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Letters to the Editor
(Fig. 1D) support this notion. Li and colleagues have previously reported three cases of phaeochromocytomas (original tumour size between 5.5 and 6.5 cm) recurring 3–4 years after apparently successful laparoscopic adrenalectomies, also thought to be secondary to laparoscope-associated tumour seeding.5 Furthermore, a recent study has reported five further cases of peritoneal seeding after laparoscopic surgery for phaeochromocytomas; three of these patients had large tumours between 7.0 and 11.0 cm, while the remaining two patients had tumours sized 4 cm and 2.5 cm respectively.6 Together, these observations suggest that laparoscopic adrenalectomy carries a potential risk of tumour seeding from phaeochromocytomas, particularly with large
References 1 Conzo G, Pasquali D, Colantuoni V, Circelli L, Tartaglia E, Gambardella C et al. Current concepts of pheochromocytoma. Int J Surg 2014; 12: 469–74. 2 Soon PS, Yeh MW, Delbridge LW, Bambach CP, Sywak MS, Robinson BG et al. Laparoscopic surgery is safe for large adrenal lesions. Eur J Surg Oncol 2008; 34: 67–70. 3 Carter YM, Mazeh H, Sippel RS, Chen H. Safety and feasibility of laparoscopic resection for large (≥6 cm)
tumours, and support the current Endocrine Society guidelines, which recommend open resection for large (e.g. >6 cm) or invasive phaeochromocytomas to ensure complete tumour resection, prevent tumour rupture and avoid local recurrence.7 Received 14 July 2014; accepted 22 October 2014. doi:10.1111/imj.12678 1
1
J. Wilson, A. Brooke, J. Dunn,2 A. Goodman,3 C. Hamilton-Wood4 and B. Vaidya1 Departments of 1Endocrinology, 2Surgery, 3Oncology, and 4 Radiology, Royal Devon and Exeter Hospital, Exeter, UK
pheochromocytomas without suspected malignancy. Endocr Pract 2012; 18: 720–6. 4 Conzo G, Musella M, Corcione F, De Palma M, Ferraro F, Palazzo A et al. Laparoscopic adrenalectomy, a safe procedure for pheochromocytoma. A retrospective review of clinical series. Int J Surg 2013; 11: 152–6. 5 Li ML, Fitzgerald PA, Price DC, Norton JA. Iatrogenic pheochromocytomatosis: a previously unreported result of laparoscopic adrenalectomy. Surgery 2001; 130: 1072–7.
6 Rafat C, Zinzindohoue F, Hernigou A, Hignette C, Favier J, Tenenbaum F et al. Peritoneal implantation of pheochromocytoma following tumor capsule rupture during surgery. J Clin Endocrinol Metab 2014; 99: E2681–5. 7 Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99: 1915–42.
General correspondence
Improving the system for managing cancer pain People living with metastatic cancer experience a high burden of symptoms, which have a significant impact on their quality of life. Untreated symptoms and the associated distress, as we read with sadness in Watts’ letter,1 have a significant and long-lasting impact on the dying and their family and friends. There are effective evidencebased strategies for managing pain and other symptoms in people with cancer, yet evidence shows that Watts’ experience is not an isolated one.2,3 Watts’ call to prevent this from happening to others in the future is a timely one and one we echo. Watts suggests that a management plan be formed following detection of discomfort. There are Australian
guidelines on the Cancer Council Australia wiki to assist in the formulation of such plans. The key elements are screening for pain, comprehensive assessment of pain, regular analgesia, breakthrough analgesia, prevention of adverse effects of opioids (constipation and nausea) and patient education.4 These guidelines have been developed following a robust process of systematic reviews, surveys of current practice and consultation with relevant stakeholders.5,6 Watts also highlights the risks associated with transitions of care, waiting in one facility for a bed in another. Thoroughly documented pain management plans would be one step to reducing the risk of uncontrolled pain. People need to be confident they can have access to good pain relief in primary care or any other healthcare setting, and the guidelines mentioned above are available
© 2015 Royal Australasian College of Physicians
361
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Letters to the Editor
to all health professionals. Health professional education is essential to reduce the barriers to pain management. The implementation of the guideline with the strategies outlined above is currently the subject of a National Breast Cancer Foundation-funded randomised controlled trial across 14 sites (ACTRN12615000064505). Let no one else be diminished by inadequate treatment of the discomfort of the dying.
