Modern Plastic Surgery, 2012, 2, 77-79 Published Online October 2012 ( 77
A Case of CD34-Negative Superficial Acral Fibromyxoma
Marie Furuichi, Keisuke Okabe, Kazuo Kishi
Departments of Plastic and Reconstructive Surgery, Keio University School of Medicine, Tokyo, Japan.
Received May 18th, 2012; revised June 15th, 2012; accepted July 13th, 2012
Superficial acral fibromyxoma (SAF) is a tumor that occurs on the distal phalanges of the digits. As it does not sponta-
neously regress and is often associated with pain, the primary treatment is surgical resection. It is often associated with
the nail component of the affected digit, and thus cosmesis is an important goal of the operation. We herein describe a
case of SAF on the distal phalanges of the fifth digit of the foot beneath the nail, which was successfully resected with
the nail component kept intact. Moreover, although SAF is most commonly CD34-positive, the present case was
CD34-negative ex cept for endothelial cells within the tumor. While CD34-n egative SAF has been previously reported,
the current case further indicates that CD34-positivity is not essential for the diagnosis of SAF.
Keywords: Superficial Acral Fibromyxoma; CD34; Nail
1. Introduction
There are a variety of tumors that can affect the acral
extremities including superficial acral fibromyxoma
(SAF), fibroma, exostosis, enchondroma, tyloticums, and
gloms tumor. The growth of some of these is accompa-
nied with pain and digital deformity. SAF is a tumor ob-
served in the apical area and is usually accompanied with
pain secondary to the stiffness of tumor within its capsule.
When the tumor is associated with the nail, cosmesis is
an important factor during tumor resection. We report a
case of SAF growing beneath the nail of the right fifth
toe which was successfully removed with the nail com-
ponent kept intact. Immunohistochemical analysis indi-
cated that the resected tumor did not stain positive for
CD34, which is rare in cases of SAF.
2. Case Report
A 40-year-old woman noticed a small growth on her
right fifth toe five years prior to her presentation with us.
The tumor regressed, but it eventually grew again and
was accompanied by pain. As the pain increased in se-
verity it became hard for her to wear a shoe on the af-
fected foot, and she subsequently presented to our hospi-
tal. There was no history of trauma or other event related
to the onset of the tumor. The mass measured 8 mm in
length and consisted of greyish-white tissue with well-
defined margins, and felt cartilaginous in consistency
(Figure 1). The epidermis did not show parakeratiniza-
tion or hyperkeratinization and was grossly intact. X-ray
images showed no calcification. Magnetic resonance ima-
ging (MRI) of the mass demonstrated low signal inten-
sity on T1 and T2-weighted images (Figure 2).
As the tumor did not invade the surrounding tissue on
these images and was presumed to be benign, a simple
resection was performed. Under spinal anesthesia, a
transverse incision on the to e was made with the nail bed
kept intact. The tumor was resected and the excess skin
was trimmed away. Then the skin was approximated with
5 - 0 nylon. The contour of the toe after the operation did
not affect of the shape of the nail (Figure 3). Histological
examination showed a proliferation of collagen, fibro-
blastic cells, and edematous change compatible with SAF
(Figure 4(a)). Immunohistochemical examination for the
presence of CD34 in the specimen was negative except
for within endothelial cells (Figure 4(b)).
Figure 1. Macroscopic view before operation.
Copyright © 2012 SciRes. MPS
A Case of CD34-Negative Superficial Acral Fibromyxoma
Figure 2. MRI images. MRI of the mass demonstrated low
signal intensity on T1 (a) and T2 (b)-weighted images.
Figure 3. Macroscopic view three months after operation.
The contour of the toe was preserved.
Figure 4. Histological section of SAF. H-E staining showing
that cells in the tumor were spindle and stellate in shape
and associated with the myxoid matrix and a delicate vas-
cular network (a). Immunohistochemical staining of the
tumor for CD34 shows that no cells except for endothelial
cells (arrow) were positive for CD34 (b). Bar = 100 μm.
3. Discussion
Fibromyxoma is a fibroma that has undergone myxoma-
tous degeneration. Superficial acral fibroma was reported
by Fetsch et al. in 2001 [1] as an uncommon tumor of the
superficial soft tissue of the acral extremities. Most cases
present in middle-ag ed adults as a long-standing, so litary
mass measuring between 1 and 2 cm on the hands or feet,
and are often observed in a subungal location [1,2].
In regards to operative management, the tumor should
be be resected at the margin of the tumor as SAF is be-
nign. Although the tumor exists in close proximity to the
nail bed, it is of mesenchymal origin and can be removed
without affecting the nail component [3].
Pathologically, SAF is characterized by a slight to
moderate cellular proliferation of spindle and stellate-
shaped fibroblastic cells arranged in a random or fas-
Copyright © 2012 SciRes. MPS
A Case of CD34-Negative Superficial Acral Fibromyxoma
Copyright © 2012 SciRes. MPS
cicular growth pattern, with myxoid stroma and promi-
nent vascularity [2,4]. It has been reported that the tumor
cells of the SAF are diffusely positiv e for CD34 and may
be focally positive for EMA, CD10, and CD99 [4,5-8].
In the present case, most of the tumor consisted of fi-
brous tissue with some areas showing m y xomatous change.
This is associated with the deposition of mucin among
the fibrous part. From these histological findings, we dia-
gnosed this tumor as SAF.
Immunohistochemical examination for CD34 was ne-
gative except for the endothelial cells within the tumor.
CD34-negative SAF has been reported previously [4],
and as supported by another example in the present case,
the diagnosis of SAF should not be made solely on the
basis of immunostaining for CD34.
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