2015, Volume 31, Number 2, Page(s) 131-135
Cutaneous Myeloid Sarcoma of the Penile Foreskin
Ruquiya AFROSE1, Deepa NEBHNANI2, Neelam WADHWA1
1Department of Pathology, University College of Medical Sciences, DELHI, INDIA
2Department of Uro-Pathology, Seth G S Medical College and KEM Hospital, MUMBAI, INDIA
Keywords: Myeloid leukemia, Chronic phase, Circumcision, Phimosis, Priapism
Myeloid sarcoma, considered to herald the onset of a blast crisis in the
setting of chronic myeloproliferative neoplasm/dysplasia, typically
presents during the course of the disorder. Cutaneous involvement is
uncommon and lesions on genital skin are seldom seen. We present
a case of a well-differentiated myeloid sarcoma in the penile foreskin
in an apparently healthy 29-year-old male presenting with phimosis.
The unusual composition of the inflammatory cell infiltrate, and
characteristic sparing of dermal blood vessels, nerves and smooth
muscle fibres led to the correct diagnosis. Absence of commonly
observed changes in the circumcision skin like those of balanitis
xerotica was also helpful. Detailed hematological work up revealed a
previously undiagnosed chronic myeloid leukemia in chronic phase.
The patient also had simultaneous priapism, another rare presentation
of chronic myeloid leukemia. One year hence, the patient is in
hematological remission with no evidence of extramedullary disease.
Although priapism has been described as a rare presenting symptom
in chronic myeloid leukemia, the present case is unique as this is the
first time a cutaneous myeloid sarcoma has been documented in the
Myeloid sarcoma (MS) refers to a rare solid tumor
composed of immature myeloid cells at an extramedullary
. Most cases develop in the course of a leukemia;
its presentation concurrent to or preceding leukemia is
. While its recognition is easy in the setting
of a known hematologic malignancy, up to 75% of MS
cases may be misdiagnosed2
. Cutaneous involvement is
uncommon and lesions on the genital skin are seldom seen1,2-4
. Priapism, sustained penile erection in the absence
of physiologic stimuli, indicates the disturbance of the
regulatory mechanisms responsible for penile erection and
. It is an uncommon but well-documented
complication of sickle cell disease but sparingly reported in
. We report a case of simultaneous phimosis with priapism as the first presentation of a previously
undiagnosed case of chronic myeloid leukemia (CML). The
present case is unique as a cutaneous MS was identified
on histopathological examination of the circumcision
specimen of the penile foreskin.
A 29-year-old uncircumcised male presented with the
inability to retract the prepuce and sustained painful penile
erection for the last 10 and 3 days, respectively. There was
no history of trauma, previous episode of similar erection,
flaccidity, or symptoms of urinary tract infection. The
patient did not have a history of chronic medication or
drug abuse, or heavy consumption of alcohol. There were
no systemic complaints besides a history of easy fatigability for the past few weeks. Genital examination revealed an
erect, tense and tender penis with a relatively softer glans.
The overlying skin showed no discoloration. The prepuce
could not be retracted from the glans penis. Abdominal
examination revealed an enlarged spleen and liver that were
palpable 5 and 2 cm below the left and right costal margins,
Routine biochemical and preliminary hematological
investigations including a peripheral blood smear
examination were performed. Color Doppler and
abdominal ultrasound were also performed to assess penile
blood flow and visceral organ size, respectively. The complete
blood counts revealed marked leukocytosis (total leukocyte
count 366.34 x 103/L), anemia (hemoglobin 76 gm/L) and
thrombocytosis (platelet count 622 x 103/L). Immature cells
of myeloid lineage at all stages of maturation including
myeloblasts (4%) were seen in the peripheral blood smear;
there was also basophilia (6%). The hematological features
were consistent with CML, chronic phase. The biochemical
test results including blood sugar were non contributory.
Color Doppler showed normal blood flow in the dorsal
arteries but no flow in the cavernosal arteries, consistent
with low flow/ischemic type priapism. Abdominal
sonogram confirmed splenomegaly and hepatomegaly.
Winter shunting procedure was performed under local
analgesia to provide immediate relief to the patient and to
reduce the risk of long term sequelae. Briefly, the corpora
cavernosa were allowed to evacuate by shunts created by
inserting a needle between them and corpora spongiosum.
