Adrenocortical tumors

Where’s the delay?

Dr Aftab Aziz, Dr Antonia Brooke, Diabetes and Endocrine department,

RD&E

• Adrenocortical tumors are rare (incidence 1-2 per 1 million) (1)

• They present in a variety of ways and carry a poor prognosis

• 5 cases of adrenocortical tumors and their outcomes

1. B. Allolio & M. Fassnacht. Clinical Review. Adrenocortical Carcinoma: Clinical update. JCEM June 2006. 91(6):2027-2037

Case overview....

• n=5 • Age range 38-76• ♀> (4:1)♂

• Non-specific symptoms• CT Abdomen confirmed adrenal mass

with staging• There were some delays

Case 1: LB, 64 ♀ • Jan 2013: Presented with incidental 9cm right adrenal

mass on US after investigation of fever and pain• Jan – May : further images and endocrinology DELAY in

investigation• 2 May 2013: discussed at MDT• 5 June 2013: open R adrenalectomy and nephrectomy• 5 July 2013: Mitotane started (histology: high mitotic

activity, capsular rupture). • Oct 2013: unable to tolerate therapeutic levels of

Mitotane (tremors and nausea). No evidence of metastases

Case 2: TB, 38 ♀ • Nov 2012: Presented to DGH with abdominal pain. CT

20cm left adrenal mass and probable lung nodules• 13 Dec 2012: Transferred to tertiary centre – L

adrenalectomy. Not thought clinically cushingoid but no secretory studies pre or post operatively. Not seen by endocrinology. Histology showed high mitotic rate, Ki67 index and capsular invasion

• 17 Jan 2013: Admitted SOB with tumour in R ventricle and IVC. Mitotane started DELAY from surgery

• Etoposide, Doxorubicin and Cisplatin discussed but RIP 4 Feb 2013

Case 3: KG, 39 ♂ • Feb 2012: 6M history in features of Cushings DELAY in

presentation• 17 Feb 2012: seen by endocrinology and discussed at

MDT. Referred to tertiary oncology centre due to invasion of IVC (and noted to have pulmonary nodules)

• 24 May 2012: R adrenalectomy, IVC thrombectomy and para-aortic lymphadenectomy

• Surgery at tertiary centre delayed to control Cushings and enable 3 surgeons to operate together DELAY in surgery

• June 2012: started Mitotane (achieved therapeutic levels) and declined chemotherapy

• RIP May 2013

Case 4: RH, 78 ♀ • 12 months history of Cushings and repeated GP visits –

self diagnosis DELAY in referral• 1 March 2012: Private referral (geographically far) DELAY

in local referral• 19 April 2012: R adrenalectomy (noted probable lung

metastases)• 29 May 2012: 2nd opinion from tertiary cancer centre but

opted for local care• June 2012: Mitotane initiated (unable to tolerate

therapeutic levels) and declined chemotherapy• RIP Feb 2013

Case 5: SB, 54 ♀ • 6 month history of vague abdominal ache (declined US

abdo by GP) DELAY by patient to investigate symptoms• 3 April 2013: incidental finding after admission for PE

(11cm adrenal mass invading IVC with liver and lung mets). Cortisol secreting

• 8 April 2013: referred to tertiary hospital MDT for discussion of whether resection an option

• 10 April 2013: Mitotane started• 15 April 2013: Readmitted • 18 April 2013: MDT felt inoperable DELAY of ?2 weeks

for MDT decision (whilst patient was inpatient)• 26 April 2013: rapid decline with hepatic obstruction -

RIP

AgeFirst

presentationBiochemistry Imaging First MDT Surgery Mitotane

Post-op Steroid Rx

Other Rx Outcome

64♀ LBJan 2013

Secondary care(incidental)

Secretory(A4 28.3nmol/l

(0.8-11.9),ODST 9am Cortisol 147nmol/l)

8.5cm R adrenal tumor

(Stage 3)

22/05/13DELAY

05/06/13(Open)

Adrenal surgeonY Y None

Alive+

Mitotane Rx

38♀ TBNov 2012

Secondary care(mass symptoms)

Secretory(cortisol secreting post op,30min Cortisol 875nmol/l)

DELAY

20cm L adrenal tumor & lung

metastasis(Stage 4)

15/01/1313/12/12

(Open)Urologist

YDELAY

Y Palliative RTXRIP

04/02/13

39♂ KG

Feb 2012Secondary care

(secretory symptoms)

Secretory(24 hr Ur Cortisol 2308, 3495,

3868 nmol/l (10-147))

8cm R adrenal tumor, IVC & lung

metastasis(Stage 4)

17/02/12

24/05/12(Open)

Cancer centre (joint approach)

DELAY

Ytherapeutic

YKetoconazoleMetyrapone

RIP07/05/13

76♀ RH

Sep 2011Private

(secretory symptoms)

DELAY

Secretory(24 hr Ur Cort

426, 173, 299 nmol/l (10-147))

13cm L adrenal tumor & lung

metastasis(Stage 4)

N/A

19/04/12(Open)

UrologistDELAY

Y YKetoconazoleMetyrapone

RIP18/02/13

54♀ SB

April 2013Secondary care

(mass symptoms)DELAY

Secretory(24 hr Ur Cortisol 229, 172,

220umol/l (10-147), A4 63.2nmol/l(0.8-11.9), DHEAS

27.1umol/l (0.5-5.56), )

11cm R adrenal tumor, cardiac,lung & liver metastasis

(Stage 4)

11/04/13DELAY

Unstable for surgery

Y Y MetyraponeRIP

26/04/13

Summary…

• All patients had delay in their pathway of adrenocortical cancer

• There was no one point of delay and all patients with invasion of IVC were discussed at tertiary cancer centres

Summary…• Delays– Presentation and referral to secondary care– Referral for surgery, decision by MDTs, operation

date and small delays in starting mitotane

• Mitotane started in all patients: only 1 able to tolerated therapeutic levels, 2 managed subtherapeutic and 2 died prior to therapeutic levels

• Chemo offered to 4 patients with progressive disease: all declined

unanswered questions:•What could have been done better in terms of diagnosis and intervention planning via MDT?

•Does multiple opinions improve outcomes and survival including virtual MDTs?

•How do you increase the awareness of urgent referrals to endocrinology from primary care?