A Rare Case of Scalp Dermato-Fibrosarcoma-Protuberans with Intracranial Extension and Distant Soft Tissue and Liver Metastasis
A.K. Gupta*, Hari Singh**, Aneesh M.M.***, Nitin Jaiswal***, Nitish Arora***
*Professor and Head, **Consultant, ***Residents, Department of Radiodiagnosis, S. N. Medical College, Agra, U.P., India
Abstract
Dermato-fibrosarcoma-protuberans (DFSP) is a rare skin tumour which typically
arises in the dermis as a multinodular mass, and then spreads into the subcutaneous
tissues and muscle but rarely metastasise. Only few cases of scalp DFSP with
intracranial and distant metastasis have been reported. Here we report a
histopathologically proven case of DFSP of scalp with intracranial extension and
distant metastasis in liver and soft tissue metastasis to chest wall and thigh,
evaluated with ultrasound and CT.
Introduction
ermato-fibrosarcoma-protuberans D(DFSP) is a relatively uncommon
slow growing locally aggressive fibrous 1,2tumour of skin. Regional and distant
metastases from DFSP do occur but are 3rare, probably less than 1%. The term
"dermato-fibrosarcoma-protuberans" was 4coined by Hoffman in 1925.
Case Report
A 42 year old male presented with a large
gradually increasing swelling in right occipital region
since 2 years. He also complained of similar swellings
in chest wall, right arm and right thigh since 10
months. At the time of presentation to our
department, FNAC was done from thigh, chest wall
and scalp swellings, which showed clusters of spindle
and oval shaped cells in myxoid stroma, likely
suggestive of benign spindle cell lesion. The chest
wall lesion was operated and histopathology of chest
wall lesion was done, which showed, fascicles of
spindle shape cells arranged in storiform pattern (Fig.
1), suggestive of dermato-fibrosarcoma-protuberans.
Examination by surgeons revealed a patient in
general good health with a large, non-tender, firm
and non pulsatile mass with smooth surface in right
occipital region. The tumour was tethered to the
overlying skin. No lymph node in the neck, occipital
region, or axilla palpable.
Contrast enhanced CT scan of head showed
mildly enhancing, soft tissue attenuation mass lesion
in right parieto-occipital region measuring
approximately 7.1 x 3.3 cms in size. Intracranial
extension was seen (extradural in location measuring
3.9 x 1.5 cms), through a defect in right side of
parieto-occipital bone measuring 3.5 cms in size (Fig.
2). The intracranial component was displacing the
underlying parietal lobe. Abdominal ultrasound
showed extensive metastatic deposits scattered in
whole of liver parenchyma measuring 1.5-4 cms in
size. The metastases were hypoechoic, hyperechoic,
and also showing target and reverse target signs.
Some metastatic deposits were necrotic. No evidence
of ascites/peritoneal deposits seen. Chest X-ray
showed no evidence of lung metastasis. The patient
was advised palliative treatment. Since FNAC of the
chest wall swelling, as well as thigh and scalp
swelling showed similar findings, the scalp swelling
was assumed to be the primary lesion as it was the
largest and first lesion to appear.
Fig. 1 (a) and (b) : Microscopical view with 10X
Bombay Hospital Journal, Vol. 54, No. 1, 2012 145
magnification (a) and 40X magnification (b) of the
chest wall specimen showing fascicles of spindle
shape cells arranged in 'storiform' pattern.
Fig. 2 (a), (b) and (c) : Non-contrast CT scan of
head (a) showing soft tissue attenuation mass lesion
in right parieto-occipital region with intracranial
extension (extradural) through underlying bony
defect. Contrast enhanced CT scan (b) showing mild
enhancement of both extra and intracranial
component. Bone window (c) clearly showing a defect
in right side of parieto-occipital bone.
Discussion
DFSP is a low grade sarcomatous
tumour resembling keloid as it has a
marked tendency to recur locally if
inadequate surgical resection margins are 1obtained. Several studies reveal almost
equal sex distribution or a slight male 2preponderance. Maximum incidence was
nd rd 2in the 2 and 3 decades. Lesions ranging
from 1 cm to more than 25 cm have been
described in the literature. Most lesions
occur over the trunk or proximal 5extremities.
