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Differential Diagnosis of Isolated Myeloid Sarcoma: A Case Report and Review of the Literature.

Hagen PA, Singh C, Hart M, Blaes AH - Hematol Rep (2015)

Bottom Line: Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics.Once a diagnosis of MS has been made, moving quickly to induction therapy is important.Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology/Transplantation, Department of Medicine , MN, USA.

ABSTRACT
Myeloid sarcoma (MS) is a rare disease entity identified as a variety of manifestations defined by the occurrence of extramedullary myeloid cell masses with or without bone marrow involvement. This case describes an unusual presentation of isolated MS in a 60-year-old otherwise healthy male, who initially presented to his primary care physician with vague abdominal pain. After extensive workup including three omental biopsies, umbilical core biopsy, and inguinal lymph node biopsy, he was ultimately diagnosed with isolated MS with extensive extramedullary tumor burden. Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics. The patient underwent induction chemotherapy and is now greater than 100 days post myeloablative unrelated donor marrow transplantation with no evidence of disease recurrence and 100% donor status with full chimerism. This case demonstrates that making a prompt diagnosis with rapid initiation of treatment in myeloid sarcoma can be challenging due to its varied clinical presentation, cytomorphology, cytochemistry, and cytogenetic overlap with other lymphoid malignancies. Once a diagnosis of MS has been made, moving quickly to induction therapy is important. Several studies have shown that improved overall survival is attained when MS is treated as acute myeloid leukemia and increased survival is noted for patients undergoing bone marrow transplantation. Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease.

No MeSH data available.


Related in: MedlinePlus

Representative images of omental tissue sections stained for CD43 (left panel), CD68 (middle panel), and high power Hematoxylin & Eosin showing tumor infiltrating fat (right panel).
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fig001: Representative images of omental tissue sections stained for CD43 (left panel), CD68 (middle panel), and high power Hematoxylin & Eosin showing tumor infiltrating fat (right panel).

Mentions: Aleukemic leukemia with myelocytic sarcoma was suspected during outpatient oncology follow-up. Bilateral bone marrow biopsies of the iliac spine showed normal cellular lineages, iron findings, morphology, and cytogenetics. To obtain additional tissue for definitive immunophenotyping and cytogenetics, a minimally invasive umbilical core biopsy of the hardened umbilicus and a fine needle aspiration of an enlarged inguinal lymph node noted on previous CT imaging were performed. The umbilical core biopsy stained positive for CD68-KP1, CD117, and MPO and negative for CD34 and CD56; this immunophenotypic profile is consistent with MS. However, due to extensive crush artifact, additional cytogenetics and immunophenotyping were unable to be performed. The inguinal lymph node biopsy was unremarkable. A positron emission tomography CT (PET-CT) scan showed advanced metastatic disease throughout the chest, abdomen, and pelvis with multiple hypermetabolic lymph nodes. Advanced pleural/omental caking was observed along with a 7.7×6.1 cm mass at the root of the mesentery invading the adjacent small intestine. Upon re-admission for planned induction chemotherapy, a final laparoscopic omental biopsy was performed with tissue adequate to perform the necessary studies needed to make a definitive diagnosis of lymphoma versus MS. Myeloid cell markers including CD99, CD117, CD68-KP1, CD43, MPO, and lysozyme were evident from the omental and umbilical biopsies; however, T and B cell markers were absent. These findings differentiated MS from B and T cell lymphomas and poorly differentiated carcinoma. Representative stains of important myeloid markers as well as the aggressive nature of this myeloproliferative disease with tumor infiltrating fat are shown in Figure 1. Molecular abnormities frequently encountered in AML including FLT3-ITD and NPM1-mutant were found. On the basis of these observations, a definitive diagnosis was made of MS 46XY, FLT3-ITD and NPM1-mutant involving the peritoneum and pleural cavity. The patient underwent 7+3 induction chemotherapy with idarubicin and cytarabine followed by two cycles of consolidation with intermediate-dose cytarabine. The patient then received myeloablative unrelated donor bone marrow transplantation. Follow-up PET-CT scan and bone marrow biopsy at 100 days post-transplantation showed no evidence of disease recurrence and 100% donor status with full chimerism.


