Baylor University Medical Center Proceedings April 2017 - 196

involvement to airway obstruction
from a mediastinal mass (6, 7). Due
to the broad range of symptoms that
can be caused by primary myeloid
sarcoma, a high index of suspicion is
necessary to avoid missing or delaying
this diagnosis.
These lesions often are initially diagnosed or worked up and treated as
a lymphoma. In our case, the patient
initially was treated with high-dose
steroids for presumed lymphoma
while the tissue was being examined
by pathology. The ﬁrst suspicion that
this might be a myeloid sarcoma came
Figure 2. (a) Hematoxylin and eosin stain showing cells with prominent nucleoli. (b) Myeloperoxidase immunohistochemical when the initial round of immunostain showing positive-staining cells.
histochemical stains, which were
designed to identify a high-grade lymphoma,
failed
to
establish
that
diagnosis. On the day of marrow
elucidate the origin of the tumor cells, and myeloperoxidase, TdT,
biopsy, the clinical team visited pathology to discuss the failure of
CD15, and CD33 were positive (Figure 2). With this immunostaining proﬁle, the tumor was diagnosed as a myeloid sarcoma.
the patient to respond to high-dose steroids. Further immunohisThe patient then had a bone marrow biopsy that showed 25%
tochemical stains for myeloid sarcoma were ordered and the correct
cellularity with trilineage hematopoiesis and only 1% myeloblasts
diagnosis was established. It is important to distinguish between a
by morphology, 1% to 2% by CD34 immunohistochemistry,
large-cell lymphoma and a myeloid sarcoma for treatment purposes.
and no evidence of a high-grade hematopoietic neoplasm by ﬂow
Acute myeloid sarcomas are generally treated in the same
cytometry. Mild megakaryocytic dyspoiesis was identiﬁed. A myemanner as acute myeloid leukemia. Overall prognosis is extremelodysplastic syndrome ﬂuorescence in situ hybridization study
ly poor, with a median survival of 9.5 months; this includes both
on the patient's bone marrow came back positive for deletions of
primary and secondary myeloid sarcomas (8). One older series
chromosomes 5q and 20q in 74% and 22% of the cells examined,
of 90 patients with primary myeloid sarcoma showed a median
survival of 22 months (9). Due to the numerous locations in
respectively, which is suﬃcient in the context of refractory cytowhich myeloid sarcoma can present, it is likely that the prognosis
penia for a presumptive diagnosis of myelodysplastic syndrome,
is largely dependent on the location and symptoms.
even in the absence of overt morphologic evidence.
The patient remained intubated without any clinical im1. Dores GM, Devesa SS, Curtis RE, Linet MS, Morton LM. Acute leukemia
provement following high-dose corticosteroid therapy. Due to
incidence and patient survival among children and adults in the United
the patient's multiple comorbidities and age, chemotherapy was
States, 2001-2007. Blood 2012;119(1):34-43.
considered to be detrimental to the patient. By request of the
2. Yamauchi K, Yasuda M. Comparison in treatments of nonleukemic
family, he was transferred to the local Veteran's Administration hosgranulocytic sarcoma: report of two cases and a review of 72 cases in the
pital, intubated, and sedated without plans for further treatment.
literature. Cancer 2002;94(6):1739-1746.
a

DISCUSSION
This patient developed a myeloid sarcoma likely arising in a
lymph node or the soft tissue of the mediastinum leading to immediately life-threatening airway obstruction. Myeloid sarcoma is an
extremely aggressive extramedullary manifestation of acute myeloid
leukemia. Primary myeloid sarcoma, deﬁned as myeloid sarcoma
without evidence of acute myeloid leukemia in the bone marrow, is
rare (3). It is generally considered a precursor lesion for acute myeloid
leukemia; one series prior to the advent of eﬀective chemotherapy
showed 29 of 30 cases progressing to overt acute myeloid leukemia
in a median time of 7 months (4). Granulocytic sarcomas have been
described in myelodysplastic and myeloproliferative disorders (5).
Previous case reports of myeloid sarcomas described compression-related symptoms as the initial presentation. The symptoms
can vary dramatically based on the site of presentation, ranging
from back pain and bilateral leg weakness from spinal cord compression to intestinal obstruction and appendicitis from mesenteric
196

8.
9.

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Baylor University Medical Center Proceedings

Volume 30, Number 2

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