A case of myxoid dermatofibrosarcoma protuberans.
ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is a slowly growing dermal spindle cell tumor and its myxoid variant, a rare type of DFSP, is characterized by extensive myxoid degeneration. We present the case of a 69-year-old woman with a multinodular reddish plaque on her trunk. Histopathologically, the tumor was located in the dermis and consisted of uniform spindle-shaped cells, showing strongly positive reaction for CD34, and negative for both S-100 and desmin. In addition to the typical storiform pattern, prominent myxoid stromal changes were demonstrated. Herein, we report an interesting case of myxoid DFSP, rarely reported in the dermatology literature.
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ABSTRACT: We present a typical case of dermatofibrosarcoma protuberans with local recurrence 2 months after surgery and, motivated by this patient, make a review of the most important aspects. This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery.Case Reports in Dermatology 05/2014; 6(2):134-9.
Vol. 23, No. 3, 2011
Received April 26, 2010, Revised July 21, 2010, Accepted for
publication July 21, 2010
Corresponding author: Hae Young Choi, M.D., Department of
Dermatology, Ewha Womans University Mokdong Hospital, 911-1
Mok-dong, Yangcheon-gu, Seoul 158-710, Korea. Tel: 82-2-2650-
5159, Fax: 82-2-2652-6925, E-mail: firstname.lastname@example.org
This is an Open Access article distributed under the terms of the
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Ann DermatolVol. 23, No. 3, 2011DOI: 10.5021/ad.2011.23.3.379
Fig. 1. Multinodular reddish plaques, with partial gelatinous
appearance on the abdomen.
A Case of Myxoid Dermatofibrosarcoma Protuberans
Yeon Ju Hong, M.D., You Won Choi, M.D., Ki Bum Myung, M.D., Hae Young Choi, M.D.
Department of Dermatology, School of Medicine, Ewha Womans University, Seoul, Korea
Dermatofibrosarcoma protuberans (DFSP) is a slowly
growing dermal spindle cell tumor and its myxoid variant, a
rare type of DFSP, is characterized by extensive myxoid
degeneration. We present the case of a 69-year-old woman
with a multinodular reddish plaque on her trunk. Histopatho-
logically, the tumor was located in the dermis and consisted
of uniform spindle-shaped cells, showing strongly positive
reaction for CD34, and negative for both S-100 and desmin.
In addition to the typical storiform pattern, prominent
myxoid stromal changes were demonstrated. Herein, we
report an interesting case of myxoid DFSP, rarely reported in
the dermatology literature. (Ann Dermatol 23(3) 379∼
Dermatofibrosarcoma protuberans, Myxoid variant
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally
aggressive dermal mesenchymal neoplasm. The character-
istic histologic feature of DFSP is the proliferation of
densely packed monomorphous spindle cells, arranged in
a storiform pattern. To date, various histological patterns,
including the myxoid, fibrosarcomatous and granular cell
types, have been described in the English literature1, but
myxoid variant of DFSP has not yet been reported in the
Korean literature. Herein, we report a case of myxoid
DFSP, rarely reported in the Korean dermatology literature.
A 69-year-old woman was presented with an enlarging
nodular lesion on her trunk. The lesion had been growing
over a period of 6 months and she denied any discomfort,
except the occasional itching sensation. There was no
history of trauma or any preexisting skin lesions in the
affected area. Clinical examination revealed multiple
erythematous to brownish nodules, with reddish plaque
on her abdomen, and some part of the lesion had a
gelatinous appearance (Fig. 1).
A skin biopsy was performed and the lesion showed a
diffusely infiltrating tumor, involving the entire dermis
(Fig. 2). In some parts of the lesion (Fig. 3A), the tumor
showed the characteristic storiform pattern, but most of
the tumor cells were randomly embedded in the palely
eosinophilic stroma (Fig. 3B). The tumor consisted of
relatively uniform spindle cells, with elongated nuclei (Fig
3C), and the stroma was diffusely positive for the alcian-
YJ Hong, et al
Fig. 2. The diffusely infiltrating tumor was located in the entire
dermis (H&E, ×40).
