a rare case of dermatofibrosaroma protuberans
TRANSCRIPT
![Page 1: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/1.jpg)
DEPT. OF OBG- I AND PLASTIC SURGERY
A RARE LESION OF VULVA
![Page 2: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/2.jpg)
• Mrs. X , 57 years old
• Housewife
• W/O Mr. Y
• Resident of Bangalore,
• Middle socio- economic status
![Page 3: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/3.jpg)
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
![Page 4: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/4.jpg)
History of similar swelling 20 years ago; for which patient underwent excision.
On 18/03/13 – underwent biopsy; showing dermatofibrosarcoma protuberans
Contd…
![Page 5: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/5.jpg)
• 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
![Page 6: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/6.jpg)
• 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
![Page 7: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/7.jpg)
![Page 8: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/8.jpg)
![Page 9: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/9.jpg)
Plan for
Wide excision followed by skin grafting
![Page 10: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/10.jpg)
![Page 11: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/11.jpg)
![Page 12: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/12.jpg)
![Page 13: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/13.jpg)
![Page 14: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/14.jpg)
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
![Page 15: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/15.jpg)
• 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.
![Page 16: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/16.jpg)
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
![Page 17: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/17.jpg)
![Page 18: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/18.jpg)
Cytogenetic studies of DFSP cells revealed specific chromosomal abnormalities as supernumerary ring chromosomes containing chromosome 17 and 22.
![Page 19: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/19.jpg)
- 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
![Page 20: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/20.jpg)
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…
![Page 21: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/21.jpg)
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…
![Page 22: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/22.jpg)
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.
![Page 23: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/23.jpg)
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
![Page 24: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/24.jpg)
![Page 25: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/25.jpg)
![Page 26: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/26.jpg)
Prolonged hospital stay.
Immediate complications – pain ,fever, infection, immobilization
Long term complications – graft failure, impairment of sensation, contarture,recurrence
Post operative period
![Page 27: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/27.jpg)
Rook’s Textbook of Dermatology
Obstetrics and Gynecology International, Hindawi
Journal of surgical case reports
Pubmed
REFRENCES
![Page 28: A rare case of dermatofibrosaroma protuberans](https://reader035.vdocument.in/reader035/viewer/2022062514/55beb5eebb61eb363c8b46de/html5/thumbnails/28.jpg)