Abstract:Failure to achieve CR with induction therapy, and age >65 years are associated with poor outcomes in MS. Allogeneic stem-cell transplant in first remission appears to be the most effective modality for achieving long-term remissions.
“…Kaur et al . studied 23 patients and found a significantly worse prognosis in patients older than 65 years . Interestingly, younger patients had a higher likelihood of having a ‘better prognosis’ tumour location, as previously discussed.…”
Section: Discussionsupporting
confidence: 59%
“…Kaur et al studied 23 patients and found a significantly worse prognosis in patients older than 65 years. 22 Interestingly, younger patients had a higher likelihood of having a 'better prognosis' tumour location, as previously discussed. Other studies failed to established a correlation between patient age and clinical outcomes in MS 6,23 (Table 2).…”
Aims
Myeloid sarcoma (MS) is a rare extramedullary neoplasm composed of immature myeloid precursor cells thought to be a unique clinical presentation of acute myeloid leukaemia (AML). Like AML, MS has a poor prognosis, but due to the rare nature of MS there are limited studies examining potential prognostic factors. We report our institutional experience, with the aim of investigating and establishing salient clinicopathological and molecular features of MS.
Methods and results
We retrospectively examined all clinicopathological and molecular data on MS patients between 2001 and 2017 from the University of Alabama at Birmingham (UAB) electronic medical records. The UAB electronic medical records were also reviewed and compared with the literature for other potential prognostic factors. Sixty‐three patients were included in the study. The median overall survival was 24 months in the group with normal karyotype and 12 months in patients with an abnormal karyotype.
Conclusions
We found that an abnormal karyotype was associated with a statistically significant worse prognosis.
“…Kaur et al . studied 23 patients and found a significantly worse prognosis in patients older than 65 years . Interestingly, younger patients had a higher likelihood of having a ‘better prognosis’ tumour location, as previously discussed.…”
Section: Discussionsupporting
confidence: 59%
“…Kaur et al studied 23 patients and found a significantly worse prognosis in patients older than 65 years. 22 Interestingly, younger patients had a higher likelihood of having a 'better prognosis' tumour location, as previously discussed. Other studies failed to established a correlation between patient age and clinical outcomes in MS 6,23 (Table 2).…”
Aims
Myeloid sarcoma (MS) is a rare extramedullary neoplasm composed of immature myeloid precursor cells thought to be a unique clinical presentation of acute myeloid leukaemia (AML). Like AML, MS has a poor prognosis, but due to the rare nature of MS there are limited studies examining potential prognostic factors. We report our institutional experience, with the aim of investigating and establishing salient clinicopathological and molecular features of MS.
Methods and results
We retrospectively examined all clinicopathological and molecular data on MS patients between 2001 and 2017 from the University of Alabama at Birmingham (UAB) electronic medical records. The UAB electronic medical records were also reviewed and compared with the literature for other potential prognostic factors. Sixty‐three patients were included in the study. The median overall survival was 24 months in the group with normal karyotype and 12 months in patients with an abnormal karyotype.
Conclusions
We found that an abnormal karyotype was associated with a statistically significant worse prognosis.
“…The neoplastic cells in MS often express precursor markers CD34 and/or CD117 together with granulocytic or monocytic markers such as MPO, CD13, CD33, CD68, and lysozyme. Different studies reported variable expression frequencies of MS for MPO (63–92%), CD13 (48%), CD33 (48–52%), CD68 (35–61%), and lysozyme (26–100%) [2, 5–7]. MS with monocytic differentiation can be particularly challenging to differentiate from histocytic sarcoma (HS) as both can express one or more monocytic antigens, such as CD68, CD163 and lysozyme.…”
Backgroud
Myeloid sarcoma (MS) is a rare neoplasm of immature myeloid precursors that form tumor mass outside the bone marrow. The diagnosis of de novo MS can be challenging, particularly in patients with no prior history of hematologic malignancies or when MS involves unusual anatomic sites.
Case presentation
The patient was a 53-year-old woman with a history of uterine fibroids and vaginal bleeding for many years who presented with a vaginal wall mass. The tumor had histologic and phenotypic features of histiocytic sarcoma, however, overlapping with a possible extramedullary MS. Using a comprehensive genomic profiling, we were able to identify recurrent chromosomal aberrations associated with MS including a rare
KMT2A
-
ELL
fusion, losses of chromosomes 1p, 9, 10, 15, 18, and gain of chromosome 1q and mutations in
FLT3
and
PTPN11
, and achived the final diagnosis of a de novo MS. The patient received standard treatment for acute myeloid leukemia regimen with stem cell transplantation and achieved complete remission.
Conclusion
Our case illustrates the clinical utility of comprehensive genomic profiling in assisting the diagnosis or differential diagnosis of challenging MS or histiocytic sarcoma cases, and in providing important information in tumor biology for appropriate clinical management.
“…Especially, after allogenic bone marrow transplantation an increased incidence has been described 5. Very rarely, MS can occur in the absence of a systemic disease and predate onset of an underlying hematologic malignancy by months to years 6. Treatment options, including radiation and chemotherapy are based upon an early and accurate diagnosis is crucial 7.…”
Background
Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a solid tumor of extramedullary localization composed of malignant primitive myeloid cells. The purpose of the study was to identify clinical and imaging features in a large patient sample.
Patients and methods
Overall, 71 cases (34 females (47.9%) and 37 males (52.1%) with a median age of 56 (± 16 years) of histopathologically confirmed myeloid sarcoma were included into this study. The underlying hematological disease, occurrence, localizations and clinical symptoms as well as imaging features on computed tomography and magnetic resonance imaging were investigated.
Results
In 4 cases (5.63%) the manifestation of MS preceded the systemic hematological disease by a mean value of 3.8 ± 2.1 months. In 13 cases, first presentation of MS occurred simultaneously with the initial diagnosis of leukemia, and 51 patients presented MS after the initial diagnosis of the underlying malignancy with a mean latency of 39.8 ± 44.9 SD months. The visceral soft tissue was affected in 26 cases, followed by the cutis/subcutis was affected in 21 cases. Further localizations were bones (n = 13), central nervous system (n = 9), lymph nodes (n = 4) and visceral organs (n = 9).
Conclusions
MS is a rare complication of several hematological malignancies, predominantly of acute myeloid leukemia, which can affect any part of the body. In most cases it occurs after the diagnosis of the underlying malignancy, and affects frequently the cutis and subcutis.
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