Extramedullary myeloid tumour of the stomach and duodenum presenting without acute myeloblastic leukemia: A diagnostic and therapeutic challenge (original) (raw)
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Diagnostic cytopathology, 2003
Trilineage extramedullary myeloid tumor (EMT) is an uncommon medical condition mostly diagnosed in patients affected by acute or chronic myeloid leukemia or, more rarely, by a myelodysplastic syndrome, among which the most frequent is refractory anemia with excess of blasts in transformation (RAEB-t). The prognostic significance of EMT is still unclear, although the appearance of trilineage EMT is often considered to affect the outcome adversely. A 70-year-old lady with previous history of intestinal resection for colonic adenocarcinoma in 1995 and subsequently treated with 5-fuorouracyl developed a refractory anemia with excess of blasts (RAEB) in 1998. During the follow-up, a progression to RAEB-t was recorded. During chemotherapy for this condition, slight enlargement of left supraclavicular and right submandibular nodes was noticed. Fine-needle biopsy was performed with ancillary studies. A diagnosis of trilineage extramedullary myeloid tumor was reached. The patient was treated...
Leukemia & Lymphoma, 2023
Chronic myelomonocytic leukemia (CMML) is an aggressive hematological malignancy with overlapping myeloproliferative and myelodysplastic features and an inherent risk for leukemic transformation [1]. Nonhepatosplenic extramedullary manifestations (EMM) in CMML have previously been described in small case series and include both, extramedullary hematopoiesis and extramedullary monocytic infiltration [2-4]. While the occurrence of nonhepatosplenic EMM in other myeloid neoplasms such as acute myeloid leukemia and chronic myelogenous leukemia have been associated with adverse outcomes and shorter survivals, in CMML the exact prevalence and prognostic impact remain to be documented [5,6]. We carried out this study in a large informative cohort of World Health Organization (WHO) defined CMML patients to (i) describe clinical and laboratory correlates of nonhepatosplenic EMM in CMML, (ii) to assess the prognostic impact of nonhepatosplenic EMM, and (iii) to document survival outcomes. Four hundred and fifty-two patients with 2016 WHO-defined CMML were identified from the institutional database [7]. All patients had bone marrow (BM) biopsies and cytogenetic studies performed at diagnosis. Nonhepatosplenic EMM for the purpose of this study were defined as: palpable lymphadenopathy, clinical/ radiological or biopsy-proven leukemia cutis, gingival infiltrates, testicular involvement, central nervous system (CNS) involvement and myeloid sarcomas. The presence of palpable hepatosplenomegaly was also recorded. EMM were included if present either at the time of CMML diagnosis or within six months of stable disease following diagnosis. A 29 gene panel next-generation sequencing (NGS) assay was carried out on BM DNA specimens on patients obtained at diagnosis for the following genes: TET2,
Isolated Myeloid Sarcoma of the Gastrointestinal Tract
Internal Medicine, 2010
Here, we report the case of a 28-year-old woman with a primary isolated myeloid sarcoma which originated in the gastrointestinal (GI) tract. Two months after intial presentation, bone marrow tests led to a diagnosis of AML. This case is noteworthy because GI tract infiltration with leukemic cells is very rare, and it is even more rare as an occurrence preceding the development of systemic leukemia.
Extramedullary Manifestations of Myeloid Neoplasms
American journal of clinical pathology, 2015
This session of the 2013 Society of Hematopathology/European Association for Haematopathology workshop focused on extramedullary manifestations of myeloid neoplasms. We divided the submitted cases into four subgroups: (1) isolated myeloid sarcoma (MS); (2) MS with concurrent acute myeloid leukemia (AML), with a focus on karyotypic and molecular findings; (3) extramedullary relapse of AML, including relapse in the posttransplant setting; and (4) blast phase/transformation of a myeloproliferative neoplasm or chronic myelomonocytic leukemia. Establishing a diagnosis of isolated MS requires a high index of suspicion and use of immunophenotypic methods. Recurrent cytogenetic abnormalities or gene mutations that occur in MS mirror those known to occur in AML. In the era of targeted therapy and sophisticated risk stratification, every attempt must be made to perform a complete workup on MS cases (or concurrent AML) since the diagnosis of MS, in itself, is no longer adequate for patient man...
Isolated Gastric Myeloid Sarcoma: A Case Report and Review of the Literature
Case Reports in Hematology, 2014
Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach-an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed.
A case report of myeloid sarcoma of the gastrointestinal system
Journal of Hematopathology, 2012
Myeloid sarcoma is a tumor mass of myeloblasts or immature myeloid cells occurring in an extramedullary site. The clinical presentation of myeloid sarcoma varies, and many organs or tissues can be involved. Myeloid sarcoma may be found in patients with acute myeloid leukemia (AML), myelodysplastic syndromes (MDS), myeloproliferative neoplasm (MPN), or without any history. Morphologically, these cells may appear like myeloblasts, promyelocytes, or more mature granulocytes. Cytochemical and immunohistochemical stains are extremely important to make the right diagnosis. We report here a case of myeloid sarcoma in an unusual site in an 80-year-old male, without history of AML, MDS, or MPN. The patient presented with perigastric mass and numerous tumor nodules in the liver, mesentery, and omentum. His bone marrow was reactive, without evidence of AML, MDS, or MPN. The tumor masses were proven to be myeloid sarcoma by immunohistochemical stains (positive for CD34, CD68, and lysozyme) and cytogenetic studies.