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Letters to the Editor
fibrous-walled cysts, lined by a single flattened layer of attenuated cells (Fig. 1c,d) without evidence of cytological atypia. There were lymphatic vascular channels, separated by fibrous material; some contained aggregates of adrenal cells. Immunoperoxidase staining showed positive staining for factor VIII and D2-40 immunostain; epithelial markers (AE1/AE3, CAM 5.2) and CD34 were negative. Cystic adrenal masses have an incidence approximating 0.06% of the general population.1 Within the differential diagnosis of these lesions, adrenal lymphangiomas are a very rare cause. Lymphangiomas are benign tumours of the lymphatic system, arising from malformations originating in embryogenesis.2 They are characterised by thin-walled, cystic lesions lined by endothelial cells and filled with lymph fluid.2 The literature suggests that adrenal lymphangiomas are more frequently rightsided, with a female predominance.3 While most adrenal lymphangiomas are clinically asymptomatic,3 patients can present with flank pain, gastrointestinal symptoms or a palpable mass, relating to the size and position of the cyst.4 The lack of distinctive symptoms and laboratory findings make preoperative diagnosis challenging.5 The acute pain presentation of this case may have occurred due to bleeding into a cyst. Adrenal lymphangiomas on CT imaging are characteristically non-enhancing, hypodense masses with an
References 1 Bellantone R, Ferrante A, Raffaelli M, Boscherini M, Lombardi CP, Crucitti F. Adrenal cystic lesions: report of 12 surgically treated cases and review of the literature. J Endocrinol Invest 1998; 21: 109–14. 2 Wiegand S, Eivazi B, Barth PJ, Berens von Rautenfeld D, Folz BJ,
appearance indistinguishable to other cystic adrenal lesions, including cystic pheochromocytoma and adrenal cortical carcinoma.6 Surgical resection is usually necessary to distinguish these benign lesions from potentially aggressive malignant lesions, in addition to relieving symptoms.3 A possible diagnosis of a lymphangioma should be considered when a hypodense, non-enhancing cystic, nonfunctioning adrenal mass is found in youth or middle age. Histological diagnosis of non-functioning adrenal masses must be undertaken with care, preferably in a unit specialising in endocrinology or endocrine surgery. As the differential diagnosis of larger non-functioning masses includes adrenal or metastatic carcinoma, excision biopsy is preferred, rather than fine-needle aspiration biopsy.3,4 Due to the rarity of these tumours, their natural history is not known. Whether these tumours may be observed (after evaluation of functional status and exclusion of malignancy) is not known. Received 24 June 2013; accepted 3 October 2013. doi:10.1111/imj.12361
E. Blanchard,1 P. Brenner,2 W. Delprado3 and K. Samaras1,4 1
Diabetes and Obesity Program, Garvan Institute of Medical Research, Departments of 2Urology and 4Endocrinology, St Vincent’s Hospital and 3School of Medicine Sydney, Notre Dame University, Sydney, New South Wales, Australia
Mandic R et al. Pathogenesis of lymphangiomas. Virchows Archiv 2008; 453: 1–8. 3 Ellis CL, Banerjee P, Carney E, Sharma R, Netto GJ. Adrenal lymphangioma: clinocopathologic and immunohistochemical characteristics of a rare lesion. Hum Pathol 2011; 42: 1013–8. 4 Erickson LA, Lloyd RV, Hartman R, Thompson G. Cystic adrenal neoplasms.
Recurrence of Carney complex atrial myxoma causing embolic stroke Carney complex is a rare genetic disease that can lead to the formation of multiple atrial myxomas. These tumours have the propensity to fragment, causing strokes and other embolic events. We present the case of a patient who suffered a stroke due to recurrence of a Carney complex atrial myxoma. © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
American Cancer Society 2004; 101: 1537–44. 5 Cobanoglu B, Karatas P, Serhatlioglu S, Dogru O. Cystic adrenal lymphangioma: differential diagnosis. Turkiye Klinikleri J Med Sci 2009; 29: 566–8. 6 Dunnick NR, Korobkin M, Francis I. Adrenal radiology: distinguishing benign from malignant adrenal masses. Am J Roentgenol 1996; 167: 861–7.
