Spinal Myeloid Sarcoma Presenting as Paraplegia: A Case Report
Maryum Nouman*, Mehwish Shaikh, Ghulam Haider, and Khalil Meher
Department of Clinical Oncology, Jinnah Postgraduate Medical Center, Pakistan
Maryum Nouman, Department of Clinical
Oncology, Jinnah Postgraduate Medical Center, Karachi, Pakistan, Tel: +92-332-3393166; Email: firstname.lastname@example.org
Submitted: 19 January 2019; Accepted: 19 April 2019; Published: 22 April 2019
Cite this article:
Nouman M, Shaikh M, Haider G, Meher K (2019) Published:
6-Dec-18Spinal Myeloid Sarcoma Presenting as Paraplegia: A Case Report. JSMC Clin Oncol Res 3: 3.
Objective: Myeloid sarcoma comprising of immature granulocytic cells is a rare, extra medullary tumor. Spinal cord compression
caused by a myeloid sarcoma is even more so. This study reports a case of spinal myeloid sarcoma presenting with paraplegia.
Patient and method: 18-year-old male presented with progressively worsening back pain leading to paraplegia. Magnetic resonance
imaging (MRI) dorsal spine revealed a soft tissue mass extending from D4 through D8 causing compression of cord in mid dorsal region.
The patient underwent surgical decompression. Histology confirmed the diagnosis of myeloid sarcoma. Post operatively bone marrow
biopsy showed no evidence of myeloproliferative neoplasm. The patient then received systemic chemotherapy in line of Acute Myeloid
Result: After 9 months patient reports complete relief of symptoms. Post treatment MRI shows no evidence of compressive or residual disease.
Conclusion: Spinal myeloid sarcoma presenting as paraplegia is very rare. We suggest that clinical suspicion can lead to early
diagnosis and appropriate management.
Keywords: Myeloid sarcoma; Myeloid leukemia; Spinal tumor; Paraplegia
Myeloid sarcoma or chloroma comprising of immature
granulocytic cells is a rare, extramedullary tumor. It may be seen
in the setting of acute or chronic leukemia or myeloproliferative
disorders but seldom reported in otherwise healthy patients
without any evidence of systemic disease . Spinal epidural
myeloid sarcoma is uncommon, and spinal cord compression
caused by a myeloid sarcoma is even more so . This study reports
a rare case of spinal myeloid sarcoma presenting as paraplegia. It
was treated successfully with surgical decompression of spinal
cord followed by chemotherapy.
An 18-year-old male presented to us with progressively
worsening back pain for the past 18 months. It was dull and
continuous, not radiating. There were no predisposing factors
like trauma. Initially he consulted a local medical practitioner
and received systemic and local analgesics along with muscle
relaxants. In spite of these measures, his pain continued to
increase over the time and eventually led to paraplegia.
Following this, the patient consulted a neurosurgeon who
referred him for imaging of the spine.
MRI dorsal spine with contrast revealed large enhancing
extradural soft tissue mass extending from D4 through D8 with
intra the cal extension causing compression of cord in mid dorsal
region. It was predominantly on the right side with extension
in the right paraspinal muscles with cranio-caudal extension of
about 10.2cm (Figure 1).
Keeping in view the worsening of symptoms and paraplegia, he
underwent excisional biopsy of dorsal spinal mass, laminectomy
Following surgical decompression, he underwent radiation
therapy of the spinal cord for cord compression; total dose of
Histopathology revealed a neoplastic lesion comprising
of intermediate to large sized cells arranged in sheets with background showing abundant eosinophil infiltrate. These cells
stained positive for LCA, MPO, CD 68, CD 117 with increased Ki-67
proliferative index. It was negative for Pan B (CD 20/ 79a), Pan T
(CD 3), Tdt, CD 30, CD 138 and PAX 5. These immunohistochemical
findings were consistent with the diagnosis of myeloid sarcoma.
