A Young Man with Paraganglioma and Angiosarcoma

Authors: V. Dimov, M.D., Cleveland Clinic
Reviewer: S. Randhawa, M.D.

A 29-year-old CM is admitted to the hospital with CP and a large left-sided pleural effusion. Prior to admission, he had a thoracentesis at an OSH during which 1200 ml of fluids was removed. CT scan of the chest showed a retroperitoneal mass and pleural thickening.

Negative prior to the OSH admission

Metoprolol, furosemide, acetaminophen, oxycodone ER, pregabalin, phenoxybenzamine, esomeprazole

Physical examination:
VS 37.8-132-99/56-24
Lungs: decreased sounds L lung
Heart: Tachycardic, no m/r/g
Abdomen: Soft, non-tender. Bowel sounds normal. No masses or organomegaly
Extremities: + 3 BLE edema

Laboratory results:

Plasma catecholamines in pheochromocytoma

Urine catecholamines in pheochromocytoma

What happened?
The patient was diagnosed with right paraganglioma which is an extra-adrenal pheochromocytoma. Thoracotomy and lung biopsy showed angiosarcoma. Peripheral edema was due to IVC compression by the tumor.

General surgery and oncology services were consulted and an exploratory laparotomy was recommended.

What happened next?
He was started on preoperative alpha and beta-blockers 2 weeks before the surgery. Laparotomy revealed inoperable retroperitoneal tumor with known lung angiosarcoma and paraganglioma. The oncology service will discuss options for chemotherapy with the patient and his family.

Final diagnosis:
Paraganglioma and Angiosarcoma

Pheochromocytoma. eMedicine.

More information:

Adrenal Histopathology: Pheochromocytoma, on YouTube.

Created: 04/13/2007
Updated: 05/07/2007

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