Isolated pancreatic myeloid sarcoma: A potential mimicker of pancreatic adenocarcinoma

Myeloid sarcoma (MS) is an extramedullary proliferation of immature myeloid cells which may occur as a progression of myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN), or myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) and as acute myeloid leukemia (AML) relapse. Rarely may it be de novo. Lymph nodes, skin, lungs, intestine are the commonly involved sites. However, an isolated pancreatic MS is seldom reported in the literature. Herein, we report one such case which was misdiagnosed as pancreatic adenocarcinoma on the clinico-radiological examination which misled us away from preoperative diagnostic sampling, and a Whipple pancreaticoduodenectomy was performed. Histopathological examination in conjunction with immunohistochemistry revealed the final diagnosis of isolated MS of the pancreas. We emphasize that although rare, a clinical suspicion along with preoperative histopathological examination may lead to early diagnosis, targeted management, and a better clinical outcome in such cases.

CYTOLOGICAL DIAGNOSIS OF CUTANEOUS MYELOID SARCOMA–A RARE CASE REPORT

Introduction: Granulocytic sarcoma or myeloid sarcoma also known as chloroma is a rare extramedullary tumour which may occur as a manifestation of acute myeloid leukaemia, myelodysplastic syndrome or blast crisis in chronic myeloproliferative disorder or may precede systemic leukaemia. Most common site includes skin, soft tissue and lymph nodes. Orbit is most commonly involved in paediatric age group. Case Report: A case of 51 years old female was admitted in department of haematology, presented with multiple nodules in nasal cavity, forehead, bilateral arms and whole abdomen. Bone marrow aspiration cytology shows 21% myeloid blast with transformation of the CML to AML.FNAC was done from multiple nodules which showed plenty of myeloid precursors and blast and diagnosis of granulocytic sarcoma was given. BCR-ABL study came out positive and karyotyping for haematological malignancy showed t (5; 12)(q31;24.3). Patient was given chemotherapy, but showed no improvement. Conclusion: Granulocytic sarcoma (GS) is a rare malignant solid tumour in adults. Diagnosis of GS has been a problem for pathologist because of relatively immature nature of tumour cells and mostly misdiagnosed as Non Hodgkin's lymphoma. Diagnosis of GS is considered as an adverse prognostic factor but early confirmation of diagnosis and treatment initiation might improve the prognosis.

Granulocytic sarcoma of breast:clinical histopathologic and biological behavior / 中华内分泌外科杂志

granulocytic sarcoma (GS) is rare in the breast, histologically and immunophenotypically similar to myelogenous leukemia, radiologically lacking specificity, similar to breast cancer or mammary abscess. It should not be misdiagnosed as invasive cancer, especially in the case of frozen diagnosis, leading to excessive surgery. Three cases of GS were collected, specimens were fully drawn, microscopic pathologic examinations and immunohistochemistry (SP method) granulocytic sarcoma of breast were performed. The clinicopathological, immunohistochemical features, diagnosis and prognosis of GS are discussed to improve the awareness of the disease.

Granulocytic sarcoma of the breast: A case report in Lao PDR / Lao Medical Journal

@#Granulocytic sarcoma (GS) also known as myeloid sarcoma, chloroma, myeloblastoma, or extramedullary myeloid tumor is a neoplasm composed of immature myeloid cells. The common sites of involvement include bone, central nervous system, soft tissue, lymph nodes, and skin. The involvement of GS in breast tissue is very rare. The incidence of breast GS is 2/1,000,000 in adults. Those affected range in age from 16 to 72 years, with the mean age of 31 years. Primary, isolated, or non-leukemic GS of the breast is defined when bone marrow biopsy confirms the absence of other hematologic malignancy. We here report a case of granulocytic sarcoma of the left breast in a 33 year-old woman who presented with a breast mass. She was initially diagnosed as having diffuse lymphoma, large cell type on Hematoxylin and eosin stain (H&E) histopathology. The tumor cells were, however, strongly positive for myeloperoxidase (MPO), CD117, CD34, and CD43 but negative for CD45, CD20, CD3, or cytokeratin. Although further clinical information, such as complete blood count or aspiration biopsy of bone marrow tissue, was absent, we finally diagnosed this case as GS by additional immunohistochemical study. What happened to the patient?

