Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma

Abstract

A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor.

Clinico‐radiological features and next generation sequencing of pulmonary epithelioid hemangioendothelioma: A case report and review of literature

“…The development of the electron microscope and immunohistochemical techniques confirmed the endothelial lineage of the tumor cells and the term epithelioid hemangioendothelioma was coined in 1986 . Clinically, EH is a rare vascular tumor with low to borderline malignancy between a benign hemangioma and a malignant angiosarcoma . It is reported to arise from many organ systems, including the liver, bone, soft tissue, mediastinum, and lungs …”

“…Pulmonary EH is often diagnosed by chance because in most cases patients do not present with obvious symptoms . Nevertheless, in most cases, patients complain of chest problems, such as dyspnea, cough, chest pain, and hemoptysis .…”

“…4 Clinically, EH is a rare vascular tumor with low to borderline malignancy between a benign hemangioma and a malignant angiosarcoma. 5,6 It is reported to arise from many organ systems, including the liver, bone, soft tissue, mediastinum, and lungs. 7,8 The mortality rate associated with EH varies according to the location of the tumor, reported as 13% in soft tissue, 35% in the liver, and 65% in lung tumors.…”

“…Pulmonary EH is often diagnosed by chance because in most cases patients do not present with obvious symptoms. 6,7,11,12 Nevertheless, in most cases, patients complain of chest problems, such as dyspnea, cough, chest pain, and hemoptysis. [13][14][15] Previous studies have found out that women tend to suffer from PEH more often than men and the mean age of patients suffering from PEH is around 40 years.…”