Granulocytic sarcoma (GS) is an extramedullary tumor composed of immature myeloid cells, typically occurring during the course of acute myelogenous leukemia. Nonleukemic GS, that is, GS with no evidence of overt leukemia and no previous history of leukemia, is very rare, and even more unusual is nonleukemic GS of the bile duct. We report a case of nonleukemic GS of the bile duct. The patient was initially misdiagnosed as a bile duct carcinoma arising in the hilum of the liver (so-called Klatskin tumor), and received a right lobectomy of the liver. Histological examination of the tumor yielded the diagnosis of GS, and the bone marrow biopsy did not show any evidence of leukemia. Considering the risk of subsequent development of overt leukemia, the patient was treated with two cycles of combination chemotherapy as used in the cases of acute myelogenous leukemia. To date, he has remained free of disease 15 months after treatment.
Sarcoma, Granulocytic; Bile Ducts; Leukemia, Myelocytic, Acute
Paraplegia due to a spinal cord epidural mass is an extremely rare presentation of undiagnosed leukemia. We are reporting a case of 14-year-old girl, who presented with paraplegia due to thoracic epidural mass, as the initial presenting manifestation of acute myeloid leukemia. Granulocytic sarcoma or chloroma should be considered in the differential diagnosis of an epidural mass in patients with or with out leukemia granulocytic sarcoma, which are rare extramedullary tumor-like proliferation of myelogenous precursor cells that may de novo precede acute leukemia or coincide with the first manifestation or relapse of acute myeloid leukemia.
Acute myeloid leukemia; chloroma; granulocytic sarcoma
Granulocytic sarcoma is an extramedullary tumor composed of immature granulocytic cells. These tumors usually occur simultaneously with or follow after the onset of acute myeloid leukemia (AML) or other myeloproliferative disorders. Rarely, it is the first manifestation of AML which appears several months before the onset of leukemia. We report a case of a 48-year-old man presenting with symptoms of small bowel obstruction. Laparotomy and open biopsy were performed. Immunohistochemical studies showed that the neoplastic cells were of myeloid lineage positive for myeloperoxidase and leukocyte common antigen, but negative for CD3, 20, 56, 79a, and cytokeratin. Initially, there was no evidence of blood or bone marrow involvement suggesting acute leukemia or other myeloproliferative disorders. The findings were consistent with the diagnostic findings of solitary granulocytic sarcoma (preleukemic). However, one month later, bone marrow biopsy revealed 57% myeloblasts. Sequentially, the patient developed FAB M2 acute myeloid leukemia. Induction chemotherapy including cytarabine and idarubicine was done which led to complete remission. Allograft bone marrow transplantation was performed later, and there is no evidence of recurrence till present.
Granulocytic sarcoma; Acute myeloid leukemia; Small bowel obstruction
Granulocytic sarcoma is an extramedullary tumor which is composed of myeloblasts and immature myeloid cells. It usually occurs in association with acute myeloid leukemia and most commonly involves skin, soft tissue, lymph nodes, bone, and periosteum. We report a case of isolated ureteral granulocytic sarcoma without hematologic manifestations. Our patient presented with bloody urine and left-sided lumbar pain. Preoperative clinical and radiologic features raised the suspicion of an upper urinary tract transitional cell carcinoma, and he was scheduled for nephroureterectomy. However, perioperative pathologic feedback and the unusual endoscopic appearance of the tumor altered our surgical strategy towards segmental ureterectomy and ureteroneocystostomy. Eventual pathologic diagnosis was granulocytic sarcoma of the ureter. Postoperative workup failed to demonstrate any sign of an accompanying hematologic disorder. He started receiving the chemotherapy protocol of acute myeloblastic leukemia. To our knowledge, this is the first documented case of nonleukemic ureteral granulocytic sarcoma which came to attention due to urologic complaints.
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.
Aleukemic leukemia cutis; AML M1; Granulocytic sarcoma
Granulocytic sarcoma (GS) is a form of acute myeloid leukemia (AML), also known as extramedullary myeloid tumor or chloroma. It forms a solid malignant tumor consisting of myelocytes or granulocytes and is typically located in bone while occurrence in other parts of the body is rare.
