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August 7, 2013A 76 year old man presented to his physician with vague right sided chest pain. During his workup, a CT scan of the abdomen and pelvis was performed, which revealed an incidental right sided adrenal nodule measuring 2.2 cm. The liver and kidney showed no abnormalities, and there was no clinical or radiographic evidence of significant lymphadenopathy. Initial blood work revealed normal blood counts and the patient denied any history of night sweats, fever, or weight loss (B-symptoms). Follow-up CT and MRI scans performed six months later showed a persistent adrenal mass without any change in size. At that time, a full biochemical workup was performed, including dexamethasone suppression test, plasma free metanephrines, aldosterone, and plasma renin values. All parameters were within normal limits. Given the small size of the mass and the negative biochemical workup, the decision was made to follow the patient with imaging and laboratory studies. Approximately eight months later – a total of fourteen months after the mass was initially discovered – follow-up CT of the abdomen and pelvis revealed the mass had doubled in size, measuring up to 4.5 cm. A biochemical workup was repeated, including cortisol level (12.2 mcg/dL), plasma renin activity (0.65 ng/mL/h), aldosterone level (2 ng/dL), plasma free metanephrines (47 pg/mL), free normetanephrines (146 pg/mL), and total free metanephrines (193 pg/mL), all falling within normal reference limits. Due to the rapid increase in size, the patient underwent a right adrenalectomy.
On gross examination, the adrenal gland weighed 55.6 grams and measured 9.0 cm×5.5 cm×2.8 cm. Cut sections revealed a pale-tan, nodular, septated, firm, and focally hemorrhagic tumor with a surrounding compressed rim of normal appearing adrenal tissue (see Figure 1). Histologically, the tumor was composed of a multinodular and variably cellular mass with broad intervening fibrous bands (see Figure 2A). Cellular areas demonstrated scattered large Reed-Sternberg cells with two or more nuclei, uneven chromatin distribution, and prominent macronucleoli (see Figure 2B-C). Other large cells with irregular nuclear contours, pale, bubbly chromatin, and prominent nucleoli were also seen (see Figure 2D). A background inflammatory infiltrate was evident which was composed of eosinophils, small lymphocytes, and plasma cells. Scattered mitotic figures and apoptotic bodies were present. A rim of benign adrenal gland tissue was seen at the periphery of the tumor. By immunohistochemistry, the neoplastic Hodgkin cells were positive for CD30, CD15, and Fascin, weakly positive for PAX-5, and negative for CD45 and CD20 (see Figure 3A-D). EBV EBER in situ hybridization was also positive in neoplastic cells, with a subset positive for EBV LMP (see Figure 3E). The lymphocytes in the background were predominantly CD3-positive T-cells with a smaller population of CD20-positive B-cells. Based on the histologic and immunophenotypic findings, a diagnosis of Hodgkin lymphoma, nodular sclerosis subtype was rendered.
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(Author: Matthew M DeNicola, Jonathan W Said, Sheeja T Pullarkat