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1.  Granulocytic sarcoma: a systematic review 
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.
PMCID: PMC3875275  PMID: 24396704
Granulocytic sarcoma; treatment; chemotherapy; leukemia
2.  Nonleukemic Granulocytic Sarcoma in the Bile Duct: A Case Report 
Journal of Korean Medical Science  2006;21(4):745-748.
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.
doi:10.3346/jkms.2006.21.4.745
PMCID: PMC2729902  PMID: 16891824
Sarcoma, Granulocytic; Bile Ducts; Leukemia, Myelocytic, Acute
3.  Solitary Preleukemic Granulocytic Sarcoma as a Cause of Small Bowel Obstruction 
Gut and Liver  2007;1(1):82-86.
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.
doi:10.5009/gnl.2007.1.1.82
PMCID: PMC2871656  PMID: 20485664
Granulocytic sarcoma; Acute myeloid leukemia; Small bowel obstruction
4.  Granulocytic sarcoma presenting as presenting as monoparesis: A rare case report 
Granulocytic sarcomas (GSs) or myeloid sarcoma or chloroma are rare, destructive, extramedullary tumor masses that consist of immature granulocytic cells. We present case of a 35-year-old man presenting as monoparesis, diagnosed to have cervical intradural extramedullary mass lesion with an extradural extension. Although the history or physical examination had no symptoms and signs suggestive of leukemia, bone marrow study and blood picture indicated chronic myeloid leukemia. Surgical decompression was done, and histopathological examination was consistent with GS. GSs have been observed in patients with acute myelogenous leukemia, chronic myelogenous leukemia, and other myeloproliferative disorders, but rarely have been reported as first presentation of the disease.
doi:10.4103/1793-5482.146641
PMCID: PMC4323979
Acute myeloid leukemia; chronic myeloid leukemia; epidural mass; granulocytic sarcoma
5.  Nonleukemic Ureteral Granulocytic Sarcoma Presenting with Unilateral Urinary Obstruction and Hematuria 
Case Reports in Urology  2013;2013:861232.
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.
doi:10.1155/2013/861232
PMCID: PMC3760117  PMID: 24024067
6.  Unusual case of paraplegia 
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.
doi:10.4103/0972-2327.56321
PMCID: PMC2824938  PMID: 20174502
Acute myeloid leukemia; chloroma; granulocytic sarcoma
7.  Multiple Granulocytic Sarcomas in a Patient with Longstanding Complete Remission of Acute Myelogenous Leukemia 
Annals of Dermatology  2011;23(Suppl 2):S270-S273.
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.
doi:10.5021/ad.2011.23.S2.S270
PMCID: PMC3229084  PMID: 22148069
Aleukemic leukemia cutis; AML M1; Granulocytic sarcoma
8.  Lateral pharyngeal wall myeloid sarcoma as a relapse of acute biphenotypic leukemia: a case report and review of the literature 
Introduction
Myeloid sarcoma is a rare extramedullary malignant tumor composed of immature myeloid cells. The tumor can affect any part of the body. Involvement of the oral cavity and nasopharynx has been reported in 50 cases. We report a case describing myeloid sarcoma affecting the lateral pharyngeal wall.
Case presentation
A 31-year-old Arabian man who had acute biphenotypic leukemia treated with chemoradiation and allogeneic stem cell transplant was referred to our department with sore throat and a mass lesion in his lateral pharyngeal wall after failed antibiotic therapy. Biopsy of his lesion revealed myeloid sarcoma. He was referred to the Department of Hematology-Oncology for further evaluation that showed no other lesions.
The patient was diagnosed with isolated extramedullary myeloid sarcoma of his lateral pharyngeal wall as a relapse of acute biphenotypic leukemia and managed with chemoradiation.
