QIUYU YU1, YANG YANG1, RENJUN HUANG1, YONGGANG LI1, NAIHUI ZHOU2, PING LI1, PENG WU3, and MENGXIAO TANG4
1Radiology, The First Affiliated Hospital of Soochow University, SUZHOU, China, 2Dermatology, The First Affiliated Hospital of Soochow University, SUZHOU, China, 3Philips Healthcare, SHANGHAI, China, 4Radiology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, HANGZHOU, China
Synopsis
Dermatofibrosarcoma
protuberans (DFSP) is a type of intermediately malignant cutaneous spindle cell
neoplasms, which is easy
to be confused with several benign ones even after needle biopsy, especially
cellular fibrous histiocytoma (cFH), resulting in inadequate excision and local
recurrence. We found that as a novel skin imaging technique, high-resolution
(HR) DCE MRI could distinguish DFSP. The features include
infiltration of surrounding fat, ill-defined margins and large quantitative
parameters. Both DFSP and cFH have type-III time-signal intensity curves (TICs).
In contrast, other confused lesions presented type-II-or-I TIC. The
recommendation of preoperative HR-MRI could assist dermatologists to perform
surgical plan more confidently.
Introduction
DFSP is an uncommon cutaneous
fibrohistiocytic neoplasm, which accounts for 1% of all soft tissue sarcomas,
with an incidence of 0.8 to 5 cases per million population each year1.
Typically, DFSP presents slowly progressive, painless cutaneous protuberance that
may begin as a pink plaque. It is an intermediately malignant (rarely metastasizing)
cutaneous spindle cell neoplasm2, presenting diffuse spindle cells
arranged in a storiform pattern under light microscopes. The tumor cells
infiltrate subcutaneous fat in a honeycomb pattern3. CD34 is usually
positively expressed while factor XIII-a and CD68 are negative1.
DFSP is notable for its high local recurrence after surgical resection. The recurrent
lesions are prone to sarcomatous transformation and metastasis4 peculiarly.
Wide local excision (WLE) is recommended to decrease the recurrence rates, and
2 to 3 cm margins are considered to be appropriate5 generally. The determination
of invasion layers is crucial especially when tumors invade the underlying
fascia or muscle. DFSP is easy to be confused with several benign spindle cell
neoplasms, including fibrous histiocytoma (FH, also known as dermatofibroma) especially cellular variants (cFH),
keloids and nodular fasciitis (NF). The
aim of this study was to explore clinical values of dynamic contrast-enhanced (DCE)
HR-MRI in differential diagnosis of DFSP.Methods
Subjects: A total of 28 patients with clinical suspicion of
DFSP (15 males, age range: 6-69 years) were enrolled.
Acquisition Protocol: All examinations were performed using a dedicated 7
centimeter-diameter single-element surface coil with 32 receiver channels on a
3T MRI scanner. The coil was placed as
close to the lesions as possible, meanwhile keeping the lesion as central as
possible6. High-resolution non-contrast T1-and-T2-weighted
images (T1WI and T2WI) were acquired firstly. Parameters
were as follows: T1WI, repetition time (TR) 612 microsecond (ms), echo
time (TE) 14ms, 1 time signal averaging, field of view (FOV) 80*80 mm2,
matrix 320*320, section thickness 1-5 mm, turbo factor 3, voxel size
0.3*0.3*2.0 mm3. T2WI, TR 4790 ms, TE 96 ms, 2 signal
averaging, FOV 80*80 mm2, matrix 384*384, section thickness 1-5 mm, turbo factor 6,
voxel size 0.2*0.2*2.0 mm3. Bi-flip-angle T1 mapping
sequences were performed prior to DCE scanning, with a flip angle of 2° and
15°. DCE-MRI was performed after intravenous administration of 0.2 mmol/kg
gadopentetate dimeglumine (Gd-DTPA) at a rate of 3 mL/s. 36 phases were
collected. The relevant scanning parameters were as follows: TR 4.21ms, TE
1.42ms, 1 signal averaging, FOV 142*142 mm2, matrix 256*256, section
thickness 3 mm, voxel size 0.6*0.6*3.0 mm3.
Data Processing: Two radiologists who were blinded to all histopathological
results evaluated HR-MRI features of all cases independently. Consensus was reached
through consultation in case of any disagreement. Evaluated features included T1 and T2
signal, depth, major
diameter, morphology, margin, cutaneous involvement
layer and enhancement features. One radiologist, who was
also blinded to the pathological data, drew oval regions of interest (ROIs) on
the images using commercial post-processing software called Tissue 4D. 1-2 cm2
ROIs were placed several times in homogeneous enhancement regions as large as
possible. Quantitative parameters of ROIs were automatically generated based on
a modified hemodynamic two-compartment Tofts model, including Ktrans,
Kep, Ve and iAUC. MeanCurve, a commercial software, was
used to generate TICs.
