SFEBES2023 Poster Presentations Adrenal and Cardiovascular (78 abstracts)
Composite Pheochromocytoma with Adrenocortical Carcinoma - a rare coexistence
Aysha Sarwani 1 , Martina Morrin 1 , Jarushka Naidoo 1 , Odharnaith O’Brien 1 , Kassiani Skordilis 2 , Cliona Ryan 1 , Neal Dugal 1 , Arnold Hill 1 , Shari Srinivasan 1 , Mark Sherlock 1 & Michael O’Reilly 1
1Beaumont hospital, Dublin, Ireland. 2University Hospital Birmingham, Birmingham, United Kingdom
A 65-year-old man presenting with urinary symptoms was incidentally discovered to have an adrenal mass. Abdominal computed tomography (CT) revealed a 9.5x8cm heterogenous solid right adrenal tumor with no evidence of metastatic disease. The initial differential lay between adrenocortical carcinoma (ACC) or a phaeochromocytoma. The elevated plasma fractionated normetanephrine at 14439 pmol/l (0-1180), metanephrine 15202 pmol/l (0-510), 3-MT 415 pmol/l (0-180) led to a presumed preoperative diagnosis of phaeochromocytoma. However, there was evidence of autonomous cortisol secretion with a positive overnight dexamethasone suppression test of 209 nmol/l, a suppressed ACTH and an elevated DHEAS of 14.9umol/l. The lesion demonstrated heterogenous I-123 metaiodobenzylguanidine (MIBG) avidity. Right adrenalectomy via an open right-subcostal approach was opted with a possible capsule breach. Histology demonstrated an 8cm, 222g phaeochromocytoma with a PASS of 11/20 with areas of atypical mitoses, tumour stain positive for synaptophysin and chromogranin-A, S100, melan-A and inhibin, however calretinin was negative. Ten weeks post operatively CT scan demonstrated significant disease recurrence in the tumor bed with a 9.4 x 5.2cm mass encasing the IVC, other vessels and invading a segment of the liver. Plasma metanephrines were normal and the lesions were not MIBG-avid with a significant elevation in DHEAS to >27umol/l. A CT guided biopsy was consistent with ACC. Histology demonstrated pleomorphic cells with mitotic rate 12/10hpf. Immunohistochemistry was positive for adrenocortical markers and a Ki67 proliferation 80-90%. The appearance was morphologically similar to the previous resected specimen, although presence of medullary neoplasia made this a challenging histological diagnosis. He had negative genetic testing for the MEN1 gene, DP53, AIP MEN1, CDKN1B, RET and CDC73. He was diagnosed as metastatic adrenocortical carcinoma with malignant Cushing’s, commenced on mitotane and received six cycles of Cisplatin/Etoposide/Doxorubucin. As serial imaging showed disease progression, Gemcitabine and Capecitabine was initiated with consideration of immunotherapy with Pembrolizumab.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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