Dermatofibrosarcoma Protuberans (DFSP)

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The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional. Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question. My husband had dermatofibrosarcoma protuberans on his thigh. Will it spread to other parts of the body by the lymph; If it spreads, what are the symptoms?

Can dermatofibrosarcoma affect heart, lungs, liver, kidney or other internal organs?

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If so, what are the symptoms? Can any symptoms be found during a complete body checkup before it spreads?

What is dermatofibrosarcoma protuberans?

See answer. Is this very harmful like other cancers? Is there any chance it could spread to other parts of the body? What are the available treatments? National Institutes of Health. Menu Search Home Diseases Dermatofibrosarcoma protuberans. You can help advance rare disease research! Title Other Names:. Summary Summary. Symptoms Symptoms. Showing of 6 View All. Skin tumors. Tumor of the skin. Firm lump under the skin. Growth of abnormal tissue under the skin.

Thick skin. Open skin sore. Do you have more information about symptoms of this disease? We want to hear from you. Do you have updated information on this disease? Cause Cause. The cause of DFSP is unknown but an injury to the affected skin may be a predisposing factor. The fused gene produces a protein which some believe may stimulate cells to multiply, leading to the tumor formation seen in dermatofibrosarcoma protuberans.

This type of gene change is generally found only in tumor cells and is not inherited. Treatment Treatment. Prognosis Prognosis. Regular follow-up is needed after tumor removal to monitor for recurrence.

What causes dermatofibrosarcoma protuberans?

FS-DFSP tumors are more likely to metastasize than the tumors in other types of dermatofibrosarcoma protuberans. Find a Specialist Find a Specialist.

Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report

Healthcare Resources To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Related Diseases Related Diseases. Conditions with similar signs and symptoms from Orphanet. The differential diagnosis should include fibrosarcoma see this term , dermatofibroma, neurofibroma, and other soft tissue tumors. Visit the Orphanet disease page for more information. Research Research. Clinical Research Resources ClinicalTrials.

Pathology Outlines - Dermatofibrosarcoma protuberans (DFSP)

Click on the link to go to ClinicalTrials. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study. Organizations Organizations. Organizations Supporting this Disease. Organizations Providing General Support. Do you know of an organization? Learn More Learn More.

DermNet NZ provides information about this condition. This website is maintained by the National Library of Medicine.

S1 guidelines for dermatofibrosarcoma protuberans (DFSP) – update 2018

In-Depth Information Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free. RT can reduce the morbidity or functional impairment associated with extensive resection. As seen in the present case, locally recurrent DFSP can be devastating. Salvage by further resection increases the risk of functional deficits and metastatic disease.

Such dose is easily reached orally, with non negligible side effects. Some clinical reports showed Imatinib induced regression in patients with recurrent or metastatic DFSP [ 6 — 17 ]. However, response was not consistent in all cases as described in our patient. To date, no other targeted therapy for DFSP has been reported. It has shown great value in the treatment of angiosarcomas [ 49 ] Maki et al.

Since it has been reported to have an important role in the treatment of some sarcomas, we elected Sorafenib as a salvage option for our patient. The response was remarkable. The tumor almost totally regressed and the patient improved clinically. This case suggests that Sorafenib could be an important targeted therapy for DFSP especially in case of recurrence or metastasis.

Invasive DSFP could be uncontrollable and challenging to cure. We present a case of recurrent and infiltrative DSFP that failed all conventional therapeutic options. Sorafenib was administered as final resort with impressive results. Sorafenib could represent a therapeutic alternative in such cases of DFSP. Written informed consent was obtained from the patient for publication of this report and any accompanying images. No external funding, apart from the support of the authors' institution, was available for this study.

Dermatofibrosarcoma protuberans. National comprehensive cancer network. Fluorescence in situ hybridization. Platelet-derived growth factor receptor. This article is published under license to BioMed Central Ltd. Case Report Open Access.


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Dermatofibrosarcoma protuberans DFSP successfully treated with sorafenib: case report. Clinical Sarcoma Research 3 Abstract DFSP is a locally invasive, slow-growing tumor of the subcutaneous tissue that rarely metastasizes but recurs frequently after surgical excision. He was offered to start on Sorafenib as it was made available for us on compassionate use. There was no significant side effect reported beside moderate fatigue, and dehiscence of prior surgical wounds. However, he suffered from recurrent pulmonary infections despite decompression of his left main bronchus since attaining a very good partial response, and unfortunately passed away after an episode of septic shock.

He remained progression free during the whole treatment time. Therapy was discontinued only one week prior to his death. Figure 1 Radiological response to Sorafenib. Figure 2 Clinical improvement and dramatic shrinking of the tumor. Box , Beirut, Jounieh, Lebanon. References Darier J: Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peu.

Ann Dermatol Syphiligr Paris. Br J Oral Maxillofac Surg. Am J Surg Pathol. J Natl Compr Canc Netw. Curr Opin Oncol. Int J Cancer. J Clin Oncol. Br J Dermatol. Anticancer Drugs. Pediatr Blood Cancer. Curr Oncol Rep. Dermatol Surg. J Dtsch Dermatol Ges. Plast Reconstr Surg. Eur J Surg Oncol. J Am Acad Dermatol. Arch Otolaryngol. A study of cases. Ann Surg Oncol. J Cancer Res Clin Oncol. Ann Plast Surg.



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