A 58-year-old woman presented to the emergency department following a collapse with no associated loss of consciousness. In the department, she was confused and agitated, and reported blurred vision. A neurological examination revealed a left-sided motor deficit, visual agnosia and perseveration. The patient had a known diagnosis of Carney complex. She had previously undergone surgical excision of myxomas of her left atrium, bowel and uterus, as well as
307
Letters to the Editor
a
b
c
d
bilateral adrenalectomies for cortisol-secreting tumours. The previous left atrial myxoma had been excised 21 years prior to this presentation. Her last screening transthoracic echocardiogram was performed 2 years prior to this presentation when she underwent excision of her uterine myxoma. This showed no recurrence of cardiac myxoma. Before this, she had been lost to follow up after moving interstate 6 years previously. Initial non-contrast brain computed tomography showed no acute haemorrhage or gross ischaemia; however, the images produced were noted to be of poor quality due to movement artefact. Over the next day, her symptoms resolved and she was able to undergo brain magnetic resonance imaging. This showed extensive chronic and acute cardioembolic infarcts involving multiple cerebral artery territories (Fig. 1). 308
Figure 1 Figure 1 shows both the acute ischaemia seen on diffusion-weighted imaging and the chronic haemorrhage seen on susceptibility-weighted imaging. Acute ischaemia can be seen in both cerebellar hemispheres (a), as well as both occipital lobes and the right frontal lobe (b). Old haemorrhage can be seen in both occipital lobes (c) and both parietal lobes (d).
Following this, transthoracic echocardiography was performed, which showed a large left atrial mass, suspicious of atrial myxoma. This was confirmed with transoesophageal echocardiography and cardiac magnetic resonance imaging. The patient was then referred to the regional cardiothoracic centre for definitive excision of the tumour. Carney complex is an autosomal dominant condition that predisposes the affected individual to skin lentignoses, myxomas of the heart and skin, and a wide variety of endocrine tumours. It has two known genotypes: a mutation of the tyrosine kinase PRKAR1A gene located at 17q23-24 and several mutations located at 2p16. The phenotypes of these two genotypes are indistinguishable. Carney complex accounts for only a small proportion of all atrial myxomas. Since the condition was first © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
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Letters to the Editor
described in 1995, only around 500 cases have been recorded.1 Atrial myxomas, while histologically benign, can nevertheless cause significant morbidity by impairing cardiac output, or by fragmenting and causing embolic events. Diagnosis of intracardiac tumours is with echocardiography with the transoesophageal approach having a sensitivity demonstrated up to 100%.2 The patient described in this case report had a primarily histological diagnosis for myxoma as she had tested negative for the PRKAR1A mutation. She is now under ongoing surveillance by the local cardiology team, with her next echocardiogram due in 2 years time. The hereditary nature of the disease was discussed with the patient and her family. They did not want to undergo screening echocardiography at that time but were happy to be referred to a clinical geneticist for ongoing follow up. Stroke or transient ischaemic attack from cardiac myxoma emboli is a condition that, if incorrectly diagnosed, can lead to inappropriate treatment with
References 1 Courcoutsakis NA, Tatsi C, Patronas NJ, Lee CCR, Prassopoulos PK, Stratakis CA. The complex of myxomas, spotty skin pigmentation and endocrine overactivity (Carney complex): imaging findings with clinical and pathological correlation.
anticoagulation as opposed to surgical resection. Despite atrial myxoma being a fleetingly rare condition, with a prevalence estimated at 0.0005–0.015%,3 it should be considered in all patients presenting with stroke or transient ischaemic attack without an obvious precipitous cause. Furthermore, for patients with a previous history of Carney complex atrial myxoma presenting with abnormal neurological findings, a diagnosis of recurrence of the myxoma should be considered.
