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Clinical A 62-year-old woman was admitted to hospital with shortness of breath and a non-productive cough of a few days duration. She was noted to have extensive hirsutism involving the face (Figure 1a), lower abdomen and extremities along with male pattern baldness, greasy skin and deepening of voice. She also had facial plethora and bilateral proximal myopathy.Abdominal examination revealed an ill-defined, non-tender right upper quadrant mass. Abdominal imaging by ultrasound, computed tomography (Figure 1b) and magnetic resonance imaging (Figure 1c) confirmed the presence of a large heterogeneous suprarenal mass infiltrating the kidney along with evidence of liver and pulmonary metastasis and tumour thrombus in the inferior vena cava. Figure 1. (a) Thick, terminal hair on the upper lip, cheek and chin and greasy skin was noted; (b) Cross-sectional imaging by computed tomography demonstrated a large, poorly-enhancing mass in the right suprarenal region; (c) The heterogeneous appearance of the right adrenal tumour was better delineated on magnetic resonance imaging Laboratory tests revealed high levels of serum testosterone at>52.0 (normal <2.4) nmol/L, androstendione at>35.0 (1.0-11.5) nmol/L and oestradiol at 743 (postmenopausal <201) pmol/L with suppressed luteininsing and follicle stimulating hormone levels. Serum human chorionic gonadotrophin, dehydroepiandrosterone sulphate and 24-hour urinary metanephrine and normetanephrine levels were normal. Serum potassium and aldosterone levels were likewise normal. Urinary free cortisol levels were raised on two occasions at 587 and 479 (0-180) nmol/24 h. Her basal serum cortisol level at 0930 h was raised at 1430 (250-650) nmol/L, and failed to suppress on overnight dexamethasone (1 mg) suppression test with serum cortisol level at 1369 (normal suppression <50) nmol/L the following morning at 0900 h. Histological examination of tumour tissue obtained by ultrasound-guided biopsy confirmed adrenocortical cancer with a high proliferation index. A computed tomographic pulmonary angiogram also showed pulmonary tumour emboli and metastases. Despite anticoagulation and palliative treatment with metyrapone she soon succumbed to progressive respiratory failure. Discussion Although hirsutism is a common complaint in women and most frequently secondary to functional causes such as polycystic ovarian syndrome,1 rapidly progressive extensive hirsutism or virilising features should prompt further investigation for androgen-secreting tumours. Adrenocortical carcinoma is a rare malignancy with an incidence of 1-2 per million population.2 There is evidence of adrenal steroid hormone excess in approximately 60% of cases and rapidly progressing Cushings syndrome with or without virilisation is the most common presentation.2 Cross-sectional imaging by CT and MRI, and more recently functional imaging by 18F-fluorodeoxyglucose positron emission tomography, have been used to distinguish between benign and malignant lesions and to plan appropriate treatment.3 Definitive treatment by en bloc primary surgical resection of the tumour offers the best prognosis. Adjuvant medical therapy with mitotane, radiotherapy or cytotoxic chemotherapy may offer some benefit in more advanced disease. Adrenostatic drugs such as metyrapone, ketoconazole, aminoglutethimide, and etomidate may be used to block steroidogenic enzymes and alleviate hormonal symptoms.2,4 Learning points Hirsutism is a common complaint in women, most frequently secondary to functional causes such as polycystic ovarian syndrome. Rapidly progressive hirsutism, virilising features, or a palpable abdominal or pelvic mass should prompt further investigation for androgen-secreting tumours.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Akheel Syed, Consultant Physician and Endocrinologist, Salford Royal NHS Foundation Trust & University Teaching Hospital, Salford, UK (and the University of Manchester, Manchester, UK); Yared Demssie, Consultant Physician and Endocrinologist, Royal Blackburn Hospital, Blackburn, UK

Acknowledgements

Correspondence

Dr Akheel Syed, Department of Endocrinology, Salford Royal NHS Foundation Trust, Salford M6 8HD, United Kingdom

Correspondence Email

aas@DrSyed.org

Competing Interests

Bode D, Seehusen DA, Baird D. Hirsutism in women. Am Fam Physician 2012;85(4):373-80.Allolio B, Fassnacht M. Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.Bharwani N, Rockall AG, Sahdev A, et al. Adrenocortical carcinoma: the range of appearances on CT and MRI. AJR Am J Roentgenol 2011;196(6):W706-14.Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. J Surg Oncol 2012;106(5):586-94.

