cases have been reported in the gastrointestinal tract, genitourinary tract, or breast. Most commonly, a neoplastic extramedullary proliferation of myeloid precursors in a patient would have systemic involvement of a myeloid neoplasm, including in the bone marrow and peripheral blood. Infrequently, extramedullary disease may be the only site of involvement. It may also occur as a localized antecedent to more generalized disease or as a site of recurrence. Herein, we present the first case in the English literature of a patient presenting with an isolated site of myeloid sarcoma arising in the form of a colonic polyp which, after subsequent bone marrow biopsy, was found to be a harbinger of chronic myelogenous leukemia.

Spinal Epidural Myeloid Sarcoma with Paraplegia in Acute Myeloid Leukemia Treated with Radiation and Chemotherapy: A Case Report

Myeloid sarcoma occurs in 1% - 9% of patients with myelogenous leukemia. Spinal epidural myeloid sarcoma is particularly rare, and its treatment has not been established. A 27-year-old woman complained of pain on her left chest, back around the scapula, and neck. Magnetic resonance imaging (MRI) showed a thoracic epidural tumor. One week after her visit, she developed motor weakness of her lower extremities and dysuria, and she was diagnosed with acute myelogenous leukemia (AML) on peripheral blood analysis. The epidural tumor was strongly suspected to be myeloid sarcoma. The paralysis of the lower extremities and bladder dysfunction were not progressive, and chemotherapy and local radiation therapy to the spine were performed. Improvement of paralysis and complete reduction of tumor volume were achieved by the combination of local low-dose radiation therapy and chemotherapy.

Myeloid sarcoma as a differential diagnosis of small bowel obstruction

We are presenting the case of a 42-year-old ?male patient, who was hospitalized due to an acute small bowel obstruction caused by a tissue mass of the mesentery. The patient reported that he had a history of a testicular tumour. For therapy of intestinal obstruction as well as for diagnostic reasons we decided to perform ?an explorative laparotomy. On histopathological examination the immunohistological staining was positive for myeloperoxidase (MPO) and KP-1 (CD68). Staining was slightly positive for Bcl-2, CD117, CD34, but negative for CD3, CD4, CD5, CD8, CD20, CD30, CD79, Bcl-6 and S-100. This leads to the diagnosis of a myeloid sarcoma. After recovery from surgery and chemotherapy, allogenic bone marrow transplantation was performed. Most intestinal obstructions are caused by postoperative adhesions or hernias and only in rare cases caused by a myeloid sarcoma.

Isolated myeloid sarcoma of the pancreas:a case report

Myeloid sarcoma(MS)is a rare solid tumor,and the diagnose is ambiguity.On account of the rarity of MS,it is often misdiagnosed.In order to promote clinicians to have a better understanding of the disease,a case of isolated myeloid sarcoma of the pancreas has been admitted by the Center for Pancreas,the First Affiliated Hospital of Nanjing Medical University.A 36-year-old male patient presented to our department with recurrent postprandial abdominal distension and pain,and weight loss.The initial radiologic diagnose of the lesion was pancreatic cancer and a pancreatectomy was performed.Eventually,the histopathology result confirmed the final diagnosis:myeloid sarcoma(MS).To date,this type of disease has hardly been reported in the literature.This case report describes the diagnostic and treatment process and discusses the better way for diagnosis,which will guide such diseases in the future.

A case of steroid-dependent myeloid granulocytic sarcoma masquerading as Crohn's disease

Small bowel tumors and Crohn's disease are common causes of small bowel obstruction.Early stage neoplasms can easily be mistaken for Crohn's disease.Therefore,thorough work-ups including imaging studies and endoscopic evaluation with biopsies are critical for accurate diagnosis.Here we report a case of an otherwise healthy female with progressive onset of multiple,recurrent obstructive symptoms secondary to terminal ileal narrowing who was referred for management of steroid-dependent Crohn's disease.After thorough evaluation,the diagnosis was revised to myeloid granulocytic sarcoma involving the terminal ileum.In this case,a delay in diagnosis can be detrimental for prognosis,as myeloid granulocytic sarcoma is highly predictive of underlying acute myeloid leukemia and needs urgent referral for chemotherapy and/or resection.

Misdiagnosed myeloid sarcoma of the vulva

A 25-year-old woman presented with a lump in the vulva associated with progressive difficulty in urination that had persisted for approximately half a year. A diagnosis of Bartholin＇s abscess had been made in another hospital. Incision revealed no pus and ultrasonography showed a solid lesion in the left vulva. Partial excision of the tumor was implemented and histopathology showed a small round cell tumor. There were no other significant findings in her present or past medical history. She was admitted to our hospital with vulvar cancer. On gynecological examination, a 7 cm × 6 cm finn, hard, painless mass was found in the left labium majora. The right labium was inflamed but painless.

原发性髓系肉瘤遗传学和分子学改变的临床意义

目的:探讨原发性髓系肉瘤遗传学和分子生物学改变的临床意义。方法:选择2010年9月-2021年12月于上海健康医学院附属嘉定区中心医院、连云港市第一人民医院诊断的14例原发性髓系肉瘤患者为研究对象,应用荧光原位杂交技术检测其AML1-ETO融合、PML-RARα融合、CBFβ断裂情况,应用下一代基因测序技术检测其NPM1、CEBPA、FLT3、RUNX1、ASXL1、KIT、TP53等基因突变情况。结果:14例患者的髓系肉瘤发生于骨、乳腺、附睾、肺、胸壁、宫颈、小肠、卵巢、淋巴结、中枢神经系统等组织器官,肿瘤细胞表达MPO(13例)、CD34(7例)、CD43(8例)、CD68(7例)、CD99(8例)、CD117(6例)。4例检测出细胞遗传学异常(AML1-ETO融合3例,CBFβ断裂1例),未检出PML-RARα融合。发生AML1-ETO融合/CBFβ断裂病例的总体生存期、无白血病生存期与未发生AML1-ETO融合/CBFβ断裂者比较差异均无统计学意义(P>0.05)。分别有5例、3例、5例、3例、2例、2例和1例患者发生NPM1、CEBPA、FLT3-ITD、RUNX1、ASXL1、KIT、TP53基因突变,其中7例至少发生FLT3-ITD、RUNX1、ASXL1、TP53基因中的一个突变。发生FLT3-ITD/RUNX1/ASXL1/TP53突变患者的总体生存期、无白血病生存期均明显短于未发生突变者(P<0.01)。结论:应用荧光原位杂交技术和下一代基因测序技术可以检出原发性髓系肉瘤的遗传学和分子学异常。FLT3-ITD/RUNX1/ASXL1/TP53突变提示预后更差,但仍需进一步临床研究证实。