Related Experiment Video
Updated: Aug 10, 2026
Multifocal Electroretinograms
Published on: December 4, 2011
Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma
Susmita Kundu1, Swapnendu Misra2, Debabani Biswas3
1Department of Respiratory Medicine, R G Kar Medical College, Kolkata, India.
Insights
Multifocal epithelioid hemangioendothelioma, a rare vascular tumor, presented diagnostic and management challenges in a young patient. This rare tumor exhibited unpredictable behavior over a four-year follow-up.
Area of Science:
- Vascular Oncology
- Rare Tumors
- Diagnostic Pathology
Background:
- Epithelioid hemangioendothelioma is a rare vascular neoplasm with intermediate malignancy potential.
- Multifocal presentation can mimic common conditions like lymphadenopathy, complicating diagnosis.
Observation:
- A young female presented with recurrent hemoptysis, neck swelling, and mediastinal mass.
- Initial diagnostic workup including imaging and cytology created a dilemma.
- Diagnosis was confirmed as multifocal epithelioid hemangioendothelioma post-surgical resection and immunohistochemistry.
Findings:
- The patient opted for palliative care due to the rarity and lack of standard chemotherapy.
- After four years of being asymptomatic, the patient relapsed with hemoptysis and mass recurrence.
- The recurrent tumor compressed vital structures, including the superior vena cava and right pulmonary artery.
Implications:
- This case highlights the diagnostic challenges posed by rare vascular tumors.
- The unpredictable clinical course necessitates careful long-term monitoring.
- Management strategies for epithelioid hemangioendothelioma require individualized approaches due to its rarity and variable behavior.
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.