Fig. 3. (A) The tumor showed the characteristic storiform pattern
in some parts of the lesion (H&E, ×100). (B) In addition to the
typical storiform pattern, prominent myxoid stromal changes
were demonstrated (H&E, ×100). (C) Bland spindle cells with
oval nuclei were randomly embedded in the loose myxoid
stroma (H&E, ×400).
Fig. 4. (A) The tumor cells were strongly positive for CD34
blue stain, indicating the prominent myxoid degeneration.
Immunohistochemically, the tumor cells were strongly
positive for CD34 (Fig. 4). Other markers, including des-
min, S-100, and epithelial membrane antigen (EMA), were
The diagnosis of myxoid DFSP was made and the patient
was referred to a surgeon for the excision. At the 5-month
follow-up, the patient remained lesion free.
A Case of Myxoid Dermatofibrosarcoma Protuberans
Vol. 23, No. 3, 2011
DFSP is a slowly growing, but locally aggressive tumor,
and it usually develops as a multinodular reddish-blue
plaque on the trunk. Occurrence at sites of previous
trauma has been reported, and many patients have a
previous, long preoperative history1. Our patient had
neither previous trauma nor operation history, except
vaginal hysterectomy for cervical cancer, 16 years ago.
Histologically, DFSP consists of relatively uniform spindle
cells containing elongated nuclei, without significant
cytologic atypia or pleomorphism, and arranged in a
predominantly storiform typical pattern. Although the
tumor is usually located in the dermis, it invariably shows
the infiltrative growth pattern, with trapping of the
subcutaneous fat tissue in the characteristic honeycomb
The myxoid DFSP is a rare variant of DFSP, characterized
by the prominent myxoid stromal changes. Since the first
case was cited in 1983 by Frierson and Cooper2, only a
few cases have been reported in the literature. The
pathogenesis of the myxoid change remains uncertain,
and the majority of cases were presented with a slowly
growing, firm subcutaneous mass. The most commonly
involved sites were the extremities, followed by the head
and neck3. In addition to the typical histological features
of the ordinary DFSP, the tumor cells of the myxoid DFSP
are embedded in an abundant, palely eosinophilic myxoid
stroma, and prominent, thin-walled vessels are frequently
present throughout the tumor3. Immunohistochimical find-
ings are consistent with the typical DFSP, with the positive
staining for CD34 ranging from 84% to 100%, and
negative for other markers, such as S-100, desmin and
Prominent myxoid changes are often obscured by the
typical storiform pattern, and this may cause considerable
diagnostic problems, especially in the distinction between
more and less aggressive myxoid mesenchymal neo-
plasms5. The differential diagnosis of myxoid DFSP is
diverse and includes myxoid liposarcoma, myxofibro-
sarcoma, myxoid neurofibroma and superficial angio-
myxoma. Myxoid liposarcoma is mainly seen in deep soft
tissues of the thigh and is distinguished from the presented
case by the presence of lipoblasts and atypical, undifferen-
tiated mesenchymal tumor cells, which are usually CD34
negative. Superficially located low-grade myxofibrosarcoma
can be easily confused with the myxoid DFSP, because
both consist of spindle-shaped cells embedded in a
myxoid stroma, rich in small vessels. However, the low-
grade myxofibrosarcoma is a rather well-circumscribed
tumor, with overall increased cellularity, compared with
the myxoid DFSP, and the myxoid areas show a more
whorled pattern. In addition, in many cases of low-grade
myxofibrosarcoma6, the tumor cells are stained positively
for the EMA. The myxoid neurofibroma can be distinguished
from the presented case by the presence of S-100 protein
positivity, wavy buckled nuclei and intralesional axons.
The superficial angiomyxoma can be difficult to distinguish
from the myxoid DFSP, because the former may also
express CD34. However, in contrast to the myxoid DFSP,
the superficial angiomyxoma lesions tend to be displayed
in a lobular growth pattern, and scattered neutrophils
surrounding the vessels are characteristically seen in a
number of cases.
In summary, we presented an interesting case of myxoid
DFSP, rarely reported in the Korean dermatology literature,
and awareness of this rare variant of DFSP is important to
avoid misdiagnosis of more or less aggressive myxoid
tumors, which could lead to either under-treatment or
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