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Adrenal myelolipomas are benign and hormonally inactive tumours that have been described in 1929 by the French pathologist, Professor Charles Oberling.1 Microscopically it is made up of adipose and hematopoietic tissue. Its pathogenesis is unclear but the favoured mechanism is reticuloendotherlial call metaplasia that is triggered by necrosis, infection or stress.2This lesion is still not clearly defined as it is a rare entity. The incidence is 0.08-0.2% as quoted by autopsy series.3 Our knowledge is mainly based on published case series and case reports.The case reported here is the sixth largest such lesion reported in the literature. Moreover it has some malignant radiological features that distinguish it from other such reported lesions.Case reportA 47-year-old man with schizophrenia presented to the hospital in July 2008 with a urinary tract infection. On examination he had abdominal distension and right flank tenderness. An abdominal ultrasound revealed a large homogeneous mass, about 3700cc in volume, displacing the right kidney inferomedially (Figure 1). Figure 1. Ultrasound of right flank The sonographic finding prompted a CT of the abdomen to further define the lesion. A 22 00d722 00d716cm right retroperitoneal well circumscribed mass was seen with mixed fat and soft tissue density. The right lobe of the liver was indented, suggestive of invasion. A lesion was noted in segment 8, which raised the possibility of metastatic liposarcoma (Figure 2). Figure 2. CT abdomen - coronal view showing the invasive lesion with a liver nodule Interestingly there was no paraortic lymphadenopathy noted on radiological investigation. On 23 July 2008, a trial of excision of this lesion was attempted. A J-shaped incision was made on his right upper quadrant. A large retroperitoneal mass was seen, with clear planes around the kidney, liver and IVC. The adrenal gland was resected together with the mass. The liver lesions noted on CT were haemangiomas. The specimen was 220 00d7180 00d7120mm, and weighed 3695g (Figure 3). Figure 3. Right retroperitoneal mass with adrenal gland in the lower left corner The adrenal gland was distinct from the mass. On cut section the lesion was fatty yellow with less than 20% patchy necrosis seen. The tumour was made up of mature adipocytes and haematopoietic cells. No malignant tissue was seen. The features were consistent with an adrenal myelolipoma. Apart from an episode of opiate overdose the patient made an uneventful recovery and was discharged from hospital eight days after admission. Discussion A PubMed search reveals that our case is the sixth largest adrenal myelolipoma that has been reported (Table 1). Boudreaux et al4 reported the second biggest one (5900g), although it included the kidney and some retroperitoneal tissue too. Table 1. Largest cases reported in literature Cases reported Size (cm) Weight (g) Akamatsu et al7 Boudreaux et al4 Wilhelmus et al5 ODaniel-Pierce et al6 Lamont et al8 31 34 30 30 40 6000 5900 5500 4370 4254 Apart from its size, its radiological appearance was singular. Adrenal lesions greater than 6cm with an inhomogeneous consistency are more likely to be malignant.9 This lesions bosselated appearance with mixed soft and fat tissue density on CT was unusual for an adrenal myelipoma. Those lesions are typically asymptomatic. However, they can present with abdominal or flank pain as a result of haemorrhage, necrosis or pressure effect on surrounding organs. A study of the largest series published shows that they are mostly incidentally picked up on imaging. 58% and 75% of the adrenal myelolipomas studied by Meyer et al10and Han et al11 respectively were asymptomatic. Interestingly in Meyers series symptomatic myelolipomas were smaller than ones picked up incidentally. We also looked at the therapeutic options offered to patients in both series. Meyer et al resected all the lesions from their patients. Han et al on the other hand were selective, offering surgery to large or symptomatic myelolipomasi.e. to only 25% of their patients. Their 3-year clinical and radiological follow-up did not reveal any complications from the adrenal myelolipomas. We hope that as more cases get reported more light will be shed on this rare lesion.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Kheman Rajkomar, Surgical Registrar, General Surgery, Waikato Hospital, Hamilton; Isaac Cranshaw, Endocrine Surgeon Auckland City Hospital, Auckland

Acknowledgements

Correspondence

Kheman Rajkomar, 51 Ohaupo Road, Hamilton, New Zealand.