Acknowledgement Gold Coast University Hospital radiology department for the assistance with radiographs for inclusion in the article. Received 11 October 2013; accepted 4 December 2013. doi:10.1111/imj.12365
R. J. Gordon, R. Q. J. Latona, D. T. Sonigra, L. A. K. Embling and S. Niranjan Gold Coast University Hospital, Southport, Queensland, Australia
Insights Imaging 2013; 4: 119–33. 2 Manning WJ, Weintraub RM, Waksmonski CA, Haering JM, Rooney PS, Maslow AD et al. Accuracy of transoesophageal echocardiography for identifying left atrial thrombi. A prospective, intraoperative study.
Ventricular fibrillation storm in a young man with early repolarisation abnormality: the role of isoprenaline and quinidine A 28-year-old man was admitted to the emergency department with refractory ventricular fibrillation (VF) requiring repeated shocks from an implantable cardioverter defibrillator (ICD). He originally presented with an out-of-hospital VF arrest 6 years earlier and underwent implantation of a single-chamber ICD. He was otherwise well with no family history of premature sudden cardiac death. The presenting electrocardiogram (ECG) demonstrated 1 mm of J-point elevation in lead III. Transthoracic © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
Ann Intern Med 1995; 123: 817–22. 3 Burke A, Virmani R. Classification and incidence of cardiac tumors. In: Burke A, Virmani R, eds. Tumors of the Heart and Great Vessels: Atlas of Tumor Pathology, vol. 16. Washington, DC: Armed Forces Institute of Pathology; 1996; 1–11.
echocardiogram, stress thallium and flecainide challenge were unremarkable. Over the next 6 years, he received appropriate shocks for VF on three occasions, having trialled atenolol and sotalol. He presented to the hospital in near-intractable VF having woken feeling not unwell. ECG prior to each VF episode revealed more marked inferior J-point elevation (Fig. 1a) than seen previously. In the emergency department, VF was recurrent despite amiodarone, magnesium, lignocaine and overdrive pacing through the ICD at 110 b.p.m. VF became incessant with unsuccessful ICD and external defibrillation. Cardiopulmonary resuscitation was commenced, and the patient was intubated and placed on venoarterial extracorporeal membrane oxygenation (ECMO) support. 309
Letters to the Editor
fibrous-walled cysts, lined by a single flattened layer of attenuated cells (Fig. 1c,d) without evidence of cytological atypia. There were lymphatic vascular channels, separated by fibrous material; some contained aggregates of adrenal cells. Immunoperoxidase staining showed positive staining for factor VIII and D2-40 immunostain; epithelial markers (AE1/AE3, CAM 5.2) and CD34 were negative. Cystic adrenal masses have an incidence approximating 0.06% of the general population.1 Within the differential diagnosis of these lesions, adrenal lymphangiomas are a very rare cause. Lymphangiomas are benign tumours of the lymphatic system, arising from malformations originating in embryogenesis.2 They are characterised by thin-walled, cystic lesions lined by endothelial cells and filled with lymph fluid.2 The literature suggests that adrenal lymphangiomas are more frequently rightsided, with a female predominance.3 While most adrenal lymphangiomas are clinically asymptomatic,3 patients can present with flank pain, gastrointestinal symptoms or a palpable mass, relating to the size and position of the cyst.4 The lack of distinctive symptoms and laboratory findings make preoperative diagnosis challenging.5 The acute pain presentation of this case may have occurred due to bleeding into a cyst. Adrenal lymphangiomas on CT imaging are characteristically non-enhancing, hypodense masses with an
References 1 Bellantone R, Ferrante A, Raffaelli M, Boscherini M, Lombardi CP, Crucitti F. Adrenal cystic lesions: report of 12 surgically treated cases and review of the literature. J Endocrinol Invest 1998; 21: 109–14. 2 Wiegand S, Eivazi B, Barth PJ, Berens von Rautenfeld D, Folz BJ,
