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10/2011; , ISBN: 978-953-307-574-7
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ABSTRACT: GeneScan (GS) analysis is a highly sensitive method for the early detection of cutaneous T-cell lymphoma (CTCL) and allows the identification of clonal heterogeneity, defined as the coexistence of two or more different T-cell clones in multiple samples from the same patient. We analyzed by GS the incidence and the significance of long-lived oligoclonal expansions in multiple skin and blood samples from 24 Sézary syndrome (SS) patients, and tried to correlate them with the clinical outcome. A skin clonal heterogeneity with additional reproducible TCRgamma-gene rearrangements (TCRgamma-GRs) was detected at diagnosis in 19/24 patients, 13 of whom had a constant prevalence of pathological TCRgamma-GRs in both skin and blood (dominant clonal pattern). During follow-up, an increase in oligoclones that were present at diagnosis or the appearance of new oligoclones was observed in 10 patients; all of them achieved a clinical response to treatment with extracorporeal photochemotherapy (ECP). The TCRgamma pattern (homogeneity or heterogeneity) in the skin at diagnosis showed a relevant prognostic value, and patients with an oligoclonal pattern had a significantly longer survival than those with a homogeneous pattern. In conclusion, multiple-sample approach GS analysis allows the identification of clonal heterogeneity and could also help in identifying SS patients with a potential higher response to therapy.
Journal of Investigative Dermatology 09/2010; 130(9):2312-9. · 6.31 Impact Factor
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ABSTRACT: Unknown melanoma occurs as metastasis to skin, nodes or viscera, without a detectable cutaneous primary tumour. We reviewed our database of 4881 melanoma patients, diagnosed and followed up prospectively for a 33-year period. We identified 93 cases of metastatic melanoma without evidence of primary; however, five of these patients had a history of a previous excision of a presumed benign lesion without histological examination and were excluded from analyses. At diagnosis, metastases were cutaneous in 35.3% of cases, nodal in 43.2% and visceral in 17% of cases; in 4.5% of patients, both skin and nodes were involved. In all cases, clinical inspection and staging procedures performed at diagnosis of metastatic disease failed to identify a primary melanoma. In 11 cases (11.8%), extensively regressed pigmented lesions (without evidence of melanoma cells at the histological examination) were documented; moreover, we identified in our series five patients with unknown primary affected by vitiligo. The 5-year and 10-year overall survival rates were 49.6 and 41.4%, respectively, with a median of 4.9 years. The 5-year and 10-year time to progression rates were 39.4 and 32.3%, respectively, with a median of 2.3 years. Survival was longer in females and showed significant differences among patients with skin, lymph node or visceral involvement at diagnosis. In melanoma patients, unknown primary represents a not so rare event, with an uncertain origin. We confirmed the relatively good prognosis of unknown primary melanoma patients, a fact that has to be taken into consideration for their management.
Melanoma research 06/2010; 20(3):227-32. · 2.06 Impact Factor
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ABSTRACT: GeneScan (GS) analysis is a highly sensitive method for the early detection of cutaneous T-cell lymphoma (CTCL) and allows the identification of clonal heterogeneity, defined as the coexistence of two or more different T-cell clones in multiple samples from the same patient. We analyzed by GS the incidence and the significance of long-lived oligoclonal expansions in multiple skin and blood samples from 24 Sézary syndrome (SS) patients, and tried to correlate them with the clinical outcome. A skin clonal heterogeneity with additional reproducible TCRγ-gene rearrangements (TCRγ-GRs) was detected at diagnosis in 19/24 patients, 13 of whom had a constant prevalence of pathological TCRγ-GRs in both skin and blood (dominant clonal pattern). During follow-up, an increase in oligoclones that were present at diagnosis or the appearance of new oligoclones was observed in 10 patients; all of them achieved a clinical response to treatment with extracorporeal photochemotherapy (ECP). The TCRγ pattern (homogeneity or heterogeneity) in the skin at diagnosis showed a relevant prognostic value, and patients with an oligoclonal pattern had a significantly longer survival than those with a homogeneous pattern. In conclusion, multiple-sample approach GS analysis allows the identification of clonal heterogeneity and could also help in identifying SS patients with a potential higher response to therapy.Abbreviations: CTCL, cutaneous T-cell lymphoma; ECP, extracorporeal photochemotherapy; GR, gene rearrangement; GS, GeneScan; SC, Sézary cell; SS, Sézary syndrome
Journal of Investigative Dermatology 04/2010; 130(9):2312-2319. · 6.31 Impact Factor
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ABSTRACT: Among primary cutaneous B-cell lymphomas, follicle center lymphomas represent, according to the World Health Organization-European Organisation For Research and Treatment of Cancer classification, a subgroup with a favorable prognosis. We describe the case of a 45-year-old man who presented with large infiltrated tumors and nodules coalescing into a wide ulcerated plaque of the scalp, extending from the frontal to the occipital region. At the vertex, 2 large ulcerations were present, reaching the subcutaneous tissues and the underlying bone structures with osseus infiltration and erosion and consequent meningeal exposure. A left retroauricular lymphadenopathy was also present. Histology and immunohystochemistry diagnosed a relapse of primary cutaneous follicle center lymphoma with multilobated histomorphology and lymph node involvement. The histological picture was unchanged from the first sample of 1989. Due to a refusal to treatment, the lesion progressively grew until now. After 6 courses of chemotherapy (cyclophosphamide, vincristine, liposomal doxorubicin, prednisone-Rituximab), the tumor displayed an impressive complete regression with the persistence of a 4-cm occipital ulceration and underlying bone erosion. The adenopathy disappeared as well. This case gave us the opportunity to observe the natural development of the disease, leading to local mutilating and destroying lesions but with low tendency to systemic spread and an impressive response to chemotherapy.