Correspondence Email

kheman205@yahoo.com

Competing Interests

Oberling C. The formation of myelolipomas. Bull. Assoc Fr Cancer. 1929;18:234-246.Meaglia JP, Schmidt JD. Natural history of an adrenal myelolipoma. J Urol. Apr 1992;147(4):1089-90.Plaut A. Myelolipoma in the adrenal cortex. Am J Pathol 1958;34:487-515.Boudreaux D, Waisman J, Skinner DG, Low R , et al. Giant adrenal myelipoma and testicular interstitial cell tumour in a man with congenital 21-hydroxylase deficiency. Am J Surg Pathol 1979; 3: 109-123.Wilhelmus JL, Schrodt GR, Alberhasky M, et al. Giant adrenal myelolipomas: Case report and review of the literature. Arch. Pathol. Lab. Med. 1981;105:532-535.ODaniel-Pierce ME, Weeks JA, McGrath PC. Giant adrenal myelolipoma. Southern Med J. 1996;89:1116-1118.Akamatsu H, Koseki M, Nakaba H, et al. Giant adrenal myelolipoma: report of a case. Surg Today 2004;34(3):283-5.Lamont JP, Lieberman ZH, Stephens JS. Giant adrenal myelipoma. Am Surg 2002 Apr; 68(4):392-4.Grumbach MM, Biller BMK, Bruanstein GD, et al. Management of the clinically inapparent adrenal mass (cIncidentalomad). Annals of internal medicine. 2003;138:424-429.Meyer A, Behrend M. Presentation and therapy of myelolipoma. International Journal of Urology 2005;12:239-243.Han M, Burnett AL, Fishman EK, Marshall FF. The natural history and treatment of adrenal myelolipoma. 1997;157(4):1213-1216.

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Adrenal myelolipomas are benign and hormonally inactive tumours that have been described in 1929 by the French pathologist, Professor Charles Oberling.1 Microscopically it is made up of adipose and hematopoietic tissue. Its pathogenesis is unclear but the favoured mechanism is reticuloendotherlial call metaplasia that is triggered by necrosis, infection or stress.2This lesion is still not clearly defined as it is a rare entity. The incidence is 0.08-0.2% as quoted by autopsy series.3 Our knowledge is mainly based on published case series and case reports.The case reported here is the sixth largest such lesion reported in the literature. Moreover it has some malignant radiological features that distinguish it from other such reported lesions.Case reportA 47-year-old man with schizophrenia presented to the hospital in July 2008 with a urinary tract infection. On examination he had abdominal distension and right flank tenderness. An abdominal ultrasound revealed a large homogeneous mass, about 3700cc in volume, displacing the right kidney inferomedially (Figure 1). Figure 1. Ultrasound of right flank The sonographic finding prompted a CT of the abdomen to further define the lesion. A 22 00d722 00d716cm right retroperitoneal well circumscribed mass was seen with mixed fat and soft tissue density. The right lobe of the liver was indented, suggestive of invasion. A lesion was noted in segment 8, which raised the possibility of metastatic liposarcoma (Figure 2). Figure 2. CT abdomen - coronal view showing the invasive lesion with a liver nodule Interestingly there was no paraortic lymphadenopathy noted on radiological investigation. On 23 July 2008, a trial of excision of this lesion was attempted. A J-shaped incision was made on his right upper quadrant. A large retroperitoneal mass was seen, with clear planes around the kidney, liver and IVC. The adrenal gland was resected together with the mass. The liver lesions noted on CT were haemangiomas. The specimen was 220 00d7180 00d7120mm, and weighed 3695g (Figure 3). Figure 3. Right retroperitoneal mass with adrenal gland in the lower left corner The adrenal gland was distinct from the mass. On cut section the lesion was fatty yellow with less than 20% patchy necrosis seen. The tumour was made up of mature adipocytes and haematopoietic cells. No malignant tissue was seen. The features were consistent with an adrenal myelolipoma. Apart from an episode of opiate overdose the patient made an uneventful recovery and was discharged from hospital eight days after admission. Discussion A PubMed search reveals that our case is the sixth largest adrenal myelolipoma that has been reported (Table 1). Boudreaux et al4 reported the second biggest one (5900g), although it included the kidney and some retroperitoneal tissue too. Table 1. Largest cases reported in literature Cases reported Size (cm) Weight (g) Akamatsu et al7 Boudreaux et al4 Wilhelmus et al5 ODaniel-Pierce et al6 Lamont et al8 31 34 30 30 40 6000 5900 5500 4370 4254 Apart from its size, its radiological appearance was singular. Adrenal lesions greater than 6cm with an inhomogeneous consistency are more likely to be malignant.9 This lesions bosselated appearance with mixed soft and fat tissue density on CT was unusual for an adrenal myelipoma. Those lesions are typically asymptomatic. However, they can present with abdominal or flank pain as a result of haemorrhage, necrosis or pressure effect on surrounding organs. A study of the largest series published shows that they are mostly incidentally picked up on imaging. 58% and 75% of the adrenal myelolipomas studied by Meyer et al10and Han et al11 respectively were asymptomatic. Interestingly in Meyers series symptomatic myelolipomas were smaller than ones picked up incidentally. We also looked at the therapeutic options offered to patients in both series. Meyer et al resected all the lesions from their patients. Han et al on the other hand were selective, offering surgery to large or symptomatic myelolipomasi.e. to only 25% of their patients. Their 3-year clinical and radiological follow-up did not reveal any complications from the adrenal myelolipomas. We hope that as more cases get reported more light will be shed on this rare lesion.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Kheman Rajkomar, Surgical Registrar, General Surgery, Waikato Hospital, Hamilton; Isaac Cranshaw, Endocrine Surgeon Auckland City Hospital, Auckland

