Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade ( …

© 2002 European Academy of Dermatology and Venereology

441

OR IG INAL AR T I CLE

JEADV

(2002)

16

, 441–446

Blackwell Science, Ltd

Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases

R

Oliveira-Soares,†*

I

Viana,‡

E

Vale,‡

LM

Soares-Almeida,†

A

Picoto‡

†

Clínica Dermatológica Universitária, Hospital Santa Maria, R Prof. Egas Moniz, Lisbon, Portugal,

‡

Centro de Dermatologia Médico Cirúrgica de Lisboa,

R. José Estêvão, 135, 1150–201 Lisboa.

*

Corresponding author, R Prof Alfredo Sousa, lote H1, 1

°

Dto, 1600–188 Lisboa, Portugal, tel. +217572721;

E-mail: [email protected]

ABSTRACT

Aim

To review the clinical and histological data of 20 cases of dermatofibrosarcoma protuberans presentingat two dermatology centres in Lisbon from 1978 to 1998.

Patients and methods

The 20 subjects comprised nine males and 11 females ranging in age from 25 to79 years, with highest frequency of subjects in the 30–50 year olds. We reviewed the clinical features, his-topathological aspects, including morphologic variants and immunohistochemical studies.

Results

Median age at diagnosis was 51 years and the trunk was the most frequent location. The character-istic histologic storiform pattern was seen in all cases. Three subjects presented fibrosarcomatous areas, onewith myoid differentiation and another with multinucleated giant cells. Immunohistochemical stainsrevealed CD34 expression in the 18 specimens tested, FXIIIa was negative, and these two antigens provedimportant for the differential diagnosis of this neoplasm. Local wide excision was performed in 13 cases andseven patients underwent Moh’s micrographic surgery. Follow-up ranged from 2 months to 17 years andthree recurrences were recorded, two following classical surgery and one after Moh’s surgery; there was nodifference in the rate of local recurrence (15%) for the two kinds of treatment in our series.

Key words:

dermatofibrosarcoma protuberans, clinicopathological features, CD34, histological variants,

treatment

Received: 29 December 2000, accepted 8 March 2002

Introduction

Dermatofibrosarcoma rotuberans (DFSP) is an unusual, locally

aggressive, cutaneous neoplasm of low-grade malignancy. It is

has been considered a distinct clinicopathological entity since

Darier and Ferrand’s description in 1924

1

but its characteristic

microscopic pattern was first noted only in 1962 by Taylor and

Helwig.

2

The exact origin of DFSP remains a matter of con-

troversy, although fibroblastic,

3

myofibroblastic,

4

histiocytic

5

and neuroectodermal

6

histogenic lines have been proposed,

based on immunohistochemical, ultrastrucural and tissue

culture studies.

This lesion affects mainly the trunk and proximal extremities

of young and middle-aged adults and, in spite of its slowly

infiltrative growth and tendency to local recurrence, metastasis

is very unusual. Histologically, DFSP is characterized by a mono-

morphous storiform proliferation of spindle cells, involving the

dermis and hypodermis, often with a honeycomb pattern of

infiltration of the subcutaneous fat.

7

Several histological

variants have been described: pigmented (Bednar tumour),

8–10

myxoid,

11,12

myoid,

4

sclerosing,

13

with granular cells,

14

with

multinucleated giant cells (resembling giant cell fibroblast-

oma),

15,16

atrophic

17

and with fibrosarcomatous areas.

18,19

This

latter form is believed to have a more aggressive behaviour than

the others, none of which change the usual course. The histo-

logical diagnosis of DFSP is often easy, but some cases may be

difficult to distinguish from other ‘fibrohistiocytic’ neoplasms,

particularly dermatofibroma with extension to the subcutis. In

such cases, immunohistochemical markers are very important

for the differential diagnosis, especially CD34, a highly sensitive

marker for DFSP.

20

Surgery has long been the treatment for

DFSP and recently Moh’s micrographic surgery has been cho-

sen by many investigators as the treatment of choice for this

neoplasm.

