Manifestation of Giant Bilateral Symptomatic Adrenal Myelolipomas in an Adult Patient with Congenital Adrenal Hyperplasia

Adrenal myelolipoma is an uncommon non-functioning tumor that is composed of variable amounts of mature adipose tissue and scattered islands of hematopoietic elements, including erythroid, myeloid, lymphoid series, and megakaryocytes. Adrenal myelolipoma should be differentiated from other fat-containing adrenal masses, such as teratoma, lipoma, and liposarcoma. This case report describes a 50-year-old adult who was raised as a male and developed giant adrenal myelolipomas that presented as symptomatic adrenal masses, and which were misdiagnosed as liposarcoma on radiologic examination. The patient had been raised as a male despite ambiguous genitalia, and a thorough investigation was never carried out because of his poor socioeconomic status. Physical examination showed profound short stature (<-3.0 standard deviation score), hyperpigmentation, and a micropenis without palpable gonads. Both a uterus and ovaries were evident in the pelvic cavity on abdominopelvic computed tomography. Adrenocorticotropic hormone stimulation tests confirmed adrenal insufficiency. Steroid replacement therapy was initiated before bilateral adrenalectomy and the histologic findings indicated myelolipoma. The patient's karyotype was 46, XX and mutation analysis of the CYP21A2 gene identified compound heterozygosity consisting of p.I173N and p.Q319*. The patient was treated with once-daily 0.5 mg dexamethasone and once-daily 0.1 mg fludrocortisone. Because the subject had been raised as a male, additional procedures such as an oophorohysterectomy are currently under consideration. We here describe an adrenal myelolipoma in this case that was complicated by a 21-hydroxylase deficiency. We conclude from our analysis that patients with congenital adrenal hyperplasia should be screened for incidental adrenal masses to avoid unnecessary surgical procedures.

Role of Clinical Stage, PSA and Gleason`s Score in Predicting Pathologic Outcome in Prostate Cancer / 대한비뇨기과학회지

Preoperative clinical staging in the prostate cancer does not always accurately predict the surgical-pathological outcome. We evaluated how the clinical staging, and other clinical parameters including preoperative PSA and Gleason`s score could reflect on the surgicopathological findings in 30 patients with prostate cancer, who underwent radical prostatectomy. Twelve of 24 patients with clinical T1 or T2 disease were understaged by clinical staging determined by digital rectal examination, bone scan, and radiologic studies including CT and MRI with endorectal coil. MRI with endorectal coil accurately reflected the extracapsular disease only in 59.1% of 22 patients studied. At the same time, it also showed low sensitivity (50%) with high specificity (100%) in detecting lymph node metastasis. Preoperative levels of PSA in patients with P2, P3, and N+ disease were 17.8 +/- 4.5, 47.9 +/- 11.3, 93.5 +/- 20.5ng/ml, respectively. The level of PSA was less than 20ng/ml in 9 of 12 patients with P2 disease, while they were greater than 20ng/ml in 9 of 12 patients with P3 disease. PSA may have a role to rule out lymph node metastasis when its level is less than 10ng/ml, although it did not reach the statistical significance because of small sample size. Gleason`s scores in patients with P2 disease were quite similar to those in patients with P3 disease (5.92 +/- 0.69 vs 5.67 +/- 0.56), whereas Gleason`s scores in all 6 patients with N+ disease were 9 or greater. Neoadjuvant hormonal therapy with LH-RH analogue and androgen receptor blocker for 1.5 to 3 months had no impact on the reduction of margin positivity or downstaging in 10 patients. PSA failure rate in patients with P2 and P3 disease was 25% at 1 year after operation. PSA is a good marker for differentiating between P2 and P3 disease (,p=0.0214) and can safely rule out N+ disease if its level is below 10ng/ml, while Gleason`s score may reflect the lymph node metastasis when it is 9 or greater (p=0.0012). Among the candidates for radical prostatectomy, selection of the patients on the basis of PSA and Gleason`s score might improve the surgical-pathological outcome.