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.

Cytogenetic Profiles of 472 Indian Children with Acute Myeloid Leukemia

Objective: To analyze the cytogenetic abnormalities of a largecohort of consecutive pediatric Acute Myeloid Leukemia (AML)patients, treated on a uniform protocol.Design: Review of case records.Setting: Pediatric Cancer Center of tertiary care hospital betweenJune 2003 and June 2016.Participants: 617 consecutive de novo pediatric AML patientswere screened and 472 patients were found eligible. Eligibilitycriteria included non M3 patients, successful cytogenetic profileand availability of complete recordsMain outcome measure: Cytogenetic profile.Results: Gum-hypertropy, chloromas and rate of completeremission were significantly different between EuropeanLeukemia Network classification (ELN) cytogenetic risk groups(P<0.01). t (8;21) (141, 29.8%), loss of Y chromosome (61,12.9%)and trisomy 8 (39, 8.3%) were the most common abnormalities.Among the chromosomal gains, trisomy 8 and trisomy 21 (bothP<0.01) were significantly different among the three ELN riskgroups. Among the chromosome losses, monosomy 5, 7 (bothP<0.01) and 9 (P=0.03), loss of X and loss of Y (both P<0.01)were statistically different amongst three cytogenetic risk groups.Event-free survival (P<0.01) and overall survival (P<0.01) werefound to be significantly different among the three risk groups.Conclusions: The higher frequency of t (8; 21) and its associationwith chloroma in Indian pediatric patients is different from otherstudies around the world.

A Case of Chronic Myeloid Leukemia with Multiple Chloromas Treated Successfully with Dasatinib / 계명의대학술지

Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of the primitive hematopoietic stem cells. CML is characterized by the overproduction of myeloid cells, which results in marked splenomegaly and leukocytosis. CML presented by multiple chloromas is extremely rare. Multiple chloromas in the skin and brain are quite rare as the initial presentation of CML. These rare manifestation should alert clinicians to include CML in the differential diagnosis of patients presenting with multiple non-pruritic skin nodules or neurologic symptoms. Dasatinib has promising therapeutic potential for managing intracranial leukemic disease. Here, we report the case of a patient who visited the hospital with multiple chloroma which is unusual presentation of CML, and treated with dasatinib successfully.

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.

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.

Sarcoma granulocítico multicêntrico como recidiva de leucemia mieloide aguda / Multicentric granulocytic sarcoma as relapse of acute myelogenous leukemia

Sarcoma granulocítico (SG) é um tumor sólido extramedular, constituído por células precursoras de granulócitos. É geralmente associado a leucemia mieloide aguda ou raramente a outras desordens mieloproliferativas. O tumor geralmente ocorre precedendo uma leucemia mieloide aguda, durante o seu curso ou após a remissão ter sido alcançada. O prognóstico é pobre e tem como principais modalidades terapêuticas a quimioterapia e a radioterapia. Relata- se um caso de SG multicêntrico, de evolução rápida, com acometimento difuso de pele, mamas, gânglios linfáticos, tecido celular subcutâneo e líquor, em mulher de 45 anos, fora de tratamento para leucemia mieloide aguda e em remissão hematológica há 18 meses. A paciente apresentava dor intensa em membro inferior direito há uma semana e estava em anticoagulação oral há seis meses por trombose venosa profunda neste membro. Diagnosticado o SG, a paciente foi tratada com radioterapia e quimioterapia com boa resposta. Após três meses de seguimento, em vigência do tratamento quimioterápico, evoluiu com recidiva do SG neste membro, associado ao acometimento das mamas e posteriormente do sistema nervoso central, evoluindo para óbito em aplasia e sepses.

Granulocytic sarcoma is an extramedullary solid tumor consisting of immature granulocytic cells. It is often associated with acute myelogenous leukemia and more rarely with other myeloproliferative disorders. The tumor generally occurs before acute myeloid leukemia, during its course or after disease remission. It has a poor prognosis with the main therapeutic options being chemotherapy and radiotherapy. A multicentric accelerated case of granulocytic sarcoma of a 45- year- old woman with diffuse skin, breast, lymphatic ganglia and subcutaneous tissue presentations no longer undergoing treatment for acute myeloid leukemia and in hematologic remission for 18 months is reported. The patient presented with severe pain of right lower limb for a week and was undergoing oral anticoagulation for 6 months due to deep venous thrombosis. After diagnosis of granulocytic sarcoma she received radiotherapy and chemotherapy with good response. After a 3- month follow- up, under chemotherapy, she presented relapse of granulocytic sarcoma in the limb followed by breast and central nervous system presentations leading to death in aplasia and sepsis.

