RESUMO
Based on the literature, there are only three reports available to date on synchronous Kaposi sarcoma (KS) and renal cell carcinoma (RCC), at least to the best of our knowledge. The present study reports a rare case of synchronous classic KS and clear cell RCC. A 69-year-old male presented with painful, purplish nodular lesions on the dorsal aspect of his hands and feet. He had no chronic medical illnesses or prior surgical interventions. An excisional biopsy of one of the lesions revealed a nodular dermal lesion with numerous vascular channels and interlacing spindle cells. A 2.5 cm-enhancing mass was found in a contrast-enhanced computed tomography scan of the abdomen, suggesting RCC or metastasis. A partial nephrectomy was performed, and the histopathological findings were consistent with clear cell RCC. The patient responded well to paclitaxel and topical imiquimod (5%), and the skin lesions disappeared. Both KS and RCC are vascular tumors, and their pathogenesis is commonly affected by an angiogenic factor known as vascular endothelial growth factor (VEGF). A complete response of KS was observed after sorafenib, an inhibitor of VEGF receptors, was administered for the treatment of metastatic renal cancer. This reinforces the fact that there is a common therapeutic and pathogenetic pathway between these two neoplasms. Synchronous KS and clear cell RCC are rare findings. Their simultaneous appearance may be triggered by the common enhancing angiogenic factor, VEGF.
RESUMO
INTRODUCTION: Juvenile hyaline fibromatosis (JHF) is a rare genetic condition characterized by impaired collagen production or metabolism. This study aims to present a rare case of JHF. CASE REPORT: An 11-year-old boy presented with bilateral keloid-like lesions on his ears and admitted intermittent reappearance of such lesions since he was seven. He was born to second-degree relative consanguineous parents. Physical examination revealed bilateral soft pink masses on the ears, multiple scars on the scalp, severe gingival hypertrophy, multiple small soft white papules on the anterior neck, broadly shaped enlargements on the ends of the fingers and toes, and multiple reticulated hard livedoid and hyperpigmented macules on the back and anterior lower extremities. A 5 mm biopsy was taken from the lesion on the ear and histopathological examination of the specimen revealed a normal epidermis but dermal and subcutaneous deposits of nodules composed of abundant amorphous eosinophilic hyaline material with sparse embedded fibroblast associated with areas of congestion and focal hemorrhage. The ear lesions were managed by surgical excision with intraregional steroid injections to prevent relapse. To improve eating ability and oral hygiene, a gingivectomy was planned. DISCUSSION: JHF presents with bone lesions, gingival hypertrophy, joint contractures, and skin lesions. The clinical features usually appear late in infancy and up to 5 years. The condition occurs mostly sporadically. A portion of the cases can be in siblings born to consanguineous parents. CONCLUSION: JHF is a rare genetic disorder that can present even beyond five years. There is no standard treatment for these cases.