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Clinical A 62-year-old woman was admitted to hospital with shortness of breath and a non-productive cough of a few days duration. She was noted to have extensive hirsutism involving the face (Figure 1a), lower abdomen and extremities along with male pattern baldness, greasy skin and deepening of voice. She also had facial plethora and bilateral proximal myopathy.Abdominal examination revealed an ill-defined, non-tender right upper quadrant mass. Abdominal imaging by ultrasound, computed tomography (Figure 1b) and magnetic resonance imaging (Figure 1c) confirmed the presence of a large heterogeneous suprarenal mass infiltrating the kidney along with evidence of liver and pulmonary metastasis and tumour thrombus in the inferior vena cava. Figure 1. (a) Thick, terminal hair on the upper lip, cheek and chin and greasy skin was noted; (b) Cross-sectional imaging by computed tomography demonstrated a large, poorly-enhancing mass in the right suprarenal region; (c) The heterogeneous appearance of the right adrenal tumour was better delineated on magnetic resonance imaging Laboratory tests revealed high levels of serum testosterone at>52.0 (normal <2.4) nmol/L, androstendione at>35.0 (1.0-11.5) nmol/L and oestradiol at 743 (postmenopausal <201) pmol/L with suppressed luteininsing and follicle stimulating hormone levels. Serum human chorionic gonadotrophin, dehydroepiandrosterone sulphate and 24-hour urinary metanephrine and normetanephrine levels were normal. Serum potassium and aldosterone levels were likewise normal. Urinary free cortisol levels were raised on two occasions at 587 and 479 (0-180) nmol/24 h. Her basal serum cortisol level at 0930 h was raised at 1430 (250-650) nmol/L, and failed to suppress on overnight dexamethasone (1 mg) suppression test with serum cortisol level at 1369 (normal suppression <50) nmol/L the following morning at 0900 h. Histological examination of tumour tissue obtained by ultrasound-guided biopsy confirmed adrenocortical cancer with a high proliferation index. A computed tomographic pulmonary angiogram also showed pulmonary tumour emboli and metastases. Despite anticoagulation and palliative treatment with metyrapone she soon succumbed to progressive respiratory failure. Discussion Although hirsutism is a common complaint in women and most frequently secondary to functional causes such as polycystic ovarian syndrome,1 rapidly progressive extensive hirsutism or virilising features should prompt further investigation for androgen-secreting tumours. Adrenocortical carcinoma is a rare malignancy with an incidence of 1-2 per million population.2 There is evidence of adrenal steroid hormone excess in approximately 60% of cases and rapidly progressing Cushings syndrome with or without virilisation is the most common presentation.2 Cross-sectional imaging by CT and MRI, and more recently functional imaging by 18F-fluorodeoxyglucose positron emission tomography, have been used to distinguish between benign and malignant lesions and to plan appropriate treatment.3 Definitive treatment by en bloc primary surgical resection of the tumour offers the best prognosis. Adjuvant medical therapy with mitotane, radiotherapy or cytotoxic chemotherapy may offer some benefit in more advanced disease. Adrenostatic drugs such as metyrapone, ketoconazole, aminoglutethimide, and etomidate may be used to block steroidogenic enzymes and alleviate hormonal symptoms.2,4 Learning points Hirsutism is a common complaint in women, most frequently secondary to functional causes such as polycystic ovarian syndrome. Rapidly progressive hirsutism, virilising features, or a palpable abdominal or pelvic mass should prompt further investigation for androgen-secreting tumours.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Akheel Syed, Consultant Physician and Endocrinologist, Salford Royal NHS Foundation Trust & University Teaching Hospital, Salford, UK (and the University of Manchester, Manchester, UK); Yared Demssie, Consultant Physician and Endocrinologist, Royal Blackburn Hospital, Blackburn, UK

Acknowledgements

Correspondence

Dr Akheel Syed, Department of Endocrinology, Salford Royal NHS Foundation Trust, Salford M6 8HD, United Kingdom

Correspondence Email

aas@DrSyed.org

Competing Interests

Bode D, Seehusen DA, Baird D. Hirsutism in women. Am Fam Physician 2012;85(4):373-80.Allolio B, Fassnacht M. Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.Bharwani N, Rockall AG, Sahdev A, et al. Adrenocortical carcinoma: the range of appearances on CT and MRI. AJR Am J Roentgenol 2011;196(6):W706-14.Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. J Surg Oncol 2012;106(5):586-94.

