FIBROHISTIOCYTIC TUMORS OF INTERMEDIATE MALIGNANCY

1.Dermatofibrosarcoma protuberans
2.Sarcoma arising in dermatofibrosarcoma protuberans
3.Bednar tumor
4.Giant cell fibroblastoma
5.Angiomatoid fibrous histiocytoma
6.Plexiform fibrohistiocytic tumor
7.Soft tissue giant cell tumor of low malignant potential

->All are characterized by a significant risk of local recurrence but a limited risk
of regional and distant metastasis
->in the younger population
->dermatofibrosarcoma and its juvenile counterpart, giant cell fibroblastoma,
seem to be most closely related to a fibroblast; and indeed the discovery of
CD34 immunoreactivity in these two lesions provides a linkage to the CD34+
dendritic cells that populate the dermis
->fibrohistiocytic. It has a bimodal population of cells, one of which has the
histologic and immunophenotypic properties of a histiocyte and the other
resembling a myofibroblast.=> +/- cd68; = myoid markers!!

1.DERMATOFIBROSARCOMA PROTUBERANS
firm, plaque-like lesion of the skin, often with surrounding red to blue discoloration.
[5]

These lesions have been compared with the morphea of scleroderma or morphea-like
basal cell carcinoma. Rarely-atrophy or multiple small subcutaneous nodules.
The skin overlying these tumors is taut or even ulcerated. Occasionally, areas of the tumor
have a translucent or gelatinous appearance corresponding microscopically to myxoid
change. Hemorrhage and cystic change are sometimes seen in the tumors, but necrosis, a
common feature of malignant fibrous histiocytoma, is rare.

Micro:
the overlying epidermis does not usual ly display the hyperplasia that characterizes some
cutaneous fibrous histiocytomas (dermatofibromas).
In deep regions, the tumor spreads along connective tissue septa and between adnexae
or it intricately interdigitates with lobules of subcutaneous fat, creating a lace-l ike or
honeycomb effect
Superficial (A) and deep (B) extensions of
dermatofibrosarcoma protuberans. Spread of the
tumor between preexisting col lagen of the dermis
may simulate the appearance of a cutaneous fibrous
histiocytoma (A). At the deep margin the tumor
intricately interdigitates with normal fat (B).
Dermatofibrosarcoma protuberans infi ltrating
between adnexal structures /\
uniform population of slender fibroblasts
arranged in a distinct, often monotonous,
storiform pattern around an
inconspicuous vasculature; giant
cel ls, xanthoma cel ls, and inflammatory
elements are few in number or absent;
occasional tumors contain
myxoid areas - the storiform pattern
becomes less distinct and the vascular
pattern more apparent-such tumors can
resemble myxoid l iposarcoma

(A) Myxoid change in dermatofibrosarcoma protuberans. (B) When the myxoid change is
prominent, the storiform pattern may be lacking and the tumor may resemble a myxoid
liposarcoma.
An unusual feature of dermatofibrosarcoma protuberans is the myoid nodule. It may represent
an unusual nonneoplastic vascular response to the tumor .
Fibrosarcomas have more than 5 mitotic figures/10 high-power fields(HPF), in contrast to areas
of conventional dermatofibrosarcoma protuberans,which usual ly have fewer than 5 mit/10
HPF.
(A) Dermatofibrosarcoma protuberans showing the transition to fibrosarcoma (lower left).
(B) CD34 in dermatofibrosarcoma(upper right) with fibrosarcomatous areas. marked diminution
of CD34 immunostain in the fibrosarcomatous portion of the tumor (lower left).
Fibrosarcomatous areas within
dermatofibrosarcoma protuberans
Dermatofibrosarcoma protuberans is characterized by the nearly consistent presence of CD34;
its presence in dermatofibrosarcoma protuberans
suggests a close l inkage to the normal CD34+ dendritic cel ls of the dermis, including those that
ensheath the adnexae, nerves, and vessels.
Apolipoprotein D ~

CD34 immunoreactivity within a conventional dermatofibrosarcoma (A) and markedly reduced
immunoreactivity within a fibrosarcomatous area of dermatofibrosarcoma protuberans (B).
Differential diagnosis: benign fibrous histiocytoma ; Malignant fibrous histiocytoma
(pleomorphic undifferentiated sarcoma); neurofibroma; myxoid liposarcoma

1b. SARCOMA ARISING IN DERMATOFIBROSARCOMA PROTUBERANS (FIBROSARCOMATOUS VARIANT OF
DERMATOFIBROSARCOMA PROTUBERANS)
the sarcomatous foci should constitute at least 510% of the tumor , in contrast to simply a
rare to occasional microscopic focus. These zones are characterized by a fascicular (rather than
storiform) architectural pattern and are composed of plump spindle cel ls of high nuclear grade.
Mitotic activity is increased in these areas, whereas CD34 immunoreactivity is often diminished
(Fig. 13-19B), compared to the surrounding dermatofibrosarcoma. In addition, fibrosarcomatous
areas are also characterized by a higher MIB-1 label ing index and increased p53
immunostaining than the classic areas. Although we have never required an absolute level of
mitotic activity to diagnose sarcomatous change, mitotic activity within these sarcomatous areas
averages 715/10 HP F[10,] [41] compared to 1-3/10 HP F in dermatofibrosarcoma.