Granulocytic Sarcoma in the Head and Neck: CT and MR Imaging Findings

OBJECTIVES: To evaluate characteristic computed tomography (CT) and magnetic resonance (MR) imaging findings of granulocytic sarcomas of the head and neck. METHODS: The CT (n=11) and MR (n=1) images obtained from 11 patients (7 males and 4 females; mean age, 23.5 yr; age range, 1 to 69 yr) with histologically-proven granulocytic sarcomas of the head and neck were retrospectively reviewed. Histological confirmation was done by bone marrow biopsy in 9 patients, and/or local biopsy in 4 patients. The imaging findings were analyzed with particular attention to location, size, shape, margin, bone destruction, internal architecture, pattern and degree of enhancement, and multiplicity of the lesions. RESULTS: The masses were most commonly located in the orbital cavity (n=8); other locations included lymph nodes (n=5) and palatine/pharyngeal/lingual tonsils (n=3). The mass sizes varied from a mean diameter of 1.3 to 5.8 cm (average, 2.6 cm). Multiple lesions were found in 6 patients. The shapes of the tumors were ovoid in 12 patients and irregular in 4 patients. Most lesions had poorly-defined margins (13/16) and invaded adjacent bony structures (5/16). On the pre-contrast CT images, the masses were iso- (5/8) or low-density (3/8) in comparison with muscle. The MRI, which was obtained in one patient in this study, showed that the mass was iso-signal intensity on T1-weighted images and iso-signal intensity on T2-weighted images compared to the gray matter of the brain. On the post-contrast CT images, there was homogenesous (n=12) or heterogeneous (n=4) enhancement, with mild (n=10), moderate (n=4), and marked (n=2) enhancement in the solid portions of the lesions. CONCLUSION: Although rare, granulocytic sarcomas arise in various locations in the head and neck area (most commonly in the orbit) in the form of well-demarcated, and mildly- and homogenously-enhancing masses with adjacent bony invasion.

Four cases of chloroma treated with hematopoietic stem cell transplantation / 대한내과학회지

Chloroma is an invasive extramedullary tumor composed of immature myeloid cells, which complicates the clinical course in a minority of patients with acute myeloid leukemia (AML). The presence of myeloid sarcoma is known to be a poor prognostic indicator in patients with AML. However, the optimal treatment of AML with concurrent chloroma has not been determined. We report four patients with AML accompanied by concurrent chloroma from the time of initial diagnosis. All of the patients underwent hematopoietic stem cell transplantation after complete remission. We also present a review of the literature.

Granulocytic Sarcoma Presenting as Epidural Mass in Lumbosacral Spine: A Case Report

Granulocytic sarcoma, as known as chloroma, is a localized solid tumor consisting of immature myeloid cells. It is a infrequent extramedullary manifestation of acute or chronic leukemias and can often precede their bone marrow involvement. Spinal involvement of granulocytic sarcoma is very rare. A 17-year-old man presented with low back pain, gluteal pain and perianal numbness for 1 month. Lumbar spine magnetic resonance imaging (MRI) was taken under impression of herniated lumbar intervertebral disc and showed an epidural mass at the lumbosacral spinal canal. Leukocytosis, thrombocytopenia and anemia were found on blood cell count. Excisional biopsy of mass was done and the histopathologic examination confirmed the diagnosis of granulocytic sarcoma. Subsequent bone marrow biopsy revealed myelodysplastic syndrome. He had received anti-leukemic chemotherapy and the size of lumbosacral mass was markedly decreased. As a granulocytic sarcoma of spinal column is uncommon, high index of suspicion is inevitable to diagnose it. Therefore it must be included as a differential diagnosis of spinal epidural mass.

Intracranial Chloroma(Granulocytic Sarcoma) by Lymphocytic Leukemia

Intracranial chloroma may occur in leukemia, although they are rare. A 23-year-old female complained diplopia. Brain magnetic resonance MR imaging showed tumors in the both cavernous sinus , both tentorial and anterior falx. Gamma-Knife radiosurgery was performed with maximal dose; 20Gy, marginal dose; 10Gy. Peripheral blood smear revealed leukemia, and bone marrow aspiration biopsy showed acute lymphocytic leukemia. Two weeks later, MR image for the stereotactic biopsy noticed markedly decreased tumor size. Biopsy result was lymphocytic leukemia. She received conventional radiation therapy, chemotherapy, and bone marrow transplantation. Brain involvement by acute lymphocytic leukemia is very rare. Even though chloroma are sensitive to radiation therapy, prognosis is poor because of the gravity of the underlying disease and association with impending blast transformation. The authors reports a intracranial chloroma by acute lymphocytic leukemia.

A Case of Gastric Extramedullary Tumor after Lymphoblastic Crisis in a Patient with Chronic Myelogenous Leukemia / 대한소화기내시경학회지

A localized extramedullary tumor mass composed of immature cells has been reported in association with acute myeloid leukemia, myeloproliferative disorders, myelodysplasia in blast transformation or chronic myeloid leukemia with trilineage hematopoiesis, as well as in patients with no known hematological disorder. Although this tumor may involve anywhere in the body and give rise to a variety of signs and symptoms, there are several case reports of extramedullary tumor in Korea which described the involvement of the gastrointestinal tract. We report the occurrence of gastric extramedullary tumor and lymphoid blast crisis in a patient with complete remission after lymphoblastic transformation of chronic myelogenous leukemia. Endoscopic biopsy for gastric elevated lesion showed diffuse lymphoblast infiltration with TdT (terminal deoxynucleotidal transferase) and CD20 positive immature cells.

