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Myeloid sarcoma of the urinary bladder and epididymis as a primary manifestation of acute myeloid leukemia with inv(16).

Myeloid sarcoma (MS) of the lower urinary tract is rare. We describe a 47-year-old man with hematuria, who was subsequently found to have MS involving bladder and epididymis. The neoplasm was composed predominantly of blasts that expressed CD68, CD117, myeloperoxidase, and lysozyme, with occasional immature eosinophils. Although blood and bone marrow examinations showed no morphologic evidence of leukemia, conventional cytogenetic studies of marrow demonstrated inv(16)(p13q22) in 4 of 20 metaphases; fluorescence in situ hybridization of the bladder neoplasm also showed inv(16). Following chemotherapy, the patient has been in complete remission for 32 months. In our literature review, we identified 7 cases of MS involving bladder, only 3 without evidence of an associated myeloid neoplasm in marrow, none with cytogenetic data. A high index of suspicion is required to establish the diagnosis of MS involving bladder. Cytogenetic analysis is useful for both demonstrating minimal marrow disease and classifying MS in paraffin-embedded tissue sections.

Antineoplastic Combined Chemotherapy Protocols↗

Myeloid sarcoma.

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Humans↗

Myeloid sarcoma of both kidneys, the brain, and multiple bones in a nonleukemic child.

A myeloid sarcoma (MS) is an extramedullary tumor consisting of primitive granulocytic precursor cells. Although most such tumors have been reported in patients with acute myelogenous leukemia, MS is rarely recognized as an isolated tumor without any evidence of leukemia. However, in such cases, the initial diagnosis of MS can be difficult, so initial misdiagnosis rates of up to 75% have been reported. This report describes an unusual case of MS in a 3-year 5-month-old girl presenting as bilateral renal enlargements, and brain masses, with multiple bone involvements, but no hematological abnormalities.

Bone Neoplasms↗

Myeloid sarcoma: clinical and morphologic criteria useful for diagnosis.

Extramedullary accumulation of myeloblasts or immature myeloid cells form tumors called myeloid sarcoma in the WHO classification. Such tumors develop in lymphoid organs, bone (skull, orbit, etc.), skin, soft tissue, various mucosae and organs, and the CNS. They may precede or occur concurrently with acute myeloid leukemia, or reveal blastic transformation of chronic myeloproliferative disorders or myelodysplastic syndromes. They may also reveal relapses in treated patients. They are constituted by a diffuse infiltrate made up of medium-to-large cells. The cells are difficult to identify. Imprints are very useful. Immunohistochemistry can help diagnose and distinguish four variants: granulocytic myeloperoxidase (MPO+, CD 68+ [KP1+/-, PGM1-] lysozyme+, CD 34+/-), monoblastic (MPO-, CD 68+, [KP1+, PGM1+] lysozyme+, CD 34-), myelomonoblastic (MPO-, CD 68+, [KP1+, PGM1+] lysozyme+, CD 34-), or megakaryoblastic (positivity for factor VIII, CD 61, CD 31). Immunohistochemistry sometimes demonstrates expression of CD 43, CD 7, CD 79a, and CD 56 (particularly the monoblastic variant with t[8;21]). Recently the demonstration of CD 99 and CD 117, which can now be done on paraffin sections, may be useful to identify blasts of granulocytic origin. The diagnosis is missed in about 50% of cases when immunohistochemistry is not used. Patients with myeloid sarcomas should be treated in the same way as patients with acute myeloblastic leukemia. Disease progression and prognosis are similar for the two conditions.

Antigens, CD↗

Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice.

We report a rare case of myeloid sarcoma (MS) of the extrahepatic bile ducts presenting as obstructive jaundice in a patient without leukemia at time of diagnosis. A 75-year-old female presented with a one-month history of abdominal pain and jaundice. Computerized tomography scan of the abdomen showed stenosis of the extrahepatic bile ducts. Endoscopic retrograde cholangiography disclosed an irregular narrowing of the common biliary duct, suggestive of a cholangiocarcinoma, and resection was performed. Histologic examination showed diffuse transmural infiltration of malignant cells. These cells exhibited medium-sized round nuclei with central nucleoli and eosinophilic cytoplasm, and were strongly positive for myeloperoxidase, CD68, lysozyme, CD45, CD117 (c-kit protein) and CD43. Eight months following surgery the patient presented with multiple cutaneous nodules and bone marrow trephine biopsy showed acute myelomonocytic leukemia. A literature search identified two previously reported cases of MS of the extrahepatic biliary duct. MS should be taken into consideration in the differential diagnosis of a patient with obstructive jaundice. Immunohistochemistry is essential for a correct diagnosis.

Aged↗

Myeloid sarcoma occurring in the maxillary gingiva: a case without leukemic manifestations.