For the PDF of this article,
contact nzmj@nzma.org.nz

View Article PDF

Clinical A 62-year-old woman was admitted to hospital with shortness of breath and a non-productive cough of a few days duration. She was noted to have extensive hirsutism involving the face (Figure 1a), lower abdomen and extremities along with male pattern baldness, greasy skin and deepening of voice. She also had facial plethora and bilateral proximal myopathy.Abdominal examination revealed an ill-defined, non-tender right upper quadrant mass. Abdominal imaging by ultrasound, computed tomography (Figure 1b) and magnetic resonance imaging (Figure 1c) confirmed the presence of a large heterogeneous suprarenal mass infiltrating the kidney along with evidence of liver and pulmonary metastasis and tumour thrombus in the inferior vena cava. Figure 1. (a) Thick, terminal hair on the upper lip, cheek and chin and greasy skin was noted; (b) Cross-sectional imaging by computed tomography demonstrated a large, poorly-enhancing mass in the right suprarenal region; (c) The heterogeneous appearance of the right adrenal tumour was better delineated on magnetic resonance imaging Laboratory tests revealed high levels of serum testosterone at>52.0 (normal <2.4) nmol/L, androstendione at>35.0 (1.0-11.5) nmol/L and oestradiol at 743 (postmenopausal <201) pmol/L with suppressed luteininsing and follicle stimulating hormone levels. Serum human chorionic gonadotrophin, dehydroepiandrosterone sulphate and 24-hour urinary metanephrine and normetanephrine levels were normal. Serum potassium and aldosterone levels were likewise normal. Urinary free cortisol levels were raised on two occasions at 587 and 479 (0-180) nmol/24 h. Her basal serum cortisol level at 0930 h was raised at 1430 (250-650) nmol/L, and failed to suppress on overnight dexamethasone (1 mg) suppression test with serum cortisol level at 1369 (normal suppression <50) nmol/L the following morning at 0900 h. Histological examination of tumour tissue obtained by ultrasound-guided biopsy confirmed adrenocortical cancer with a high proliferation index. A computed tomographic pulmonary angiogram also showed pulmonary tumour emboli and metastases. Despite anticoagulation and palliative treatment with metyrapone she soon succumbed to progressive respiratory failure. Discussion Although hirsutism is a common complaint in women and most frequently secondary to functional causes such as polycystic ovarian syndrome,1 rapidly progressive extensive hirsutism or virilising features should prompt further investigation for androgen-secreting tumours. Adrenocortical carcinoma is a rare malignancy with an incidence of 1-2 per million population.2 There is evidence of adrenal steroid hormone excess in approximately 60% of cases and rapidly progressing Cushings syndrome with or without virilisation is the most common presentation.2 Cross-sectional imaging by CT and MRI, and more recently functional imaging by 18F-fluorodeoxyglucose positron emission tomography, have been used to distinguish between benign and malignant lesions and to plan appropriate treatment.3 Definitive treatment by en bloc primary surgical resection of the tumour offers the best prognosis. Adjuvant medical therapy with mitotane, radiotherapy or cytotoxic chemotherapy may offer some benefit in more advanced disease. Adrenostatic drugs such as metyrapone, ketoconazole, aminoglutethimide, and etomidate may be used to block steroidogenic enzymes and alleviate hormonal symptoms.2,4 Learning points Hirsutism is a common complaint in women, most frequently secondary to functional causes such as polycystic ovarian syndrome. Rapidly progressive hirsutism, virilising features, or a palpable abdominal or pelvic mass should prompt further investigation for androgen-secreting tumours.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Akheel Syed, Consultant Physician and Endocrinologist, Salford Royal NHS Foundation Trust & University Teaching Hospital, Salford, UK (and the University of Manchester, Manchester, UK); Yared Demssie, Consultant Physician and Endocrinologist, Royal Blackburn Hospital, Blackburn, UK

Acknowledgements

Correspondence

Dr Akheel Syed, Department of Endocrinology, Salford Royal NHS Foundation Trust, Salford M6 8HD, United Kingdom

Correspondence Email

aas@DrSyed.org

Competing Interests

Bode D, Seehusen DA, Baird D. Hirsutism in women. Am Fam Physician 2012;85(4):373-80.Allolio B, Fassnacht M. Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 2006;91(6):2027-37.Bharwani N, Rockall AG, Sahdev A, et al. Adrenocortical carcinoma: the range of appearances on CT and MRI. AJR Am J Roentgenol 2011;196(6):W706-14.Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. J Surg Oncol 2012;106(5):586-94.

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