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The authors describe a case of a 5 cm mixed desmoplastic melanoma occurring on the cheek of an 88-year-old white woman. The epidermis showed the features of lentigo maligna. Within the dermis, there was a mixed desmoplastic melanoma with 2 components. The first component consisted of infiltrative malignant spindled cells with prominent stromal fibrosis and had the typical appearance of desmoplastic melanoma. The second component was within the deep half of the tumor and consisted of a densely cellular nodule composed of spindled melanocytes admixed with many osteoclast-like giant cells. There was a peripheral neurotropism and tumor invaded bone. The Breslow thickness was 14 mm. On followup, a sacral metastasis was discovered, which had a similar morphology to the deep cellular nodule. Immunohistochemistry of spindled cells both inside and outside the nodule showed S100 positivity with the absence of other melanocytic markers (HMB-45, Melan-A). Smooth muscle actin and p63 were focally positive. The osteoclast-like giant cells expressed CD68 and MiTF. Array comparative genomic hybridization of the typical desmoplastic melanoma region had a flat profile, whereas the cellular osteoclast-like giant cell–rich region displayed important cytogenetic anomalies, some of which have been previously described in melanomas. The main array comparative genomic hybridization findings were confirmed by fluorescence in situ hybridization using specific probes. The differences in morphology and molecular cytogenetics between the 2 areas suggest that these might represent the progression or emergence of a more aggressive clone within the tumor. Subsequent metastatic spread to the bone may be a result of accumulated cytogenetic abnormalities.

Primary angiosarcoma of the breast is a rare malignant tumour that presents in young women as a painless mass or a sensation of fullness in the breast. To report two cases of primary breast angiosarcoma presenting with unusual and misleading cutaneous lesions. A clinical investigation including ultrasound, MRI and histological examination. In the first patient, the lesion appeared as a superficial, acquired angioma; in the second as an indolent superficial haematoma. This type of primary presentation is exceptional and the benign appearance of the lesion, combined with a lack of breast mass, is misleading. The benign appearance and the pathological aspect of these lesions can lead to misdiagnosis. Comparison of clinical and pathological data is necessary to prevent delay in diagnosis. We believe that all acquired angiomatous lesions developing on the breasts of young women should raise suspicion of angiosarcoma.

BRCA-1-associated protein 1 (BAP1) is a tumor suppressor gene located at chromosome position 3p21.1. It encodes a nuclear protein involved in multiple cellular activities: transcriptional regulation, chromatin modulation and DNA repair [1]. Germline mutations in BAP1 are responsible for a tumor predisposition syndrome (OMIM database #614327) [2]. Carriers are at greater risk of developing cutaneous and ocular melanomas, clear-cell renal cancer, mesothelioma and multiple basal cell carcinomas [3]. [...]

AIMS: Aggressive epidermotropic cutaneous CD8+ lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8+ lymphomas.METHODS & RESULTS: Sixty-one CD8+ cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed & recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD8+ lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and have striking pagetoid epidermotropism histologically. A CD8+ CD45RA+ CD45RO- CD2- CD5- CD56- phenotype, with 1 or more cytotoxic markers was found in 7/18 with a very similar phenotype in the remainder.. The tumours seldom involve lymph nodes but mucosae and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD8+ mycosis fungoides, lymphomatoid papulosis and Woringer-Kolopp presented the typical features well documented in the CD4+ forms of those diseases.CONCLUSIONS: Aggressive Epidermotropic Cutaneous CD8+ Lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications. This article is protected by copyright. All rights reserved.

A case of primary adenocarcinoma of the renal pelvis occurring in a 57-year-old woman who had no previous history is reported. The lesions were thought to be a renal destruction by pyelolithiasis upon symptomatology and imaging study data. A nephrectomy was thus performed. Macroscopic examination revealed voluminous and exophytic lesions surrounding lithiasis. Diagnosis of intestinal-type adenocarcinoma of the renal pelvis was established on histological examination. No evidence of other tumor localization was revealed by complete exploration. Primary adenocarcinoma of renal pelvis is a rare and often mucinous intestinal-type tumour. Chronic inflammation and renal lithiasis seem to be associated with this tumour. Few cases are reported and prognosis is doubtful. The main differential diagnosis to eliminate is secondary lesions to the kidney of adenocarcinoma from another origin. A careful pathological examination in case of pyelonephritic kidney is necessary to look for an associated tumour.