|Ahead of print publication
Neglected giant dermatofibrosarcoma protuberans
Gautam Prakash, Manojit Midya, Pankaj Sharma, Rakesh K Jain
Department of Burns, Plastic and Reconstructive Surgery, SMS Medical College, Jaipur, Rajasthan, India
|Date of Submission||24-May-2018|
|Date of Decision||18-Jun-2018|
|Date of Acceptance||10-Sep-2018|
F-27, Resident's Doctors Hostel, SMS Medical College, Jaipur - 302 004, Rajasthan
Source of Support: None, Conflict of Interest: None
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, intermediate to low grade malignant soft tissue neoplasm originating from the dermal layer of the skin. Scalp DFSP is even a rarer entity occurring at less than 1% of all DFSP. We recently encountered a massive DFSP on the scalp which presented to us in the emergency settings with acute bleeding. This was successfully managed by wide local excision of the tumour. This case is remarkable due to the large size of tumour on the rather rare site on the scalp presenting unusually as acute bleeding and its successful management with wide local excision as a life saving procedure.
Keywords: Dermatofibrosarcoma protuberans, Mohs micrographic surgery, scalp, wide local excision
| Introduction|| |
Dermatofibrosarcoma protuberans (DFSP) is a rare,, slow growing,,, intermediate to low-grade malignant soft-tissue neoplasm originating from the dermal layer of the skin. It is most commonly seen on the trunk followed by proximal extremities and the head and neck region. It accounts for approximately <5% of adult soft tissue sarcomas and <1% of all malignant tumors of the head and neck region. Scalp DFSP is even a rarer entity occurring at <1% of all DFSP. We describe a remarkable case of a giant DFSP of the scalp that came to us in the emergency department and discusses the salient clinicopathological aspects of the tumor along with its management.
| Case Report|| |
A 28-year-old moderately built patient came to us in the emergency department with bleeding from the scalp. He was referred from community health center of a nearby subdivisional town. His brother gave a history of the presence of mass lesion on the head for the last 12 years. It was insidious in onset and gradually progressive in nature. They also revealed quack treatment for all these years. There was no history of trauma to the head although the relatives gave a history of on and off bleeding from a tumor over his head. We were not aware of that history till then.
On presentation, we could find a bulky cotton wool dressing over his head which was soaked in blood. He was conscious, alert but not responding to our query probably fearing that any movement could ensure further bleeding.
There was evident pallor and sweat over his face although he was calm all this while. His Pulse was 118/min, thready, blood pressure 86/56 mmHg on the right arm supine position. He was tachypneic with a respiratory rate of 28/min. A quick survey of rest of general physical and systemic examination was essentially normal.
Two wide bore intravenous cannulae were introduced over the forearm veins, and bolus Ringer's lactate was started. In the meantime, cross-matching of the blood sample was done to arrange packed cells, and emergency operation theatre (EOT) staff kept ready for any eventuality.
After initial resuscitation, the dressings were gently removed to inspect the bleeding source. We were astonished to find a huge tumor arising from the vertex of the scalp. It was profusely bleeding from the exposed interiors.
He was immediately shifted to the EOT. It was decided to excise a tumor in its entirety as a life-saving procedure. A formal informed written consent was taken regarding the procedure and poor prognosis of the situation in general. Under general anesthesia, the tumor was gently exposed on the OT table [Figure 1].
|Figure 1: The tumor shows variegated surface appearance with necrotic, sloughed out tip|
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Initially, deep quilting sutures were taken all around the tumor to cut off the feeding arterial supply and keep bleeding under control [Figure 2]. Once bleeding was controlled, we proceeded to excise the tumor using diathermy. A wide margin of 5 cm was ensured all around the tumor in the periphery.
|Figure 2: Deep quilting sutures (arrow head) in place all around the tumor|
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On dissecting the under surface of the tumor, it was found to abut the outer cortex of the skull bone. An intraoperative neurosurgery intervention was done, and the involved outer cortex of skull bone was chiselled out. The small exposed underlying dura mater of size 5 cm × 4 cm was inspected thoroughly and was found to be free of any tumor deposits. Split-thickness skin graft was placed over the exposed scalp defect as an interim procedure in the emergency settings [Figure 3]. The excised specimen measured approximately 16 cm × 14 cm × 9 cm in the greatest dimensions [Figure 4] and weighed 2575 g. The excised specimen was sent for histopathological examination which confirmed the diagnosis of DFSP with negative surgical margins [Figure 5].
