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.

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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.

BMJ 2011;342:609