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Background: The treatment of stage I Merkel cell carcinoma (MCC) usually includes wide local excision (WLE) combined with irradiation of the tumor bed (ITB). No randomized study has ever been conducted in MCC. The purpose of this study was to assess the efficacy and safety of prophylactic adjuvant radiotherapy on the regional nodes., Patients and Methods: In this randomized open controlled study, patients for a stage I MCC treated by WLE and ITB were randomly assigned to regional adjuvant radiotherapy versus observation. Overall survival (OS) and probability of regional recurrence (PRR) were primary end points. Progression-free survival (PFS) and tolerance of irradiation were secondary end points., Results: Eighty-three patients were included before premature interruption of the trial, due to a drop in the recruitment mainly due to the introduction of the sentinel node dissection in the management of MCC. No significant improvement in OS (P = 0.989) or PFS (P = 0.4) could be demonstrated after regional irradiation, which, however, significantly reduced the PRR (P = 0.007) with 16.7% regional recurrence rate in the observation arm versus 0% in the treatment arm. The treatment was well tolerated., Conclusion: The adjuvant regional irradiation significantly decreased the PRR in MCC, but benefit in survival could not be demonstrated.

Introduction: Cutaneous T-cell lymphoma subtypes are now better identified thanks to progress in immunohistochemistry. This article describes a new case of primary cutaneous natural killer/T-cell lymphoma of nasal type (NKTL-NT) and reviews 18 other cases of this rare neoplasm., Case Report: A 79-year-old man presented with a 3-cm nodular tumor of the left leg occurring on a primary chronic lymphedema of the legs. The lesion was CD56+, CD3 intracytoplasmic+, CD45+ and Epstein-Barr virus+. A comprehensive workup including CT scan and bone marrow biopsy was negative and a diagnosis of NKTL-NT with a primary cutaneous involvement was made. The patient was free of disease under multi-agent chemotherapy after 24 months of follow-up., Discussion: After reviewing 18 other cases of primary cutaneous NKTL-NT, we conclude that the prognosis of these lymphomas is usually poor. However, limited cutaneous forms have a longer median survival than extracutaneous variants., (Copyright (c) 2006 S. Karger AG, Basel.)

Background: Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin., Objectives: To assess the prevalence of these DFSPs and further to describe their presentation and course., Methods: One hundred and forty-three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis., Results: Eighty-one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7.6 years. Twenty-nine per cent of npDFSPs were 'morphoea-like', 19% were 'atrophoderma-like' and 42% were 'angioma-like'. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians., Conclusions: Nearly half the patients first identified their early DFSP-related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.

The early diagnosis of cutaneous melanoma alone is capable of significantly reducing the morbidity and mortality associated with this tumour. It is pertinent to all physicians who normally observe skin during the clinical examination; who must have a degree of suspicion for all recent or modified pigmented lesions, and should offer a cutaneous follow-up and advise decreased sun exposure in all patients with multiple naevi. The modification of a lesion, intensely black in colour, are the key elements which arouse suspicion of a melanoma and incite either its removal or a specialist opinion. The follow-up of the patient treated surgically for a melanoma is mainly clinical (skin, subcutaneous tissues, lymph node examination) but has to be consistent (more often on a trimesterly basis), systematic, and annual after 5 to 8 years, during the whole life of the patient with a high risk of a second melanoma. Equally, family members of a patient with a melanoma should also be systematically examined.

The main objective of this prospective multicentre randomized phase III study was to compare a combined regimen of fotemustine plus whole brain irradiation with fotemustine alone in terms of cerebral response and time to cerebral progression in patients with melanoma cerebral metastases. Seventy-six patients were randomized to receive either fotemustine (arm A, n = 39) or fotemustine plus whole brain irradiation (arm B, n = 37). Fotemustine was administered intravenously at 100 mg/m(2) on days 1, 8 and 15, followed by a 5 week rest period, then every 3 weeks in non-progressive patients. In arm B, concomitant whole brain irradiation was performed at a total dose of 37.5 Gy (2.5 Gy/day on days 1-5 for three consecutive weeks). Although patients who received fotemustine alone had worse prognostic factors, there was no significant difference in cerebral response (arm A, 7.4%, arm B, 10.0%) or control rates (objective responses plus stable disease) after 7 weeks (arm A, 30%; arm B, 47%) or in overall survival (arm A, 86 days; arm B, 105 days). However, there was a significant difference in favour of arm B for the time to cerebral progression (P = 0.028, Wilcoxon test). In conclusion, fotemustine plus whole brain irradiation delayed the time to cerebral progression of melanoma cerebral metastases compared with fotemustine alone but without a significant improvement in terms of objective control or overall survival.

To evaluate the value of morphologic, immunohistochemical and molecular analyses, we studied 21 skin biopsy specimens from 19 patients with primary cutaneous B-cell infiltrates. Morphologic review by two independent dermatopathologists confirmed the consensus diagnoses of lymphoma (n = 6) or benign lymphoid hyperplasia (n = 6). A discordant diagnosis was made for the other samples (n = 9), which were thereafter considered as unclassified lymphoid infiltrates. Immunohistochemical analysis showed either a monotypic expression of immunoglobulin light chain or a positive staining with anti-bcl-2 antibodies in three and four samples, respectively, of lymphoma. Polymerase chain reaction was used to analyze immunoglobulin heavy chain and T-cell receptor gamma chain gene rearrangement and to amplify t(14;18) and t(11;14) break points. A clonal molecular marker was detected in 12 of 19 patients. Among these 12 patients, a final diagnosis of lymphoma was confirmed in 8 patients, including the 6 with a morphologic diagnosis of lymphoma. Two patients with clonal benign lymphoid hyperplasia and two with clonal unclassified lymphoid infiltrate presented a benign clinical outcome; one patient was lost to follow-up. Alternatively, no clonal molecular marker was found in two of the patients with lymphoma. The morphologic and molecular criteria, therefore, provided complementary and partially overlapping information for the diagnosis of cutaneous B-cell infiltrates. We proposed a practical use for these data.

