A Rare Neoplasm With A Deceptively Benign Face: A Case Report

Abstract

 Aim : To report a rare case of retiform hemangioendothelioma arising in the parotid region and to emphasize the importance of histopathological evaluation in diagnosing atypical vascular neoplasms. Introduction: Retiform hemangioendothelioma (RHE) is a rare, lowgrade malignant vascular tumor that resembles the rete testis. It is primarily affects young to middle-aged adults with a mean age of onset around 36 years, with a higher incidence in females. RHE presents as a slow-growing, painless mass, often in the distal extremities. Despite its low metastatic potential, RHE is locally aggressive and has a high recurrence rate. Accurate diagnosis relies on histopathological examination and immunohistochemical staining for endothelial markers. The most common locations include the skin and subcutaneous tissue of the lower extremities. Case Description: A 31-year-old male from West Bengal, a shopkeeper, had a gradually progressive, non-tender swelling in front of his right ear for the past 8 years. The swelling, initially small and asymptomatic, grew to 8 x 4 cm. Despite two previous surgical interventions in 2012 and 2015, the swelling recurred and enlarged, indicating a persistent underlying pathology. The patient's swelling was painless and non-tender, without any associated symptoms. The recurrence suggested a persistent pathology, possibly due to incomplete excision or the lesion's nature. Clinical Significance: The present case underscores the need for histopathological confirmation in atypical parotid lesions to avoid misdiagnosis and ensure appropriate surgical management of rare vascular tumors like retiform hemangioen dothelioma. Conclusion: This case highlights the diagnostic challenge posed by rare vascular neoplasms such as retiform hemangioendothelioma, especially when presenting at atypical sites such as the parotid region. Accurate diagnosis through histopathological evaluation is crucial for guiding appropriate surgical management and ensuring long-term disease control.