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Rhabdomyosarcoma (RMS), a high-grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16-year-old girl. Because of the tumor size, local Invasiveness, and occurence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/N0/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, α-sarcomeric actin, musclespecific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease-free interval during three years of follow-up underlines the importance of muitidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence. [ABSTRACT FROM AUTHOR]

Gougerot-Carteaud syndrome or confluent and reticulated papillomatosis (CRP), was first described by Gougerot and Carteaud as dermatosis. It is generally considered a rare condition. The eruption consists confluent, flat, brown papules localized primarily to the intermammary and interscapular regions with subsequent spread to the breast and abdomen; at the periphery, the papules spread out forming a pigmented reticulated pattern. At present, the aetiology of CRP remains unknown. The two prominent theories are an abnormal host response to fungi and a keratinization defect. Other hypothesis include photosensitivity, genetic factor, amyloidosis cutis and endocrinopathy.

We report the morphological, immunohistochemical and ultrastructural cutaneous findings of two sisters, aged 72-74, with pseudoxanthoma-elasticum-like papillary dermal elastolysis (PDE), recently defined as an age-related condition. To our knowledge, these are the first familial cases of PDE reported in the literature. The lesions appeared as small, asymptomatic, soft papules around the neck and axillary regions. The affected skin revealed a marked decrease of normal elastic network of papillary dermis without alterations in either the relevant collagen or reticular dermis. Ultrastructural and immunohistochemical examinations showed activated dermal fibroblasts with abundant elongated dendritic cytoplasmic processes and the absence of myofibroblasts. The well documented avoidance of sun exposure (the patients are both nuns), the rare incidence of the disorder (only six cases reported), and the familial occurrence suggest that genetic or inherited predisposition should also be considered in the pathogenesis of PDE.

Multidrug-resistant (MDR) variants of a human osteosarcoma cell line (U-2 OS) have been recently obtained by continuous exposure to doxorubicin (DX). The growth and phenotypic characteristics of these cell lines have been demonstrated to be related to the level of expression of P-glycoprotein. In this work, the morphological changes associated with MDR have been evaluated by quantitative image analysis and transmission electron microscopy. Resistant cells present morphological changes with respect to sensitive cells at both cytoplasmic and nuclear level. Some of these changes appear to be related to the degree of resistance but not to the direct presence of DX, since deprived cells maintain some modified characters, while others are partly lost. These findings suggest that DX exposure affects cell metabolism causing progressive changes of the cell morphotype.