Granulocytic sarcoma of the small intestine with CBFβ/MYH11 fusion gene: report of an aleukaemic case and review of the literature

Xavier, Sandra Guerra; Fagundes, Evandro M.; Hassan, Rocı́o; Bacchi, Carlos E.; Conchon, Mônika; Tabak, Daniel; Spector, Nelson; Zalcberg, Ilana

doi:10.1016/s0145-2126(03)00070-5

Cited by 36 publications

(34 citation statements)

References 22 publications

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“…Several investigators have reported an association between inv16 and the development of GS in the abdominal cavity, especially in the small intestine. (19,20) However, only one patient in the present study developed GS in the pancreas and no patient developed GS in the small intestine.…”

Section: Discussioncontrasting

confidence: 58%

“…(18,19) To fill the research need for broad examination of a larger AML patient population, the present study conducted a large-scale analysis of adult patients with AML to explore the clinical significance of GS among this general population.…”

Section: Discussionmentioning

confidence: 99%

“…Several studies have reported that specific factors, including French-American-British (FAB) M4 and M5 classification (11,12) and the expression of CD56 (13,14) or T-cell antigens (CD2, CD4 and CD7) (15,16) on leukemia cells, are associated with GS, while others have identified an association between GS and t(8;21) (17,18) or inv (16). (19,20) Establishment of the clinical characteristics of GS has been further impeded by the mixed results obtained by several studies that investigated the prognosis of GS in limited subpopulations of AML patients. Byrd et al (21) found that GS was associated with unfavorable outcomes and a shorter survival time (median 5.4 months with GS vs 59.5 months without GS) among AML patients carrying t(8;21), whereas Felice et al (22) argued that GS had no adverse prognostic impact on AML patients carrying t (8;21).…”

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Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia

et al. 2012

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To investigate the clinical significance of granulocytic sarcoma (GS) in adults with acute myeloid leukemia (AML), 434 consecutive patients with AML were analyzed retrospectively. Forty-five patients (10.4%) with GS at diagnosis were younger (P < 0.001), presented with higher white blood cell counts (P = 0.03) and were more likely to conform to French-American-British M4 (P = 0.001) and M5 (P = 0.045) classifications than those without GS. In contrast, no significant difference in frequency of cytogenetic abnormalities was found between the GS and non-GS groups. Treatment outcomes in 260 patients (40 with GS) who underwent intensive chemotherapy, excluding patients with acute promyelocytic leukemia, were investigated. Complete remission rates did not differ significantly between the GS and non-GS groups (75.0% vs 79.1%; P = 0.192, respectively) or the 5-year overall survival (OS) rates (39.9% vs 38.7%; P = 0.749, respectively). However, the GS group had a significantly higher relapse rate than the non-GS group (74.2% vs 55.3%; P = 0.048) and a significantly lower 5-year disease-free survival rate (8.2% vs 25.7%, respectively; P = 0.005). When considered together with the results of multivariate analysis, which identified the presence of GS as an independent predictor for disease-free survival time, these findings indicate that GS might identify a high-risk subset of patients with AML. (Cancer Sci 2012; 103: 1513-1517 G ranulocytic sarcoma (GS), a rare extramedullary tumor composed of malignant granulocytic precursor cells that affects 3-9% of patients diagnosed with acute myeloid leukemia (AML), (1)(2)(3) occurs concomitantly with or after a diagnosis of AML. Granulocytic sarcoma can occur in any organ and common sites are bones, lymph nodes, soft tissues and skin. (4) Other organs, including the brain, (5,6) orbit (7,8) and genitourinary system, (9,10) are infrequently affected by GS. Such variation in its site of occurrence complicates accurate diagnosis of GS, and, moreover, the low incidence of GS has impeded establishment of the clinical characteristics of patients with GS. Several studies have reported that specific factors, including French-American-British (FAB) M4 and M5 classification (11,12) and the expression of CD56 (13,14) or T-cell antigens (CD2, CD4 and CD7) (15,16) on leukemia cells, are associated with GS, while others have identified an association between GS and t(8;21) (17,18) or inv(16). (19,20) Establishment of the clinical characteristics of GS has been further impeded by the mixed results obtained by several studies that investigated the prognosis of GS in limited subpopulations of AML patients. Byrd et al. (21) found that GS was associated with unfavorable outcomes and a shorter survival time (median 5.4 months with GS vs 59.5 months without GS) among AML patients carrying t(8;21), whereas Felice et al. (22) argued that GS had no adverse prognostic impact on AML patients carrying t(8;21). In light of these discrepant findings, this study aimed to clarify the clinical significance of GS...