21–23

JDV_558.fm Page 441 Tuesday, September 10, 2002 5:14 PM

442

Oliveira-Soares

et al.


JEADV

(2002)

16

, 441–446

Materials and methods

This study was based on clinical and histological data of patients

with DFSP diagnosed between 1978 and 1998 at Centro de

Dermatologia Médico Cirúrgico (CDMC) and Clínica Derma-

tológica Universitária do Hospital Santa Maria (CDUHSM) in

Lisbon. The following clinical information was obtained from

patient records: sex, age, clinical presentation, location, surgical

treatment, follow-up period and recurrence.

All specimens were fixed in 10% formalin, embedded in par-

affin and stained with haematoxylin-eosine. In 18 cases, an

immunohistological study was performed, using antibodies to

the following antigens: CD34 (Novocastra 1:20), XIIIa factor

(Calbiochem 1:500), vimentine (Dako 1:150) and S100 (Dako

1:4000), and in one case desmin (Novocastra 1:50) and smooth

muscle alpha actin (Novocastra 1:20).

Results

A total of 20 cases of DFSP were identified (15 from CDMC and

five from CDUHSM) and the relative clinical data are outlined

in Table 1. There was no sex predominance. The age ranged

from 25 to 79 years old (median, 51 years). The trunk was the

most frequent location (chest, three patients; abdomen, eight

patients; lumbar, five patients). There was one case each of the

tumour on the face, buttock, shoulder and thigh. Clinically the

lesions were reported mainly as plaques (fig. 1) and the pro-

posed diagnosis were DFSP, dermatofibroma, scar, morphea,

lymphoma, sarcoma and cutaneous metastasis. Preoperative

duration ranged from months to years. Thirteen patients were

treated by classical surgery and seven underwent Moh’s surgery.

Of the three recurrences, two were treated by Moh’s surgery and

one by classical local wide-excision surgery. Follow-up ranged

from 2 months to 17 years.

Histopathological findings

All cases shared extensive involvement of the dermis and

subcutis by dense, uniform and monomorphous array of bland

spindle-shaped cells with elongated nuclei with little or no

pleomorphism. Most of the cells were arranged in a classic

whorled storiform pattern (fig. 2). The epidermis was normal

in ten cases, had some degree of hyperplasia in eight (five with

hyperpigmentation) and was atrophic in two. A Grenz zone

(narrow zone of sparing in the upper dermis) was seen in 11

Table 1 Clinical findings (DF, Dermatofibroma; CS, Classical surgery; R, Recurrence; y, years)

Case N°°°° Sex Age Location Duration PresentationClinical diagnosis

Surgical treatment

Recurrence and its treatment

Years of Follow-up

1 F 72 Lumbar 4 y Nodule DFSP CS 17

2 M 27 Abdomen 10 y Plaque DFSP CS R 15 years-Mohs Lost

3 M 34 Abdomen 2 y Plaque Morphea Mohs R 3 years-Mohs Lost

4 F 42 Abdomen 15 y Macule DFSP Mohs 3

5 M 25 Chest 1 y Plaque DFSP Mohs 3

6 F 78 Abdomen 5 y Plaque Scar CS 4

7 F 35 Face 3 y Nodule ? Mohs 3

8 F 51 Lumbar 1 y plaque? DFSP CS 5

9 F 65 Chest 1 y Plaque Scar CS 5

10 M 45 Lumbar ? y Plaque DFSP Mohs Lost

11 F 44 Lumbar < 1 y Plaque Lymphoma CS 3

12 M 55 Chest 20 y Nodule DF Mohs Lost

13 F 46 Abdomen ? y Nodule Metastasis CS 3

14 F 52 Abdomen 3 y Plaque DF CS 2 months

15 F 37 Abdomen 4 y Tumour DFSP CS 6

16 M 63 Shoulder 1 y Tumour Sarcoma CS R 2 years-CS 10

17 M 56 Lumbar < 1 y Plaque DF Mohs 8

18 F 71 Buttock ? y Nodule DF CS 8

19 M 49 Abdomen 1 y Nodule DFSP CS 2

20 M 79 Thigh 3 y Plaque DF CS 6

fig. 1 Bosselated plaque on the lumbar region.


