Case report:
A 19-year-old male was diagnosed with AML M2 with a normal karyotype in
2018, and underwent standard treatment with induction 7+3, re-induction
5+2, and consolidation therapy (Cytarabine 3 g/m2).
And had achieved complete remission (CR) for three years. However, in
2022, the patient began experiencing neurological symptoms, including
headaches, loss of vision, weakness in both lower limbs, and difficult
to walk. A Brain MRI revealed a massive heterogeneous mass 9× 7.5×3cm in
the left sphenoid wing region, This mass exerted pressure on critical
structures such as the carotid artery, optic nerve, and cavernous sinus.
Figure (1).
The patient underwent surgery using the frontotemporal orbit zygomatic
approach (FTOZ) which successfully identified a tumor mass pressing
against the carotid artery, median cerebrum, optic nerve, on the left
side, optic chiasm and left cavernous sinus. The patient underwent
neurosurgical surgery using the frontotemporal-orbit zygomatic approach
(FTOZ), which detected a tumor mass compressing the carotid artery,
median cerebrum, optic nerve on the left side, optic chiasm, and left
cavernous sinus. The tumor mass was removed until intact edges were
reached with orbital scraping and optic nerve severing Figure (2).
Pathological analysis of the mass confirmed the presence of a myeloid
sarcoma involving both the brain and optic nerve, with orbital
involvement. Immunohistochemistry further revealed positive staining for
LCA, MPO, and CD34, while CD20 and CD3 tests returned negative results.
Ki67 proliferative marker showed intermediate activity.
The patient received high-dose cytarabine (1.5 mg/m2) combined with
intrathecal injections (cytarabine, dexamethasone, Methotrexate), and
achieved clinical improvement and remission. Despite initial progress,
the patient died four months after treatment began due to chemotherapy
side effects and neutropenic fever.
Discussion :
Myeloid Sarcoma (MS) is classified as a subtype of Acute Myeloid
Leukemia (AML) and related neoplasms by the World Health Organization
(WHO) (6). MS is similarly classified by the European Society for
Hematology (ESH) into four separate categories: [1] MS in
conjunction with AML, [2] extramedullary relapse of AML, including
cases after bone marrow transplantation, [3] blast
phase/transformation from myeloproliferative neoplasms or Chronic
Myelomonocytic Leukemia (CMML), and [4] isolated MS, occurring
without a history of myeloid dysplasia and with normal bone marrow
aspirate findings.
The incidence of central nervous system (CNS) involvement in Myeloid
Sarcoma (MS) is relatively rare,(7) accounting for only 0.4% of cases
involving cranial bone marrow, vertebrae, or orbital bones. Its
migration to the brain parenchyma is attributed to the disruption of the
blood-brain barrier (8,9). While Orbital involvement as an initial
manifestation of AML is uncommon, it is less than 3% of cases.(10) In
our case, brain MRI revealed a heterogeneous mass measuring 9× 7.5×3cm
in the left temporal region, extending into the left sphenoid wing and
causing destruction of the pilgrims. The mass compressed the left middle
cerebral artery and bulged into the left cavernous sinus figure (1). MS
can sometimes express B-cell antigens (CD19 and CD79a), which may lead
to misdiagnosis as CNS lymphoma. However, in our case, immunostaining
confirmed AML with myeloperoxidase (MPO) positivity.
Treatment approaches for MS lack consensus due to its rarity and limited
randomized controlled trials (RCTs). Therapeutic decisions are
influenced by factors such as tumor location, the timeline of MS
occurrence (before AML onset or AML relapse), patient age, and
performance status. Chemotherapy, surgery, radiotherapy, allogeneic
hematopoietic stem cell transplantation (allo-SCT), targeted therapy,
and immunotherapy are available therapeutic options [10]. Surgery
plays a vital role in relieving mass effect symptoms, confirming
diagnosis, and debulking large-sized MS before initiating systemic
therapy [3]. In cases of isolated MS with inadequate response to
chemotherapy or when rapid relief of vital function impairment is
necessary, radiotherapy may be recommended [1,10]. In our case, we
opted for neurosurgical surgery utilizing the frontotemporal-orbit
zygomatic approach (FTOZ), followed by an induction chemotherapy
protocol involving high-dose cytarabine (1.5 g/m2). Cytarabine has a
good outcomes in MS.
The prognosis of Myeloid Sarcoma (MS) remains uncertain due to limited
available data. However, it is generally acknowledged that MS occurring
concomitantly with Acute Myeloid Leukemia (AML) or as a relapsed AML is
associated with a poor prognosis (11). Patients who received
chemotherapy showed better prognosis compared to those who did not (12).
The life expectancy of individuals with MS varies based on several
factors like age, performance status, and location of the disease, with
a reported 5-years survival rate of approximately 24% (12,13). Disease
relapse and infections are the most common causes of mortality in MS
patients, in our case the patient dead with infection after 6 months
from diagnosis MS.
In our case, the patient developed MS in multiple organs three years
after achieving complete remission (CR) from AML, and unfortunately, his
condition rapidly deteriorated within Six months of chemotherapy
protocols. This highlights the challenges associated with MS and
emphasizes the need for further research and advancements in treatment
strategies to improve patient outcomes.
Conclusion :
Myeloid sarcoma can occur in patients with AML who have been in complete
remission, and can manifest in various organs. Awareness of MS in
various organs in relapsed AML is essential, and this diagnosis demands
further individualized treatment due to the very high mortality risk.
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