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Section: Discussioncontrasting

confidence: 58%

Section: Discussionmentioning

confidence: 99%

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Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia

et al. 2012

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“…The literature has reported varied cytogenetic abnormities in myeloid sarcomas (12), such as the inversion of chromosome 16 and its associated molecular genetic change resulting in the core binding factor β/myosin, heavy chain 11, smooth muscle (CBFβ/MYH11) fusion gene (13). In the present case, the patient exhibited a normal karyotype of 46,XX and was negative for the CBFβ/MYH11 fusion gene; however, EVI1 gene overexpression was recorded.…”

Section: Discussionmentioning

confidence: 52%

Myeloid sarcoma derived from the gastrointestinal tract: A case report and review of the literature

et al. 2016

Oncology Letters

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Abstract. Myeloid sarcoma is a type of malignant neoplasm composed of myeloblasts that locates extramedullary. The present study reports the case of a 31-year-old female who presented with upper abdominal pain, melena, vomiting and jaundice. The abdominal computed tomography revealed a mass in gastric antrum area and possible infiltration of the duodenum, gallbladder and head of the pancreas, with possible retroperitoneal lymph node metastasis. The tumor grew quickly and led to serious obstructive jaundice. New masses developed in the bilateral orbits and left breast within 2 months of admission. The pathological results of the gastroscopic biopsy and the fine-needle biopsy of the breast revealed myeloid sarcoma. Transhepatic cholangial drainage, radiotherapy and chemotherapy were administered, but the disease reoccurred and became resistant to chemotherapy, so salvage allogenetic peripheral blood stem cell transplantation was performed. The disease relapsed at 5 months post-transplantation, and chemotherapy and donor lymphocytes transfusions were then administered. The patient declined further treatment and succumbed to disease on May 19, 2015. The present study could improve the understanding of myeloid sarcoma and provide a reference for standardized and individualized treatments for this disease.

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“…103 Mechanistically, neural cell adhesion molecule is also highly expressed in breast, testicular, ovarian, and gut tissue, which could account for EM homing to these sites. 85,104 In the case of inv (16), it has been speculated that the deregulation of CBF transcription factors involved in cellular adhesion and recognition may play a role in the pathogenesis of MS 45 ; however, mechanistic studies are needed to support this.…”

Section: What Is Our Current Understanding Of the Pathogenesis Of Em?mentioning

confidence: 99%

How I treat extramedullary acute myeloid leukemia

Bakst¹,

Tallman

Douer

et al. 2011

Blood

415

586

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IntroductionAcute leukemia may present in a variety of extramedullary (EM) tissues with or without bone marrow disease. EM involvement by acute leukemia is a relatively rare, but clinically significant, phenomenon that often poses therapeutic dilemmas. Myeloid sarcoma (MS) and leukemia cutis (LC) represent 2 well-known EM manifestations. MS (also known as granulocytic sarcoma or chloroma) is a rare EM tumor of immature myeloid cells. It was first described in 1811 1 and later named "chloroma" by King 2 in 1853 because of its green color caused by the presence of myeloperoxidase (MPO). 3 Five decades later, the relationship of MS to acute leukemia was identified. 4 The term "granulocytic sarcoma" was introduced later by Rappaport to describe only tumors of granulocytic origin 5 ; however, the term is now often applied to any tumor related to acute leukemia or myelodysplastic syndrome (MDS).LC is the infiltration of the epidermis, dermis, or subcutis by neoplastic leukocytes (leukemia cells) resulting in clinically identifiable cutaneous lesions. LC commonly results in subcutaneous nodules and can be confusingly referred to as cutaneous granulocytic sarcoma. 6,7 For the purposes of this article, LC will refer to cutaneous involvement only. LC has been described mostly in acute myeloid leukemia (AML), but also in the accelerated phase of chronic myeloid leukemia, MDS, and rarely in acute lymphocytic leukemias. 7 In this article, we describe an approach and therapeutic strategies for patients with EM manifestations of leukemia by addressing a series of questions commonly raised by the practicing clinician. How common are MS and LC, respectively?MS is reported in 2.5%-9.1% 8-10 of patients with AML and occurs concomitantly, following, or, rarely, antedating the onset of systemic bone marrow leukemia. 11 Isolated MS, defined by the absence of a history of leukemia, MDS, or myeloproliferative neoplasm and a negative bone morrow biopsy, has been described in limited case reports. 12 Many of these patients are often misdiagnosed with lymphoma. 13,14 Certain known AML cytogenetic abnormalities, in particular t(8,21), have been associated with a higher incidence. 15 MS can also develop at relapse with or without marrow involvement. The incidence of patients developing MS after allogeneic hematopoietic cell transplantation (HCT) has been reported to be 0.2%-1.3% with poor overall survival. 16,17 LC occurs in ϳ 3% of patients with AML 18 and less frequently in chronic leukemias. 19 The reported incidence may be overestimated if biopsy is not performed because skin lesions similar to LC have a wide range of inflammatory, neoplastic, and infectious etiologies. 19 Certain subtypes of AML are more commonly associated with skin infiltration. The most frequent association occurs with acute myelomonocytic and monocytic differentiation with involvement in up to 50% of patients. 18,20,21 The incidence of LC may be higher in children, particularly infants with myeloid leukemia. 22 How do MS and LC most often present clinically?MS most...

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Granulocytic sarcoma of the small intestine with CBFβ/MYH11 fusion gene: report of an aleukaemic case and review of the literature

Cited by 36 publications

References 22 publications

Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia

Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia

Myeloid sarcoma derived from the gastrointestinal tract: A case report and review of the literature

How I treat extramedullary acute myeloid leukemia

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