Dermatofibrosarcoma protuberans

443


JEADV

(2002)

16

, 441–446

cases. Invariably there was local infiltration of subcutaneous

tissues, mostly with a honeycomb pattern (16 cases) (fig. 3); in

seven of these there was also a multilayered pattern of bundles

orientated parallel to the skin surface and in two there was

extension of spindle-shaped cells along septae; one case shared

all three of these patterns. In one specimen the tumour bulged

into the subcutaneous tissue. Most of the tumour cells were

bland and monomorphous, and some degree of pleomorphism

was observed only in two cases. Mitotic activity was not a

pronounced feature and just one case showed seven mitoses per

ten high power fields. Skin appendages (or remnants) could be

seen in eight cases, surrounded but never invaded by the

tumour. Nerve and muscle invasion was not observed, and

although there was a high degree of vascularity in some cases,

necrosis and angiolymphatic invasion were not seen. Focal

infiltration of lymphocytes and plasma cells was present at the

periphery of the tumour in six cases.

The following morphologic variants of DFSP were observed.

DFSP with fibrosarcomatous areas (DFSP-FS): three speci-

mens showed areas of larger cellular fascicles of fusiform cells

separated by variable amounts of collagen, sometimes with a

herring-bone pattern and showing a more pronounced level of

pleomorphism and mitosis (fig. 4); two of these specimens

referred to patients with recurrence. DFSP with areas of myoid

differentiation: in one case of classic DFSP there were some

bundles and nodules of muscular appearance (fig. 5). DFSP with

multinucleated giant cells: giant cells were seen in one case, located

at the periphery of the tumour, embedded in a myxoid stroma,

and no angiectoid or sinusoidal spaces were seen (fig. 6).

fig. 2 (a) The characteristic monomorphous storiform pattern was present in

most cases. (b) High magnification.

fig. 3 Infiltration of subcutaneous tissue in a typical honeycomb pattern and

in horizontal layers.

fig. 4 The cells were arranged in fascicles with herring-bone pattern in fibro-

sarcomatous areas.

fig. 5 DFSP with areas of myoid differentiation.


444

Oliveira-Soares

et al.


JEADV

(2002)

16

, 441–446

CD34 immunostaining was strongly positive in all the 18

specimens studied (two of the blocks were not available).

Fibrosarcomatous areas showed weaker CD34 positivity than

the surrounding tumour. Unreactivity to XIIIa factor was the

rule, although some scattered dendritic cells were observed in

nine cases. The areas with myoid differentiation were negative

for CD34 and desmin, and positive for smooth-muscle-actine

and pan-muscle-actine. Multinucleated giant cells labelled only

with vimentin.

Discussion

Clinical aspects

DFSP is a rare tumour. Estimated incidence is one case per

million per year.

2,24,25

Although it can occur at any age, DFSP is rare in children, where

its clinical findings are similar to adult DFSP.

26–28

We observed a

clear peak in the fourth and fifth decades, coincident with epidemio-

logical data.

24,25

There was no sex predominance, while in other

series a slight male-female preponderance is reported.

2,7,29

The trunk was the location in 80% of tumours, a percentage

slightly above the 50–60% reported in other studies.

2,24,25

One

tumour was located on the head, and none was found on the

hands or feet. Large series demonstrate that acral sites are a rare

location of DFSP.

7,24

Clinical presentation was usually an

asymptomatic indurate plaque, sometimes a nodule or a

tumour. No multiple primary lesions were found in our series,

although some cases have been reported in the literature.

30

A

considerable delay between the onset of symptoms and con-

sultation was the rule.

The most frequent clinical diagnosis at presentation were

DFSP and dermatofibroma. The others were scar, fibrosarcoma,

and metastasis, all conditions that have been previously referred

in the clinical differential diagnosis of DFSP.

2,7,24

Standard surgical local excision was performed in 65% of

the patients and 35% underwent Moh’s micrographic surgery.