Conclusions
Myeloid sarcoma of the pharynx is a rarely encountered malignancy in the practice of otolaryngology-head and neck surgery. It can develop de novo, but may also represent relapse of leukemia. Thus, it should be considered in the differential diagnosis of any pharyngeal lesions in patients with leukemia.
doi:10.1186/1752-1947-7-292
PMCID: PMC3880180  PMID: 24377982
Acute biphenotypic leukemia; Extramedullary tumor; Lateral pharyngeal wall; Myeloid sarcoma
9.  Multiple Jejunal Myeloid Sarcomas Presenting with Intestinal Obstruction in a Non-leukemic Patient: A Case Report with Ultrastructural Observations 
Korean Journal of Pathology  2012;46(6):590-594.
Myeloid sarcoma is a rare extramedullary myeloid tumor, which is frequently misdiagnosed when no evidence of leukemia is initially observed. Here, we report on a peculiar case of a 49-year-old man afflicted with multiple masses in the jejunum, the superior mesentery, and the serosa of the transverse colon, without leukemic manifestation. The tumor was composed of undifferentiated small round cells containing eosinophilic cytoplasm, which were negative for myeloperoxidase, nonspecific esterase, lysozyme, terminal deoxynucleotidyl transferase, leukocyte common antigen, CD3, CD4, CD15, CD20, CD30, CD43, CD56, CD68/PG-M1, CD79a, human melanoma black-45, c-kit, and CD34 with positivity only for CD68/KP1, CD99, and vimentin. Under electron microscopy, those cells had abundant membrane-bound cytoplasmic granules that measured 200 to 300 nm in diameter, which were consistent with granulocytic azurophilic granules. The tumor was finally diagnosed as a myeloid sarcoma. The presence of non-leukemic myeloid sarcomas showing immunonegativity for conventional myeloid-leukemic markers necessitated a diagnosis by ultrastructural observation.
doi:10.4132/KoreanJPathol.2012.46.6.590
PMCID: PMC3540339  PMID: 23323112
Sarcoma, myeloid; Jejunum; CD68/KP1; CD99; Microscopy, electron
10.  Granulocytic sarcoma of the pancreas: A case report and review of the literatures 
BMC Gastroenterology  2010;10:80.
Background
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.
Case presentation
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.
Conclusions
The accurate diagnosis of GS is necessary for determining prognosis and deciding appropriate therapy.
doi:10.1186/1471-230X-10-80
PMCID: PMC2912803  PMID: 20624285
11.  Generalized Lymphadenopathy as the First Presentation of Granulocytic Sarcoma: A Diagnostic Challenge 
Case Reports in Medicine  2013;2013:483291.
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.
doi:10.1155/2013/483291
PMCID: PMC3824813  PMID: 24282418
12.  Unsuspected gastric granulocytic sarcoma in a patient with myelodysplastic syndrome. 
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.
PMCID: PMC3053922  PMID: 8703373
13.  Acute Appendicitis Secondary to Acute Promyelocytic Leukemia 
Patient: Female, 43
Final Diagnosis: Myeloid sarcoma appendicitis
Symptoms: Abdominal pain • chills • fever
Medication: —
Clinical Procedure: Laparoscopic appendectomy, bone marrow biopsy
Specialty: Gastroenterology and Hepatology
Objective:
Rare disease
Background:
The gastrointestinal tract is a rare site for extramedullary involvement in acute promyelocytic leukemia (APL).
Case Report:
A 43-year-old female with no past medical history presented complaining of mild abdominal pain, fever, and chills for the past day. On examination, she was tachycardic and febrile, with mild tenderness of her right lower quadrant and without signs of peritoneal irritation. Laboratory examination revealed pancytopenia and DIC, with a fibrinogen level of 290 mg/dL. CT of the abdomen showed a thickened and hyperemic appendix without perforation or abscess, compatible with acute appendicitis.
The patient was given IV broad-spectrum antibiotics and was transfused with packed red blood cells and platelets. She underwent uncomplicated laparoscopic appendectomy and bone marrow biopsy, which revealed neo-plastic cells of 90% of the total bone marrow cellularity. Flow cytometry indicated presence of 92.4% of immature myeloid cells with t (15: 17) and q (22: 12) mutations, and FISH analysis for PML-RARA demonstrated a long-form fusion transcript, positive for APL. Appendix pathology described leukemic infiltration with co-expression of myeloperoxidase and CD68, consistent with myeloid sarcoma of the appendix.