The authors have analyzed and compared the clinicopathologic and molecular features of 16 cases of large cell cutaneous lymphomas expressing CD30 antigen. Three main clinical groups were defined: (1) a group of localized skin disease (7 cases); (2) a group of multicentric skin disease (5 cases); and (3) a group of concomitant skin and extracutaneous disease. Good prognosis was associated with localized skin disease and no history of lymphoma. Interestingly, a majority of Reed Sternberg-like cells was only observed in this group (5 of 6 cases). The two other groups did not show distinctive evolutive nor morphologic features. Southern blot and/or polymerase chain reaction (PCR) technique showed clonality and a T-cell genotype in respectively 13 of 14 and 12 of 12 analyzed cases. Viral infection of tumoral cells was investigated by PCR, in situ hybridization (ISH) or electron microscopy. Epstein-Barr virus (EBV) sequences were detected by PCR and ISH in tumoral cells of cutaneous lesions in one case of skin lymphoma with extracutaneous spreading. No EBV sequence was detected by ISH in the localized lymphomas, whereas HIV particles were visible in tumoral cells in one of these cases. No human T-cell lymphotropic virus (HTLV) tax sequence was amplified by PCR in any case of our series. Our results confirm that CD30-positive cutaneous large cell lymphomas are different clinical and molecular entities. However, a combined clinical and morphologic analysis may help to identify a subset of CD30 cutaneous lymphomas with favorable prognosis.

Background: The major factor influencing the prognosis of cutaneous malignant melanoma (MMs) is the maximum thickness of the tumor as measured by Breslow's method. However, it has been reported that thin melanomas, which should have an excellent prognosis, may have the potential to metastasize, some with an unusually rapid course., Objective: Our purpose was to examine prognostic indicators in relation to unusually rapid aggressive behavior in patients with thin MMs (<0.76mm)., Methods: We describe nine cases of thin MM (<.76mm) that exhibited a recurrence or metastasis during a follow-up period ranging from 3 to 10 years, among computerized records of 1118 MMs treated in a multicenter epidemiologic study. The data obtained from these nine cases were compared with nonrecurring thin MM (149 cases) of the same cohort., Results: The particular aggressiveness of these thin melanomas was reflected by the short disease-free interval (3 years or less) in all ine patients. The recurring thin MM more frequently involved head and neck sites, occurred in male patients, and showed Clark's level III and IV., Conclusion: Our review suggests that the head and neck area is particularly involved by unusually rapidly recurring thin MM. Possible explanations are the specific problems of surgical management and the greater sun exposure of this location.

Introduction: Merkel cell carcinoma or cutaneous neuroendocrine carcinoma is an uncommon severe disease. The carcinogenic effect of ionizing radiations has been suspected in exceptional observations. We report the sixth case of Merkel cell neuroendocrine carcinoma in a patient with prior radiotherapy., Case Report: An 86-year-old man underwent radiotherapy for a basal cell carcinoma of the tip of the nose and developed a highly aggressive Merkel cell carcinoma at the same location 6 years later., Discussion: The development of Merkel cell carcinoma on irradiated tissue accounts for 2.6 p. 100 of the 227 publications where dermatological history was reported. This percentage may be underestimated. The similar localizations of the irradiated zone and the site of cancer development 5 years later suggest that the Merkell cell carcinoma may be a radio-induced tumor. The delay may vary from 5 to 47 years. The similarity of the carcinogenic factors involved in Merkel cell carcinoma and squamous cell or basal cell carcinomas (ultraviolet, ionizing irradiation) and the frequent association of different types favor an epidermal origin for Merkel cell carcinoma. In clinical practice, past history of radiotherapy in an area where Merkell cell carcinoma develops indicates that therapeutic management must exclude post-operative radiotherapy.

Background: Ocular paraneoplastic syndromes are rare, and consist of optic neuropathy or retinopathy. Classically, these syndromes are related to carcinoma. Melanoma-associated retinopathy is extremely rare, and unrecognized., Methods: A patient with metastatic cutaneous melanoma discovered and operated 18 months before. Visual complains consisted of xanthopsia and shimmering light vision, then hemeralopia, which dramatically worsened. Classical clinical examination, visual field and electroretinogram were performed., Results: Visual acuity was 20/25, and fundus examination was normal. The visual field showed a tubular aspect, with V4 isopter remained, like an advanced retinitis pigmentosa. The photopic electroretinogram was negative, and the scotopic one was flat., Conclusion: This recent hemeralopia with normal fundus and "negative" electroretinogram, ruled out congenital stationary night blindness diagnosis, and suggested the diagnosis of melanoma-associated retinopathy. This is a rare paraneoplastic syndrome since to date only 7 cases have been reported. Immunochemistry studies, that show antibodies directed against bipolar cells, are consistent with selective reduction of the electroretinogram b wave.

Cutaneous metastases are seldom encountered in digestive cancers. The mechanism of their formation explains their low rate. The metastases spread mainly by the lymphatic system (especially in diffuse, spotty or pseudo-elephantiasic forms and in regional forms), however numerous lymph node filters found through out the lymphatic system limit the progression of neoplasic cells. On the contrary, metastases most often spread by retrograde involvement due to blocking of the lymphatic system. As for hematogenous metastases (generalized nodular forms or aberrant localizations), they are observed even less frequently than lymphatic ones; metastatic embolisms may effectively become blocked in capillaries, especially in the lungs and the liver, which constitutes a double barrier before the skin may be involved.