To improve cure rates with conventional surgery, a wide local

excision with 3 cm margins beyond the tumour border down to

the fascia has been recommended.

21

Such a margin is not pos-

sible when DFSP involves the face or distal extremities. Moh’s

micrographic surgery is considered by many investigators to be

the best treatment for DFSP.

21–23,31

The recurrence rate with this

method ranges from 0% to 6% in different series.

7,16,17

Moh’s

surgery enables the surgeon to map the location of all the tissue

that has been removed and microscopically examine the entire

deep and lateral margins of a horizontally sectioned excision

specimen. This is important in a tumour with a macroscopic-

ally indistinguishable border. Such a tissue-sparing technique

is particularly important when the tumour involves the face or

distal extremities. As recommended,

24

our patients were exam-

ined every 3 months for 2 years and annually for life, as late

recurrences of up to 10 years have been reported. We observed

local recurrences in three patients (15%), appearing after 2–

15 years of follow-up. In this series, there was no significant

difference in the recurrence rate for the two types of surgical

treatment: 2/13 (15%) after classical surgery and 1/7 (14%)

after Moh’s micrographic surgery. Two of the patients with

recurrence were treated with Moh’s micrographic surgery, but

unfortunately both were lost to follow-up. The other patient

underwent classical surgery with a 3 cm margin and is free of

disease after 10 years. We did not find regional or distant metas-

tases in any of the patients. Prophylactic lymphadenectomy is

unnecessary as metastases of DFSP are uncommon, and in such

a case dissemination is haematic, usually to the lung. Radiation

therapy can be done in addition to surgery when complete

resection is not possible.

32

Chemotherapy is not useful in the

treatment of localized DFSP. It may have a role in metastatic

DFSP, but significant benefits remain to be demonstrated.

24

Histological aspects

Diagnosis is not difficult when the characteristic histological

features of DFSP are present, however, sometimes we must

differentiate DFSP from other dermal spindle cell tumours,

namely benign fibrous histiocytoma (dermatofibroma).

33,34

Some authors refer to the presence of epidermal hyperplasia

and the existence of a Grenz zone between the epidermis and

the underlying tumour as a feature to distinguish it from

DFSP.

35

In our series, however, we found eight cases with some

degree of hyperplasia of the epidermis and in 11 of these there

was a clear zone separating the epidermis from the neoplasm.

The pattern of extension in the subcutis is also used by many

authors to differentiate these two tumours:

36,37

dermato-

fibroma is usually well demarcated and bulging or penetrating

along the hypodermis septa and DFSP spreads in a honeycomb-

like or multilayered fashion, parallel to the skin surface. In most

of our cases the lesion extended to the subcutaneous tissue in

one or sometimes both patterns. In two cases, however, there

was extension along the septa and one showed a bulging

fig. 6 In an otherwise typical DFSP there were areas with multinucleated

giant cells. The other features of giant-cell fibroblastoma were missing.


Dermatofibrosarcoma protuberans

445


JEADV

(2002)

16

, 441–446

deep margin, but all the other features were typical of DFSP.

Malignant fibrous histiocytoma (MFH), atypical fibroxanthoma

(AT) and fibrosarcoma are other ‘fibrohistiocytic tumours’ said

to be possibly confused with DFSP, but we think that the

morphologic features of MFH and AT – marked cellular atypia

and pleomorphism, high mitotic activity and characteristic

bizarre giant cells – allows one to make the correct diagnosis.

The problem may be more difficult with spindle cell atypical

fibroxanthoma. In such cases immunohistochemistry can play

a very important role (see below). Fibrosarcoma is a deeper

tumour of spindle cells arranged in a characteristic herring-

bone pattern. In DFSP, areas of fibrosarcomatous differenti-

ation may be found, and that was the case in three of our specimens,

this change has been associated with unfavourable course,

higher tendency to recur and increased risk of metastasis,

18,19,38

but its true prognostic significance is still controversial. Two of

our cases showed initially fibrosarcomatous foci (cases five and

ten) and the other one (case three) was already a recurrence

after an initial Moh’s treatment; we know that this last case had

another recurrence 6 months after being submitted a second

time to Moh’s surgery and was then unfortunately lost to

follow-up. The patient of case five was free of disease after

3 years. The patient of case ten was lost to follow-up.

