Paraffin-embedded micrographic surgery for the treatment of dermatofibrosarcoma protuberans: Analysis of 33 patients.

BACKGROUND: Dermatofibrosarcoma protuberans is a rare malignant tumor with a high rate of recurrence after surgery. Moh's micrographic surgery allows examination of all surgical margins to ensure complete removal. OBJECTIVE: To evaluate the use of Moh's micrographic surgery using paraffin-embedded sections for the treatment of dermatofibrosarcoma protuberans. METHODS: We performed a retrospective analysis of 33 patients with dermatofibrosarcoma protuberans treated in our department with paraffin-embedded micrographic surgery between January 2002 and June 2015. Our cases included patients with primary untreated disease and also those with persistent disease previously treated surgically elsewhere, with histologically positive margins. RESULTS: Tumors were most commonly located on the trunk. After the first stage of micrographic surgery, including an initial lateral margin, 20 (60.6%) tumors were completely excised, 11 (33.3%) tumors required two stages and one tumor each (3.0%) required 4 and 6 stages respectively. Patients were monitored for recurrence for a mean duration of 6.5 years. There was no recurrence in any of our 33 patients. CONCLUSIONS: Our results indicate that Moh's micrographic surgery with paraffin-embedded sections may be the method of choice to treat dermatofibrosarcoma protuberans with a low recurrence rate, while preserving surrounding normal healthy tissue.

Extramammary Paget's disease: analysis of 17 Chinese cases.

BACKGROUND/OBJECTIVE: Extramammary Paget's Disease (EMPD) seems to be more common in Caucasians than Chinese. We report the clinical manifestations, management, and prognostic characteristics in 17 Chinese patients. METHODS: Medical records and biopsies of 17 patients who had been treated at a large university hospital in China between March 2005 and January 2012 were reviewed. RESULTS: Of the 17 patients, 14 were men. They had lesions on the scrotum and the penis. Of the three women, two had vulvar and one had inguinal lesions. All patients underwent Mohs micrographic surgery (MMS). Three men had metastasis to the inguinal lymph nodes and underwent an extensive local excision with inguinal lymphadenectomy. Eight patients who had positive excision margins received additional radiation therapy. The mean follow-up duration was 54 months (4-85 months). One patient had two recurrences. Three had metastasis to the inguinal lymph node. One had metastasis to the bone and concomitant prostate cancer. Two patients died of the disease. CONCLUSION: A striking difference in presentation of EMPD in Chinese compared with Caucasians is the male predominance and location on the penis and scrotum. Mohs micrographic surgery followed by radiotherapy is an effective treatment. Long-term follow-up suggests that the disease has a good prognosis when it does not metastasise.

Autosomal dominant epidermodysplasia verruciformis: a clinicotherapeutic experience in two cases.

Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by a unique susceptibility to cutaneous infection by a group of phylogenetically related human papilloma viruses (HPVs). These patients show a defect in cell-mediated immunity specific toward the causative HPVs that lead to lifelong disease. The defect is usually inherited as autosomal recessive trait and presents clinically with plane warts, pityriasis versicolor-like lesions and reddish verrucous plaques. Dysplastic and malignant changes in the form of actinic keratoses, Bowen's disease and squamous cell carcinoma (SCC) are common but metastasis occurs rarely. A totally effective treatment against EV is as yet highly desirable. Two siblings having autosomal dominant EV presented with multiple actinic keratoses in addition to classic lesions. One of them had also developed well-differentiated SCC over forehead with metastases to regional lymph nodes. They were treated with combination of excision of small malignant/premalignant lesions, topical 5-flurouracil and sun protection. Additionally, elective excision/grafting of large SCC was performed after chemotherapy/radiotherapy in patient with metastatic SCC. Oral acitretin (25 mg/day) was of benefit in the other patient. Overall clinicotherapeutic experience in both the patients is discussed here.