Clinical outcome of allogeneic hematopoietic stem cell transplantation in the treatment of 9 myeloid leukemia patients with granulocytic sarcoma / 中华内科杂志

To explore the efficacy and influencing factors of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in patients with myeloid leukemia and granulocytic sarcoma (GS).Clinical outcome including hematopoietic reconstitution,transplant-related complications,survival and relapse were collected and retrospectively analyzed in 9 patients with myeloid leukemia and GS after allo-HSCT.Hematopoiesis reconstitution was achieved in all the 9 recipients.Four cases developed acute graft-versus-host disease (GVHD),and 1 with chronic GVHD.The median follow-up time after transplantation was 10(4-81) months.Only 2 cases survived,the other 7 died of relapse.The median time of relapse after transplantation was 5(3-19) months.Allo-HSCT is relatively effective treatment for patients with myeloid leukemia and GS.Relapse after transplantation remains the major factor of mortality.

Chronic Myeloid Leukemia Relapsing as Isolated Extramedullary Disease Presenting as a Cardiac Mass / 대한내과학회지

Allogeneic hematopoietic stem cell transplantation is the only option for chemotherapy-refractory chronic myeloid leukemia (CML). Extramedullary relapse after transplantation is rare and usually accompanies marrow relapse. Generally, the prognosis of extramedullary relapse is poor. Here, we report a man with extramedullary relapsed CML after stem cell transplantation that presented as an isolated cardiac mass, which has shown an indolent course for more than 2 years, without evolving to medullary relapse during that period. This case implies that the CML clone might contribute to the development of quiescent extramedullary relapse with a benign course.

Case of Acute Myeloid Leukemia with Mass of Eyelid and Conjunctiva in the Early Stage

PURPOSE: To report a case of an 82-year-old male with acute myeloid leukemia presenting with bilateral isolated conjunctival and eyelid masses. CASE SUMMARY: An 82-year-old male presented with a bilateral conjunctival mass and right eyelid mass occurring 10 days prior. He was diagnosed with prostate cancer 8 years ago and complete recovery was achieved using selective mass chemotherapy. He experienced a stroke 4 years ago and was treated using a carotid artery stent insertion and medication. In the initial laboratory test, hemoglobin was 13.7 g/dL and leukocyte count 5,530/mm3(neutrophil 74.4%, lymphocyte 10%, monocyte 11.8%). Light reflex, movement of extraocular muscle and fundus examination were all normal. Biopsy was performed 1 week after the first visit. Seven days after biopsy, he complained of sudden dyspnea and febrile sense and was admitted to the intensive care unit via the emergency room (ER). The laboratory tests performed in the ER showed hemoglobin was 9.6 g/dL and leukocyte count was 78,020/mm3(neutrophil 0%, lymphocyte 7%, monocyte 5%, promyelocyte 1%, metamyelocyte 4%, myelocyte 6%, blast 67%). The biopsy revealed diffuse proliferation of atypical plasmacytoid cells, consistent with leukemic infiltration. Under the diagnosis of acute myeloid leukemia, chemotherapy was administered. However, the patient died due to aggravated pneumonia. CONCLUSIONS: Even if non-specific findings appear on the peripheral blood tests, eyelid and conjunctival masses should be considered as possible tumors in acute myeloid leukemia.

Neoplasmas em suínos: 37 casos / Neoplasia in swine: 37 cases

Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

Imaging diagnosis of granulocytic sarcoma / 实用放射学杂志

Objective To summarize radiographic manifestations of granulocytic sarcoma (GS),in order to investigate its radio-graphic feature.Methods Nine patients of GS confirmed by surgery pathology and immunohistochemistry were collected.There were four cases underwent CT scan,2 underwent MRI,3 underwent CT and MRI meanwhile.Results There were 4 cases in the lymph nodes,4 cases in the bone,1 case in the enterocoelia.The density of enlarged lymph node was inhomogeneous,and smaller lymph node was homogeneous density,both enhanced obviously.The density or intensity of the lesions occurred in the canalis verteb-ralis and paravertebra was also homogeneous,slightly enhanced,with bone and medulla ossea invasion.Lesions in the enterocoelia was inhomogeneous desity with clear boundary,enhanced inhomogeneously.Conclusion GS is a rare extramedullary malignant tumor composed of immature myeloid cells.CT and MRI can provide a certain diagnostic value.The perplexity in diagnosing such lesions highlighted the need of careful interpretation of all clinacal,radiographic,histopathological and immunochistochemical details as it is one of the most frequently misdiagnosed disorder.

Myeloid Sarcoma of Maxillo-Ethmoidal Sinus: An Uncommon Presentation of Acute Myeloid Leukemia.