We found one case of myoid differentiation in an otherwise

common type of DFSP; this variant was described mainly in

DFSP with fibrosarcomatous areas but it can also be seen,

although less frequently, in ordinary DFSP.

4

The finding of multinucleated giant cells that only expressed

vimentin in one of our cases is similar to some reports in the liter-

ature.

16

We looked, but were unable to find other characteristics

of giant cell fibroblastoma (GCF); this tumour shares many

clinical, histological and immunohistochemical features with

DFSP and the histogenic relationship between the two tumours

has been debated.

15,39,40

GCF is a mesenchymal tumour of

childhood that commonly recurs locally; it consists of dermal

and hypodermal proliferation of spindle cells arranged in a

storiform way, with myxoid areas, multinucleated floret-like

giant cells and pseudovascular sinusoidal-like spaces. Like

DFSP, these structures stain positively with CD34. There are

reports of cases of DFSP recurring with histological aspects of

GCF and vice-versa.

38,41

It is also possible to find ‘common’

cases of DFSP with areas resembling GCF. Cytogenetic studies

evidencing a common chromosomal aberration are a further

feature pointing to a relationship between the two neoplasms.

42

CD34, a human haematopoietic progenitor cell antigen is

a marker of endothelial cells and tumours of vascular origin; it

is also present in 20–30% of dermal dendritic cells (around

eccrine glands and the mid-portion of the hair follicle and inter-

stitially in the reticular dermis).

34

It has been considered for the

past few years to be the most important immunohistochemical

marker for the diagnosis of DFSP,

24,34,43,44

and our findings con-

cord with this idea. In the cases with fibrosarcomatous areas we

found CD34 expression in these foci (in a less intense way); this

finding, considered uncommon by some authors

19

is regarded

by Diaz-Cascajo

et al.

as important to distinguish DFSP with FS

areas from true fibrosarcoma, which does not express CD34.

38

Its use may become more common in the future in patients

submitted to several surgical treatments, as an adjuvant in

Moh’s surgery, to improve the accuracy of surgical margins

free of disease

45

because sometimes it is difficult to recognize

the difference between images of fibrosis and persistence of

tumour.

Acknowledgements

We thank Dr E Calonje for the diagnostic confirmation of the

case with myoid differentiation and Ms Luísa Borges, Ms Aldina

Curado, Mr Amadeu Ferro and Ms Isabel Silva for their work at

the histopathological laboratory.

References

1 Darier J, Ferrand M. Dermatofibromes progressifs et récidivants ou

fibrosarcomes de la peau.

Ann Dermatol Syphiligr

1924;

5

: 545–562.

2 Taylor RW, Helwig EB. Dermatofibrosarcoma protuberans: a study

of 115 cases.

Cancer

1962;

15

: 717–725.

3 Lautier R, Wolff HH, Jones RE. An immunohistochemical study

of dermatofibrosarcoma protuberans supports its fibroblastic

character and contradicts neuroectodermal or histiocitic

components.

Am J Dermatopathol

1990;

12

: 25–30.

4 Calonge E, Fletcher CDM. Myoid differentiation in

dermatofibrosarcoma protuberans and its fibrosarcomatous

variant: clinicopathological analysis of 5 cases.

J Cutan Pathol

1996;

23

: 30–36.

5 Shindo Y, Akiyama J, Takase. Tissue culture of dermato-

fibrosarcoma protuberans.

J Dermatol

1998;

15

: 220–223.

6 Hashimoto K, Brownstein MH, Jakobiec FA. Dermatofibrosarcoma

protuberans: a tumor with perineural and endoneural features.

Arch Dermatol

1974;

110

: 874–885.

7 Fletcher CDM, Evans BJ, Macartney JC,

et al.

Dermatofibrosarcoma

protuberans: a clinicopathological and immunohistochemical

study with a review of the literature.

Histopathology

1985;

9

:

921–938.