appearance indistinguishable to other cystic adrenal lesions, including cystic pheochromocytoma and adrenal cortical carcinoma.6 Surgical resection is usually necessary to distinguish these benign lesions from potentially aggressive malignant lesions, in addition to relieving symptoms.3 A possible diagnosis of a lymphangioma should be considered when a hypodense, non-enhancing cystic, nonfunctioning adrenal mass is found in youth or middle age. Histological diagnosis of non-functioning adrenal masses must be undertaken with care, preferably in a unit specialising in endocrinology or endocrine surgery. As the differential diagnosis of larger non-functioning masses includes adrenal or metastatic carcinoma, excision biopsy is preferred, rather than fine-needle aspiration biopsy.3,4 Due to the rarity of these tumours, their natural history is not known. Whether these tumours may be observed (after evaluation of functional status and exclusion of malignancy) is not known. Received 24 June 2013; accepted 3 October 2013. doi:10.1111/imj.12361
E. Blanchard,1 P. Brenner,2 W. Delprado3 and K. Samaras1,4 1
Diabetes and Obesity Program, Garvan Institute of Medical Research, Departments of 2Urology and 4Endocrinology, St Vincent’s Hospital and 3School of Medicine Sydney, Notre Dame University, Sydney, New South Wales, Australia
Mandic R et al. Pathogenesis of lymphangiomas. Virchows Archiv 2008; 453: 1–8. 3 Ellis CL, Banerjee P, Carney E, Sharma R, Netto GJ. Adrenal lymphangioma: clinocopathologic and immunohistochemical characteristics of a rare lesion. Hum Pathol 2011; 42: 1013–8. 4 Erickson LA, Lloyd RV, Hartman R, Thompson G. Cystic adrenal neoplasms.
Recurrence of Carney complex atrial myxoma causing embolic stroke Carney complex is a rare genetic disease that can lead to the formation of multiple atrial myxomas. These tumours have the propensity to fragment, causing strokes and other embolic events. We present the case of a patient who suffered a stroke due to recurrence of a Carney complex atrial myxoma. © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
American Cancer Society 2004; 101: 1537–44. 5 Cobanoglu B, Karatas P, Serhatlioglu S, Dogru O. Cystic adrenal lymphangioma: differential diagnosis. Turkiye Klinikleri J Med Sci 2009; 29: 566–8. 6 Dunnick NR, Korobkin M, Francis I. Adrenal radiology: distinguishing benign from malignant adrenal masses. Am J Roentgenol 1996; 167: 861–7.
A 58-year-old woman presented to the emergency department following a collapse with no associated loss of consciousness. In the department, she was confused and agitated, and reported blurred vision. A neurological examination revealed a left-sided motor deficit, visual agnosia and perseveration. The patient had a known diagnosis of Carney complex. She had previously undergone surgical excision of myxomas of her left atrium, bowel and uterus, as well as
307
Letters to the Editor
a
b
c
d
bilateral adrenalectomies for cortisol-secreting tumours. The previous left atrial myxoma had been excised 21 years prior to this presentation. Her last screening transthoracic echocardiogram was performed 2 years prior to this presentation when she underwent excision of her uterine myxoma. This showed no recurrence of cardiac myxoma. Before this, she had been lost to follow up after moving interstate 6 years previously. Initial non-contrast brain computed tomography showed no acute haemorrhage or gross ischaemia; however, the images produced were noted to be of poor quality due to movement artefact. Over the next day, her symptoms resolved and she was able to undergo brain magnetic resonance imaging. This showed extensive chronic and acute cardioembolic infarcts involving multiple cerebral artery territories (Fig. 1). 308
Figure 1 Figure 1 shows both the acute ischaemia seen on diffusion-weighted imaging and the chronic haemorrhage seen on susceptibility-weighted imaging. Acute ischaemia can be seen in both cerebellar hemispheres (a), as well as both occipital lobes and the right frontal lobe (b). Old haemorrhage can be seen in both occipital lobes (c) and both parietal lobes (d).
