A 26-year-old white woman with an approximately 10-year history of hypertension that is poorly responsive to medical therapy. The patient has noted increasing headaches, sweating, and hot flashes over the past 6 months. She denies palpitations, dyspnea, fevers, nausea, vomiting, diarrhea or constipation.
Vital signs: BP: 130/90, HR: 90, RR: 20, T: 36.6.
No focal physical findings
24 hour urine collection:
Norepi: 790 mcg
Epi: <5.0 mcg
Dopa: 302 mcg
VMA: 17.1 mg
Metanephrines: 4.0 mg
Glucagon and Clonidine tests positive.
Small ovoid para-aortic mass with very intense signal on T2WI. It enhances on images not provided. The adrenals are normal.
It corresponds with a focus of MIBG uptake on nuclear medicine imaging.
If no clinical information or MIBG imaging were available, the MR findings would still be fairly specific for a paraganglioma, but other possibilities for an enhancing retroperitoneal mass would still have to be considered, including adenopathy, fibromatosis, peripheral nerve sheath tumors, and sarcomas.
Dx: Paraganglioma (extra-adrenal paraganglioma)
Dx Confirmed by: surgery-pathology
A large mass was found to be intimate with the Left renal artery, but was ultimately resectable. This mass was contiguous with Left adrenal gland, but also extended more medially over top of SMA. Dense lymphatic tissue from tumor extended anterior and posterior to SMA. Mass was noted to be partially fed by arterial source which branched directly from Aorta. Mass was resected and sent to pathology. Upon final abdominal inspection, oozing was noted from splenic capsule. Attending physician believed splenic capsule was likely nicked during surgery and safest option was splenectomy. Splenectomy was performed without further complications.
1) Soft tissue mass - Left peri-adrenal tissue containing firm, 4 x 3.5 x 3.0 cm nodule in fibro-fatty tissue with yellow and brown/tan appearance. Most consistent with paraganglioma.
2) Left adrenal gland - no tumor noted.
3) Spleen - no tumor noted.
DIAGNOSIS: hormonally active para-aortic paraganglioma ("extra-adrenal pheochromocytoma").
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