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PubMed1978

Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in Turkish children.

In a series of 166 leukemic children from Turkey, 56 had acute myelomonocytic leukemia (AMML). Seventeen boys and 3 girls presented with chloroma-like deposits (granulocytic or myeloid sarcomas) in the eye and orbit, all showing AMML on initial study of blood and marrow. The ocular lesions responded rapidly to antileukemic therapy. Laboratory studies of AMML cases revealed no cytogenetic or immune defects, and Epstein-Barr virus titers were normal. A group-specific (GS-3) antigen (type-C virus?) was identified in one patient by radioimmunoassay of orbital tumor extracts. It is not clear what factors contribute toward the myelomoncytic differentiation of leukemia and its localization in the eye and orbit, but opportunities for further study are enhanced by reports of a predisposition to ocular chloroma among leukemic children in Africa, Egypt, and Japan.

Adolescent↗
PubMed1995

Granulocytic sarcoma in patients with chronic myeloid leukaemia.

Granulocytic sarcoma is an unusual variant of myeloid malignancy most commonly encountered in the course of chronic or acute myeloid leukaemia. Of 60 patients of chronic myeloid leukaemia studied over 21 months, we encountered 6 (10%) cases of granulocytic sarcoma. Four of these had granulocytic sarcoma on their first presentation. All those who were receiving hydroxyurea did not fare well but one who was put on DAT (daunorubicin, cyftosine arabinocide, 6 thioguanine)regimen went into remission with complete disappearance of lesions.

Adolescent↗
PubMed2005

Myeloid leukemias and lung involvement.

Myeloid leukemias are clonal malignancies characterized by the presence of increased numbers of immature myeloid cells in the marrow and peripheral blood. Pulmonary involvement by myeloid leukemia is relatively uncommon and seen mainly in patients with severe disease. The most common form of pulmonary involvement consists of leukemic infiltration along the lymphatics in the peribronchovascular, septal, and pleural interstitial tissue. Less common manifestations include myeloid sarcoma, leukostasis, leukemic cell lysis pneumopathy, and hyperleukocytic reaction. The radiological manifestations of pulmonary leukemic cell infiltration and leukostasis consist mainly of bilateral thickening of the peribronchovascular interstitium and interlobular septa, a pattern that resembles that of interstitial pulmonary edema. The radiological manifestations of leukemic cell lysis pneumopathy and hyperleukocytic reaction consist of symmetric bilateral areas of consolidation. This manuscript reviews the histological and radiological intrathoracic manifestations of myelogenous leukemias.

Diagnosis, Differential↗
PubMed2006

Advances in the pathologic diagnosis and biology of acute myeloid leukemia.

In a general surgical pathology practice, cases of acute myeloid leukemia (AML), including myeloid sarcoma, are relatively rare; the diagnosis is very often difficult, however, and consequences of a missed or improper diagnosis compromise patient care. Currently, accurate diagnosis of every case of AML requires integration of the morphological features and results of cytochemical and immunohistochemical stains, flow cytometric immunophenotyping, cytogenetics, and molecular studies. This review focuses on a practical approach to diagnosis of AML according to current standard of practice and discusses some of recent changes in the field of AML.

Acute Disease↗
PubMed2001

[Ocular infiltration in the anterior chamber in a female infant with acute non-lymphoblastic leukemia].

Unlike myeloid sarcoma, ocular involvement is unusual in acute non-lymphoblastic leukemia.A 9-month-old female infant with acute non-lymphoblastic leukemia M5 and evidence of active central nervous system (CNS) disease showed infiltration of the anterior chamber during therapy. At that time, the CNS disease was in completed remission. She was treated with topical corticosteroids, chemotherapy and bilateral ocular radiotherapy (total dose 1,000 cGy). The ocular manifestations responded well to treatment but hematologic response was poor. The patient died a few months later. Any ophthalmic manifestation in children with leukemia should be detected and treated early. Radiotherapy is warranted in infiltration of the anterior chamber of the eye. The presence of ocular, central CNS or bone marrow involvement indicates poor prognosis in acute childhood leukemia.