M. R. Lovell,1,2,3 J. Phillips,4 T. Luckett3,4,5 and M. Agar1,3,5,6 1 Palliative Care, HammondCare, 2Sydney Medical School, The University of Sydney, 3Improving Palliative Care through Clinical Trials (ImPaCCT), 4Centre for Cardiovascular and Chronic Care, University of Technology Sydney, Faculty of Health, 5South Western Sydney Clinical School, University of New South Wales, 6 Ingham Institute of Applied Medical Research, Sydney, New South Wales, Australia
Received 7 December 2014; accepted 17 December 2014. doi:10.1111/imj.12677
References 1 Watts MR. Stakes are high when caring for the dying. Intern Med J 2014; 44: 822–3. 2 Deandrea S, Montanari M, Moja L, Apolone G. Prevalence of undertreatment in cancer pain. A review of published literature. Ann Oncol 2008; 19: 1985–91. 3 National Pain Summit Initiative. National Pain Strategy. Melbourne: Faculty of Pain Medicine; 2010.
4 Australian Adult Cancer Pain Management Working Party. Cancer pain management in adults. 2014 [cited 2014 Dec 7]. Available from URL: http://wiki. cancer.org.au/australiawiki/index.php? oldid=89991. 5 Luckett T, Davidson PM, Green A, Boyle F, Stubbs J, Lovell M. Assessment and management of adult cancer pain: a systematic review and synthesis of recent qualitative studies aimed at developing insights for managing barriers and
Familial pulmonary arterial hypertension at a tertiary referral unit: patterns in presentation and prognosis Familial and sporadic pulmonary arterial hypertension (PAH) fall into the World Health Organisation Group 1 for PAH. The familial form of the disease accounts for at least 6% of all cases of PAH.1 It has previously been thought that familial pulmonary arterial hypertension (fPAH) has a worse prognosis when compared with sporadic PAH,2 although with the widespread use of advanced PAH therapies, this is less clear. In addition, there is uncertainty whether fPAH shows genetic anticipation with patients developing symptomatic PAH at an earlier age as the disease is passed on to later generations.3,4 The aim of this study was to review a group of patients with fPAH at a tertiary referral centre to see if we could clarify these two key clinical questions. Clinical case notes of six families, 13 patients in total, with fPAH, managed at a single tertiary referral centre, were retro-
optimizing facilitators within a comprehensive framework of patient care. J Pain Symptom Manage 2013; 46: 229–53. 6 Lovell M, Agar M, Luckett T, Davidson PM, Green A, Clayton J. Australian survey of current practice and guideline use in adult cancer pain assessment and management: perspectives of palliative care physicians. J Palliat Med 2013; 16: 1403–9.