The stagnant blood could then exit the erect penis, leading to gradual detumescence. Circumcision was performed for
the phimosis in the same setting. Analgesics and hydration
were continued for the next week.
The excised foreskin was formalin fixed, processed routinely
and 4 micron thick hematoxylin and eosin stained sections
prepared. Myeloid cells at different stages of maturation were
seen infiltrating the mid and deep dermis (Figure 1). The
maturing myeloid cells could be identified easily owing to
their nuclear lobation. The immature forms were few; they
had round to oval nuclei with scant cytoplasm. Eosinophilic
precursors had prominent cytoplasmic granules. The
dermal vessels were characteristically uninvolved by the
infiltrate and there was no evidence of vasculitis in the form
of thrombosis, fibrinoid necrosis or leukocytoclasia in the
vessel wall (Figure 2). Similarly, neither the dermal nerve
bundles nor the smooth muscle fibres were infiltrated.
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|Figure 1: Diffuse infiltration of dermis by myeloid cells and
dermal edema (H&E; x100).
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|Figure 2: Myeloid cells at various stages of maturation are
identified. Note that the blood vessels are characteristically
uninvolved by the dense infiltrate of myeloid cells (H&E; x400).
Megakaryocytes or erythroid precursors could not be
identified. Immuno-staining with the standard avidin
biotin method was performed for leukocyte common
antigen (LCA), CD34 (for blasts), CD15 (for myeloid
differentiation), CD20 and CD3 (for B and T lymphoid
lineage respectively). The reaction for LCA and CD15 was
strong and observed in almost all cells (Figure 3). This
confirmed the myeloid differentiation observed on the
hematoxylin and eosin stained sections. The results for
CD20 and CD3 were expectedly negative. CD34 expression
was occasional. There was no evidence of balanitis xerotica
obliterans (BXO) in the form of epidermal changes, dermal
hyalinization or fibrosis (Figure 4). A diagnosis of cutaneous
MS of penile foreskin was made.
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|Figure 3: CD15 decorating almost all cells confirming the myeloid
differentiation; the vascular sparing is highlighted (CD15; x200).
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|Figure 4: Essentially unremarkable epidermis; note absence of
vacuolization of the basal layer (H&E; x200).
Bone marrow aspiration, biopsy and fluorescence in situ
hybridization (FISH) were also performed subsequently.
The bone marrow was markedly hypercellular with
reduction of fat spaces. There was preponderance of myeloid
precursors and maturing forms outnumbered the blasts.
The erythroid precursors were scanty and megakaryocytes
numerous including abnormal forms. Using the LSI bcr/
abl dual color dual fusion probe (Vysis) for interphase
FISH, 83.5% (167/200) of the nuclei showed a fusion signal
indicative of translocation9,22. Oral imatinib (400
mg/day) therapy was started and 12 months hence the
patient is in hematological remission with no evidence of
MS refers to an extramedullary manifestation of a non
lymphoid malignancy. Also called granulocytic sarcoma, it
occurs more often in acute leukemia. Its presence in chronic
myeloproliferative neoplasm/dysplasia heralds the onset
of a blast crisis. Presentation concurrent or preceding a
hematological malignancy, as happened in our case, is seen
in only up to one third of the cases. Any site of the body can
be affected, particularly the bone, soft tissues and lymph
. The skin is an uncommon site of involvement;
cutaneous lesions include erythematous to violaceous
papules, nodules, plaques, or persistent non healing ulcer3,7
. The legs are involved most often, followed by the
arms, back, chest, scalp and face. Involvement of genital
skin is very rare and only one case involving the scrotal skin
has been described in the English literatüre4
Depending upon the degree of differentiation, MS is
classified as well differentiated, poorly differentiated and
blastic type1. The presence of numerous maturing
myeloid elements qualifies for the well differentiated
category, as was the present case. These cases need to be
distinguished from extramedullary hematopoiesis (EMH),
small vessel vasculitis and infections1,8. MS differs from
EMH in lacking erythroid and megakaryocytic cells. We did
not find megakaryocytes or erythroid precursors despite
extensive search. Cutaneous EMH is commoner in neonates
where it is often associated with TORCH (toxoplasma,
rubella, and herpes virus) infection8. In adults, it is
described mostly in myelofibrosis; although it may occur
rarely in CML. New evidence suggests that cutaneous
EMH in chronic myeloid disease should be considered as
metastasis of the abnormal neoplastic cells and subsequent
differentiation along divergent myeloid lineages9. Recent
demonstration of the characteristic Janus kinase 2 gene
mutation in the cutaneous lesion of a patient with primary
myelofibrosis also supports the metastasis hypothesis10. Poorly differentiated MS cases have scanty maturing
cells. Lesions lacking morphological evidence of myeloid
differentiation are said to be blastic. The latter are likely to
be confused with lymphoma infiltrate, poorly differentiated
carcinoma and malignant melanoma; these cases require
immunohistochemistry for the diagnosis1,3.