Following this, transthoracic echocardiography was performed, which showed a large left atrial mass, suspicious of atrial myxoma. This was confirmed with transoesophageal echocardiography and cardiac magnetic resonance imaging. The patient was then referred to the regional cardiothoracic centre for definitive excision of the tumour. Carney complex is an autosomal dominant condition that predisposes the affected individual to skin lentignoses, myxomas of the heart and skin, and a wide variety of endocrine tumours. It has two known genotypes: a mutation of the tyrosine kinase PRKAR1A gene located at 17q23-24 and several mutations located at 2p16. The phenotypes of these two genotypes are indistinguishable. Carney complex accounts for only a small proportion of all atrial myxomas. Since the condition was first © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
bs_bs_banner
Letters to the Editor
described in 1995, only around 500 cases have been recorded.1 Atrial myxomas, while histologically benign, can nevertheless cause significant morbidity by impairing cardiac output, or by fragmenting and causing embolic events. Diagnosis of intracardiac tumours is with echocardiography with the transoesophageal approach having a sensitivity demonstrated up to 100%.2 The patient described in this case report had a primarily histological diagnosis for myxoma as she had tested negative for the PRKAR1A mutation. She is now under ongoing surveillance by the local cardiology team, with her next echocardiogram due in 2 years time. The hereditary nature of the disease was discussed with the patient and her family. They did not want to undergo screening echocardiography at that time but were happy to be referred to a clinical geneticist for ongoing follow up. Stroke or transient ischaemic attack from cardiac myxoma emboli is a condition that, if incorrectly diagnosed, can lead to inappropriate treatment with
References 1 Courcoutsakis NA, Tatsi C, Patronas NJ, Lee CCR, Prassopoulos PK, Stratakis CA. The complex of myxomas, spotty skin pigmentation and endocrine overactivity (Carney complex): imaging findings with clinical and pathological correlation.
anticoagulation as opposed to surgical resection. Despite atrial myxoma being a fleetingly rare condition, with a prevalence estimated at 0.0005–0.015%,3 it should be considered in all patients presenting with stroke or transient ischaemic attack without an obvious precipitous cause. Furthermore, for patients with a previous history of Carney complex atrial myxoma presenting with abnormal neurological findings, a diagnosis of recurrence of the myxoma should be considered.
Acknowledgement Gold Coast University Hospital radiology department for the assistance with radiographs for inclusion in the article. Received 11 October 2013; accepted 4 December 2013. doi:10.1111/imj.12365
R. J. Gordon, R. Q. J. Latona, D. T. Sonigra, L. A. K. Embling and S. Niranjan Gold Coast University Hospital, Southport, Queensland, Australia
Insights Imaging 2013; 4: 119–33. 2 Manning WJ, Weintraub RM, Waksmonski CA, Haering JM, Rooney PS, Maslow AD et al. Accuracy of transoesophageal echocardiography for identifying left atrial thrombi. A prospective, intraoperative study.
Ventricular fibrillation storm in a young man with early repolarisation abnormality: the role of isoprenaline and quinidine A 28-year-old man was admitted to the emergency department with refractory ventricular fibrillation (VF) requiring repeated shocks from an implantable cardioverter defibrillator (ICD). He originally presented with an out-of-hospital VF arrest 6 years earlier and underwent implantation of a single-chamber ICD. He was otherwise well with no family history of premature sudden cardiac death. The presenting electrocardiogram (ECG) demonstrated 1 mm of J-point elevation in lead III. Transthoracic © 2014 The Authors Internal Medicine Journal © 2014 Royal Australasian College of Physicians
Ann Intern Med 1995; 123: 817–22. 3 Burke A, Virmani R. Classification and incidence of cardiac tumors. In: Burke A, Virmani R, eds. Tumors of the Heart and Great Vessels: Atlas of Tumor Pathology, vol. 16. Washington, DC: Armed Forces Institute of Pathology; 1996; 1–11.
echocardiogram, stress thallium and flecainide challenge were unremarkable. Over the next 6 years, he received appropriate shocks for VF on three occasions, having trialled atenolol and sotalol. He presented to the hospital in near-intractable VF having woken feeling not unwell. ECG prior to each VF episode revealed more marked inferior J-point elevation (Fig. 1a) than seen previously. In the emergency department, VF was recurrent despite amiodarone, magnesium, lignocaine and overdrive pacing through the ICD at 110 b.p.m. VF became incessant with unsuccessful ICD and external defibrillation. Cardiopulmonary resuscitation was commenced, and the patient was intubated and placed on venoarterial extracorporeal membrane oxygenation (ECMO) support. 309