spectively evaluated. Data were collected on age at diagnosis of each generation and survival. Survival data were compared with survival data in patients with sporadic PAH at the same centre. The mean follow-up time for patients was 6.4 years. Survival in the group with fPAH was similar to that of sporadic PAH (Fig. 1). Almost all patients remain alive and median survival could not be estimated. However, 6-year survival rates (80% confidence interval (CI) 65–95 for fPAH and 72.5% CI 67–75.8 for sporadic PAH) are significantly higher than documented survival in previous studies.2 There were no significant differences in gender or cardiopulmonary haemodynamics between the two groups at diagnosis. Patients with sporadic PAH tended to be older than patients with fPAH at diagnosis. Median (range) of sporadic patients was 48 (14–88) years compared with 26 (12–57) for fPAH (P < 0.05). Within each family, the age of diagnosis was younger in later generations. This was also the case when assessed in the group as a whole with the mean age of diagnosis for generations 1, 2 and 3 being 50.6, 21.7 and 14, © 2015 Royal Australasian College of Physicians
362
Letters to the Editor
(Fig. 1D) support this notion. Li and colleagues have previously reported three cases of phaeochromocytomas (original tumour size between 5.5 and 6.5 cm) recurring 3–4 years after apparently successful laparoscopic adrenalectomies, also thought to be secondary to laparoscope-associated tumour seeding.5 Furthermore, a recent study has reported five further cases of peritoneal seeding after laparoscopic surgery for phaeochromocytomas; three of these patients had large tumours between 7.0 and 11.0 cm, while the remaining two patients had tumours sized 4 cm and 2.5 cm respectively.6 Together, these observations suggest that laparoscopic adrenalectomy carries a potential risk of tumour seeding from phaeochromocytomas, particularly with large
References 1 Conzo G, Pasquali D, Colantuoni V, Circelli L, Tartaglia E, Gambardella C et al. Current concepts of pheochromocytoma. Int J Surg 2014; 12: 469–74. 2 Soon PS, Yeh MW, Delbridge LW, Bambach CP, Sywak MS, Robinson BG et al. Laparoscopic surgery is safe for large adrenal lesions. Eur J Surg Oncol 2008; 34: 67–70. 3 Carter YM, Mazeh H, Sippel RS, Chen H. Safety and feasibility of laparoscopic resection for large (≥6 cm)
tumours, and support the current Endocrine Society guidelines, which recommend open resection for large (e.g. >6 cm) or invasive phaeochromocytomas to ensure complete tumour resection, prevent tumour rupture and avoid local recurrence.7 Received 14 July 2014; accepted 22 October 2014. doi:10.1111/imj.12678 1
1
J. Wilson, A. Brooke, J. Dunn,2 A. Goodman,3 C. Hamilton-Wood4 and B. Vaidya1 Departments of 1Endocrinology, 2Surgery, 3Oncology, and 4 Radiology, Royal Devon and Exeter Hospital, Exeter, UK
pheochromocytomas without suspected malignancy. Endocr Pract 2012; 18: 720–6. 4 Conzo G, Musella M, Corcione F, De Palma M, Ferraro F, Palazzo A et al. Laparoscopic adrenalectomy, a safe procedure for pheochromocytoma. A retrospective review of clinical series. Int J Surg 2013; 11: 152–6. 5 Li ML, Fitzgerald PA, Price DC, Norton JA. Iatrogenic pheochromocytomatosis: a previously unreported result of laparoscopic adrenalectomy. Surgery 2001; 130: 1072–7.
6 Rafat C, Zinzindohoue F, Hernigou A, Hignette C, Favier J, Tenenbaum F et al. Peritoneal implantation of pheochromocytoma following tumor capsule rupture during surgery. J Clin Endocrinol Metab 2014; 99: E2681–5. 7 Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99: 1915–42.
General correspondence
Improving the system for managing cancer pain People living with metastatic cancer experience a high burden of symptoms, which have a significant impact on their quality of life. Untreated symptoms and the associated distress, as we read with sadness in Watts’ letter,1 have a significant and long-lasting impact on the dying and their family and friends. There are effective evidencebased strategies for managing pain and other symptoms in people with cancer, yet evidence shows that Watts’ experience is not an isolated one.2,3 Watts’ call to prevent this from happening to others in the future is a timely one and one we echo. Watts suggests that a management plan be formed following detection of discomfort. There are Australian
guidelines on the Cancer Council Australia wiki to assist in the formulation of such plans. The key elements are screening for pain, comprehensive assessment of pain, regular analgesia, breakthrough analgesia, prevention of adverse effects of opioids (constipation and nausea) and patient education.4 These guidelines have been developed following a robust process of systematic reviews, surveys of current practice and consultation with relevant stakeholders.5,6 Watts also highlights the risks associated with transitions of care, waiting in one facility for a bed in another. Thoroughly documented pain management plans would be one step to reducing the risk of uncontrolled pain. People need to be confident they can have access to good pain relief in primary care or any other healthcare setting, and the guidelines mentioned above are available
© 2015 Royal Australasian College of Physicians
361
bs_bs_banner
Letters to the Editor
to all health professionals. Health professional education is essential to reduce the barriers to pain management. The implementation of the guideline with the strategies outlined above is currently the subject of a National Breast Cancer Foundation-funded randomised controlled trial across 14 sites (ACTRN12615000064505). Let no one else be diminished by inadequate treatment of the discomfort of the dying.
