Saturday, November 17, 2018

CCH_ some skin disorders _dermatofibrosarcoma protuberans (DFSP)

uncommon locally malignant skin tumors

They arise in the deeper layer of the skin (the dermis). It grows slowly but has a tendency to recur after excision. Luckily, it rarely spreads to other sites beyond the skin.



I had a classmate who is the CEO of a chain of multispeciality hospitals in Hyderabad, first had this  locally excised when we were in medical school. ( this was  all hush hush I became privy to this information  after a long time )
It recurred after 5 or 6 years when I excised it when I was an assistant professor of general surgery.

when the histology came back I advised him to have an extended excision ,he waited for few more years  before  getting  a wider excision done by Oncological surgeon Dr.Chandrashekar working  at MNJ cancer hospital Hyderabad. I have not asked him recently how he is doing.

The cause of DFSP is unknown, but an injury to the affected skin may be a predisposing factor. Recent advances show tumor cells carry abnormal chromosomes within the tumor cells
DFSP is rare, and affects less than 1 person in every 100,000

presents as a painless thickened area of skin (plaque) and/or nodule that feels rubbery or firm to touch and is fixed to the underlying skin. It may be red-brown or skin coloured. It usually grows very slowly over months to years.
Often diagnosed when it enters a more rapid growth phase giving rise to larger lesions. Neglected tumours may reach large proportions
 wide excision of the lesion including deep fascia, with 1–3 cm margin of normal skin. This may take more than one surgical procedure to ensure complete removal of the tumour.

rarely 
radiotherapy for locally nonresectable tumors  or  The tyrosine kinase inhibitor imatinib mesylate is used to treat rare cases of local advanced inoperable or metastatic dermatofibroscarcoma

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