Recognition of MS is often straightforward In the setting
of a known hematological malignancy. However, as
many as three-fourths of MS cases may be misdiagnosed
in the absence of this useful information2. The well
differentiated type of MS is likely to be overlooked as
a benign inflammatory/infectious lesion owing to the
presence of a large number of mature neutrophils. Blastic type MS is easily recognized as a malignant lesion, the issue
being differentiation from other high-grade malignancies.
Early chemotherapy in MS is known to improve survival
outcome. Local therapy may improve symptoms but does
not influence survival11. Our patient received immediate
chemotherapy and is doing well after one year.
Histopathology of the circumcision skin invariably reveals
an inflammatory dermatosis: either a specific lesion like
balanitis xerotica obliterans (BXO) or a chronic non
specific infiltrate12. BXO, the male variant of lichen
sclerosus et atrophicus and limited to the genital regions, is
a chronic inflammatory dermatosis characterized by basal
layer vacuolization, hyalinization of dermal collagen and
loss of elastic fibres. A history of diabetes mellitus should
be sought in cases with a non specific inflammatory pattern13. So far no case of phimosis has been demonstrated
to harbor hematopoietic elements. This case is the first to
histologically document a cutaneous MS in the phimosis
circumcision foreskin specimen. Attention to the unusual
composition of the inflammatory cell infiltrate, and the
characteristic sparing of dermal blood vessels, nerves and
smooth muscle fibres led to the correct diagnosis. Absence
of commonly observed changes in circumcision skin such
as the epidermal changes of balanitis xerotica was also
helpful. The real etiology of phimosis in the present case
remains obscure although it may be hypothesized that the
dermal leukemic infiltration and edema made the skin
thicker and rigid, making the physiological movement of
the foreskin difficult. Rarely, cutaneous lesions harboring
hematopoietic elements may show dermal sclerosis. The
demonstration of transforming growth factor beta in the
immature hematopoietic cells may explain the fibrogenic
activity14. However, there was no evidence of fibrosis in
the present case. Phimosis is a well known manifestation
of chronic graft versus host disease in male patients with
hematological disease treated with allogenic bone marrow/
stem cell transplant. Suzuki et al reported phimosis in 15/46
(32.6%) uncircumcised male patients under the age of 15
years treated with allogenic stem cell transplantation15.
However, the histopathological details of the circumcision
skin are not mentioned in their report.
Priapism refers to sustained (more than 4 hours) penile
erection in the absence of physiologic stimuli. Rare by
itself in the healthy population, it is notably associated
with sickle cell anemia5. Male patients with CML may
uncommonly also develop priapism. Its occurrence is more
typical in the course of the evolution of CML rather than
an initial presentation as happened in our case. It has been
reported as the first complaint in only 3.2% of male CML patients6. Hyperleukocytosis is a significant risk factor
for developing priapism, as was present in our case. The
sludging of the rigid leukemia cells in hyperleukocytosis
states prevents the outflow of blood, resulting in failure of
To conclude, we have reported an unusual presentation
of a previously undiagnosed CML. This case is unique as
it is the first time a cutaneous MS has been demonstrated
in a phimosis circumcision foreskin specimen. Features
suspicious of myeloid sarcoma were the unusual
composition of dermal infiltrate, absence of erythroid
precursors, megakaryocytes and characteristic sparing of
dermal vessels and nerves.
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