CT showing Right sided pelvic mass lesion of size 73x 93 x 110,causing erosion of right iliac wing and infiltrationof right illiacus muscle Rt sided pelvic mass lesion causing erosion of
right iliac wing and infiltration of right illiacus muscle
The lung fields showed multiple metastatic lesions on chest x ray.Tru cut biopsy of the pelvis mass was done . Microscopic examination, which included histopathologic and immunohistochemical
analysis with CD-34, confirmed the diagnosis of DFSP.
It was first described in 1924 as a progressive and recurrent dermatofibroma(6). The trunk and proximal extremities are the most frequent locations of the disease, but it can occur at any
DFSPs rarely progress to a high-grade fibrosarcomatous component(7) Histologically, DFSP is identified by a pattern of monomorphous proliferation of cytological bland spindle cells with
a visible storiform or whorled (rushmat-like) architecture (8).
Other characteristic features are low mitotic activity and deep, honeycomb infiltration into subcutaneous adipose tissue .
The expression of CD34 is almost a consistent finding and it is extremely useful in differentiation of DFSP from benign fibrous histiocytoma , dermatofibroma and other soft tissue tumors .
The sensitivity of CD34 staining in DFSP ranges from 84 to 100% .
The Metastases occurs mostly to lungs, followed by the regional lymph nodes. Generally, distant metastases is seen after recurrent local relapses.(9,10)
MRI is useful to demonstrate deep tissue invasion, especially in the advanced stages. Nuclear study is useful for ruling out bone invasion.
The preferred therapy of DFSP is radical (wide) surgical excision. The most significant prognostic factor in patients with DFSP has proved to be the extent of surgical resection. The success
of the initial surgical excision has a major effect on the outcome as well. In fact, if this procedure fails and hence, the tumor recurs, it could lead to an uncontrollable local growth, as seen
in our case.
Due to its infiltrative nature, DFSP is characterized by a high recurrence
rate varying in the literature from 10-80% (11) Radiotherapy is of limited value in the treatment of DFSP. However, it might have some role to play when the resection border is positive or when extensive excision is not possible due to cosmetic or functional difficulties.Imatinib mesylate, a drug
used in chronic myelogenous leukemia, has been successful in metastatic disease and/or relapses of the entity.
We discussed this case in our department and patient was kept on Tab imatinib.800mg/Day.
DSFP could be uncontrollable and challenging to cure. So Aggressive management of the dermatofibro sarcoma protuberans should be done .