Introduction: Myeloid sarcoma (MS) is a rare localized extramedullary tumor of myeloid precursor cells. Short clinical history: An 11-year-old male presented with nasal bleed, two days; breathlessness and loss of appetite, one month and bilateral nasal blockade for two months. There was no fever. On clinical examination, bilateral cervical lymphadenopathy and hepatosplenomegaly was found. On opening mouth, a pink mass hanging behind the soft palate was noted. Magnetic resonance imaging of head and neck revealed a nasopharyngeal mass in maxillo-ethmoidal sinus. Gross pathology: Excised mass was greenish pink, measuring 2 × 2 × 2 cm. Microscopic examination: Hemogram revealed total leukocyte count 1.2 lac/mm3, hemoglobin 10.1 g/dl, and platelet count 41,000/mm3. Differential count showed 24% blasts with marked left shift. Bone marrow aspirate revealed 27% blasts. No auer rod seen. Cytochemistry showed myeloperoxidase (MPO) positivity. Trephine biopsy showed myeloid hyperplasia with excess blasts. Histopathological examination of lymph nodes and soft tissues showed leukemic infiltration. Immunohistochemistry showed blasts positive for MPO and negative for CD3. On multicolor flow cytometry blasts were positive for MPO, CD34 and CD45, and negative for CD3 and CD79a. A diagnosis of MS with acute myeloid leukemia (AML) was made. Discussion: It is rare in children (1 month-89 years). Any site of body, most commonly skin, lymph node, bone, etc. are affected. Prediction of first appearance of MS or AML was difficult in this case. MS may progress to AML simultaneously or may remain localized, never progressing to AML. Conclusion: Any extramedullary tumor showing myeloid precursor cells, should be investigated for MS, easily misdiagnosed as solid tumors.

Granulocytic Sarcoma in Breast after Bone Marrow Transplantation / 한국유방암학회지

Granulocytic sarcoma is a localized extramedullary solid tumor composed of immature myeloid cell and is usually associated with acute myeloid leukemia or myelodysplastic syndrome. Although it can involve any site, commonly in lymph nodes, skin, bone and soft tissue, the involvement of breast is unusual. Especially, the involvement of the breast as a pattern of relapse after bone marrow transplantation is extremely rare. We have experienced 2 cases of granulocytic sarcoma after bone marrow transplantation. One case was a 39-year-old woman with right breast mass diagnosed with granulocytic sarcoma. She had received an unrelated bone marrow transplantation due to biphenotype acute leukemia 3 years before our presentation. Another case was a 48-year-old woman with acute myeloid leukemia, who was diagnosed with granulocytic sarcoma on both breasts 8 months after allogenic bone marrow transplantation. We also discuss the clinicopathologic features of granulocytic sarcoma in breast after bone marrow transplantation.

Acute Myeloid Leukemia Presenting as Obstructive Jaundice.

Jaundice as a presenting feature of pediatric acute myeloid leukemia is rare. We report two cases of AML who presented with obstructive jaundice, one with a malignant stricture at the common bile duct and other with a granulocytic sarcoma obstructing the bile duct. The prognosis is poor in these patients.

Sarcoma granulocítico en una paciente con leucemia mieloide crónica / Granulocytic sarcoma in a female patient with chronic myeloid leukemia

El sarcoma granulocítico es un tumor localizado poco común, compuesto de células granulocíticas inmaduras. Generalmente se presenta en pacientes con leucemia mieloide aguda, síndromes mielodisplásicos o leucemia mieloide crónica. Puede ocurrir en cualquier localización anatómica. Reportamos el caso de una mujer de 41 años con el diagnóstico de leucemia mieloide crónica que presentó edema en brazo derecho que no remitía a pesar de tratamiento antibiótico y fasciotomía. El estudio histológico de la biopsia la lesión tomada mostró un sarcoma granulocítico. En este trabajo se revisa la citogenética, presentación clínica, diagnóstico y pronóstico de este síndrome tumoral.

Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. It may present in patiens with acute myeloid leukaemia, myelodysplastic syndrome or chronic myelogenous leukaemia. It may occur in any anatomical site. We report on the case of a 41 year old female diagnosed with chronic myelogenous leukaemia who presented with right arm swelling that did not resolve after antibiotic and surgical fasciotomy. The histological examination showed granulocytic sarcoma. In this report the citogenetics, clinical presentation, diagnosis and outcome of granulocytic sarcoma was reviewed.

