Project Title

RENAL AND TESTICULAR MYELOID SARCOMA DEFINES ACUTE MYELOID LEUKEMIA EVEN IN THE ABSENCE OF BLOOD AND MARROW INVOLVEMENT

Authors' Affiliations

East Tennessee State University

Location

WhiteTop Mountain Room 225

Start Date

4-5-2018 8:00 AM

End Date

4-5-2018 12:00 PM

Poster Number

122

Name of Project's Faculty Sponsor

Devapiran Jaishankar MD

Faculty Sponsor's Department

Oncology (Internal Medicine)

Type

Poster: Competitive

Classification of First Author

Medical Resident or Clinical Fellow

Project's Category

Biomedical and Health Sciences

Abstract Text

Acute Myeloid Leukemia (AML) is an aggressive hematopoietic neoplasm characterized by rapid clinical progression and universally fatal outcome if left untreated. The neoplastic cells are immature precursor cells that usually originate in the bone marrow and are often noted in the peripheral circulation. Myeloid sarcomas are rare abnormal collection of these cells in extramedullary sites and are now defined as a unique subset of AML as per the WHO classification. Here we describe a patient with AML presenting with a renal and testicular mass, which on biopsy revealed myeloid sarcoma in the absence of blood and bone marrow involvement. A 71 year old male was hospitalized with complaints of nausea, vomiting, confusion and jaundice associated with worsening pruritus in the absence of constitutional symptoms. Medical history notable for chronic kidney disease, Type 2 Diabetes Mellitus and history of localized prostate cancer treated with TURP. Family history of thyroid cancer in his mother. Clinical exam revealed small right testicular non tender mass. Labs revealed a total bilirubin of 11.5mg/dL, creatinine of 3.09 mg/dL, Hemoglobin 13.4g/dL, WBC 9.5k/uL and Platelet count of 283K/uL. Abdominal imaging studies demonstrated a biliary stricture, a 16mm right testicular lesion and a additional right renal mass measuring 4.0 x 3.9 x 2.4 cm. He underwent a right inguinal orchiectomy, which confirmed myeloid sarcoma. Histopathologically, numerous neoplastic cells with enlarged nuclei and mitotic figures were identified with immunohistochemical staining positive for CD45, MPO, CD117, CD68, CD71 and BCL2. A biopsy of the right renal mass also demonstrated myeloid sarcoma, with similar features. He had an ERCP with stent placement for his biliary stricture with negative cytology. Bone marrow biopsy was negative for AML. His challenging clinical presentation, advanced age, medical comorbidities, hyperbilirubinemia, and impaired kidney function, precluded aggressive treatment with standard acute myeloid leukemia induction chemotherapy regimens so a trial of hypomethylating agent (Azacitidine) was successfully initiated. AML is characterized by a rapid clonal proliferation of immature hematopoietic cells in the peripheral blood and bone marrow. It is a heterogeneous disease with regard to acquired genetic alterations, including cytogenetic aberrancies as well as gene mutations and changes in gene expression. The signs and symptoms of AML mostly reflect predominant cytopenias or cytoses and usually a short history (1 to 8 weeks) of constitutional complaints. Myeloid sarcomas are rare extramedullary solid tumors that have resulted in infiltration of organs such as gingiva, skin, lymph nodes and other organs with immature granulocytic precursor cells. The majority of cases are reported in association with coexisting acute myeloid leukemia and infrequently can present in isolation (a harbinger for future blood and marrow involvement). Treatment of myeloid sarcomas follows the AML paradigm. The overall survival of AML is dictated by cytogenetics/ molecular markers and age. Complete remission can be achieved in a group of patients. Relapses occur in the first two years. Leukemic infiltration of the kidney or testicle with myeloid sarcoma is extremely rare and the concurrent presentation is documented in isolated case reports only.

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Apr 5th, 8:00 AM Apr 5th, 12:00 PM

RENAL AND TESTICULAR MYELOID SARCOMA DEFINES ACUTE MYELOID LEUKEMIA EVEN IN THE ABSENCE OF BLOOD AND MARROW INVOLVEMENT

WhiteTop Mountain Room 225

Acute Myeloid Leukemia (AML) is an aggressive hematopoietic neoplasm characterized by rapid clinical progression and universally fatal outcome if left untreated. The neoplastic cells are immature precursor cells that usually originate in the bone marrow and are often noted in the peripheral circulation. Myeloid sarcomas are rare abnormal collection of these cells in extramedullary sites and are now defined as a unique subset of AML as per the WHO classification. Here we describe a patient with AML presenting with a renal and testicular mass, which on biopsy revealed myeloid sarcoma in the absence of blood and bone marrow involvement. A 71 year old male was hospitalized with complaints of nausea, vomiting, confusion and jaundice associated with worsening pruritus in the absence of constitutional symptoms. Medical history notable for chronic kidney disease, Type 2 Diabetes Mellitus and history of localized prostate cancer treated with TURP. Family history of thyroid cancer in his mother. Clinical exam revealed small right testicular non tender mass. Labs revealed a total bilirubin of 11.5mg/dL, creatinine of 3.09 mg/dL, Hemoglobin 13.4g/dL, WBC 9.5k/uL and Platelet count of 283K/uL. Abdominal imaging studies demonstrated a biliary stricture, a 16mm right testicular lesion and a additional right renal mass measuring 4.0 x 3.9 x 2.4 cm. He underwent a right inguinal orchiectomy, which confirmed myeloid sarcoma. Histopathologically, numerous neoplastic cells with enlarged nuclei and mitotic figures were identified with immunohistochemical staining positive for CD45, MPO, CD117, CD68, CD71 and BCL2. A biopsy of the right renal mass also demonstrated myeloid sarcoma, with similar features. He had an ERCP with stent placement for his biliary stricture with negative cytology. Bone marrow biopsy was negative for AML. His challenging clinical presentation, advanced age, medical comorbidities, hyperbilirubinemia, and impaired kidney function, precluded aggressive treatment with standard acute myeloid leukemia induction chemotherapy regimens so a trial of hypomethylating agent (Azacitidine) was successfully initiated. AML is characterized by a rapid clonal proliferation of immature hematopoietic cells in the peripheral blood and bone marrow. It is a heterogeneous disease with regard to acquired genetic alterations, including cytogenetic aberrancies as well as gene mutations and changes in gene expression. The signs and symptoms of AML mostly reflect predominant cytopenias or cytoses and usually a short history (1 to 8 weeks) of constitutional complaints. Myeloid sarcomas are rare extramedullary solid tumors that have resulted in infiltration of organs such as gingiva, skin, lymph nodes and other organs with immature granulocytic precursor cells. The majority of cases are reported in association with coexisting acute myeloid leukemia and infrequently can present in isolation (a harbinger for future blood and marrow involvement). Treatment of myeloid sarcomas follows the AML paradigm. The overall survival of AML is dictated by cytogenetics/ molecular markers and age. Complete remission can be achieved in a group of patients. Relapses occur in the first two years. Leukemic infiltration of the kidney or testicle with myeloid sarcoma is extremely rare and the concurrent presentation is documented in isolated case reports only.