M. R. Lovell,1,2,3 J. Phillips,4 T. Luckett3,4,5 and M. Agar1,3,5,6 1 Palliative Care, HammondCare, 2Sydney Medical School, The University of Sydney, 3Improving Palliative Care through Clinical Trials (ImPaCCT), 4Centre for Cardiovascular and Chronic Care, University of Technology Sydney, Faculty of Health, 5South Western Sydney Clinical School, University of New South Wales, 6 Ingham Institute of Applied Medical Research, Sydney, New South Wales, Australia
Received 7 December 2014; accepted 17 December 2014. doi:10.1111/imj.12677
References 1 Watts MR. Stakes are high when caring for the dying. Intern Med J 2014; 44: 822–3. 2 Deandrea S, Montanari M, Moja L, Apolone G. Prevalence of undertreatment in cancer pain. A review of published literature. Ann Oncol 2008; 19: 1985–91. 3 National Pain Summit Initiative. National Pain Strategy. Melbourne: Faculty of Pain Medicine; 2010.
4 Australian Adult Cancer Pain Management Working Party. Cancer pain management in adults. 2014 [cited 2014 Dec 7]. Available from URL: http://wiki. cancer.org.au/australiawiki/index.php? oldid=89991. 5 Luckett T, Davidson PM, Green A, Boyle F, Stubbs J, Lovell M. Assessment and management of adult cancer pain: a systematic review and synthesis of recent qualitative studies aimed at developing insights for managing barriers and
Familial pulmonary arterial hypertension at a tertiary referral unit: patterns in presentation and prognosis Familial and sporadic pulmonary arterial hypertension (PAH) fall into the World Health Organisation Group 1 for PAH. The familial form of the disease accounts for at least 6% of all cases of PAH.1 It has previously been thought that familial pulmonary arterial hypertension (fPAH) has a worse prognosis when compared with sporadic PAH,2 although with the widespread use of advanced PAH therapies, this is less clear. In addition, there is uncertainty whether fPAH shows genetic anticipation with patients developing symptomatic PAH at an earlier age as the disease is passed on to later generations.3,4 The aim of this study was to review a group of patients with fPAH at a tertiary referral centre to see if we could clarify these two key clinical questions. Clinical case notes of six families, 13 patients in total, with fPAH, managed at a single tertiary referral centre, were retro-
optimizing facilitators within a comprehensive framework of patient care. J Pain Symptom Manage 2013; 46: 229–53. 6 Lovell M, Agar M, Luckett T, Davidson PM, Green A, Clayton J. Australian survey of current practice and guideline use in adult cancer pain assessment and management: perspectives of palliative care physicians. J Palliat Med 2013; 16: 1403–9.
spectively evaluated. Data were collected on age at diagnosis of each generation and survival. Survival data were compared with survival data in patients with sporadic PAH at the same centre. The mean follow-up time for patients was 6.4 years. Survival in the group with fPAH was similar to that of sporadic PAH (Fig. 1). Almost all patients remain alive and median survival could not be estimated. However, 6-year survival rates (80% confidence interval (CI) 65–95 for fPAH and 72.5% CI 67–75.8 for sporadic PAH) are significantly higher than documented survival in previous studies.2 There were no significant differences in gender or cardiopulmonary haemodynamics between the two groups at diagnosis. Patients with sporadic PAH tended to be older than patients with fPAH at diagnosis. Median (range) of sporadic patients was 48 (14–88) years compared with 26 (12–57) for fPAH (P < 0.05). Within each family, the age of diagnosis was younger in later generations. This was also the case when assessed in the group as a whole with the mean age of diagnosis for generations 1, 2 and 3 being 50.6, 21.7 and 14, © 2015 Royal Australasian College of Physicians
362
