IMAGES FOR SURGEONS
ANZJSurg.com
Management of adrenocortical carcinoma
A 46-year-old woman presented with a 2-week history of a painless swelling in the left upper abdomen. Physical examination revealed a visible and palpable mass in the left upper abdominal quadrant. An abdominal ultrasound scan demonstrated a 14-cm well-defined mass, possibly left adrenal in origin. Plasma catecholamines, renin, aldosterone and cortisol were within normal ranges. Subsequent computed tomography (CT) imaging showed a well-defined, heterogenous 15.6 x 11.7 × 16.8 cm retroperitoneal mass, displacing the lower pole of the left kidney but not arising directly from it (Fig. 1). The left adrenal gland could not be identified separately. Following multidisciplinary team discussion, a laparotomy and left adrenalectomy was deemed the most appropriate management.
The mass was excised completely intact and analyzed in the laboratory. The tumour dimensions were 160 x 120 × 143 mm with a weight of 1935 grams. There was a recognizable adrenal gland present at one edge of the specimen measuring 35 × 15 mm. The cut surface of the tumour had a nodular, solid and fleshy appearance with large areas of necrosis and haemorrhage present (Fig. 2).
Histologically, the tumour was composed almost entirely of solid sheets of eosinophilic epithelial cells with foci of coagulative necro- sis (Fig. 3). The nuclei were moderately pleomorphic with foci of marked pleomorphism. Mitotic activity was frequent (21 mitotic figures per 50 high-power fields) with occasional abnormal mitotic figures noted. The tumour was encapsulated and confined to the adrenal gland by the adrenal capsule. These findings were those of an oncocytic adrenocortical carcinoma, completely excised, with staging pT2 NX R0.
VIS/THORAX PIVC
INMO L 6
A
Adrenal masses are among the most frequent tumours in humans, with the vast majority being benign. Adrenocortical carcinoma (ACC) is a rare, very aggressive malignancy affecting only 1-2 million people per year.1 ACC has a bimodal distribution, in which there is a relatively high incidence in children less than 5 years of age and in adults in their fourth and fifth decades of life.2
ACC often presents with symptoms of hormonal excess or a palpable mass. In approximately 60% of cases, the presentation of
O
3
.
5
.
1
.
e
# PH002759N
10
12
15
E
autonomous adrenocortical steroid excess, mainly Cushing’s syn- drome or worsening androgenization in females can often lead to diagnosis. In those with hormonally inactive ACC, symptoms relate to local mass effects of the tumour, including abdominal discomfort, back pain, nausea or vomiting. In these cases, most tumours exceed more than 10 cm in diameter. An increasing percentage of ACCs are picked up incidentally because of the more frequent use of abdominal imaging.2
Adrenocortical carcinomas are staged on the basis of size, local invasion and the presence or absence of metastatic disease. Adrenal surgery is the treatment of choice only when a complete resection can be achieved, therefore staging prior to surgery is essential to rule out local invasion or invasion of adjacent organs and distant metas- tases. A third of patients present with distant metastases, with lung and liver being the most common sites.2
Adrenocortical carcinoma is associated with a poor prognosis and can be managed surgically or medically depending on the stage at presentation. Surgical treatment involves open or laparoscopic adrenalectomy. In patients with ACC, which is resectable at presen- tation, up to 80% experience relapse locally or develop metastases.2 Five-year survival after complete resection is 16-47% and there is a median survival of <1 year in patients with incomplete resection.2
Medical management consists of mitotane, cytotoxic drugs or radiotherapy. Mitotane was the first anti-neoplastic drug specifi- cally introduced for the management for ACC more than 50 years ago. The exact mechanism of action remains unknown. Side effects include gastrointestinal upset, elevation of liver function tests, leukopenia and confusion.3 Due to the high risk of recurrence in ACC, most clinicians agree that the use of mitotane should be used as adjuvant therapy. Where there is advanced disease at pres- entation, or recurrence that is not amenable to a complete resec- tion, then mitotane can be given alone or in combination with cytotoxic drugs.2
Patients with ACC must be closely monitored to ensure recur- rence is detected at an early stage. Abdominal CT or magnetic resonance imaging plus chest CT should be repeated three-monthly for 2 years. Recurrence may still occur after a 2-year period, there- fore follow up in suggested for at least 10 years but the intervals of imaging may be lengthened.2
Adrenocortical carcinoma is a rare, aggressive malignancy accounting for approximately 0.02% of all cancers. Patients with ACC require a multidisciplinary team discussion at initial presenta- tion and at disease relapse and progression. Our case highlights the possible management options of such a rare tumour and the impor- tance of complete surgical resection.
Informed consent was obtained from the patient for the case report.
References
1. Wang C, Sun Y, Wu H et al. Distinguishing adrenal cortical carcinomas and adenomas: a study of clinicopathological features and biomarkers. Histopathology 2014; 64: 567-76.
2. Zini L, Porpiglia F, Fassnacht M. Contemporary management of adreno- cortical carcinoma. Eur. Urol. 2011; 60: 1055-65.
3. Terzolo M, Angeli A, Fassnacht M et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N. Engl. J. Med. 2007; 356: 2372-80.
Lianne Thomas,* MBChB, MRCP Thomas Needham, } MBBS Ursula Earl,* MBChB, MRCPath Wael Elsaify,¿ MBBCh (Hons), FRCS *Department of Histopathology, James Cook University Hospital, Middlesbrough, UK and +Department of Endocrine Surgery, James Cook University Hospital, Middlesbrough, UK
doi: 10.1111/ans.13352