Anterior Chamber↗
PubMed2002

Bethesda proposals for classification of nonlymphoid hematopoietic neoplasms in mice.

The hematopathology subcommittee of the Mouse Models of Human Cancers Consortium recognized the need for a classification of murine hematopoietic neoplasms that would allow investigators to diagnose lesions as well-defined entities according to accepted criteria. Pathologists and investigators worked cooperatively to develop proposals for the classification of lymphoid and nonlymphoid hematopoietic neoplasms. It is proposed here that nonlymphoid hematopoietic neoplasms of mice be classified in 4 broad categories: nonlymphoid leukemias, nonlymphoid hematopoietic sarcomas, myeloid dysplasias, and myeloid proliferations (nonreactive). Criteria for diagnosis and subclassification of these lesions include peripheral blood findings, cytologic features of hematopoietic tissues, histopathology, immunophenotyping, genetic features, and clinical course. Differences between murine and human lesions are reflected in the terminology and methods used for classification. This classification will be of particular value to investigators seeking to develop, use, and communicate about mouse models of human hematopoietic neoplasms.

Animals↗
PubMed1994

Two unusual neurological presentations of granulocytic sarcoma in Philadelphia positive chronic myeloid leukaemia.

Granulocytic sarcoma (GS) is a rare extramedullary tumour consisting of immature myeloid precursors. It occurs most commonly in association with myeloid leukaemias and myeloproliferative disorders. Rarely there may be no evidence of haematological malignancy. We describe neurological presentations of GS in two patients with Philadelphia (Ph) positive chronic myeloid leukaemia (CML). In both cases the bone marrow was in chronic phase at the time of presentation of the GS, but there was rapid subsequent transformation into the blastic phase.

Adult↗
PubMed2006

Mass-forming extramedullary hematopoiesis diagnosed by fine-needle aspiration cytology.

Extramedullary hematopoiesis (EMH) is usually a microscopic finding. However, it may present as a mass-forming lesion making it amenable to fine-needle aspiration biopsy (FNAB). When mass-forming EMH occurs, it can simulate a neoplasm clinically and radiologically. Additionally, the megakaryocytes can mimic malignant neoplastic cells, particularly if EMH is not a considered diagnosis. We report six cases of mass-forming EMH diagnosed by FNAB and evaluate the utility of FNAB in diagnosing EMH. Four patients had prior diagnoses of hematologic disorders, one patient had malignant mastocytosis who presented with lymphadenopathy and one patient had a history of carcinoma. The patients' ages ranged from 46 to 78 yr with an equal sex distribution. Aspirate smears showed trilineage hematopoiesis. The cytomorphologic differential diagnosis included metastatic carcinoma, Hodgkin lymphoma and myeloid sarcoma. No special stains were necessary due to the classic cytologic findings and prior hematologic history.

Aged↗
PubMed2005

Podocalyxin: a marker of blasts in acute leukemia.

Podocalyxin is a CD34 family member expressed by podocytes, vascular endothelium, mesothelium, and a subset of hematopoietic progenitors. Podocalyxin expression was not observed in the hematopoietic cells of normal adult bone marrow samples. However, podocalyxin was expressed by blasts in 30 (77%) of 39 cases of acute myeloid leukemia (AML), 22 (81%) of 27 cases of acute lymphoblastic leukemia (ALL), and 13 (87%) of 15 cases of cutaneous myeloid sarcoma. No correlation with CD34 expression by immunohistochemical analysis was seen. Wilms tumor 1 (WT1) expression was detected in blasts in 17 AML cases (44%) and 21 ALL cases (78%). There was no correlation between WT1 and podocalyxin expression. We conclude that podocalyxin is expressed commonly by blasts in ALL and AML. Analysis of the expression of CD34 and podocalyxin increases sensitivity for the immunophenotypic detection of leukemic blasts compared with the analysis of CD34 alone. Therefore, podocalyxin seems to complement CD34 as a useful hematopoietic blast marker. The physiologic role of podocalyxin in leukemic blasts remains unknown.

Adult↗
PubMed2004

t(8;21)(q22;q22) in blast phase of chronic myelogenous leukemia.

