Adrenocortical tumours

Adrenocortical tumours

• 1.
  Adrenocortical tumors Where’s the delay? DrAftab Aziz, Dr Antonia Brooke, Diabetes and Endocrine department, RD&E
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  • Adrenocortical tumorsare 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
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  Case overview.... • • • • • n=5 Age range38-76 ♀>♂ (4:1) Non-specific symptoms CT Abdomen confirmed adrenal mass with staging • There were some delays
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  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
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  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
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  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
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  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
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  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
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  Age First presentation Biochemistry Imaging First MDT Surgery Mitotane Post-op Steroid Rx OtherRx Outcome 64♀ LB Jan 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) 22/05/13 DELAY 05/06/13 (Open) Adrenal surgeon Y Y None Alive + Mitotane Rx 38♀ TB Nov 2012 Secondary care (mass symptoms) Secretory (cortisol secreting post op, 30min Cortisol 875nmol/l) DELAY 20cm L adrenal tumor & lung metastasis (Stage 4) 15/01/13 13/12/12 (Open) Urologist Y DELAY Y Palliative RTX RIP 04/02/13 39♂ KG Feb 2012 Secondary 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 Y therapeutic Y Ketoconazole Metyrapone RIP 07/05/13 76♀ RH Sep 2011 Private (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) Urologist DELAY Y Y Ketoconazole Metyrapone RIP 18/02/13 54♀ SB April 2013 Secondary 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) 11/04/13 DELAY Unstable for surgery Y Y Metyrapone RIP 26/04/13
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  Summary… • All patientshad 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
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  Summary… • Delays – Presentationand 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
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  unanswered questions: •What couldhave 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?