Relapse presenting as granulocytic sarcoma without bone marrow involvement in acute myeloid leukemia with complete remission: a case report and review of literature / 白血病·淋巴瘤

ObjectiveTo explore clinical characteristics and diagnosis of patients with granulocytic sarcoma (GS),and to evaluate the value of FCM in diagnosing it.MethodsClinical data of one patient with GS was reviewed and related literature was reviewed. ResultsThe patient was diagnosed as AML-M2,chromosomal karyotype was 46.XY, t (8;21)(q22;q22)and the AML/ETO gene was positive. Systemic chemotherapy with daunorubicin plus cytarabine was given and complete remission was received. Then a nodular in medial angle of right eye was found. Result of CT indicated the possibility of leukemia infiltration.Needle aspiration cytology was conducted and many blast cells were found by microscope.CD34+ CD117+ CD13+ CD33+CD45dim SSC+ can be found by FCM. The cytology was complete remission and minimal residual disease was negative. Finally diagnosis was GS, relapse of AML. After a systemic chemotherapy with large dose of cytarabine plus teniposide (cytarabine 6.0 g/d, d1-3;teniposide 50 mg/d, d1-3), the mass could not be touched and the follow-up was continued.Conclusion Although relapse of AML often occurs in the testicle or the central nervous system,it pay attention to the possibility of relapse of AML presenting as GS.Fine needle aspiration cytology(FNAC)combined with FCM can provide an convenient, handy, practicable and less invasive way of the diagnosis and can be a preferred detection technique.

Multiple Granulocytic Sarcomas in a Patient with Longstanding Complete Remission of Acute Myelogenous Leukemia

Granulocytic sarcoma is an extramedullary tumor composed of granulocytic precursor cells. It usually presents as a nodular mass in the course of acute myelogenous leukemia. Rarely, the tumor develops in non-hematological conditions or in a patient with complete remission from the acute myelogenous leukemia. In such cases, aleukemic granulocytic sarcoma can be a preceding sign of systemic leukemia or a first sign of hematologic relapse of leukemia. We present an unusual case of multiple granulocytic sarcomas developed in a patient with longstanding complete remission of acute myelogenous leukemia, who has not had bone marrow and peripheral blood involvement for a long time.

Aspectos imaginológicos del sarcoma granulocítico en la cara y el cráneo de niños y jóvenes: informe de cinco casos y revisión de la literatura / Radiological findings of granulocytic sarcoma in the face and skull of children and young persons: report of five cases and literature review

Introducción: el sarcoma granulocítico (SG) es una neoplasia maligna cuya incidencia es de 2,9% a3,1% en pacientes con leucemia mieloide (LM) o enfermedades mieloproliferativas. Se presentahabitualmente en hombres y en población africana, asiática y suramericana. Objetivo: describir las características imaginológicas del SG en la cara y el cráneo de cinco niños y jóvenes. Pacientes y métodos: presentamos cinco pacientes con SG en la cara y la base del cráneo. A cuatro de ellos se les hizo tomografía computarizada (TC) y a tres, resonancia magnética (RM) cerebral. Cuatro presentaron una masa de tejido blando en la órbita, dos tenían afectación ósea y otro reveló lesión en el sistema nervioso central. En cuatro se diagnosticó leucemia mieloide aguda (LMA) .Conclusión: el SG puede manifestarse con invasión orbitaria y craneofacial en niños y adultos jóvenes. Usualmente los pacientes consultan por proptosis y edema orbitario. Con este cuadro clínico el SG es la primera probabilidad diagnóstica en el contexto de la LMA o las mielodisplasias. En otras situaciones clínicas se debe hacer diagnóstico diferencial con complicaciones de sinusitis, rabdomiosarcoma, linfoma de la órbita y otras neoplasias. La imaginología demuestra invasión de tejidos blandos e infiltración ósea. Es muy característico del SG afectar en un comienzo la pared lateral o la superior de la órbita. En algunos casos simula abscesos. El diagnóstico se confirma por histopatología.

