dematofibrosarcoma of vulva

1. DEPT. OF OBG- I AND PLASTIC
SURGERY

2. • Mrs. X , 57 years old
• Housewife
• W/O Mr. Y
• Resident of Bangalore,
• Middle socio- economic status

3.  Complaints of -Swelling over the vulval region
- since past 15 years
-increased in size over past 2 years
-no complaints of any discharge, pain,
pigmentation, associated fever.

4.  History of similar swelling 20 years ago; for which
patient underwent excision.
 On 18/03/13 – underwent biopsy; showing
dermatofibrosarcoma protuberans

5. • Patient is moderately built and nourished
• Weight- 64 kg/ height- 160 cms ; BMI – 25
• No pallor,icterus, cyanosis, clubbing ,
lymphadenopathy
• Pulse- 74 bpm ; BP- 130/80 mmHg
• Breast and Thyroid - normal

6. • Per Abdomen – soft, non tender, no mass, no
organomegaly, no ascites , tubectomy scar healed
well with primary intention
• Local examination:
- firm to hard multiple lesion present
over the mons pubis and left labia majora each
measuring about 0.5 to 2 cm, spread over an area
of about 8 cms.
- largest lesion over the left labia majora
- the lesion crossing over the midline,
involving the clitoris
-induration present, non tender

9.  Plan for
Wide excision followed by skin
grafting

14.  Dermatofibrosarcoma protuberans:
- A rare neoplasm : 0.1% of all malignancies
- tumor of intermediate grade malignancy
- typically occur between 20- 50 years of age;
frequently on trunk.
- DFSP of the clitoris and vulva are rare; DFPS of
vulva typically arises in the left labium majus

15. • Clinical features:
- present as solid protuberant nodule.
-indolent in nature
-pain and ulceration can occur with tumors in
an accelerated growth phase.
- aggressive local growth.
- lymphatic spread is rare; metastasis <6% ,
lungs , bones and brain.

16.  Pathology:
- arises from stem cell within the dermis
-finger-like projections into surrounding
tissue, subsequently, can invade subcutaneous
tissue, muscle and bone.
- Histology: fibroblasts arranged in a
“storiform” pattern.
- immunohistochemical staining for CD34
differentiate from other tumors

18.  Cytogenetic studies of DFSP cells revealed
specific chromosomal abnormalities as
supernumerary ring chromosomes
containing chromosome 17 and 22.

19.  - Wide local excision :
removal of the tumor along with 2-3 cms
of normal looking skin
recurrence rate is around 20 %
- Moh’s Micrographic
- uses systematic horizontal sectioning
with 100% of the tumor margins being
microscopically examined
- local recurrence rare , 7% over period of
past 5 years.

20.  Chemotherapy
-proved useful in cases of recurrence or those
who are not amenable to surgery.
- targeted molecular therapy by arresting
tumor growth by blocking PDGF- alpha , beta,
c-abl activity.
-Imanitib mesylate , selective tyrosine
kinase inhibitor, with efficacy against the PDGF
receptors.

21.  Radiotherapy has been shown to be effective
when the lesion is not resectable or a postoperative
therapeutic option when complete excision is not
possible.

22.  Survival rates range from 91 to 100%.
 Local recurrence rates of 20 to 49% have been
reported.
 Therefore close follow-up is recommended; every
3-6 months for 3 years followed by annual
examination.

23.  Only 29 cases of DFSP in the vulva has reported.
 Out which 13 cases where followed up for a period
ranging from -2 months to 444 months.
- 9 patients there was no evidence of
disease
- 2 patients alive with the disease
- 1 patient died due disease
- 1 patient died due other cause

26.  Prolonged hospital stay.
 Immediate complications – pain ,fever, infection,
immobilization
 Long term complications – graft failure,
impairment of sensation, contarture,recurrence

27.  Rook’s Textbook of Dermatology
 Obstetrics and Gynecology International, Hindawi
 Journal of surgical case reports
 Pubmed