Acknowledgements

Correspondence

Kheman Rajkomar, 51 Ohaupo Road, Hamilton, New Zealand.

Correspondence Email

kheman205@yahoo.com

Competing Interests

Oberling C. The formation of myelolipomas. Bull. Assoc Fr Cancer. 1929;18:234-246.Meaglia JP, Schmidt JD. Natural history of an adrenal myelolipoma. J Urol. Apr 1992;147(4):1089-90.Plaut A. Myelolipoma in the adrenal cortex. Am J Pathol 1958;34:487-515.Boudreaux D, Waisman J, Skinner DG, Low R , et al. Giant adrenal myelipoma and testicular interstitial cell tumour in a man with congenital 21-hydroxylase deficiency. Am J Surg Pathol 1979; 3: 109-123.Wilhelmus JL, Schrodt GR, Alberhasky M, et al. Giant adrenal myelolipomas: Case report and review of the literature. Arch. Pathol. Lab. Med. 1981;105:532-535.ODaniel-Pierce ME, Weeks JA, McGrath PC. Giant adrenal myelolipoma. Southern Med J. 1996;89:1116-1118.Akamatsu H, Koseki M, Nakaba H, et al. Giant adrenal myelolipoma: report of a case. Surg Today 2004;34(3):283-5.Lamont JP, Lieberman ZH, Stephens JS. Giant adrenal myelipoma. Am Surg 2002 Apr; 68(4):392-4.Grumbach MM, Biller BMK, Bruanstein GD, et al. Management of the clinically inapparent adrenal mass (cIncidentalomad). Annals of internal medicine. 2003;138:424-429.Meyer A, Behrend M. Presentation and therapy of myelolipoma. International Journal of Urology 2005;12:239-243.Han M, Burnett AL, Fishman EK, Marshall FF. The natural history and treatment of adrenal myelolipoma. 1997;157(4):1213-1216.

