MedPix® Patient Chart - Case No: 2648 :: - Review Images

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.

- Review Images for PT: 2648

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.

- Review Images for PT: 2648

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.

- Review Images for PT: 2648

Dx: Paraganglioma (extra-adrenal paraganglioma)

Dx Confirmed by: surgery-pathology

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Left adrenalectomy:

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.

Pathology results:

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").