We reported a 40-year-old male patient who had jaundice, highly elevated bilirubin, and a mass highly suspicious of pancreatic head carcinoma. We performed surgery and the pathology and immunohistochemistry suggested GS; however the blood test and the bone marrow infiltration showed no evidence of AML. In our review of the published reports of GS, we only found six reports of the GS in the pancreas, and we suggested that immunohistochemical staining should be used to accurately differentiate GS from other pancreatic cancer and other types of leukemia.
The accurate diagnosis of GS is necessary for determining prognosis and deciding appropriate therapy.
Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a rare entity, and mostly accompanied by acute myeloid leukemia. It is observed during the course of myeloproliferative disorders especially in chronic myeloid leukemia and myelodysplastic syndromes. In some rare circumstances, it is detected before clinical signs of leukemia or other diseases. When the bone marrow biopsy reveals no other hematologic malignancies, the granulocytic sarcoma is described as nonleukemic, primary or isolated. It is observed at any part of the body but the most common locations are soft tissues, bone, peritoneum and lymph nodes. Presenting signs or symptoms are mainly due to mass effect of the tumor and dysfunction of the organ, or the tissue that is affected. The diagnosis is performed by biopsy of the tumor. The tumor consists of immature granulocytic cells, which could be documented by H&E, immunohistochemistry, and flow cytometric methods. Fluorescence in-situ hybridization and molecular analysis are also performed. The optimal time and type of treatment is not clear. Surgery could be an option especially for tumors, which cause organ dysfunction and/or obstruction. Systemic treatment should be considered in all patients because without systemic treatment, relapses and progression to acute myeloid leukemia is the ultimate fate of the disease in many cases. Cytarabine-containing remission-induction chemotherapies have been the most applied therapeutic strategies, but it is not clear whether the consolidation therapies are required or not, and what kind of regimens are appropriate. The role of hematopoietic stem cell transplantation (HSC) as a consolidation regimen is not clear, but, after the relapse of the disease with or without bone marrow involvement, HSC transplantation should be considered in suitable patients after the reinduction performed by AML chemotherapies. There is only limited data about the role of radiotherapy in these patients. It could be used in patients with relapsed disease, organ dysfunction which should be quickly relieved and inadequate response to chemotherapy. The effect of radiotherapy on overall survival is not known. New prospective studies and clinical trials are needed to generate guidelines for the treatment of primary granulocytic sarcomas.
Granulocytic sarcoma; treatment; chemotherapy; leukemia
Granulocytic sarcoma (GS) is an extramedullary tumor mass consisting of immature myeloid cells. Isolated pancreatic granulocyte sarcoma is extremely rare. We report a very unusual pancreatic granulocytic sarcoma in a patient without acute myeloid leukemia. The patient presented with acute epigastric pain because of splenic infarction due to a mass consisting of myeloblasts in the pancreatic tail. The patients underwent splenectomy and distal pancreatectomy. Pathology and immunohistochemistry suggested a GS. Despite local surgery, an isolated tumor recurred 2 mo after operation and the patient died 3 mo after removal of the tumor. Only 7 reported cases of pancreatic GS were identified in the literature and the mass was located in the pancreatic head. This is the first report of GS in the pancreatic tail with splenic infarction.
Granulocytic sarcoma; Pancreatic mass
Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. It may present in association with acute myeloid leukaemia, myelodysplastic syndrome and chronic myelogenous leukaemia. Granulocytic sarcoma may occur in any anatomical site but involvement of the gastrointestinal tract is rare, especially in the rectum. We report on the case of a 17 year old female who presented with rectal bleeding, abdominal pain and weight loss one mo prior to admission. Rectosigmoidoscopy revealed a rectal polypoid and ulcerated mass. The histological examination of the mass showed granulocytic sarcoma. Bone marrow examination was compatible with acute promyelocytic leukaemia (FAB type M3). This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukaemia. We also discuss diagnostic methods and analyze the disease course.
