SUN-180 A Case of Deoxycorticosterone-Producing Malignant Adrenocortical Tumor

Background: Hypermineralocorticism (hypertension, hypokalemia, and low plasma renin activity) due to deoxycorticosterone (DOC) excess associated with adrenocortical carcinoma is extremely rare. DOC-producing tumors cause primary aldosteronism-like symptoms presenting low plasma aldosterone with very high DOC levels, and due to weak hormonal DOC activity, its diagnostic is done lately. Generally, malignant cases are progressive with a dismal prognosis. Clinical case: A 61-year-old woman was admitted to our hospital presenting lumbar pain and weight loss of 8 kg, in 2018. Previously, arterial hypertension was diagnosed in 2015, showing a satisfactory control with two classes of antihypertensive drugs. Physical exam: The patient presented no features of Cushing syndrome, but a palpable abdominal mass was noticed in the right flank. Blood pressure was 160x100 mmHg, with sustained high levels, despite regular treatment. Laboratory data: a hypokalemia (K 2.4 mEq/L, nr 3.5 -5.0 mEq/L) and hypernatremia (Na 146 mEq/L, nr 135 to 145 mEq/L), with metabolic alkalosis (venous pH 7.46 and serum bicarbonate 32 mmol/L, nr 23-27 mmol/L) was confirmed. Hormonal tests excluded hypercortisolism and pheocromocytoma. Serum aldosterone and renin were suppressed. Mineralocorticoid precursors dosage was extremely high, DOC (654 ng/dL, nr < 25 ng/dL) and progesterone (5.0 ng/mL, nr <0.89 ng/mL), as well 11-deoxycortisol (7.2 ng/mL, nr <0.5 ng/mL). Radiological imaging: abdominal CT showed a heterogeneous hypervascular adrenal mass (13.0x13.0x21.0 cm) exhibiting central necrosis, suggesting malignancy. FDG-PET/CT scan showed a hypermetabolic adrenal mass (SUVmax=13.8). Also, two metabolically active pulmonary nodules (SUVmax=3.7) measuring 0.7 and 0.4 cm were detected. The patient underwent right adrenalectomy, and the tumor was removed (24x13x13 cm). According to Weiss criteria (8/9) and modified Weiss criteria (5/7), the tumor was considered an adrenocortical carcinoma. Immunohistochemistry revealed a low Ki-67 index (10%). After the surgical procedure, all adrenal steroid levels normalized, and mitotane was prescribed as adjuvant therapy. Although the pulmonary nodules were stable at the four-month follow-up, the abdominal CT-scan revealed a heterogeneous nodule (3.7cm) in the left adrenal gland, which was suspicious of metastasis. Conclusion: DOC-producing adrenocortical tumors are heterogeneous regarding tumor size, clinical behavior, hormonal and metabolites secretion, and disease-free and overall survival; however, it is common hypokalemia, hypertension, and other symptoms as abdominal pain, due to tumor growth, and weight loss. The association of arterial hypertension with hypokalemia and elevated 11-deoxycortisol, with normal aldosterone and renin, lead to the need for mineralocorticoid precursors evaluation in patients with adrenocortical tumor.

Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

Background/Aims: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. Methods: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years). Outcomes of interest were: alive with no evidence of disease, alive with evidence of disease, and dead of disease. Results: Median age at onset of symptoms was 15.7 years (n = 41; range, 0.2–21 years). Female:male ratio was 3.6: 1. Mixed symptomatology (> 1 hormone abnormality) was the most common presentation (54%, n = 22). Sixty-six percent of patients (n = 27 out of 41) underwent total adrenalectomy. Metastatic disease was more common in children aged > 12 years (p = 0.002 compared to < 4 years). The most common sites of metastases were the liver and lungs. Overall 2-year and 5-year survival rates were 61% (95% CI 45–77) and 46% (95% CI 30–62), respectively. Metastasis at the time of diagnosis was independently associated with poor prognosis (risk ratio 13.7%; 95% CI 3.9–87.7). Weiss criteria (29%) and modified Weiss criteria (33%) were less accurate in younger patients (< 12 years), compared to the Wieneke index (100%). Conclusion: The presence of metastases was an independent prognostic factor. The Wieneke index was the most accurate in predicting clinical outcomes in younger children.

Desmoplastic Medulloblastoma Metastatic to the Pancreas: Case Report

A case is reported in which a desmoplastic medulloblastoma metastasized to the pancreas and to the surrounding soft tissues but did not recur locally or disseminate within the central nervous system. Multidrug chemotherapy and local radiation therapy resulted in a complete remission. In all four previously reported cases of medulloblastoma metastasizing to the pancreas, the diagnosis was not made until the postmortem examination, and all of these patients also had extensive metastases in other organ systems. Modification of the Weiss criteria defining extraneural metastases from tumors of the central nervous system is suggested.

Metastatic Medulloblastoma

SUMMARY:The incidence of metastatic medulloblastoma is probably greater than the thirteen documented patients. Case reports of metastatic medulloblastoma must be carefully analysed. We have added two cases which conform to Weiss’ criteria for metastatic disease. The direction and factors influencing spread have been discussed.