Encephalocraniocutaneous lipomatosis: congenital alopecia treatment in a rare neurocutaneous syndrome.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that include skin, ocular, and neurological disorders. This study describes the case of a 16-year-old girl that came to observation for the treatment of a congenital alopecia causing great psychological distress. After two expansion procedures the hairless patch was restored with high patient satisfaction. The case met all the criteria for definite diagnosis of ECCL.

Plasmacytoid dendritic cells represent a major dendritic cell subset in sentinel lymph nodes of melanoma patients and accumulate in metastatic nodes.

Plasmacytoid dendritic cells (pDC) represent the main source of interferon-alpha, a cytokine with antitumor activity. However, in vitro studies point to pDC as a key subset for induction of tolerance. Herein, we investigated pDC in sentinel lymph nodes (SLN) of melanoma patients. We report that pDC were constantly found in SLN and represented, with Langerhans cells, the most frequent dendritic cell subset. Their frequency in positive (with metastasis) SLN was significantly higher than in negative (without metastasis) SLN. PDC were observed in the T cell-rich areas of lymph nodes, particularly around high endothelial venules and, in metastatic nodes, they accumulated in close vicinity with melanoma nests. Finally, pDC capability to produce interferon-alpha in situ was impaired. Consistently, pDC expressed CD86, but neither CD80 nor CD83, suggesting a not complete activation in melanoma-draining lymph nodes. These results are consistent with the hypothesis of a tolerogenic role played by pDC in tumor immunology.

Sentinel node biopsy procedures with an analysis of recurrence patterns and prognosis in melanoma patients: technical advantages using computer-assisted gamma probe with adjustable collimation.

The purpose of this study was to investigate whether a computer-assisted gamma probe with adjustable collimation could aid in the detection of sentinel nodes (SNs) and to analyse the patterns of recurrence and prognosis in SN-positive and SN-negative cases. We analysed 385 SN biopsies. The SN identification rate was 87.2% using preoperative lymphoscintigraphy and blue dye, 93.9% using preoperative lymphoscintigraphy, blue dye and different probes, and 100% using preoperative lymphoscintigraphy, blue dye and a computer-assisted probe with adjustable collimation. The computer-assisted probe was particularly advantageous in cases where the melanoma was located very close to the SN and in cases of deep-seated nodes or nodes with low uptake, due to the possibility of changing the collimation during the procedure. The SN-positive rate according to the thickness of the primary melanoma was 1.7% for melanomas < or = 1 mm in thickness and 27.5% for melanomas > or = 1 mm. In 4.9% of cases we identified nodes outside the regional nodal basin. In one case we found a micrometastasis in a blue and hot interval node of the lateral abdominal wall. Analysing the node counts registered by the computer-assisted probe, we verified that the blue-positive node for tumour metastases was not the most radioactive node in the field in six out of 52 positive cases (11.5%). Distant metastases were present in 2.0% of SN-negative patients, and in 24% of SN-positive patients (P < 0.001). Highly statistically significant differences were found between SN-negative and SN-positive patients in both the 3 year disease-free survival (86.3% versus 49.2%) and the 3 year disease-specific survival (92.3% versus 77.1%) (P < 0.001).

Vasculogenic mimicry has no prognostic significance in pT3 and pT4 cutaneous melanoma.

The concept of vasculogenic mimicry has been introduced to define periodic acid-Schiff (PAS)-positive channels and loops lined by tumor cells, instead of endothelium, able to contribute to microcirculation in uveal melanomas. Previous studies have shown that the PAS-positive patterns are associated with a poor prognosis in uveal melanoma. The aim of the current study was to investigate whether vasculogenic mimicry has a prognostic impact in pT3 and pT4 cutaneous melanoma. Fifteen patients with pT3 and pT4 cutaneous melanoma who did not experience progression after 10 years of follow-up and 30 matched controls who underwent progression were selected. Tumor sections were stained with PAS reaction, omitting the nuclear counterstaining. For immunohistochemistry, sections were stained with CD31, CD105 (endoglin), and laminin. Differences in the distribution of the PAS-positive patterns and a series of clinicopathological variables were evaluated by the Pearson chi(2) and Mann-Whitney U tests. We observed PAS-positive linear sheets, arcs, elliptical loops, and networks encircling roundish to oval aggregates of melanoma cells. The overall distribution of the PAS-positive patterns did not match with the blood microvessels' architecture as detected by immunohistochemical analysis. No statistically significant differences in the distribution of PAS-positive patterns were found between cases and controls. The presence of a parallel pattern correlated significantly with thickness (P = 0.04), whereas an inverse correlation was found with vessel area (P = 0.05). In conclusion, our results suggest that there is a mismatch between vasculogenic mimicry and tumor angiogenesis and do not support any prognostic role of vasculogenic mimicry in thick cutaneous melanoma.

Tumor angiogenesis as a prognostic factor in thick cutaneous malignant melanoma. A quantitative morphologic analysis.

The prognostic value of the extent of neovascularization in cutaneous melanoma is a highly controversial issue. The aim of the current study was to evaluate whether the morphometric analysis of tumor vascularity may be helpful in predicting the clinical outcome of patients with thick cutaneous melanomas. A series of 15 patients with melanoma (>3 mm in thickness) who did not experience disease progression after long-term follow-up (10 years) and 30 matched controls who underwent recurrence and/or metastases were selected for the study. Microvessels were immunohistochemically stained with anti-CD31 antibody. Several parameters, including vessel number, vascular density, vessel area, equivalent circle diameter, perimeter, shape factor, compactness, and the number of vascular ramifications per 100 vessel sections, were quantitatively assessed by a computer-aided semi-automatic image analysis system. Mean vessel area was 341.69 microm2 in cases without progression and 512.55 microm2 in the progressed melanomas (P=0.008, Mann-Whitney U test). The mean equivalent circle diameter was 18.95 microm in non-progressed melanomas and 22.57 microm in progressed melanomas (P=0.009). The mean number of ramifications was 0.8 in cases without progression and 1.9 in the controls (P=0.03). Microvessel count and vascular density were higher in progressed cases (17.37 vs. 11.73 and 28.94/mm2 vs. 19.55/mm2, respectively), but the difference did not reach statistical significance (P=0.06). Our results suggest that neovascularization is a critical event in the progression of thick melanoma. Its prognostic significance is better assessed by quantification of vessel area, equivalent circle diameter, and microvessel branching, whereas microvessel count and vascular density do not provide significant prognostic information.