Granulocytic sarcoma; Acute myeloid leukaemia; Rectum; Gastrointestinal bleeding; Chemotherapy
Myeloid sarcoma is a tumor which consists of myeloblasts or immature myeloid cells. This tumor presents in the lymphoid organs, bone, skin, soft tissue, various mucosae and organs, and the central nervous system. Granulocytic sarcoma, an extramedullary acute myeloid leukemia, is also referred to as chloroma (GS) because of its greenish surface color. Granulocytic sarcoma is rare and difficult to diagnose. We can easily misdiagnose this tumor as lymphoma or sarcoma, especially when there is no evidence of hematologic disorders. Immunohistochemical studies are helpful in determining the correct diagnosis. Antibodies to myeloperoxidase, lysozyme, and chloroacetate esterase are used for the diagnosis of granulocytic sarcoma. In addition, detection of cell surface markers such as CD 33, CD 34, CD 68, CD 99, and HLA-DR may be useful. We describe a case of GS that presented with bluish nodules on the right cheek of a 54-year-old woman with immunohistochemical findings for correct diagnosis.
Extramedullary acute myeloid leukemia; Immunohistochemistry; Myeloperoxidase
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; Breast; Granulocytic sarcoma
Granulocytic sarcoma (GS) is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. Most GS reported in large series were not associated with overt acute myelogenous leukemia. Gastric perforation occurred during prednisolone therapy in a 72-year-old Japanese male with a four-month history of a myelofibrosis-like state. Subtotal gastrectomy was performed for a suspected gastric ulcer perforation. Gastric histologic, immunohistochemical and cytochemical examination revealed diffuse infiltration by sheets of myeloblasts and promyelocytes with scant or moderately abundant cytoplasm including a few eosinophilic myelocytes. Bone marrow study done in one month after the operation disclosed refractory anemia with excess of blasts (RAEB). Leukemic transformation occurred two months later, and a subcutaneous tumor appeared on the forehead. The forehead tumor predominantly consisted of myeloblasts without evidence of maturation. Both the stomach and forehead tumors were examined immunohistochemically with a panel of monoclonal antibodies (LCA, L26, MT1, UCHL1, OPD4, LN-1, LN-2, LN-3, MB1, Leu-M1, PM) and polyclonal antibodies (lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S-100 protein, lactoferrin), as well as naphthol-ASD-chloroacetate esterase staining to investigate and characterize the reliable marks for GS, and the patient was diagnosed as GS. We found that gastric GS may occur in a myelofibrosis-like state followed by RAEB of myelodysplastic syndrome and that naphthol-ASD-chloroacetate esterase staining and immunohistochemical detection of MT1, lysozyme, and alpha 1-antitrypsin were the most reliable markers for confirming the diagnosis of GS.
Myeloid Sarcoma (MS), a rare extra hematopoietic carcinoma composed of blast cells, is located primarily in extramedullary sites such as skin, soft tissue, lymph nodes, and bone. MS usually presents in the setting of coexisting acute myeloid leukemia (AML) and myeloproliferative disorders. Gastrointestinal involvement (GI) is extremely rare from nonspecific abdominal symptoms to obstruction. Eight cases of myeloid sarcoma involving the duodenum including the current case have been reported, overall mean age being 40 years (range 17–71) and M : F ratio 7 : 1. The prognosis of patients with de novo MS cases has been reported to be better than those who have a coexisting leukemia. MS is a rare extramedullary tumor, which should be considered in the differential diagnosis of a soft tissue mass involving the duodenum, especially if there is a coexisting hematological disorder. De novo cases often progress to AML, and current therapy involves Daunorubicin- and Cytarabine-based chemotherapy. The wide cytogenetic and molecular heterogeneity of MS implies a potential role for more targeted MS therapies, which may offer a curative strategy.
Introduction. Granulocytic sarcoma (GS), also known as chloroma or extramedullary myeloblastoma, is a solid tumor composed of primitive precursors of the granulocytic series that include myeloblasts, promyelocytes, and myelocytes. Granulocytic sarcoma is a rare tumor that may develop during acute myeloid leukemia (AML) but less frequently may precede its presentation. Although generalized lymph node enlargement is a presentation for malignant lymphoma, it can also rarely be the early presenting sign of GS.
