RESUMO
Rhabdomyosarcoma (RMS) is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells called rhabdomyoblasts. RMS is a rare tumor in adults, and involvement of the sinonasal area is extremely rare, comprising only 1.5% of all reported head and neck RMSs. Alveolar RMS, mainly seen in adults, has the worst prognosis. Incidence of lymph node metastases is more common in this type compared with the other forms. Fine needle aspiration cytology (FNAC) has been used extensively in the diagnosis of metastatic malignancies. However, metastatic soft tissue sarcomas are often overlooked, primarily due to the low frequency with which they occur. Here, we report a rare case of metastatic alveolar RMS in the cervical lymph nodes of an 18-year-old girl that was detected by FNAC. After 6 months, the patient came with a huge mass involving the nasal vestibule and the upper lip. Histology of both the main mass and the lymph nodes revealed alveolar RMS.
RESUMO
Lewandowsky and Lutz dysplasia, also known as epidermodysplasia verruciformis (EV), is an inherited disorder in which there is widespread and persistent infection with human papilloma virus, defect in cell-mediated immunity and propensity for malignant transformation. Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail. Cases were followed up for 7 years. Detailed history, clinical features and investigations, including skin biopsy from different sites at different times, were examined. Generalized pityriasis versicolor like hypopigmented lesions in both the cases, together with variable pigmented nodular actinic keratosis like lesions on sun-exposed areas, were present. Multiple skin biopsies done from various sites on different occasions revealed features typical of EV along with lesions, i.e., actinic keratosis, Bowen's disease, basal and squamous cell carcinoma, in the elder sibling. However, skin biopsy of the other sibling showed features of EV and seborrheic keratosis only till date. This study reveals that the disease progression is variable among two individuals of the same family. Malignant lesions were seen only on sun-exposed areas and may be associated with other skin lesions or infections such as angiokeratoma of Fordyce and tinea cruris, as seen in this report.
RESUMO
Retiform hemangioendothelioma (RH) is a rare, recently described, unique kind of low grade angiosarcoma. The tumor is characterized by distinctive arborizing blood vessels that mimic the appearance of rete testis. The tumor chiefly originates from the skin and subcutaneous tissue and has a tendency to recur locally. Only one case with lymph node metastasis has been reported previously. Another such rare case of RH is being reported in an adult female who presented with a subcutaneous nodule, underwent multiple recurrences and uniquely metastasized to regional lymph node.