8 Bednar B. Storiform neurofibromas of the skin, pigmented and

nonpigmented.

Cancer

1957;

10

: 368–376.

9 Dupree WB, Langloss JM, Weiss SW. Pigmented dermatofibrosar-

coma protuberans (Bednar tumor): a pathologic, ultrastructural

and immunohistochemical study.

Am J Surg Pathol

1985;

9

:

630–639.

10 Fletcher CDM, Theaker JM, Flanagan A, Krausz T. Pigmented

dermatofibrosarcoma protuberans (Bednar tumour): melanocytic

colonization or neuroectodermal differentiation? A clinico-

pathological and immunohistochemical study.

Histopathology

1988;

13

: 631–643.

11 Frierson HF, Cooper PH. Myxoid variant of dermatofibrosarcoma

protuberans.

Am J Surg Pathol

1983;

7

: 445–450.


446

Oliveira-Soares

et al.


JEADV

(2002)

16

, 441–446

12 Betti R, Inselvini E, Crosti C. Unusual features of primary

dermatofibrosarcoma protuberans and its myxoid recurrence.

J Cutan Pathol

1996;

23

: 283–287.

13 Diaz-Cascajo C, Weyers W, Borghi S. Sclerosing dermato-

fibrosarcoma protuberans.

J Cutan Pathol

1998;

25

: 440–444.

14 Banerjee SS, Harris M, Eyden BP, Hamid BNA. Granular cell

variant of dermatofibrosarcoma protuberans.

Histopathology

1990;

17

: 375–378.

15 Shmookler BM, Enzinger FM, Weiss SW. Giant cell fibroblastoma.

A juvenile form of dermatofibrosarcoma protuberans.

Cancer

1989;

64

: 2154–2161.

16 Beham A, Fletcher CDM. Dermatofibrosarcoma protuberans with

areas resembling giant cell fibroblastoma: report of two cases.

Histopathology

1990;

17

: 165–182.

17 Zelger BW, Ofner D, Zelger BG. Atrophic variants of

dermatofibroma and dermatofibrosarcoma protuberans.

Histopatholoy

1995;

26

: 519–527.

18 Connelly JH, Evans HL. Dermatofibrosarcoma protuberans: a

clinicopathologic review with emphasis on fibrosarcomatous areas.

Am J Surg Pathol

1992;

16

: 921–925.

19 Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CDM.

Fibrosarcomatous (‘high-grade’) dermatofibrosarcoma

protuberans. Clinicopathologic and immunohistochemical study

of a series of 41 cases with emphasis on prognostic significance.

Am

J Surg Pathol

1998;

22

: 576–587.

20 Altman DA, Nickoloff BJ, Fivenson DP. Differential expression of

factor XIIIa and CD34 in cutaneous mesenchymal tumors.

J Cutan

Pathol

1993;

16

: 154–158.

21 Papadopoulos DJ, Greenway HT, Chung JY. Mohs surgery for

malignant fibrohistiocitic neoplasms.

Skin Cancer

1995;

11

: 73–79.

22 Ratner D

et al.

Mohs micrographic surgery for the treatment of

dermatofibrosarcoma protuberans.

J Am Acad Dermatol

1997;

37

:

600–613.

23 Brito C, Picoto A. Dermatofibrosarcoma Protuberans. Tratamento

por cirurgia micrográfica de Mohs.

Trab Soc Port Ven

1990;

48

: 206–

208.

24 Gloster HM. Dermatofibrosarcoma protuberans.

J Am Acad

Dermatol

1996;

35

: 355–374.

25 Chuang TY, Su WPD, Muller SA. Incidence of cutaneous T cell

lymphoma and other rare skin cancers in a defined population.

J Am Acad Dermatol

1990;

23

: 254–256.

26 Bouyssou-Gauthier ML, Labrousse F, Longis B

et al.

Dermatofibrosarcoma protuberans in childhood.

Pediat Dermatol

1997;

14

: 463–465.

27 McKee PD, Fletcher LDH. Dermatofibrosarcoma protuberans pre-

senting in infancy and childhood.