|Figure 3: The entire scalp defect covered with split thickness skin graft. The encircled area shows the split thickness skin graft over the dura mater|
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|Figure 4: The excised tumor specimen measuring 16 cm × 14 cm × 9 cm in the greatest dimensions|
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|Figure 5: Histopathological examination (H and E, ×100) reveal poorly circumscribed uniform population of spindle cells in storiform pattern|
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The postoperative period had been uneventful [Figure 6]. He has been in our follow-up for 8 months now. He has maintained stable health till now with no evidence of tumor recurrence at the local site. Postoperative metastatic workup also showed no evidence of distant metastasis. We plan to reconstruct his scalp defect soon.
|Figure 6: Postoperative photograph on day 15 of the operated area showing well healed wound with split thickness skin graft. The encircled area shows satisfactory graft take over the dura mater|
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| Discussion|| |
DFSP is a low grade, uncommon soft-tissue sarcoma that was originally described in 1924 by Darier and Ferrand. The term DFSP was coined by Hoffman in 1925. It is commonly seen in persons in the third or fourth decades of life with a slight male predominance. Congenital DFSP has been reported in literature although it is unusual in children.
In one large case series, the average tumor size has been reported to be fewer than 5 cm, and there is handful of cases of tumor size more than 10 cm. This is probably the largest size DFSP on the scalp reported in the English literature. Cases of patient's neglect resulting in giant basal cell carcinoma have reported earlier, and patient's relatives play an imperative role in the treatment process. In this case, also, patient's brother played cardinal role to instill confidence and allay any fear to seek definitive treatment in this rather timid individual.
Regional lymph nodal and distant metastases are infrequent. A high local recurrence rate has been reported. It may be as high as 18% when wide excision margins (more than 2 cm) have been taken. Mohs micrographic surgery has been recommended as the method to minimize local recurrence. This is important in the head and neck region where maximal tissue conservation is desired. In our set up, unfortunately, there is no availability of such expertise and being an emergency procedure, life-saving took precedence over other factors. We went ahead with wide local excision of the tumor.
Biopsy of the lesion is typical. The lesion is typically located in the dermis with irregular infiltration of the subcutaneous fat in lace-like pattern. The cellular arrangement in a storiform or mat-like arrangement is typical. The most frequent and difficult differential diagnosis is with the giant dermatofibromas (DF). Immunohistochemical analysis can be done to differentiate DFSP from DF. DFSP are CD34 positive and factor XIIIa, CD117 negative whereas DF is CD34 negative and factor XIIIa positive.
The ideal treatment of DFSP at the scalp is controversial. In one of the cases of DFSP of the scalp with underlying bone erosion and no intracranial extension, the tumor was removed with 3 cm wide peripheral margins and removal of the underlying bone followed by defect closure with local scalp transposition flaps. There was no local recurrence at 4-month follow-up. Similarly, in our case, as our primary aim was to stop the acute bleeding, we performed wide local excision with 5 cm peripheral margins. Since the tumor was only abetting the outer cortex of bone and considering that the postexcision scalp defect was quite large, we went ahead with chiselling of the outer bony cortex followed by split skin graft of the entire scalp defect.
Postoperative adjuvant therapy includes Imatinib, a tyrosine kinase inhibitor, has been approved to treat adult patients with unresectable, recurrent, and/or metastatic disease. The role of radiotherapy in adjuvant settings is limited. It may be of some value when the resection margins come out to be positive or functional limitations of extensive tumor excision. We did not give any adjuvant chemotherapy and radiotherapy to the patient as the excised margins were free of tumor deposits.
Long-term follow-up is essential as majority of the local recurrences occur within 3 years after the surgery. We intend to further follow-up, the patient for any local recurrences or distant metastasis due to our concerns of the massive size of the tumor. Scalp reconstruction after tissue expansion or free flap soft-tissue coverage is planned in the near future.
| Conclusion|| |
This case has been extraordinary due to several aspects. This is the largest size (16 cm × 14 cm × 9 cm) DFSP on the scalp which in itself is a rare site of presentation. In spite of its giant nature, there was no evidence of any distant metastasis. It presented to us in emergency hours with acute bleeding from the tumor. This again is an unusual presentation of DFSP. The tumor can be efficiently managed with wide local excision in emergency settings.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]