muramidase and Sarcoma--Myeloid

Myeloid Sarcoma with monocytic differentiation is rare and quite likely is missed by surgical pathologists. However it is frequently misdiagnosed because of its non-specific imaging and histological pattern.. We report the case of a 64-year-old woman with gastric primary myeloid sarcoma with monocytic differentiatio. Upper endoscopy revealed a neoplastic growth at the junction of the lesser curvature and gastric antrum. Except for a slightly increased peripheral monocyte count, no abnormalities were found on hematological and bone-marrow examination. Gastroscopic biopsy showed poorly differentiated atypical large cells with visible nucleoli and nuclear fission. Immunohistochemistry showed positive CD34, CD4, CD43, and CD56 expression, and weakly positive lysozyme expression. Immune markers for poorly differentiated adenocarcinoma, malignant melanoma, and lymphohematopoietic-system tumors were negative. The final diagnosis was myeloid sarcoma with monocytic differentiation. Chemotherapy did not shrink the tumor, so, radical surgery was performed. Although the tumor morphology did not change postoperatively, the immunophenotype did. CD68 and lysozyme expression (tumor tissue markers) changed from negative and weakly positive to strongly positive, AE1/3 expression (epithelial marker) changed from negative to positive, and CD34, CD4, CD43, and CD56 expression (common in naive hematopoietic cell-derived tumors) was greatly attenuated. Exome sequencing revealed missense mutations in FLT3 and PTPRB, which are associated with myeloid sarcoma, and in TP53, CD44, CD19, LTK, NOTCH2, and CNTN2, which are associated with lymphohematopoietic tumors and poorly differentiated cancers.. We diagnosed myeloid sarcoma with monocytic differentiation after excluding poorly differentiated adenocarcinoma, common lymphohematopoietic-system tumors, epithelioid sarcoma, and malignant melanoma. We identified that the immunophenotypic of patient had alterations after chemotherapy, and FLT3 gene mutations. We hope that the above results will improve our understanding of this rare tumor.

Topics: Adenocarcinoma; Cell Differentiation; Exome Sequencing; Female; Hematologic Neoplasms; Humans; Melanoma; Melanoma, Cutaneous Malignant; Middle Aged; Muramidase; Sarcoma, Myeloid

Myeloid sarcoma (MS) is defined as an extramedullary mass-forming lesion composed of immature myeloid cells. It is a rare but well-known manifestation of acute myeloid leukemia. Pediatrics testicular MS may pose a possible diagnostic challenge, an issue that is underscored in the few testicular pediatric MS cases reported in the literature. Herein, we report a series of 5 cases of pediatric testicular MS that are evaluated at the morphologic and immunohistochemical levels with correlation with the KMT2A (MLL) rearrangement status. Three patients presented with no prior history of acute myeloid leukemia. All 5 cases showed monoblastic morphology; positive for CD33, CD43, CD68, CD163, CD4 (dim), and lysozyme; and negative for CD10, CD34, CD117, and myeloperoxidase. KMT2A (MLL) rearrangement was detected in 4 of the 5 cases. In the literature, 8 more cases of pediatric testicular lymphoma were reported. Most of them showed monocytic differentiation and KMT2A (MLL) rearrangement was reported in 3 of the cases. In conclusions, testicular MS in pediatric patients shows monoblastic differentiation which may be attributed to the KMT2A (MLL) rearrangement. We also highlight the importance of using an extended immunohistochemistry panel in the diagnosis of MS.

Topics: Antigens, CD; Antigens, CD34; Antigens, Differentiation, Myelomonocytic; CD4 Antigens; Child; Child, Preschool; Gene Rearrangement; Histone-Lysine N-Methyltransferase; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Infant; Leukemia, Myeloid, Acute; Leukosialin; Male; Muramidase; Myeloid-Lymphoid Leukemia Protein; Neprilysin; Peroxidase; Proto-Oncogene Proteins c-kit; Receptors, Cell Surface; Sarcoma, Myeloid; Sialic Acid Binding Ig-like Lectin 3; Testicular Neoplasms

Myeloid sarcoma of the head and neck region can pose diagnostic challenges because of the low frequency of myeloid sarcoma and the potential for tumors of almost any lineage to occur in the head and neck.. To study the clinicopathologic and immunohistochemical characteristics of myeloid sarcoma in the head and neck region and to review the differential diagnosis.. We searched for cases of myeloid sarcoma involving the head and neck region for a 24-year period at our institution. The medical records and pathology slides were reviewed. Additional immunohistochemical stains were performed.. We identified 17 patients, age 17 to 85 years. Most tumors involved the oral cavity. Myeloid sarcoma was the initial diagnosis in 9 patients (53%); the remaining 8 patients (47%) had a history of bone marrow disease. Immunohistochemical analysis using antibodies specific for lysozyme, CD43, and CD68 were highly sensitive for diagnosis but were not specific. By contrast, assessment for myeloperoxidase in this study was less sensitive but more specific. We also used antibodies specific for CD11c and CD33 in a subset of cases, and these reagents seem helpful as well.. The clinical presentation of myeloid sarcoma involving the head and neck, particularly the mouth, is often nonspecific, and a high degree of suspicion for the possibility of myeloid sarcoma is needed. Immunohistochemistry is very helpful for establishing the diagnosis.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Bone Marrow Diseases; CD11c Antigen; Diagnosis, Differential; Female; Head and Neck Neoplasms; Humans; Immunohistochemistry; Leukosialin; Male; Middle Aged; Muramidase; Peroxidase; Sarcoma, Myeloid; Sialic Acid Binding Ig-like Lectin 3; Young Adult