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

View Article PDF

Adrenal myelolipomas are benign and hormonally inactive tumours that have been described in 1929 by the French pathologist, Professor Charles Oberling.1 Microscopically it is made up of adipose and hematopoietic tissue. Its pathogenesis is unclear but the favoured mechanism is reticuloendotherlial call metaplasia that is triggered by necrosis, infection or stress.2This lesion is still not clearly defined as it is a rare entity. The incidence is 0.08-0.2% as quoted by autopsy series.3 Our knowledge is mainly based on published case series and case reports.The case reported here is the sixth largest such lesion reported in the literature. Moreover it has some malignant radiological features that distinguish it from other such reported lesions.Case reportA 47-year-old man with schizophrenia presented to the hospital in July 2008 with a urinary tract infection. On examination he had abdominal distension and right flank tenderness. An abdominal ultrasound revealed a large homogeneous mass, about 3700cc in volume, displacing the right kidney inferomedially (Figure 1). Figure 1. Ultrasound of right flank The sonographic finding prompted a CT of the abdomen to further define the lesion. A 22 00d722 00d716cm right retroperitoneal well circumscribed mass was seen with mixed fat and soft tissue density. The right lobe of the liver was indented, suggestive of invasion. A lesion was noted in segment 8, which raised the possibility of metastatic liposarcoma (Figure 2). Figure 2. CT abdomen - coronal view showing the invasive lesion with a liver nodule Interestingly there was no paraortic lymphadenopathy noted on radiological investigation. On 23 July 2008, a trial of excision of this lesion was attempted. A J-shaped incision was made on his right upper quadrant. A large retroperitoneal mass was seen, with clear planes around the kidney, liver and IVC. The adrenal gland was resected together with the mass. The liver lesions noted on CT were haemangiomas. The specimen was 220 00d7180 00d7120mm, and weighed 3695g (Figure 3). Figure 3. Right retroperitoneal mass with adrenal gland in the lower left corner The adrenal gland was distinct from the mass. On cut section the lesion was fatty yellow with less than 20% patchy necrosis seen. The tumour was made up of mature adipocytes and haematopoietic cells. No malignant tissue was seen. The features were consistent with an adrenal myelolipoma. Apart from an episode of opiate overdose the patient made an uneventful recovery and was discharged from hospital eight days after admission. Discussion A PubMed search reveals that our case is the sixth largest adrenal myelolipoma that has been reported (Table 1). Boudreaux et al4 reported the second biggest one (5900g), although it included the kidney and some retroperitoneal tissue too. Table 1. Largest cases reported in literature Cases reported Size (cm) Weight (g) Akamatsu et al7 Boudreaux et al4 Wilhelmus et al5 ODaniel-Pierce et al6 Lamont et al8 31 34 30 30 40 6000 5900 5500 4370 4254 Apart from its size, its radiological appearance was singular. Adrenal lesions greater than 6cm with an inhomogeneous consistency are more likely to be malignant.9 This lesions bosselated appearance with mixed soft and fat tissue density on CT was unusual for an adrenal myelipoma. Those lesions are typically asymptomatic. However, they can present with abdominal or flank pain as a result of haemorrhage, necrosis or pressure effect on surrounding organs. A study of the largest series published shows that they are mostly incidentally picked up on imaging. 58% and 75% of the adrenal myelolipomas studied by Meyer et al10and Han et al11 respectively were asymptomatic. Interestingly in Meyers series symptomatic myelolipomas were smaller than ones picked up incidentally. We also looked at the therapeutic options offered to patients in both series. Meyer et al resected all the lesions from their patients. Han et al on the other hand were selective, offering surgery to large or symptomatic myelolipomasi.e. to only 25% of their patients. Their 3-year clinical and radiological follow-up did not reveal any complications from the adrenal myelolipomas. We hope that as more cases get reported more light will be shed on this rare lesion.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Kheman Rajkomar, Surgical Registrar, General Surgery, Waikato Hospital, Hamilton; Isaac Cranshaw, Endocrine Surgeon Auckland City Hospital, Auckland

Acknowledgements

Correspondence

Kheman Rajkomar, 51 Ohaupo Road, Hamilton, New Zealand.

Correspondence Email

kheman205@yahoo.com

Competing Interests

Oberling C. The formation of myelolipomas. Bull. Assoc Fr Cancer. 1929;18:234-246.Meaglia JP, Schmidt JD. Natural history of an adrenal myelolipoma. J Urol. Apr 1992;147(4):1089-90.Plaut A. Myelolipoma in the adrenal cortex. Am J Pathol 1958;34:487-515.Boudreaux D, Waisman J, Skinner DG, Low R , et al. Giant adrenal myelipoma and testicular interstitial cell tumour in a man with congenital 21-hydroxylase deficiency. Am J Surg Pathol 1979; 3: 109-123.Wilhelmus JL, Schrodt GR, Alberhasky M, et al. Giant adrenal myelolipomas: Case report and review of the literature. Arch. Pathol. Lab. Med. 1981;105:532-535.ODaniel-Pierce ME, Weeks JA, McGrath PC. Giant adrenal myelolipoma. Southern Med J. 1996;89:1116-1118.Akamatsu H, Koseki M, Nakaba H, et al. Giant adrenal myelolipoma: report of a case. Surg Today 2004;34(3):283-5.Lamont JP, Lieberman ZH, Stephens JS. Giant adrenal myelipoma. Am Surg 2002 Apr; 68(4):392-4.Grumbach MM, Biller BMK, Bruanstein GD, et al. Management of the clinically inapparent adrenal mass (cIncidentalomad). Annals of internal medicine. 2003;138:424-429.Meyer A, Behrend M. Presentation and therapy of myelolipoma. International Journal of Urology 2005;12:239-243.Han M, Burnett AL, Fishman EK, Marshall FF. The natural history and treatment of adrenal myelolipoma. 1997;157(4):1213-1216.

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