eMedicine Specialties > Dermatology > Malignant Neoplasms
Dermatofibrosarcoma Protuberans: Follow-up
Updated: Aug 17, 2009
Follow-up
Further Outpatient Care
Because of the high local recurrence rate of dermatofibrosarcoma protuberans (DFSP), patients require close follow-up care after treatment.
- Most recurrences occur within 3 years of the primary excision. Patients should be seen every 6 months during this period and annually thereafter.1,15
- A literature review of DFSP case series treated with Mohs surgery shows that 50% of recurrences appear within the first 3 years after operation and 25% of local recurrences are detected after 5 years. A large case review from a series of 159 patients treated at Memorial Sloan-Kettering Cancer Center (New York) showed the medium time to the development of a local recurrence was 32 months. The indolent nature of DFSP requires lifelong surveillance for recurrence.19
- In each follow-up visit, a complete history and a review of systems, as well as complete physical examinations, including skin examination and palpation of the excision site and regional lymph nodes, should be performed. An extensive workup is not warranted unless metastatic disease is suspected.
Prognosis
Dermatofibrosarcoma protuberans (DFSP) is characterized by its aggressive local invasion. The tumor invades local tissue by extending tentaclelike projections underneath healthy skin, rendering complete removal of the tumor very difficult. Incomplete removal of these neoplastic cells results in a high local recurrence rate.
Despite the local invasiveness, DFSP rarely metastasizes. For classic form of DFSP, the risk is assumed to be only 0.5%. According to the literature, the overall risk for development of metastatic disease is 5%, including 1% with regional lymph node metastasis and 4% with distant metastasis. Regional lymph node involvement represents a sign of poor prognosis; most patients die within 2 years. The lungs are the most common site of distant metastasis that occurs via hematogenous spread. Usually, metastatic disease is preceded by multiple local recurrences.15
The extent of surgical excision determines the prognosis for the patient. To reduce the local recurrence rate, a wide surgical excision with adequate margins or Mohs technique are used. The latter imparts a better outcome.
Histologic features of DFSP may also serve as prognostic indicators. A high number of mitotic figures, increased cellularity, DNA aneuploidy, TP53 gene overexpression, and the presence of fibrosarcomatous changes within the tumor are poor prognostic indicators. Of note, fibrosarcomatous variants of DFSP lacking a genetic marker of translocation between chromosomes 17 and 22 may not respond to imatinib. The loss of the t(17,22) cytogenetic marker in the fibrosarcomatous progression DFSP variant may represent progression of the malignancy.1,18,33
Age older than 50 years is also a risk factor associated with a poor clinical outcome.19
Patient Education
Patients are advised to seek for dermatologic evaluation if they have noticed a slow-growing skin lump or scarlike lesion on any part of their body.
Miscellaneous
Medicolegal Pitfalls
Because of the rarity of dermatofibrosarcoma protuberans (DFSP), misdiagnosis and delayed diagnosis often occur. When encountering an atypical scarlike lesion, the clinician should consider skin biopsy.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.
More on Dermatofibrosarcoma Protuberans |
| Overview: Dermatofibrosarcoma Protuberans |
| Differential Diagnoses & Workup: Dermatofibrosarcoma Protuberans |
| Treatment & Medication: Dermatofibrosarcoma Protuberans |
Follow-up: Dermatofibrosarcoma Protuberans |
| Multimedia: Dermatofibrosarcoma Protuberans |
| References |
| Further Reading |
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Further Reading
Clinical trials
- Imatinib in Dermatofibrosarcoma Protuberans (DFSP) (active, not recruiting)
- A Short Course of Neoadjuvant Gleevec (Imatinib Mesylate) in Dermatofibrosarcoma Protuberans (recruiting)
- Imatinib Mesylate in Treating Patients With Locally Recurrent or Metastatic Dermatofibrosarcoma Protuberans (DFSP) or Transformed Fibrosarcomatous DFSP (active, not recruiting)
- Neoadjuvant Imatinib in Dermatofibrosarcoma Protuberans (recruiting)
- Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans (recruiting)
Keywords
dermatofibrosarcoma protuberans, DFSP, sarcomatous tumors resembling keloid, hypertrophic morphea, progressive and recurring dermatofibroma, fibrosarcomatous tumors with attenuated dermal surfaces, fibrosarcoma of the skin
Follow-up: Dermatofibrosarcoma Protuberans