A Case of Bronchiolitis Obliterans Organizing Pneumonia Developed after Treatment of Acute Myelogenous Leukemia (M2) with Chloroma and Retinopathy / 대한소아혈액종양학회지

Chloroma, a tumor consisted of primitive myeloid cells and located in extramedullary tissues, usually develops during or preceding the course of systemic leukemia, particularly acute myelogenous leukemia (FAB M2) with translocation between chromosomes 8 and 21. Leukemic retinopathy which observed rarely in children than adults, generally related to thrombocytopenia and anemia in leukemic patients. Bronchiolitis obliterans organizing pneumonia (BOOP) is unusual clinicopathologic syndrome in association with a variety of causes or conditions, and histologically defined by the presentation of granulation tissue plugs consisting of fibroblasts and collagen within the lumen of the distal air space. We experienced a 12-year old girl who presented with weakness and ocular pain. She was diagnosed with acute myeloid leukemia with chloroma and retinopathy, and treated with chemotherapy (daunomycin, ara-C, thioguanine, etoposide. mitoxantrone, cyclosporin). Five weeks after the chemotherapy, she developed coughing and persistent fever, and diagnosed with BOOP, which resolved completely after prednisolone therapy.

Meningeal Chloromain in Acute Lymphoblastic Leukemia

Isolated chloromas(granulocytic sarcomas) are rare tumors. Chloromas are masses composed of immature granulocytic cells. Granulocytic sarcoma occurs primarily in patients with acute myelogenous leukemia as well as in patients with other myeloproliferative disorders, but rarely in patients with acute lymphoblastic leukemia(ALL). We now describe one patient affected by ALL with isolated granulocytic sarcoma as initial CNS relapses. These unusual clinical manifestation and radiological finding in acute lymphoblastic leukemia should be considered as recurrence of leukemia. Early detection and antileukemic treatment of granulocytic sarcoma are necessarily important for favorable prognosis.

Cutaneous Granulocytic Sarcoma: A report of two cases / 대한피부과학회지

Granulocytic sarcoma (GS), also known as chloroma, is an extramedullary tumor of granulocytic lineage. It usually occurs in patients with acute myelogenous leukemia (AML), myeloproliferative disorder, or myelodysplasia but can develop in patients with no known hematologic disorder (isolated chloroma). The sites of predilection are bone and periosteum, and the next most common sites are soft tissues of the skin, kidneys, gut and lymph nodes. We report herein two cases of GS in a 36 year-old male and a 39 year-old female. The former had been treated for AML for 4 months before GS developed as an exophytic and multiple cutaneous masses on his left arm. In the latter, whose chief complaint was plum-colored nodules on her trunk, the initial diagnosis was malignant lymphoma. A follow-up after chemotherapy revealed that she had AML and reversely the final diagnosis of GS could be made for her skin tumor.

A Case of Cardiac Chloroma Complicated by Acute Lymphocytic Leukemia

Chloroma (granulocytic sarcoma) indicates an extramedullary leukemic cell collection. It often develops in the course of, or as a presenting sign of leukemia. Cardiac chloroma is uncommon and rarely detected as a mass. We report the first case of cardiac chloroma in a patient with acute lymphocytic leukemia in Korea. A 73-year-old man was admitted because of exertional dyspnea, orthopnea and generalized weakness. Thrombocytopenia and immature leukocytes were detected in the peripheral blood. An X-ray film of the chest showed mild cardiome-galy and bilateral pleural effusion. Transthoracic and transesophageal echocardiography showed a low echogenic mass at the lateral wall of the right ventricle. The size of the mass was about 6x4 cm. MRI of the chest showed right ventricular mass with slightly increased inhomogeneous signal intensity. Bone marrow aspiration and biopsy confirmed that he had a L3 FAB subtype of acute lymphocytic leukemia. Induction chemotherapy with vincristine, prednisolone, daunorubicin resulted in hematologic complete remission. At 6 weeks after the induction chemotherapy, transesophageal echocardiography demonstrated disappearance of the right ventricular mass which suggested that it was a cardiac chloroma complicating acute lymphocytic leukemia.

Intracranial granulocytic sarcoma (chloroma) in a nonleukemic patient

Chloroma is a granulocytic sarcoma with it's characteristic greenish color. Recently there is an increased number of cases that are apparently aleukemic when the tumor mass is first presented. Recently we experienced a case of granulocytic sarcoma with characteristic green color (chloroma), which showed no evidence of leukemia in the bone marrow and peripheral blood. This patient presented headache, and was diagnosed brain tumor on computed tomography. A left parietal cranietomy was done to remove a large central dome-like mass, 8 cm, involving the dura with a slightly dusky greenish solid appearance. Compact nests of moderately mature granulocytes and immature cells comprised the tumor. Histochemical and electron microscopic studies confirmed these tumor cells as myeloid cells in varying stages of maturation. Several days after the operation, left cervical lymph nodes became palpated, and the biopsied lymph nodes revealed same neoplastic cells seen in the skull. However, bone marrow aspiration, biopsy and peripheral blood smears did not show any evidence of leukemia.