Introduction: Granulocytic sarcoma (GS) is a rare malignant neoplasia, with an incidence rate of 2.9% to 3.1% in patients with myeloid leukemia or myeloproliferative diseases. Usually it affects males, of African, Asian and South American populations. Objetive: To describe the radiological characteristics of GS in the face and skull of children and young people. Patients and methods: We report five patients with GS in the face and skull. Computerized tomography (CT) was carried out in four and orbital and brain magnetic resonance (MRI) in three. Out of the five, four had soft tissue masses in the orbit, two had bone infiltration, and in one there was a central nervous system lesion. In four patients acute myeloid leukemia was demonstrated. Conclusion: GS may affect the orbit, the face and the skull of children and young adults; it manifests with exophthalmia and orbital edema. It must be considered as a diagnostic possibility in the context of myeloid leukemia and myelodysplasias. Otherwise, differential diagnosis should be made with complications of sinusitis, orbital rhabdomyosarcoma, orbital lymphoma and other tumors. Imaging studies may help in the diagnosis by revealing soft tissue masses and areas of bone infiltration. In some cases GS may resemble abscesses. Diagnosis must be confirmed by histopathology.

A case of donor-derived granulocytic sarcoma after allogeneic hematopoietic stem cell transplantation

The occurrence of granulocytic sarcoma as a pattern of relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is rare. In this paper, we report a rare case of acute myeloid leukemia (AML) relapsed as a granulocytic sarcoma of the donor type. The patient was diagnosed as having AML and underwent an allo-HSCT from his matched sibling donor. Fifty-seven months after allo-HSCT, he developed granulocytic sarcomas of duodenum, jejunum, and left sterno-cleido-mastoid muscle. The bone marrow was normal with 100% donor chimerism. A Y chromosome PCR was performed on the patient's duodenum specimen as well as bone marrow aspirate in order to check the patient-origin cells. The duodenal specimen was found to contain 41.2% SRY-positive cells (from the donor). Repeat endoscopy on day 2 of chemotherapy showed that the granulocytic sarcoma had shrunk dramatically. The patient died of sepsis during the nadir state 35 days after starting salvage chemotherapy.

Sino-orbital Granulocytic Sarcoma Causing Bilateral Proptosis As an Initial Manifestation of Acute Myelogenous Leukemia (AML): A Case Report

Granulocytic sarcoma is a manifestation of myelogenous leukemia, which means a solid mass consisting of primitive precursors of the granulocytic series of white blood cells. We present CT and MR imaging findings of bilateral sino-orbital granulocytic sarcoma in a 22-month-old boy. The mass involved bilateral orbital fossa which resulted in bilateral proptosis. Moreover, the mass extended to the almost skull base including paranasal sinuses, maxilla, temporal bone, zygomatic bone, sphenoid bone, ethmoid, and palatine bone. The adjacent dura was continuously thickened and the lower half of cavernous sinus was also involved. The patient was diagnosed as AML (M5) with t(8,21) translocation through a chromosome study from the bone marrow.

Sino-orbital Granulocytic Sarcoma Causing Bilateral Proptosis As an Initial Manifestation of Acute Myelogenous Leukemia (AML): A Case Report

Granulocytic sarcoma is a manifestation of myelogenous leukemia, which means a solid mass consisting of primitive precursors of the granulocytic series of white blood cells. We present CT and MR imaging findings of bilateral sino-orbital granulocytic sarcoma in a 22-month-old boy. The mass involved bilateral orbital fossa which resulted in bilateral proptosis. Moreover, the mass extended to the almost skull base including paranasal sinuses, maxilla, temporal bone, zygomatic bone, sphenoid bone, ethmoid, and palatine bone. The adjacent dura was continuously thickened and the lower half of cavernous sinus was also involved. The patient was diagnosed as AML (M5) with t(8,21) translocation through a chromosome study from the bone marrow.

Isolated Recurrence of Intracranial Granulocytic Sarcoma Mimicking a Falx Meningioma in Acute Myeloblastic Leukemia

Intracranial granulocytic sarcomas are rare tumors, which are composed of immature granulocytic cells. Although it has been well known that these tumors are associated with acute myeloblastic leukemia (AML), they have been almost always related to bone marrow relapse. However, isolated recurrence of granulocytic sarcoma following complete remission from prior AML is extremely rare, especially in the central nervous system. A 44-year-old male presented with isolated recurrence of granulocytic sarcoma mimicking a falx meningioma two years after complete remission by allogenic peripheral blood stem cell transfusion (PBSCT) in the acute myelomonoblastic leukemia (FAB, M4). Because of depressed mental state and mass effect, total surgical resection was performed. Pathological findings were compatible with the granulocytic sarcoma. There was no evidence of leukemic relapse in the peripheral blood. We suggest that this phenomenon can be explained by the hypothesis that a certain barrier effect such as blood brain barrier might lead to the proliferation of intracranial leukemic cells which metastasized before PBSCT.