Analysis: The continuous variables were
given as mean ± standard deviation. All data analysis was performed using SPSS 23.0.
Fisher exact test was used to analyze whether TIC types were different between groups.
One way ANOVA was used to compare quantitative parameters of DFSP, FH and
keloid. The difference was statistically significant when P <0.05.Results
7 cases of
DFSP, 9 cases of FH (4 cFH), 11 cases of keloid and 1 case of NF were
collected. DFSP presented relatively
high T2WI signal, ill-defined margins, irregular morphology and infiltration
of surrounding fat (signs included tiny
spinous protrusion, crab-foot-like spicules of the basal, satellite lesions and
broken capsule
(Figure 1). TIC types between lesions
were significantly different. Most of DFSP and cFH had type-III TIC (10 cases
out of 11 cases). The comparison of quantitative perfusion parameters is shown
in Table 1, with DFSP having the highest values. Of all cases, HR-MRI displayed
that 1 case
involved muscle, 5 cases involved deep fascia,
11 cases involved subcutis, and 11 cases involved dermis. All patients undergoing preoperative HR-MR got pathologically
negative basal margin after the first operation.Discussion
Due to
its high-resolution presentation, HR-MRI could clearly reveal the infiltration
of surrounding fat in DFPS, which coincided with the honeycomb-pattern
infiltration of spindle cells into subcutaneous fat in pathology. This is
the most compelling evidence that supports DFSP diagnosis. CFH had vague
margins in some cases but no fat infiltration signs (Figure 2). Both cFH and
DFSP have theoretical risk of local recurrence7, similar necessity
for WLE (cFH needs 1-2cm margins )8, and type-III TIC, which means type-III TIC
might suggest potential recurrence risk and regular follow-up
after operation should be emphasized.
Quantitative perfusion parameters might help in the identification of DFSP.
When it comes to operation planning, invasion layers determined by HR-MRI are
reliable.Conclusion
HR-MRI is a
reliable imaging technique in differentiating DFSP from other confusing
cutaneous spindle cell neoplasms.Acknowledgements
This work was mainly
supported by the program for Gusu Medical talent of Suzhou city(grant number GSWS2020009, the Translational Research Grant of
NCRCH(grant number2020WSB06,
National Natural Science Foundation of China (grant number 81671743), the
clinical key diseases diagnosis and therapy special project of Health and Family
Planning Commission of Suzhou (LCZX201801), the program for Advanced Talents
within Six Industries of Jiangsu province (WSW-057), and the High-level Health
Personnel “six-one” Project of Jiangsu province in China (LGY2016035).References
1. Allen A, Ahn C, Sangüeza OP. Dermatofibrosarcoma
Protuberans. Dermatol Clin. 2019;37(4):483-488.
2. Choi JH, Ro JY. The 2020 WHO Classification of
Tumors of Soft Tissue: Selected Changes and New Entities. Adv Anat Pathol.
2021;28(1):44-58.
3. Choi JH, Ro JY. Cutaneous Spindle Cell Neoplasms:
Pattern-Based Diagnostic Approach. Arch Pathol Lab Med. 2018;142(8):958-972.
4. Grizzetti L, Gaide O, Kuonen F. Dermatofibrosarcoma
protuberans : clinico-pathological aspects and management. Rev Med Suisse.
2020;16(688):640-645.
5. Acosta AE, Santa Ve´lez C. Dermatofibrosarcoma protuberans.
Curr Treat Options Oncol. 2017;18(9):56.
6. Budak MJ, Weir-McCall JR, Yeap PM, et al.
High-Resolution Microscopy-Coil MR Imaging of Skin Tumors: Techniques and Novel
Clinical Applications. Radiographics. 2015;35(4):1077-1090.
7. Hornick JL. Cutaneous soft tissue tumors: how do we
make sense of fibrous and "fibrohistiocytic" tumors with confusing
names and similar appearances?. Mod Pathol. 2020;33(Suppl 1):56-65.
8. Berklite L, Ranganathan S, John I, Picarsic J,
Santoro L, Alaggio R. Fibrous histiocytoma/dermatofibroma in children: the same
as adults?. Hum Pathol. 2020;99:107-115.