No abstract

Fibrosarcomatous transformation represents a rare event in dermatofibrosarcoma protuberans (DFSP) with unpredictable biological behaviour. No guidelines for the adequate treatment of patients with this rare neoplasm have been published. Herein, we present a comprehensive review of the literature comprising 157 patients with transformed DFSP focussing on surgical and adjuvant treatment modalities for this tumour. In the cohort examined, local recurrence occurred in 36% of cases and was significantly lower in patients treated by wide excision with margins ≥2 cm when compared with those treated with local excision without defined margins (P = 0.01). Consistently, negative margin status was associated with a lower recurrence rate when compared with positive or unknown margin status (P = 0.01). Distant metastases were detected in 13% of patients, which is significantly higher when compared with ordinary dermatofibrosarcoma protuberans. Systemic dissemination was preceded by local recurrence in 81% of cases, and is therefore strongly associated with tumour recurrence (P ≤ 0.001). The present data confirm that wide excision with margins ≥ 2 cm represent the gold standard in the treatment of transformed dermatofibrosarcoma protuberans, and prevents recurrence as well as metastasis. When R0-resection is not feasible, adjuvant radiation should be considered for cases with incomplete resection or unknown surgical margins. Irresectable or metastatic transformed DFSP harbouring the COL1A1-PDGFB fusion gene should be treated with imatinib in the palliative setting or as an adjunctive treatment before surgery, although responses may be short-lasting.

Dendritic cells play a major role in the generation of immunity against tumour cells. They can be grown under various culture conditions, which influence the phenotypical and functional properties of dendritic cells and thereby the consecutive immune response mainly executed by T cells. Here we discuss various conditions, which are important during generation and administration of dendritic cells to elicit a tumouricidal T cell-based immune response.

The neurons which synthesize and release luteinizing hormone-releasing hormone (LHRH), are hypothesized to originate in the epithelium of the medial olfactory pit and to migrate into the brain along a scaffolding made up of neural cell adhesion molecule (NCAM)-immunoreactive branches of the terminal and vomeronasal nerves. These LHRH neurons, studied by immunocytochemical and autoradiographic procedures, were found to originate within a very short period of embryogenesis, specifically day 10, in mice, and to follow a remarkably ordered spatiotemporal course along the migration route into the brain. The purpose of the present experiments was to determine whether perturbation of the NCAM-immunoreactive migration route, at a particular time in development, would arrest the migration of LHRH neurons into the brain. We found that a 1 microliter injection of antiserum to NCAM into the area of the olfactory pit, on day 10 of embryogenesis, significantly reduced the number of LHRH-immunoreactive neurons seen in the epithelium of the medial olfactory pit, with a concomitant significant reduction in the number of LHRH-immunoreactive cells seen outside of the placode, on the migration route. These results confirm our initial hypothesis that LHRH neurons migrate from the epithelium of the olfactory pit to the brain and indicate that NCAM plays a causal role in this phenomenon.

Milia en plaque in the periocular region represent a cosmetically disturbing skin condition of unknown origin characterized by numerous tiny milia grouped around the inner canthus and the medial aspect of both eyelids. While conservative treatment and manual expression often result in local recurrence, invasive approaches harbor the risk of mechanical or thermal injury of periocular skin and lid margins. A 32-year-old female patient with refractory periocular milia was treated with the erbium:YAG laser and followed-up for 12 months. Ablative laser treatment led to nearly complete resolution of the milia and an excellent clinical result. Importantly, no scarring, dyspigmentation or ocular complications were noted. This report demonstrates the efficacy and safety of erbium:YAG laser ablation of periocular milia. The precise control of tissue ablation with minimal thermal damage makes the erbium:YAG laser an ideal tool for the treatment of the delicate periocular region where even minimal scarring can result in ocular complications.