Topics: Aged; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Biomarkers, Tumor; Bone Neoplasms; Carboxylic Ester Hydrolases; Humans; Leukemia, Myeloid, Acute; Magnetic Resonance Imaging; Male; Muramidase; Neoplasms, Multiple Primary; Peroxidase; Sarcoma, Myeloid; Skin Neoplasms; Soft Tissue Neoplasms; Subcutaneous Tissue

Extramedullary myeloid tumors (myeloid sarcomas) are rare neoplasms that are composed of myeloid precursors. They usually arise concurrently with a diagnosis of acute myeloid leukemia, chronic myeloid leukemia, or other myeloproliferative disorders. They may also indicate relapsing disease in a patient with a prior history of leukemia or myeloproliferative disorder. We present our findings of a 63-year-old female diagnosed with extramedullary myeloid tumor first presenting in the gallbladder. She subsequently developed respiratory failure; pre- and postmortem bone marrow studies were negative for leukemia by morphology, flow cytometry, and karyotypic analysis. However, the myeloid neoplasm was disseminated throughout most of her remaining organs. Immunohistochemical stains of the cells indicated a neoplasm of myelomonocytic derivation (CD4, CD43, CD45, CD68, myeloperoxidase, and lysozyme positive). To our knowledge, this is the first report of an extramedullary myeloid neoplasm of the gallbladder with disseminated disease without involvement of the bone marrow.

Topics: Antigens, CD; Antigens, Differentiation, Myelomonocytic; Bone Marrow; Female; Gallbladder Neoplasms; Humans; Middle Aged; Muramidase; Peroxidase; Respiratory Insufficiency; Sarcoma, Myeloid

Myeloid sarcoma can involve any anatomic site, but involvement of the gynecologic tract is uncommon. We describe 11 women, 17 to 60 years old, with myeloid sarcoma involving the gynecologic tract, including 5 patients in whom myeloid sarcoma presented as an isolated mass. The uterus was the most frequently involved anatomic site, in 8 patients (5 corpus, 3 cervix). Each neoplasm diffusely infiltrated normal structures, and, cytologically 7 tumors were immature, 3 were differentiated, and 1 was blastic. In 9 cases assessed, immunohistochemical stains showed that all neoplasms were positive for myeloperoxidase and lysozyme; CD117 was positive in 7 of 8 cases, and cytochemical staining for naphthol AS-D chloroacetate was positive in all 6 neoplasms analyzed. Following chemotherapy, complete remission and long-term survival were achieved in a subset of patients, as was particularly true for 2 patients (cases 8 and 10), with complete remission 12.5 and 31 years after diagnosis, respectively.

Topics: Adolescent; Adult; Biomarkers, Tumor; Fatal Outcome; Female; Genital Neoplasms, Female; Humans; Middle Aged; Muramidase; Peroxidase; Sarcoma, Myeloid

Granulocytic sarcoma is a form of extramedullary leukaemia. The intraparenchymal localisation is extremely rare. We report a case of cerebellar granulocytic sarcoma occuring in a 43 years old woman without any precedent medical history. The diagnosis of granulocytic sarcoma was established by neoplastic cells findings through morphological and immunohistochemical studies. The patient died few days after surgery. There are still no conclusive treatment strategies for this entity; however, early antileukemic chemotherapy seems to lower the probability of developing systemic disease and thus prolong survival.

Topics: Adult; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Biomarkers, Tumor; Carboxylic Ester Hydrolases; Cerebellar Neoplasms; Fatal Outcome; Female; Headache; Hepatomegaly; Humans; Immunoenzyme Techniques; Muramidase; Neoplasm Proteins; Peroxidase; Sarcoma, Myeloid; Splenomegaly; Staining and Labeling

CD117 (c-Kit) and lysozyme are frequently expressed by myeloblasts and are sensitive markers for the diagnosis of extramedullary myeloid tumor. The diagnosis of cutaneous plasmacytoma presents a degree of difficulty, particularly with the plasmablastic variant, which can mimic hematologic as well as epithelioid malignancies. Approximately 25% of multiple myelomas express CD117 in the bone marrow by flow cytometry. Lysozyme immunoreactivity has been previously shown in 30% of poorly differentiated myelomas, while it is nonreactive in nonmalignant plasma cells.. To ascertain whether CD117 and lysozyme can aid in the diagnosis of cutaneous plasmacytomas, particularly the plasmablastic type.. Pathology reports of 2357 patients with a diagnosis of multiple myeloma were reviewed to find 13 cutaneous plasmacytomas (8 Bartl grade II, 5 Bartl grade III). Formalin-fixed, paraffin-embedded tissue sections were stained with CD117 and lysozyme on the Dako Autostainer system.Setting.-Patients with the diagnosis of multiple myeloma who developed cutaneous plasmacytoma(s).. The cutaneous plasmacytomas uniformly expressed CD117 in a cytoplasmic or membranous and cytoplasmic distribution with varying degrees of staining intensity unrelated to the Bartl grade of the lesion, while they were uniformly negative for lysozyme.. CD117 is a sensitive marker for malignant plasma cells in paraffin-embedded tissue, while lysozyme does not help identify poorly differentiated malignant plasma cells. While CD117 alone does not distinguish extramedullary myeloid tumor from poorly differentiated myeloma, the combination of CD117 and lysozyme may allow their differentiation. The possibility of c-kit inhibitors being used in the treatment of other hematopoietic malignancies allows speculation regarding implications for the treatment of multiple myeloma.

Topics: Biomarkers; Diagnosis, Differential; Humans; Immunohistochemistry; Muramidase; Plasmacytoma; Proto-Oncogene Proteins c-kit; Sarcoma, Myeloid; Skin Neoplasms