Whenever imaging is used, it is often to
evaluate larger primary lesions or
recurrent disease. CT is helpful in deciding
the line of incision and avoiding
inadequate excision, which leads to 2recurrence or metastasis. MRI is
invaluable in the evaluation DFSP,
allowing accurate localisation before
surgery. T1-weighted MRI with i.v.
gadolinium administration shows
enhancement of lesions. STIR sequences
provided fat suppression allows clear
6definition of the tumour. On MRI a
subcutaneous mass lesion which crosses
the superficial fascia, malignancy is 6.88
times greater than that for lesions that did 6not cross the fascia. After review of
literature we could not find any accurate
portrayal of the typical imaging features of
DFSP.
Histologically DFSP looks like
dermatofibroma but more infiltrative.
Uniform slender fibroblasts arranged in
distinct 'storiform pattern' with low to
moderate mitosis or cells radiating from a
central hub of fibrous tissue forming a
'cartwheel pattern' is seen. CD-34
positivity (by immuno-histochemical
staining) is useful to differentiate it from 2,7dermatofibroma. Condensation of
connective tissue at the periphery may
give a false appearance of encapsulation
but actually tumour may extend well
beyond marg ins in microscopic 2projection. In general, wide surgical
excision of DFSP followed by immediate
reconstruction is considered necessary to 8avoid recurrence of the tumour. Prognosis
is excellent after adequate surgical
excision, ensuring adequate margin of 3 to 15 cm. Micrographic controlled excision
3(MCE) also showed good results.
Radiation hitherto with a limited role
recently has been used as adjunct to
surgery. Molecular targeted therapy holds
a promise for the future; Imatinib may
provide an alternative treatment for
unresectable tumours or adjunctive 3treatment in addition to surgery.
Conclusion
DFSP is a rare, usually superficial soft
tissue sarcoma, which is often diagnosed
histopathologically. CT imaging allows
146 Bombay Hospital Journal, Vol. 54, No. 1, 2012
only preoperative assessment of extent
and bony involvement, but without any
characteristic diagnostic sign. MR imaging
allows accurate preoperative assessment.
References
1. V.D Sinha, S.R Dhalkar, G.S Kalra.
Dermatofibrosarcoma protuberans of scalp: a
case Report. Neurol India. 2001; 49:81-83.
2. ME Asuquo, MS Umoh, G Ebughe.
Dermatofibrosarcoma protuberans. Ann Afr
Med. 2007; 6:80-83.
3. A Ah-Weng, J R Marsden, DSA Sanders, and R
Waters. Dermatofibrosarcoma protuberans
treated by micrographic surgery. Br J Cancer.
2002; 87: 1386-1389.
4. H o f f m a n n E . D e r m a t o f i b r o s a r c o m a
protuberans. Dermatol Z. 1925; 43:1-28.
5. Cakir B, Misirlioglu A, Gideroglu K, Akoz T.
G i a n t f i b r o s a r c o m a a r i s i n g i n
dermatofibrosarcoma protuberans on the scalp
during pregnancy. Dermatol Surg. 2003; 29:
297-299.
6. William C. Torreggiani, Khalid Al-Ismail, Peter L,
Munk et al. Dermatofibrosarcoma Protuberans:
MR Imaging Features. Am J Roentgenol. 2002;
178:989-993.
7. D i w a n A H , H o r e n s t e i n M G .
Dermatofibrosarcoma protuberans association
with nuchal-type fibroma. J Cutan Pathol. 2004;
31:62-6.
8. Parker TL, Zitelli JA. Surgical margins for
excision of dermatofibrosarcoma protuberans. J
Am Acad Dermatol. 1995; 32:233-236.
Bombay Hospital Journal, Vol. 54, No. 1, 2012 147
Excess mortality in osteoarthritis
Provides evidence for a unified approach to musculoskeletal ageing
Osteoarthritis typically affects the hands, knees, hips, spine, and feet. Previous studies have attributed higher death rates in patients with osteoarthritis to concomitant risk factors for the disorder (most notably obesity) or to treatment with non-steroidal anti-inflammatory drugs.
The 1163 participants (denominator population 26046) aged 35 years and over who had symptomatic radiographically defined osteoarthritis at these two joint sites showed a significant excess in all cause mortality; cause specific mortality was particularly high for cardiovascular disease and dementia.
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