Adrenal Mass
22 year-old F with R adrenal mass discovered on screening ultrasound in 28th week of pregnancy
- Pt delivered a healthy girl at term
- Denies flank pain
- Sometimes feels a “fast pulse”
- No diarrhea
- No visual problems or headaches
- Feels well overall
- Otherwise unremarkable pregnancy
More history:
PMH: None
PSH: None
Meds: None
All: NKDA
Social hx: Married
Family hx: No known malignancies
Exam unremarkable
Recommended initial labs for adrenal mass:
- Hypercortisolemia Eval: Low dose dexamethasone suppression test (high false positive rate in pregnancy and in women taking oral contraceptives) or urinary cortisol (now considered not appropriately sensitive, but best test in pregnancy)
- Hyperaldosteronism Eval: Plasma aldostorone:renin ratio -- only required if patients has hx of hypertension. Electrolytes can be helpful; however, hypokalemia is not routinely seen in modern series of primary aldosteronism.
Labs:
- CMP normal
- Aldosterone 5.4 ng/dl (low normal)
- Renin 13.6 mcu/ml (normal)
- Cortisol: 10.8 mcg/dl (normal)
- 24hr urine VMA, total catecholamines, metanephrine: normal
- Pt delivered a healthy girl at term
- No new headaches, night sweats, fevers, chills etc. . .
- Worsening vision
- Labs were all repeated and no significant changes noted
- Shortly after delivering, she noted oral and vaginal lesions—painful, superficial, white ulcers
Imaging: CT abdpel showed 5.6 x 3.5 cm heterogeneously enhancing mass in the right adrenal region and a 4.0 x 4.5 cm well-circumscribed hyperenhancing lesion just posterior to the main portal vein adjacent to the adrenal mass
Management: Pt taken to OR for R adrenalectomy and partial nephrectomy
- Findings: R adrenal mass extending across midline with LNs wrapping around IVC, tumor adhered to upper pole of R kidney
- Pathology: “Low grade spindle cell proliferation c/w angiomyoid proliferative tumor (Castelman’s disease)”
- No involvement of adrenal gland or kidney
Post-op:
- Pt recovered well from her surgery
- However, she had continued worsening of dermatologic sx’s (particularly mucous membranes)
- Biopsy of lesions consistent with paraneoplastic pemphigus
Paraneoplastic pemphigus:
- May be initial presentation of malignancy
- Typically progressive and often fatal when associated with malignant neoplasm
- Rx: systemic corticosteroids, cyclosporine
- Treatment of underlying disease influences skin lesions only if underlying disease is benign
Castleman’s disease:
- Lymphoproliferative disorder
- Associated with HIV, HHV-8
- Pt negative for both
- Histologic changes are those associated with response to antigenic stimulus
- Disease may be unicentric or multicentric
- Unicentric disease most often an isolated benign dz of young adults
- Typically asymptomatic, detected incidentally
- Surgical resection usually curative=
- Radiation and chemotherapy may be used for persistent disease
- Rituximab (anti-CD20 Ab) and anti IL-6 antibodies have also shown some benefit