The patient completed a course of daunorubicin, cytarabine, and all trans-retinoic acid. Repeat bone marrow biopsy demonstrated complete remission. She will follow up with her primary care physician and hematologist/oncologist.
Conclusions:
Myeloid sarcoma of the appendix in the setting of APL is very rare and it might play a role in the development of acute appendicitis. Urgent management, including bone marrow biopsy for definitive diagnosis and urgent surgical intervention, dramatically improve prognosis.
doi:10.12659/AJCR.892760
PMCID: PMC4327184  PMID: 25666852
Appendicitis; Leukemia, Promyelocytic, Acute; Sarcoma, Myeloid
14.  Granulocytic sarcoma of the rectum: Report of one case that presented with rectal bleeding 
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.
doi:10.4291/wjgp.v1.i4.144
PMCID: PMC3097956  PMID: 21607155
Granulocytic sarcoma; Acute myeloid leukaemia; Rectum; Gastrointestinal bleeding; Chemotherapy
15.  Precursor B-cell acute lymphoblastic leukemia presenting as obstructive jaundice: a case report 
Introduction
Acute leukemias very rarely present with jaundice. Herein we report a case of precursor B-cell acute lymphoblastic leukemia that presented with jaundice in an adult.
Case presentation
A 44-year-old Hispanic man presented with right upper quadrant abdominal pain and jaundice. His initial blood work revealed pancytopenia and hyperbilirubinemia. Direct bilirubin was more than 50% of the total. His imaging studies were unremarkable except for hepatomegaly. All blood screening tests for various hepatocellular etiologies were normal. A diagnosis of precursor B-cell acute lymphoblastic leukemia was made upon liver biopsy. It also showed lymphocytic infiltration of the hepatic parenchyma leading to bile stasis. The diagnosis was subsequently confirmed upon bone marrow biopsy. The patient was treated with a hyperfractionated cyclophosphamide/vincristine/doxorubicin/dexamethasone regimen.
Conclusion
Acute lymphoblastic leukemia should be one of the differential diagnoses that should be considered when initial work-up for jaundice is inconclusive. Some cases of acute lymphoblastic leukemia have been reported in both adults and children to have presented with the initial manifestation of jaundice, but only a few had no radiographic evidence of biliary obstruction. Such presentation can pose a serious diagnostic dilemma for clinicians. This manuscript attempts to highlight it. Moreover, we believe that if acute lymphoblastic leukemia presentations similar to this case continue to be reported in adults or children, a specific immunophenotypic expression and cytogenetic abnormality may be found to be associated with hepatic infiltration by leukemia. This may substantially contribute to the further understanding of the pathophysiology of this hematologic disease.
doi:10.1186/1752-1947-5-269
PMCID: PMC3141714  PMID: 21722379
16.  Granulocytic Sarcoma in Breast after Bone Marrow Transplantation 
Journal of Breast Cancer  2013;16(1):112-116.
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.
doi:10.4048/jbc.2013.16.1.112
PMCID: PMC3625757  PMID: 23593091
Acute myeloid leukemia; Breast; Granulocytic sarcoma
17.  Isolated pancreatic granulocytic sarcoma: A case report and review of the literature 
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.
doi:10.3748/wjg.v17.i4.540
PMCID: PMC3027023  PMID: 21274386
Granulocytic sarcoma; Pancreatic mass
18.  Cerebellar Granulocytic Sarcoma: A Case Report 
Turkish Journal of Hematology  2012;29(2):177-180.
Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells that is usually associated with acute myelogenous leukemia. Intraparenchymal cranial localization without skull, meningeal, or bone marrow invasion is extremely rare. The mechanisms of intraparenchymal cranial localization of GS remains unknown, as only 10 cases with cerebellar granulocytic sarcoma have been previously reported. Herein, we report a four year old boy with cerebellar localization of granulocytic sarcoma.
doi:10.5505/tjh.2012.65002
PMCID: PMC3986958  PMID: 24744651
children; Acute leukemia; Granulocytic sarcoma
19.  Primary Granulocytic Sarcoma of the Face 
Annals of Dermatology  2011;23(Suppl 2):S214-S217.