The blast phase of chronic myelogenous leukemia (CML) frequently is associated with cytogenetic evidence of clonal evolution, defined as chromosomal aberrations in addition to the t(9;22)(q34;q11.2). We identified the t(8;21)(q22;q22) and other cytogenetic abnormalities by conventional cytogenetics and fluorescence in situ hybridization in 2 patients with t(9;22)-positive CML at the time of blast phase. The t(8;21), which typically is associated with a distinct subtype of de novo acute myeloid leukemia (AML) carrying the aml1/eto fusion gene, was accompanied by increased bone marrow myeloblasts (33%) in case 1 and extramedullary myeloid sarcoma in case 2, suggesting its possible role in disease progression. In case 1, the leukemic cells in aspirate smears had salmon-colored cytoplasmic granules, and immunophenotypic studies showed that the blasts expressed CD19. These findings suggest that the pathologic features of blast phase CML with the t(8;21) resemble those of de novo AML with the t(8;21).

Blast Crisis↗
PubMed2005

Primary malignant lymphoma of the testis.

UNLABELLED: Primary testicular lymphomas are rare entities representing 1-2% of non-Hodgkin lymphoma (NHML) and 1-7% of malignant testicular tumors and they are the most common testicular tumors in men older than 50 years of age. This study included 8 cases of inpatients diagnosed by echography and NMR with testicular tumors. The age of patients was between 46 and 81 (with a mean of 52). The tumors were unilateral, with disease limited to testicle and accompanied by pain except 1 case with bone involvement. Orchectomy was performed as first therapeutic and diagnostic purpose. All patients were clinically staged according to the Ann Arbor criteria in IE and IIID stage and received a doxorubicin based chemotherapy regimen (CHOP, MTX, CVP, and Leukeran). A standard chemotherapy protocol has not been used because of reduced number of patients. Tumor fragments were fixed in 10% formallin, paraffin embedded, sectioned and standard H.E. stained. Immunohistochemistry for L26, Alphafetoprotein, NK1, CD30, and CLA was performed. Microscopy revealed in all cases a stromal proliferation with medium size cells, monomorphic shape and prominent nucleoli. Alphafetoprotein was positive in seminal tubes and negative in tumor, NK1 in small lymphocyte and negative in tumor and L26 diffuse positive in tumor. CLA diffuse positive in tumor. We were able to follow up only four patients. CONCLUSIONS: The diagnosis was of NHML in 6 cases and for 2 secondary involvement of hematopoietic malignancy (myeloid sarcoma and leukemia). Lymphoma cases were typed using REAL classification as small and large B-cell lymphoma. Unfavorable evolution with 6 months relapse and one death prove a more aggressive evolution of primitive testicular lymphoma.

Humans↗
PubMed2005

Maxillo-ethmoidal chloroma in acute myeloid leukaemia: case report.

Chloroma, also called Granulocytic Sarcoma or Myeloid Sarcoma, is a rare malignant extra-medullary neoplasm of myeloid precursor cells. It is usually associated with myelo-proliferative disorders but its appearance may precede the onset of leukaemia. Chloroma may be found in several extracranial sites. Involvement of the head and neck region is uncommon. Differential diagnosis is often difficult and includes acute lymphoblastic leukaemia, large cell NHL, lymphoblastic lymphoma and Ewing's sarcoma. The case is presented of a maxillo-ethmoidal chloroma occurring in a case of poor prognosis acute myeloid leukaemia, emphasizing the clinical and cyto-histological features and problems concerning differential diagnosis.

Acute Disease↗
PubMed1997

Granulocytic sarcoma in the absence of myeloid leukemia.

Granulocytic sarcoma is an extramedullary tumor composed of immature granulocytic precursor cells. It usually occurs in association with leukemia or other myeloproliferative disorders but can occur without overt hematologic disease. We describe a 6-year-old boy with granulocytic sarcoma in the absence of demonstrable hematologic disease and review the literature regarding the diagnosis, clinical associations, and treatment of this condition.

Antineoplastic Combined Chemotherapy Protocols↗