J Cutan Pathol

1991;

18

: 241–246.

28 Pappo AS, Rao BN, Cain A, Bodner S, Pratt CB.

Dermatofibrosarcoma protuberans: The pediatric experience at

St. Jude Children’s Res Hospital.

Pediat Hematol Oncol

1997;

14

:

563–568.

29 Sachse MF, Cabeçadas J, Pecegueiro M, Amaro J.

Dermatofibrosarcoma protuberans. A retrospectice study of 10

cases.

Skin Cancer

1997;

12

: 59–67.

30 Groetschel H, Cramer HJ. Multilokulär symmetrisches

Dermatofibrosarcoma protuberans.

Dermatol Wochenschr

1967;

153

: 574–582.

31 Garcia C, Viehman G, Hitchcock M, Clark RE. Dermatofibro-

sarcoma protuberans treated with Mohs surgery. A case with CD34

immunostaining variability.

Dermatol Surg

1996;

22

: 177–179.

32 Rinck PA, Habermalz HJ, Lobeck H. Effective radiotherapy in one

case of dermatofibrosarcoma protuberans.

Strahlentherapie

1982;

158

: 681–685.

33 Aiba S

et al.

Dermatofibrosarcoma protuberans is a unique

fibrohistiocytic tumour expressing CD34.

Br J Dermatol

1992;

127

:

79–84.

34 Kutzner H. Expression of the human progenitor cell antigen CD34

(HPCA-1) distinguishes dermatofibrosarcoma protuberans from

fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue.

J Am Acad Dermatol

1993;

28

: 613–617.

35 Elder D, Elenitsas R, Jaworsky C, Johnson B Jr editors.

Lever’s

Histopathology If the Skin

, 8th ed. Lippincott-Raven, Philadelphia,

1997.

36 Kamino H, Jacobson M. Dermatofibroma extending into the

subcutaneous tissue: differential diagnosis for

dermatofibrosarcoma protuberans.

Am J Surg Pathol

1990;

14

:

1156–1164.

37 Zelger B, Sidoroff A, Stanzl U

et al.

Deep penetrating

dermatofibroma versus dermatofibrosarcoma protuberans.

Am J

Surg Pathol

1994;

18: 677–686.

38 Diaz-Cascajo C, Weyers W, Borrego L, Iñanea JB, Borghi S.

Dermatofibrosarcoma protuberans with fibrosarcomatous areas:

a clinico-pathologic and imunohistochemic study in four cases.

Am J Dermatopathol 1997; 19: 562–567.

39 Sigel JE, Bergfeld WF, Goldbwim JR. A morphologic study of

dermatofibrosarcoma protuberans: expansion of a histologic

profile. J Cutan Pathol 2000; 27: 159–163.

40 Harvell JD, Kilpatrick SE, White WL. Histogenic relations between

giant cell fibroblastoma and dermatofibrosarcoma protuberans.

Am J Dermatopathol 1998; 20(4): 339–345.

41 Perry DA, Shultz LR, Dehner LP. Giant cell fibroblastoma with

dermatofibrosarcoma protuberans-like transformation. J Cutan

Pathol 1993; 20: 451–454.

42 Dal Cin P, Sciot R, Wever I et al. Cytogenetic and

immunohistochemical evidence that giant cell fibroblastoma is

related to dermatofibrosarcoma protuberans. Gene Chromosomes

Cancer 1996; 15: 73–79.

43 Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differencial

diagnosis of dermatofibroma and dermatofibrosarcoma

protuberans. Am J Dermatopathol 1993; 15: 429–434.

44 Goldblum JR, Tuthill RJ. CD34 and factor XIIIa immunoreactivity

in dermatofibrosarcoma protuberans and dermatofibroma.

Am J Dermatopathol 1997; 19: 147–153.

45 Haycox CL, Odland PB, Olbright SM, Pierkorn M.

Immunohistochemical characterization of dermatofibrosarcoma

protuberans with practical applications for diagnosis and

treatment. J Am Acad Dermatol 1997; 37: 438–444.


Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade ( …

Documents