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.
doi:10.5021/ad.2011.23.S2.S214
PMCID: PMC3229069  PMID: 22148054
Extramedullary acute myeloid leukemia; Immunohistochemistry; Myeloperoxidase
20.  Isolated granulocytic sarcoma of the nasopharynx: a case report and review of the literature 
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.
doi:10.2147/IMCRJ.S53612
PMCID: PMC3869915  PMID: 24368892
acute myeloid leukemia; myeloid sarcoma; chloroma; treatment
21.  Primary Vaginal Myeloid Sarcoma: A Rare Case Report and Review of the Literature 
Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour) is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML). A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen), MPO (myeloperoxidase), and c-kit were strongly positive on IHC (immunohistochemistry). The patient's routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India), 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.
doi:10.1155/2015/957490
PMCID: PMC4313514
22.  Metachronous Bilateral Granulocytic Sarcoma of the Testis in a Young Adult: A Report of an Unusual Entity 
Case Reports in Urology  2014;2014:762630.
Granulocytic sarcomas are rare tumors composed of neoplastic blood cells, typically occurring during the course of acute nonlymphoblastic leukemia or before its onset. We present a case of a 23-year-old young adult man with metachronous granulocytic sarcoma of the testis without hematologic manifestations who was diagnosed with granulocytic sarcoma (GS). The patient was treated with right orchiectomy but relapsed with a left testicular mass 16 months later when a left orchiectomy was performed. The patient has been free of disease for 13 months following the left orchiectomy. This case highlights a rare hematologic cancer that urologists and pathologists should be aware of since it can present as a testicular mass. Only 3 cases of testicular GS without an associated hematologic disorder have been described. To the best of our knowledge, our patient is the first reported case in the English literature of metachronous GS of the testis with no evidence of hematologic disorder.
doi:10.1155/2014/762630
PMCID: PMC4026881  PMID: 24883222
23.  Acute Myeloid Leukemia Presenting as Obstructive Jaundice Caused by Granulocytic Sarcoma 
Gut and Liver  2007;1(2):182-185.
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.
doi:10.5009/gnl.2007.1.2.182
PMCID: PMC2871626  PMID: 20485638
Leukemia, Myelocytic, Acute; Jaundice, Obstructive; Bile ducts; Retrograde cholangiopancreatography, Endoscopic
24.  Massive myeloid sarcoma affecting the central nervous system, mediastinum, retroperitoneum, liver, and rectum associated with acute myeloblastic leukaemia: a case report 
Journal of Clinical Pathology  2005;58(3):325-327.
Myeloid sarcomas are extramedullary tumours with granulocytic precursors. When associated with acute myelogenous leukaemia (AML), these tumours usually affect no more than two different extramedullary regions. This report describes a myeloid sarcoma associated with AML with tumour formation at five anatomical sites. The patient was a 37 year old man admitted in September 1999 with a two month history of weight loss, symptoms of anaemia, rectal bleeding, and left facial nerve palsy. The anatomical sites affected were: the rectum, the right lobe of the liver, the mediastinum, the retroperitoneum, and the central nervous system. A bone marrow smear was compatible with AML M2. Flow cytometry showed that the peripheral blood was positive for CD4, CD11, CD13, CD14, CD33, CD45, and HLA-DR. A karyotypic study of the bone marrow revealed an 8;21 translocation. The presence of multiple solid tumours in AML is a rare event. Enhanced expression of cell adhesion molecules may be the reason why some patients develop myeloid sarcomas.
doi:10.1136/jcp.2003.015651
PMCID: PMC1770587  PMID: 15735171
acute myelogenous leukaemia; myeloid sarcoma
25.  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.
doi:10.3340/jkns.2010.47.5.385
PMCID: PMC2883061  PMID: 20539800
Chloroma; Granulocytic sarcoma; Leukemia

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