Differential Diagnosis of Isolated Myeloid Sarcoma: A Case Report and Review of the Literature.

Hagen PA, Singh C, Hart M, Blaes AH - Hematol Rep (2015)

Representative images of omental tissue sections stained for CD43 (left panel), CD68 (middle panel), and high power Hematoxylin & Eosin showing tumor infiltrating fat (right panel).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508548&req=5

fig001: Representative images of omental tissue sections stained for CD43 (left panel), CD68 (middle panel), and high power Hematoxylin & Eosin showing tumor infiltrating fat (right panel).
Mentions: Aleukemic leukemia with myelocytic sarcoma was suspected during outpatient oncology follow-up. Bilateral bone marrow biopsies of the iliac spine showed normal cellular lineages, iron findings, morphology, and cytogenetics. To obtain additional tissue for definitive immunophenotyping and cytogenetics, a minimally invasive umbilical core biopsy of the hardened umbilicus and a fine needle aspiration of an enlarged inguinal lymph node noted on previous CT imaging were performed. The umbilical core biopsy stained positive for CD68-KP1, CD117, and MPO and negative for CD34 and CD56; this immunophenotypic profile is consistent with MS. However, due to extensive crush artifact, additional cytogenetics and immunophenotyping were unable to be performed. The inguinal lymph node biopsy was unremarkable. A positron emission tomography CT (PET-CT) scan showed advanced metastatic disease throughout the chest, abdomen, and pelvis with multiple hypermetabolic lymph nodes. Advanced pleural/omental caking was observed along with a 7.7×6.1 cm mass at the root of the mesentery invading the adjacent small intestine. Upon re-admission for planned induction chemotherapy, a final laparoscopic omental biopsy was performed with tissue adequate to perform the necessary studies needed to make a definitive diagnosis of lymphoma versus MS. Myeloid cell markers including CD99, CD117, CD68-KP1, CD43, MPO, and lysozyme were evident from the omental and umbilical biopsies; however, T and B cell markers were absent. These findings differentiated MS from B and T cell lymphomas and poorly differentiated carcinoma. Representative stains of important myeloid markers as well as the aggressive nature of this myeloproliferative disease with tumor infiltrating fat are shown in Figure 1. Molecular abnormities frequently encountered in AML including FLT3-ITD and NPM1-mutant were found. On the basis of these observations, a definitive diagnosis was made of MS 46XY, FLT3-ITD and NPM1-mutant involving the peritoneum and pleural cavity. The patient underwent 7+3 induction chemotherapy with idarubicin and cytarabine followed by two cycles of consolidation with intermediate-dose cytarabine. The patient then received myeloablative unrelated donor bone marrow transplantation. Follow-up PET-CT scan and bone marrow biopsy at 100 days post-transplantation showed no evidence of disease recurrence and 100% donor status with full chimerism.

Bottom Line: Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics.Once a diagnosis of MS has been made, moving quickly to induction therapy is important.Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology/Transplantation, Department of Medicine , MN, USA.

ABSTRACT
Myeloid sarcoma (MS) is a rare disease entity identified as a variety of manifestations defined by the occurrence of extramedullary myeloid cell masses with or without bone marrow involvement. This case describes an unusual presentation of isolated MS in a 60-year-old otherwise healthy male, who initially presented to his primary care physician with vague abdominal pain. After extensive workup including three omental biopsies, umbilical core biopsy, and inguinal lymph node biopsy, he was ultimately diagnosed with isolated MS with extensive extramedullary tumor burden. Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics. The patient underwent induction chemotherapy and is now greater than 100 days post myeloablative unrelated donor marrow transplantation with no evidence of disease recurrence and 100% donor status with full chimerism. This case demonstrates that making a prompt diagnosis with rapid initiation of treatment in myeloid sarcoma can be challenging due to its varied clinical presentation, cytomorphology, cytochemistry, and cytogenetic overlap with other lymphoid malignancies. Once a diagnosis of MS has been made, moving quickly to induction therapy is important. Several studies have shown that improved overall survival is attained when MS is treated as acute myeloid leukemia and increased survival is noted for patients undergoing bone marrow transplantation. Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease.

No MeSH data available.


Related in: MedlinePlus