1c. BEDNAR TUMOR (PIGMENTED DERMATOFIBROSARCOMA PROTUBERANS, STORIFORM
NEUROFIBROMA)
Bednar tumor . Gross appearance of the
tumor is identical to conventional
dermatofibrosarcoma protuberans, but the
substance of the tumor is flecked
with melanin pigment.
Pigmented dermatofibrosarcoma protuberans (Bednar tumor)(A).dendritic pigmented cells (B).
2. GIANT CELL FIBROBLASTOMA
Grossly, the lesions consist of gray to yellow mucoid masses that are poorly circumscribed and
measure 18 cm.
Micro: loosely arranged, wavy spindle cells with a moderate degree of nuclear pleomorphism
that infiltrate the deep dermis and subcutis and encircle adnexal structures in a fashion similar
to dermatofibrosarcoma protuberans; The tumors vary in cellularity from those approximating
the cellularity of dermatofibrosarcoma protuberans to those that are hypocellular with a
myxoid or hyaline stroma ;
-Large and irregular in shape, the pseudovascular spaces are lined by a discontinuous row of
multinucleated cells that represent variants of the basic proliferating tumor cell. Although these
cells appear to contain multiple overlapping nuclei, as seen by light microscopy, they actually
represent multiple sausage-like lobations of a single nucleus-ultrastructurally .
-Vimentin+
-CD34 +/-

-S100-
3.ANGIOMATOID FIBROUS HISTIOCYTOMA
Previously termed angiomatoid malignant fibrous histiocytoma;
relative rarity of metastasis and the overall excellent clinical course;
slowly growing nodular , multinodular , or cystic mass of the hypodermis or subcutis;
One of the most characteristic features is the presence of irregular blood-fi l led cystic spaces
best appreciated on cross section.This feature may be so striking as to give the impression of a
hematoma, hemangioma, or a thrombosed vessel
Gross specimen of an
angiomatoid fibrous
histiocytoma illustrating cystic
change and a hemosiderin-
stained tumor . Normal fat is
present at the periphery.
=three features: irregular sol id masses of histiocyte-like cells, cystic areas of hemorrhage, and
chronic inflammation.
In general, the solid masses of histiocyte-like cells interspersed with areas of hemorrhage
occupy the central portion of the tumor , and the inflammatory cells form a dense peripheral
cuff that blends with the surrounding pseudocapsule.
Angiomatoid fibrous
histiocytoma. Histiocyte-like
cells are arranged in solid
sheets. Lymphoid infiltrate
surrounds the tumor nodule.
4.PLEXIFORM FIBROHISTIOCYTIC TUMOR
in children and young adults and is rarely encountered after the age of 30 years;
small (13 cm), ill-defined masses with a gray-white trabecular appearance;
mixture of two components: a differentiated fibroblastic component and a round cell
histiocytic component containing multinucleated giant cells;
numerous tiny cellular nodules that occupy the dermis and subcutaneous tissue ;
histiocytic cells that often contain multinucleated, osteoclast-like giant cells and occasionally
undergo focal hemorrhage;
The nodules are circumscribed by short fascicles of fibroblastic cells that intersect slightly
or ramify in the soft tissue, creating a plexiform growth pattern;
the cells in the 2 zones appear to be in an intermediate stage between fibroblasts &histiocytes.
Immunohistochemical ly, the multinucleated giant cel ls and many of the mononuclear cells
express CD68, suggesting true histiocytic differentiation, whereas the spindle cells express
smooth muscle actin, as one would expect of myofibroblasts (Fig. 13-51). The cells do not
contain other histiocytic markers such as HLA-DR, lysozyme, or L-2; nor are S-100 protein,
keratin, desmin, and Factor XIIIa present.
5. SOFT TISSUE GIANT CELL TUMOR OF LOW MALIGNANT POTENTIAL
the term soft tissue giant cel l tumors of low mal ignant potential was proposed for a group
of lesions that represents the benign end of the spectrum of malignant giant cell tumor of soft
parts (malignant fibrous histiocytoma, giant cell type) and that seem to be the soft tissue
analogue of giant cell tumor of bone;
at any age; may develop in superficial or deep soft tissue, most commonly on the arm or hand;
bland mononuclear cells, short spindle cells, and osteoclasts;
lack the strikin atypia that is the hallmark of giant cell forms of malignant fibrous histiocytoma;
often have brisk mitotic activity; about one-half display vascular invasion (as may be seen
with giant cell tumor of bone), although necrosis is not seen;
they express CD68 and smooth muscle actin, and the osteoclastic giant cells express the
osteoclast-specific marker tartrate-resistant acid phosphatase (TRAP ) !!!!!!!
Ihc neg: CD45, S-100 protein, desmin, and lysozyme.
Giant cell tumor of low malignant potential with mild (A) and moderate (B) atypia of the
mononuclear tumor cells. This contrasts with the marked atypia of classic malignant fibrous
histiocytoma, giant cel l type.
Differential diagnosis: tenosynovial giant cell tumor , plexiform fibrohistiocytic tumor,
epithelioid sarcomas and nodular fasciitis with giant cells.