DEPT. OF OBG- I AND PLASTIC SURGERY

A RARE LESION OF VULVA

• Mrs. X , 57 years old

• Housewife

• W/O Mr. Y

• Resident of Bangalore,

• Middle socio- economic status

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.

History of presenting illness

History of similar swelling 20 years ago; for which patient underwent excision.

On 18/03/13 – underwent biopsy; showing dermatofibrosarcoma protuberans

Contd…

• 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

Examination

• 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

Plan for

Wide excision followed by skin grafting

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

DISCUSSION

• 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.

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

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

- 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.

TREAMENT

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.

Contd…

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

Contd…

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.

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

Review of literature

Prolonged hospital stay.

Immediate complications – pain ,fever, infection, immobilization

Long term complications – graft failure, impairment of sensation, contarture,recurrence

Post operative period

Rook’s Textbook of Dermatology

Obstetrics and Gynecology International, Hindawi

Journal of surgical case reports

Pubmed

REFRENCES