Methods. We present a case of GS mimicking lymphoma in a 45-year-old male. The patient presented with bilateral neck masses and had widespread, prominent lymphadenopathy secondary to AML as the first presenting manifestation of GS for the last 4 months with concurrent marrow AML. Result. A clinical diagnosis of lymphoma was suspected; fine needle aspiration cytology findings were also suggestive of lymphoma. However, peripheral blood and bone marrow examination reported as acute myeloid leukemia with monocytic differentiation and histopathology of excised lymph node confirmed it to be a GS not lymphoma. Conclusion. GS is often misdiagnosed as malignant lymphoma because of cytomorphologic and histologic similarities of the blasts to large cell lymphoma. A careful search for immature myeloid is a useful clue to the diagnosis accompanied with appropriate immunophenotyping.
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.
Chloroma; Granulocytic sarcoma; Leukemia
Granulocytic sarcoma (GS) is a rare extramedullary tumor composed of immature myeloid cells. It is usually associated with leukemia or other myeloproliferative disorders. It occurs very rarely without overt hematologic diseases. A 19-year-old man presented with left knee mass. Biopsy with pathological analysis showed lymphoma aspect. Immunostains yielded the diagnosis of GS with myeloperoxidase and CD43 positivity. There was no systemic manifestation of leukemia, and bone marrow biopsiy was negative for neoplastic infiltration. Chemotherapy by CHOP was efficient, and the patient remaind alive and healthy 40 months after the end of treatment. The case is discussed in the framework of the existing literature about the diagnosis, treatment, and prognosis of this very rare condition.
Chloroma, also called granulocytic sarcoma (GS) or Myeloid Sarcoma, is a rare malignant extra-medullary neoplasm of myeloid precursor cells. It is usually associated with myeloproliferative disorders but its appearance may precede the onset of leukaemia. The presence of a chloroma is certainly a sign of poor prognosis. We report a case of chloroma in a middle aged female without a prior history of leukemia in the perianal skin region with clinical diagnosis of perianal abscess. When there is no concomitant leukaemia, diagnosis of chloroma may be difficult. Differential diagnoses include acute lymphoblastic leukemia, large cell NHL, lymphoblastic lymphoma, Ewing's sarcoma and cutaneous T cell lymphoma.
Chloroma; granulocytic sarcoma; leukemia; myeloid sarcoma
We describe a unique case of Granulocytic Sarcoma (GS) in a male, who presented to us with a painless right breast mass without any prior history of Leukemia. GS is an extramedullary tumor of myeloproliferative precursors and may involve multiple sites of the body, but involvement of male breast is extremely rare. In the absence of clinical history or hematological abnormality, GS may be misdiagnosed, depending on the degree of myeloid differentiation present within the tumor. Often it is misdiagnosed as lymphoma. Diagnosis is made by finding eosinophilic myelocytes, myeloperoxidase, chloroacetate esterase staining, and lysozyme immunostain. Chemotherapy regimens similar to acute myeloid leukemia are recommended to treat GS. Recognition of this rare entity is important because early, aggressive chemotherapy can induce regression of the tumor and improve patient longevity.
Background. Localized granulocytic sarcoma of the uterine cervix in the absence of acute myelogenous leukemia (AML) at presentation is very rare, its diagnosis is often delayed, and its prognosis almost always ominous evolving into refractory AML. Case. We present the case of a 30-year-old woman with vaginal bleeding and a large cervical mass. Further evaluation confirmed the presence of a granulocytic sarcoma but failed to reveal systemic involvement. Results. AML type chemotherapy followed by radiotherapy of the uterus led to a durable complete remission. She remains in complete remission six years after diagnosis. Conclusion. Granulocytic sarcoma of the cervix is a rare entity for which early intensive AML type therapy is effective.
A rare case of spinal epidural granulocytic sarcoma (GS) preceding acute myelogenous leukemia is described. A 10-year-old boy presented with lower leg weakness. The initial diagnosis was a histiocytic lymphoma, and he was treated accordingly. No evidence of bone marrow involvement was found at that time. The correct diagnosis of epidural GS was made possible in retrospect by using immunoperoxidase staining for lysozyme fourteen months later when the patient showed the full-blown features of leukemia. This rare tumor should be considered in the differential diagnosis of an epidural mass with cord compression in patients with or even without acute leukemia, because early diagnosis followed by appropriate combined chemotherapy and radiation may obviate surgical intervention and eventually prevent leukemic transformation.