The patient was referred to us in Medical Oncology for further
management. We carried out thorough history and physical
examination. Karyotyping showed normal cytogenetics. We did
bone marrow biopsy to rule out myeloid sarcoma associated with
Acute Myeloid Leukemia (AML). It showed no evidence of bone
marrow involvement with myeloproliferative disorder.
The patient underwent “3+7” induction chemotherapy
with daunorubicinand cytarabine followed by four cycles of
consolidation with high dose cytarabine.
Post consolidation MRI of the dorsal spine with contrast
revealed complete response to treatment with no residual disease.
It shows post-surgical changes with evidence of laminectomy
from D2 to D8 (Figure 2). The patient reports complete resolution
of symptoms with ability to walk and perform daily activities. He
is disease and symptom free on follow up of one year.
A myeloid sarcoma also known as chloroma or granulocytic
sarcoma is a rare, extra medullary myeloid tumor. It is a
solid tumor composed of immature granulocytes . It is a rare
hematologic phenomenon in patients with AML with an incidence
as low as 2.5 to 9.1% [1, 4]. It may occur during the course or just
preceding or following leukemia [1,4].
Myeloid sarcoma has a predilection for males (male-tofemale
ratio, 1.2:1) [5,6].
Sites commonly involved include the skin, lymph nodes,
gastrointestinal tract, bone, soft tissue, and testis. [7,8] However,
spinal cord involvement is even less than 1 percent .
Spinal myeloid sarcoma may present with varied symptoms depending upon the site. Those with involvement of para-spinal
muscles may lead to spinal cord compression due to mass
effect . In the case presented here, patient initially reported
progressively increasing back pain that eventually led to
Spinal myeloid sarcoma is often challenging to diagnose
because the clinical data and imaging is usually nonspecific. It
often appears as a soft tissue mass on computed tomography
or MRI that does not point to a specific diagnosis [2,9]. Hence,
it is crucial to obtain a tissue biopsy to formulate a diagnosis of
myeloid sarcoma. Following this, immunohistochemistry and a
series of markers is a practical guide that aids in the diagnosis
and further characterization of the tumor .
In this case, the patient underwent excisional biopsy of the
dorsal spinal mass, laminectomy, and cord decompression.
Immunohistochemical stains showed positive staining with LCA,
MPO, CD 68, CD 117 with increased Ki-67 proliferative index
which helped to confirm the diagnosis as these are the most
commonly expressed markers of myeloid differentiation .
Given the rare incidence of spinal myeloid sarcoma, the clinical
suspicion of the disease should be kept high so that the treatment
can start at optimal timing. Possible treatment strategies include
chemotherapy, hematopoietic stem cell transplantation (HSCT),
radiotherapy and surgery. Literature reveals large randomized
studies, which assessed the prognosis of isolated myeloid
sarcoma. Systemic chemotherapy used in the treatment of AML
remains the best strategy for treating this set of patients, but
the optimal timing remains controversial [13,15-17]. Surgery is
however limited to the cases with symptomatic compression due
to the tumor . We reviewed cases of spinal myeloid sarcoma
presenting with cord compression from the literature (Table 1),
[18-26]. Surgical excision of the tumor appeared to be the first
treatment option in these cases.
In our case, patient underwent surgery for decompression
followed by systemic chemotherapy that led to complete
Inoue et al identified 26 patients of myeloid sarcoma from
the literature who had no evidence of myeloid leukemia, and it
was found that the treatment strategy varied from individual to
individual . Surgical decompression was performed in 22
patients, and 21 of the patients received additional AML-type
systemic treatment. However, the outcome of the patients varied
and was poor in those who showed progression to leukemia.
Overall, prompt diagnosis and adequate treatment are critical to
achieve a better outcome.
Spinal myeloid sarcoma presenting as paraplegia is very
rare. Clinical suspicion of the disease can lead to early diagnosis
and appropriate management. Keeping in view our patient
and previous cases from literature, we suggest that prompt
diagnosis followed by emergency decompression and intensive
Figure 2 Showing laminectomy with complete resolution of mass. chemotherapy in the line of management of AML can lead to complete recovery objectively and clinically and can prevent