CASE REPORT
Evan J. Cretney
1
&
Erik A. Ranheim
2
&
Gregory K. Hartig
1,3
Received: 3 December 2017 / Accepted: 1 March 2018 / Published online: 27 March 2018
#
Springer-Verlag GmbH Germany, part of Springer Nature 2018
Abstract
Myeloid sarcoma is an uncommon extramedullary manifestation of myeloproliferative malignancies, such as acute myeloid
leukemia (AML). This sarcoma most often involves structures such as subperiosteal bone, peritoneum, or pericardium, and has
only rarely been reported in the nose and paranasal sinuses. The signs and symptoms of myeloid sarcoma can overlap with those
of acute invasive fungal sinusitis (AIFS), a life-threatening surgical emergency. This report describes a 75-year-old male with a
known history of AML who developed both AIFS and myeloid sarcoma. Several months after successful treatment for biopsy
proven AIFS, this patient developed a new soft tissue mass of the nasal septum. A new biopsy was obtained which revealed
infiltrating blast cells with evidence of monocytic differentiation diagnostic of myeloid sarcoma. Given symptoms of severe nasal
obstruction, this patient received palliative radiotherapy to the nasal septum, which provided temporary improvement prior to the
patient succumbing to his underlying malignancy. We review the existing reports of mucosal myeloid sarcomas, diagnostic
overlap of myeloid sarcoma and AIFS, as well as currently available treatment strategies.
Keywords Myeloid sarcoma
.
Sinonasal mucosa
.
Acute myeloid leukemia
.
Radiotherapy
.
Chloroma
.
Granulocytic sarcoma
Introduction
Myeloid sarcoma and AIFS are both complications seen in
acute myeloid leukemia. However, myeloid sarcoma of the
sinonasal tissues is rare and acute invasive fungal sinusitis is
very uncommon as well. The presence of both entities in the
same patient is exceedingly rare and highlights their overlap-
ping presentations despite very different pathophysiologies.
Acute myeloid leukemia (AML) arises from a clonal pro-
liferation of immature cells of myeloid lineage (granulocytic,
monocytic, erythroid, and megakaryocytic cell lines) [1].
These malignant cells accumulate in the blood, bone marrow,
and other tissues, resulting in variably decreased production of
normal mature granulocytes, red blood cells, and/or platelets.
Decreased production of normal blood cells combined with
proliferation of abnormal blast cells leads to clinically hetero-
geneous levels of anemia, thrombocytopenia with associated
bleeding, and neutropenia with associated increased risk of
infection. In addition, malignant myeloid blasts can invade
extramedullary tissues to form tumor-like masses, which are
variably referred to as myeloid sarcoma, chloroma, granulo-
cytic sarcoma, and leukemia cutis. While primarily a manifes-
tation of existing AML, myeloid sarcoma has also been de-
scribed in aleukemic patients, patients with chronic
myelomonocytic leukemia (CMML), chronic myeloid leuke-
mia (CML), particularly during a blast crisis, and in
myelodysplastic syndromes [2]. Myeloid sarcoma is a rela-
tively common entity; however, manifestations of this process
involving the mucosal or submucosal surfaces of the head and
neck are exceedingly rare, with only a few cases reported in
the literature [3–11].
Initial therapy for AML typically involves induction ther-
apy with cytarabine and an anthracycline followed by consol-
idation therapy using either hematopoietic stem cell transplan-
tation or continuation of intensive chemotherapy with
cytarabine [1]. A common adverse effect of the induction
* Gregory K. Hartig
hartig@surgery.wisc.edu
1
Department of Surgery, Division of Otolaryngology, Head and Neck
Surgery, University of Wisconsin School of Medicine and Public
Health, 750 Highland Avenue, Madison, WI 53726, USA
2
Department of Pathology and Laboratory Medicine, University of
Wisconsin School of Medicine and Public Health, 750 Highland
Avenue, Madison, WI 53726, USA
3
K4/7 Clinical Science Center, 600 Highland Avenue,
Madison, WI 53792-7375, USA
Journal of Hematopathology (2018) 11:51–56
https://doi.org/10.1007/s12308-018-0319-1
Myeloid sarcoma of the nasal septum following invasive fungal
sinusitis: report of a case and review of the relevant literature
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