Professional Documents
Culture Documents
of Dermatofibrosarcoma Protuberans
Julia A. Bridge, James R. Neff, and Avery A. Sandberg
ABSTRACT: A case of dermatofibrosarcoma protuberans that occurred in an old burn scar over the
anterior chest wall of a 46-year-old man is reported. Cytogenetic analysis of the tumor cells
showed the presence of two abnormal clones: 47,XY,+8 and 48,XY,+8,+r.
INTRODUCTION
Dermatofibrosarcoma protuberans (DP) is an u n c o m m o n , locally aggressive, but rarely
metastasizing tumor of the deep dermis [1]. It typically presents during early or
m i d a d u l t life and is most frequently located on the trunk and proximal extremities.
With the exception of malignant melanoma, the literature regarding cytogenetic
findings of primary skin tumors is sparse [2-4]. To our knowledge, cytogenetic analysis of DP has not been reported previously.
CASE REPORT
RESULTS
G-banded chromosome preparations of surgically excised tumor were made after 9day culture, as previously described [5]. Twenty-five metaphase cells were analyzed,
six of which were karyotypically normal. Two abnormal clones were observed in the
r e m a i n i n g cells and consisted of the following: five cells with 47,XY,+8 and 14 cells
From the Cancer Center of Southwest Biomedical Research Institute of Genetrix, Scottsdale, Arizona
{J. A. B., A. A. S.), and the Department of Pathology and Oncologyand Departmentof Orthopaedic Surgery,
Universityof Kansas Medical Center, Kansas City, Kansas (l. A. B., J. R. N.).
Address reprint requests to: Dr. Julia A. Bridge, Department of Pathology and On cology,
University of Kansas Medical Center, 39th and Rainbow Blvd., Kansas City, KS 66103.
Received January 12, 1990; accepted February 14, 1990.
199
1990 Elsevier SciencePublishingCo., Inc.
655 Avenue of the Americas, New York, NY 10010
J. A. Bridge et al.
200
Figure 1
neoplasmconsisted of slightly
atypical spindle-shaped
cells arranged in a whorlfat (upper right-hand
corner] and skeletal muscle
(lower left-hand corner). (Original magnification x 200. reduced by 2U/ir).
like fashion
with
invasion
with 48,XY,+8,+r
ring chromosome.
of subcutaneous
of the
DISCUSSION
Dermatofibrosarcoma
protuberans was first described by Darier and Ferrand [6] as a
progressive and recurring dermatofibroma. It is a nodular cutaneous tumor characterized by a prominent storiform pattern; it tends to recur but metastasizes infrequently. It is considered a neoplasm of intermediate malignancy.
The histopathogenesis
of DP is controversial. Histologically, ultrastructurally, and
by tissue culture, DP is similar to benign and malignant fibrous histiocytoma (MFH).
These data have led some investigators to consider DP to be of fibroblastic or histocytic
origin [7-g]. Others have proposed a Schwannian derivation based on detailed morphologic and electron microscopic examinations [10, 111,
Cytogenetically,
the present case of DP was characterized by two abnormal clones,
one with trisomy 8 as the sole abnormality and the other with trisomy 8 and a marker
ring chromosome. Trisomy 8 appears to be the primary abnormality, with the ring
chromosome occurring as a secondary abnormality with evolution of an additional
clone. Trisomy 8 is not a frequently reported primary chromosomal anomaly, particu-
201
t.3
14
Ib
~9
20
IO
16
XY
~1
12
I7
18
rln9
Figure 2 Representative karyotype of secondary clone with trisomy 8 and marker ring chromosome. The Y chromosome was missing in only this one metaphase.
202
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