The American Journal of dermatopathology 11/2009; 32(1):91-4. · 1.30 Impact Factor
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ABSTRACT: Skin metastases are a frequent event in the natural history of malignant melanoma, both in the early and late phases of disease progression. In this study, we reviewed our database of 4865 melanoma patients, who were diagnosed and followed up prospectively over a 30-year period at our institution. Statistical analyses were focused on patients with secondary involvement of the skin. Seven hundred and thirty-three of the 4030 patients that met the inclusion criteria (18.2%) developed cutaneous metastases; the skin was involved as first site in 413 patients (56.3%) and after regional lymph node spreading in 208 (28.4%) patients. In a lower number of patients, cutaneous metastases developed only in advanced stages of the disease. Skin metastases were mainly locoregional, when arising as the first site of relapse (89.3%) and/or in patients with a primary melanoma of the lower limbs; in contrast, disseminated metastases are more often observed after a visceral involvement and for primary melanomas of the trunk. Moreover, despite a lower disease-free survival rate (1.3 vs. 2.9 years), we showed a significantly longer time to progression to visceral involvement for the group of patients with cutaneous locoregional metastases (62.5 vs. 17.8 months). The site of primary melanoma is strictly related to the pattern of cutaneous recurrence. The disparity in clinical outcome between patients with locoregional or disseminated skin metastases should therefore be taken into consideration in their management.
Melanoma Research 09/2009; 19(5):321-326. · 2.19 Impact Factor
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ABSTRACT: Despite frequent skin involvement with solid tumors, zosteriform metastases are a rare, not well-defined entity, with only few cases published in literature. The unifying characteristic is merely topographic: cutaneous lesions were distributed along dermatomes, despite the variety of clinical features, including vesicobullous, papular, and nodular lesions. Several theories have been proposed to explain the pathogenetic mechanism of zosteriform dissemination, even if none was adequately proved.
In this article, we report three new cases of patients with melanoma with skin zosteriform metastases and present a meta-analysis of literature data.
We collected all Entrez-PubMed articles about zosteriform skin metastasis since 1970 and reviewed 56 cases, including our own taken from a 4,774-patient series.
The histotypes mainly implicated were melanoma (18%); lymphoma (14%); breast cancer (12%); squamous cell carcinoma (12%); and digestive (10.7%), respiratory (10.7%), and urinary tumors (7%), with other histotypes accounting for 14%. In only one case in our series did we describe a typical herpetiform pattern, whereas in the others we found papulonodular lesions with a dermatomeric distribution.
Cutaneous metastases with zosteriform pattern are rare and show a wide clinicopathologic spectrum that could affect the disease course. The authors have indicated no significant interest with commercial supporters.
Dermatologic Surgery 07/2009; 35(9):1355-63. · 1.80 Impact Factor
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Dermatologic Surgery 06/2009; 35(8):1282-5. · 1.80 Impact Factor
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ABSTRACT: Vandetanib is an inhibitor of the vascular endothelial growth factor receptor 2 tyrosine kinase and the epidermal growth factor receptor tyrosine kinase, recently used in the treatment of different tumors. We describe a case of a photo-allergic reaction to vandetanib in an 80-year-old Caucasian woman affected by metastatic non-small cell lung cancer. Phototoxic reactions to vandetanib have been rarely reported in the literature. Dermatologists should be aware of this cutaneous side effect of vandetanib treatment and affected patients should be counseled to use adequate sun protection.
Dermatologic Therapy 23(5):553-5. · 1.69 Impact Factor
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ABSTRACT: Febrile ulcero-necrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta (only 41 cases described to date), characterized by an acute onset of ulcero-necrotic papules accompanied by high fever and severe constitutional symptoms. We report a case of a 23-year-old man with a steroid-resistant FUMHD treated by intravenous immunoglobulins (IVIG) combined with methotrexate. Only one case of FUMHD treated by IVIG has been reported to date in literature. Also in our case, IVIG proved to be effective in inducing a dramatic improvement of ulceration and in arresting the appearance of new lesions. Moreover, in our experience we decided to perform a maintenance treatment with extracorporeal photochemotherapy (ECP), to the best of our knowledge not previously used in the treatment of pityriasis lichenoides et varioliformis acuta. ECP, which involves extracorporeal exposure of peripheral blood mononuclear cells to photo-activated 8-methoxypsoralen, induces an immunological reaction against auto-reactive T cell clones, without immune-depression and thus could potentially be useful particularly in FUMHD avoiding the risk of an infective reactivation.
Dermatologic Therapy 23(4):419-22. · 1.69 Impact Factor