Myeloid sarcoma (MS) is a localized extramedullary mass of immature granulocytic cells that usually occurs in patients with acute myeloid leukemia (AML) or myeloproliferative disorders. It may rarely precede peripheral blood or bone marrow involvement, presenting a diagnostic challenge. Although MS may be found in any location, an intraoral occurrence is rare. In this report we describe a rare case of a patient with nonleukemic MS of the maxillary gingiva. The histologic specimen was first interpreted as non-Hodgkin's lymphoma. The correct diagnosis was reached after extensive immunohistologic studies. The malignant cells were myeloperoxidase positive, lysozyme positive, CD45+, CD68+, CD3-, CD10-, CD19-, CD20-, CD30-, CD34-, CD56-, CD79a-, S100-, and chloroacetate esterase negative. Induction therapy with FLAND (fludarabine, Ara-C, mitoxantrone, and dexamethasone) was started, but the patient did not achieve a remission. Some weeks later, the patient presented pleural effusion and paralysis of the seventh cranial nerve on the left side. She died a few days later. The present case indicates the importance of a correct initial diagnosis for adequate therapy, which is often delayed because of a high misdiagnosis rate. If the MS is treated without intensive chemotherapy for AML as soon as possible, the prognosis will be poor.

Antineoplastic Combined Chemotherapy Protocols↗

Myeloid sarcoma of the uterus presenting as vaginal bleeding.

Extramedullary relapse of acute myeloid leukaemia may occur in sites such as the central nervous system, testes, and skin. Presentations in the female genital tract are uncommon and usually asymptomatic. In contrast, symptomatic uterine myeloid sarcoma is very rare. Treatment of this is generally unsuccessful, but is improved when systemic therapies are used. We study a case of a uterine relapse of acute myeloid leukaemia presenting as vaginal bleeding and successfully managed by local irradiation. The mechanism of preferential infiltration of uterine tissue requires further study.

Diagnosis, Differential↗

Acute myeloid leukemia with concurrent myeloid sarcoma treated with autologous bone marrow transplantation: two illustrative cases and a literature review.

Myeloid sarcoma (MS) is an invasive extramedullary solid tumor composed of immature cells of the myeloid series. It complicates the clinical course of a minority of patients with acute myeloid leukemia (AML). Traditionally its presence has been regarded as an indicator of aggressive disease. Currently, the optimal treatment of AML with concurrent MS remains to be determined. We report two cases of autologous bone marrow transplantation (auto-BMT) for AML with concurrent MS followed by a review of the literature.

Adult↗

Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients.

Myeloid sarcoma (MS) is a rare neoplasm whose knowledge is largely based on case reports and/or technically dated contributions. Ninety-two MSs in adulthood with clinical data available were evaluated both morphologically and immunohistochemically. Seventy-four cases were also studied by fluorescent in situ hybridization on tissue sections and/or conventional karyotyping on bone marrow or peripheral blood. Histologically, 50% of the tumors were of the blastic type, 43.5% either monoblastic or myelomonocytic and 6.5% corresponded to different histotypes. CD68/KP1 was the most commonly expressed marker (100%), followed by myeloperoxidase (83.6%), CD117 (80.4%), CD99 (54.3%), CD68/PG-M1 (51%), CD34 (43.4%), terminal-deoxy-nucleotidyl-transferase (31.5%), CD56 (13%), CD61/linker for activation of T cells (2.2%), CD30 (2.2%) and CD4 (1.1%). Foci of plasmacytoid monocyte differentiation were observed in intestinal cases carrying inv16. Chromosomal aberrations were detected in about 54% of cases: monosomy 7(10.8%), trisomy 8(10.4%) and mixed lineage leukemia-splitting (8.5%) were the commonest abnormalities, whereas t(8;21) was rare (2.2%). The behavior was dramatic irrespective of presentation, age, sex, phenotype and cytogenetics. Most if not all, long survivors received bone-marrow transplantation. The present report expands the spectrum of our knowledge showing that MS has frequent monoblastic/myelomonocytic differentiation, displays distinctive phenotypic profile, carries chromosomal aberrations other than t(8;21), and requires supra-maximal therapy.

Adolescent↗

Clinico-pathological characteristics of myeloid sarcoma at diagnosis and during follow-up: report of 12 cases from a single institution.

The aim of this study was to describe the presenting features, the frequency and outcome of myeloid sarcoma (MS) diagnosed in our Institution from January 1995 to December 2000. Twelve MS were seen and the frequency account for only 2% of all acute myeloid leukemia (AML) patients observed in our department in the same period. Median age was 45 years (range: 4-84). All had been initially misdiagnosed as malignant lymphoma (ML) and a median of 2.9 months (range: 1-6) elapsed between the misdiagnosis and the correct of MS, effectuated in our department. At that time, a bone marrow examination revealed a myelodysplastic condition in seven patients, an infiltration by blast cells >30% in two patients, and normal features in the other three. In the non-leukemic patients a median of 5 months (range: 2-44 months) elapsed between the diagnosis of MS and acute leukemia. In all, 10 patients received intensive treatment. A total of seven patients (70%) achieved MS complete remission (CR). Patients who presented isolated skin localization and received only radiotherapy, obtained a MS-CR, but subsequently developed AML. Only in patients who were treated within 4 months from the initial ML diagnosis we achieved complete remission of both MS and leukemia, whereas in patients who were treated after this time, we obtained a complete disappearance of MS without response at the bone-marrow level, irrespectively of the specific therapy regimen. Median survival time from MS diagnosis was 7 months (range: 1-49 months), and only one patient is still alive, 49 months after bone marrow transplantation. Our data stress the importance of an accurate and prompt identification of this rare form of AML, and suggest that, even in patients with isolated MS, the early administration of AML-like intensive chemotherapy followed by bone marrow transplantation might reduce the risk of subsequently developing systemic disease.

Adolescent↗