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| Journal of the New Zealand Medical Association,
05-November-2010, Vol 123 No 1325
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Oncocytomas are neoplasms that are characterised
histologically by the appearance of epithelial cells with an abundant
eosinophilic, granular cytoplasm. Most oncocytic neoplasms are non-functioning,
however there have been seven reported cases of functioning oncocytic
adrenocortical neoplasms.2,5–7,9,10 We
report a case of a functioning oncocytic adrenocortical carcinoma in a
19-year-old female.
Case reportA 19-year-old female presented with recent onset hirsutism,
acne affecting the face and shoulders, and irregular menses after stopping the
oral contraceptive pill.
Biochemistry revealed an elevated testosterone of 12 nmol/L
(0.5–2.7) and DHEA-S of 17.5 μmol/L (0.5–12). A dexamethasone
suppression test revealed a 0800 plasma cortisol of 108 nmol/L (<50 nmol/L)
indicating incomplete suppression. She had normal post synacthen
17-hydroxyprogesterone, electrolytes, creatinine, liver function tests, urinary
catecholamines and preoperative 24-hour urinary cortisol level.
Abdominal ultrasound followed by CT scan, revealed a
five-by-four centimetre left adrenal mass with central necrosis but no evidence
of invasion into surrounding structures or lymphadenopathy.
The patient underwent a laparoscopic converted to open left
adrenalectomy. There was no evidence of invasion into surrounding structures.
Postoperative recovery was uneventful.
Six weeks postoperatively, the serum testosterone and DHEA-S
levels had both returned to normal and she reported improvements in her acne,
reduced hair growth and the return of regular menses. Follow-up
chest/abdomen/pelvis CT scans performed at 3 and 9 months postoperatively showed
no evidence of tumour recurrence. A repeat dexamethasone suppression test was
also normal 9 months post discharge.
The mass weighed 67 g. Histological findings included
oncocytic cells with granular, eosinophilic cytoplasm, highly pleomorphic nuclei
and infrequent tumour mitoses. There was necrosis but no evidence of vascular or
capsular invasion.
Based on the histological findings, the specimen fulfilled
four of the Weiss criteria and therefore was a malignant adrenocortical
neoplasm. The specimen was sent for a second opinion in Sydney, Australia and
was confirmed to be an adrenal cortical carcinoma by means of advanced
immunohistological staining using IGF2.
DiscussionOncocytic neoplasms are most commonly found in the kidney,
thyroid and salivary glands but are rare in other sites.
We have identified 42 cases of adrenal oncocytic neoplasms
in the English literature comprising 18 cases of adrenocortical oncocytoma, 17
cases of oncocytic adrenocortical carcinoma and 7 cases of adrenocortical
oncocytoma of unknown malignant potential
(UMP).1–10
Of the 17 cases of malignant oncocytic neoplasm arising in
the adrenal gland, the average tumour size was 11.9 cm with a mean weight of
628.4 g. The average age at diagnosis was 52 years and there were 8 male cases
and 9 female cases.
Of the 14 cases that included information regarding
follow-up,[2,3,4,10] 7 had no evidence of
recurrence, 6 had documented recurrence at an average of 26 months following
surgery, and 1 succumbed to their disease at 58 months.
There have been three previously reported cases of tumour
functionality in oncocytic adrenocortical carcinomas. They included two cases of
cortisol secretion alone2,9 and a case of
co-secretion of cortisol and aldosterone.10 Our
case is the first that we are aware of involving elevated serum testosterone and
DHEA-S in an oncocytic adrenocortical carcinoma.
The main histological criteria adopted for the
differentiation of adrenal oncocytic neoplasms is the Weiss criteria. Its
accuracy has been debated recently and Lin et al have proposed a modified system
for use in oncocytic neoplasms.1
In summary, we have presented a unique case of a functioning
oncocytic adrenocortical carcinoma associated with elevated testosterone and
DHEA-S levels. To our knowledge this is the only such case reported to date in
the English literature. Following surgery, the patient’s clinical signs
and symptoms resolved and the serum testosterone and DHEA-S levels returned to
normal.
Author information: Munanga Mwandila,
Surgical Registrar; Hayley Waller, Surgical Registrar; Victoria Stott,
Endocrinologist; Philippa Mercer, General Surgeon; Department of Surgery,
Christchurch Hospital, Christchurch
Acknowledgements: We thank Professor David
Stewart (Dunedin Hospital), Dr Steven Soule (Christchurch Hospital), and Dr
Alistair Murray (Christchurch Hospital) for their assistance.
Correspondence: Dr M Mwandila, Department
of Surgery, Christchurch Hospital, Private Bag 4710, Christchurch, New Zealand.
Fax +64 (0)3 3640352; email: mmunx@hotmail.com
References:
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