Granulocytic sarcoma (GS) is a rare extramedullary manifestation of acute myeloid leukemia (AML). It may also represent blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. Although usually seen in the context of advanced and poorly controlled disease, it may also present as the first manifestation of illness, without concurrent bone marrow or blood involvement. In the medical literature, chloroma and GS are terms that have been used interchangeably with myeloid sarcoma. GS usually manifests as soft tissue or bony masses in several extracranial sites, such as bone, periosteum, and lymph nodes; involvement of the head and neck region is uncommon. We report a case of a woman with insidious onset of progressive nasal congestion and diminished hearing who was diagnosed with an isolated GS of the nasopharynx. With involved field radiotherapy, she achieved a complete remission of 12-months duration before being diagnosed with overt AML. She has remained disease-free for greater than 18 months following induction and consolidation chemotherapy. Through a MEDLINE®/PubMed® search we identified an additional 13 cases of nasopharyngeal GS. The median age was 37 years (range 1 to 81 years). The cases were equally distributed among the sexes. The most common presenting symptoms were conductive hearing loss and sinonasal congestion. Isolated GS was identified in six cases, and the median time from diagnosis of GS to AML was 12 months (range 3 to 48 months). The treatment varied, but responses were seen in all the patients who received chemotherapy with or without radiotherapy.
acute myeloid leukemia; myeloid sarcoma; chloroma; treatment
We report a rare case of granulocytic sarcoma infiltrating the bile duct in a patient with acute myeloid leukemia. A 23-year-old man presented with jaundice and weight loss. A peripheral blood smear revealed blast cells, and the results of an examination of bone marrow aspirate were consistent with acute myeloid leukemia. The bilirubin level increased gradually after induction chemotherapy with cytarabine. Magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of the intrahepatic bile ducts and smooth tapering off at the level of the common hepatic bile duct. Endoscopic retrograde cholangiopancreatography (ERCP) also revealed diffuse narrowing of the proximal common hepatic bile duct. Obstructive jaundice resolved after endoscopic nasobiliary drainage. Remission induction chemotherapy with cytarabine and idarubicin was administered, and the patient remained complete hematological remission with normal liver function tests.
Leukemia, Myelocytic, Acute; Jaundice, Obstructive; Bile ducts; Retrograde cholangiopancreatography, Endoscopic
Acute Myelogenous Leukemia (AML) typically involves intramedullary proliferation of myeloid precursor cells. Extramedullary manifestations of AML are exceedingly rare, but do occur. Granu-locytic sarcoma, or chloroma, is one example of extramedullary leukemia cells forming a tumorous mass. We report a case of Chloroma in the volar forearm compartment presenting with both median nerve compressive neuropathy and apparent tenosynovitis. Abscess was at the top of the early differential, and the patient was scheduled for operative debridement. However, further evaluation indicated that chloroma was present, thus obviating the need for emergent surgical intervention and necessitating the induction of chemotherapy. To our knowledge this is the first report of chloroma in this location and with these presenting symptoms.
Granulocytic sarcoma is a rare extramedullary tumour, which most often occurs in the course of an acute or chronic leukaemia or myeloproliferative disorders. Rarely it is found before peripheral blood or bone marrow evidence of leukemia is present. We report an unusual case of acute paraplegia at first presentation of a spinal epidural granulocytic sarcoma without any haematological disorder. Therapeutic strategies are discussed in the light of the literature.
Granulocytic sarcoma also known as extramedullary myeloid sarcoma or chloroma is an uncommon manifestation of leukemia and presents as a deposit of leukemic cells outside the bone marrow. We report a case of a twenty-five-year-old pregnant woman who presented with progressive dysphagia and recurrent postprandial vomiting. Upper GI endoscopy had shown large flat laterally spread nodular lesions in the cardia and proximal body of stomach. Biopsies from the gastric lesion showed granulocytic sarcoma of the stomach. Concurrent peripheral and bone marrow picture was suggestive of acute myeloid leukemia (AML–M4). There is limited reported literature on granulocytic sarcoma of the stomach. Concurrent gastric granulocytic sarcoma involving cardia and AML in pregnancy has not been reported till date.