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Adenomyoepithelioma of the breast: a diagnostic dilemma
316
Case reports
tumour responded very well to the 5-fluorouracil treatment and shrinkage of the pancreatic tumour was confirmed radiologically in just 6 weeks. The reduction in size of the tumour coupled with the slow growing nature of this tumour prompted us to perform a resection. Thus, pre-operative adjuvant chemotherapy plays a significant role in downstaging the endocrine pancreatic tumour. Secondly, although a curative resection may not have been achieved despite radical excision, the patient remained well after 13 years following the Whipple’s procedure. Indeed her recent CT failed to show any evidence of recurrent disease. In conclusion, chemotherapy has a significant role to play in the management of malignant insulinomas. Although non-functioning malignant insulinomas presenting with obstructive features are rare, they may be successfully treated by surgery. If this is not feasible, surgical debulking of the primary and metastatic lesions produce good palliation and increased survival. Due to the slow nature of their growth it is imperative to follow-up these patients for many years.
References 1. Service FJ, McMahon MM, O’Brien PC, Ballard DJ. Functioning insulinoma: incidence, recurrence and long term survival of patients: a 60 year study. Mayo Clin Proc 1991; 66: 711–9. 2. Moldrow RE, Connelly RR. Epidemiology of pancreatic endocrine tumours of the pancreas. Analysis of autopsy cases. Dig Dis Sci 1968; 36: 933–42. 3. Bliss RD, Carter PB, Lennard TWJ. Insulinoma: a review of current management. Surg Oncol 1997; 6: 49–59. 4. Lo CY, Lam KY, Kung AW, Lam KS, Tung PH, Fan ST. Pancreatic insulinomas. A 15 year experience. Arch Surg 1997; 132: 926–30. 5. Grant CS. Surgical management of malignant islet cell tumours. World J Surg 1993; 17: 498–503. 6. Van Heerden J. Pancreatic resection for carcinoma of the pancreas: Whipples versus total pancreatectomy—an institutional perspective. World J Surg 1994; 8: 880. 7. Doppman JL, Miller DL, Chang R. Intraarterial calcium stimulation test for detection of insulinomas. World J Surg 1993; 17: 439–43.
doi:10.1053/ejso.1999.0890, available online at http://www.idealibrary.com on
Adenomyoepithelioma of the breast: a diagnostic dilemma T. Singh Gill, D. Clarke, A. G. Douglas-Jones∗, H. M. Sweetland and R. E. Mansel University Departments of Surgery and Pathology∗, University Hospital of Wales, Heath Park, Cardiff CF4 4XW, UK
Introduction Myoepithelial cells are normally present in the breast and are located between the epithelial cells and the basal lamina of secretory elements of the mammary duct system.1,2 Tumours derived from these cells have been described in skin, salivary glands, breast and lungs.3–5 Mammary tumours with predominant myoepithelial elements are extremely uncommon. The most common lesion with predominance of myoepithelial cells is sclerosing adenosis. Adenomyoepithelioma of the breast is an uncommon tumour characterized by the presence of both epithelial and myoepithelial cells.6 We describe two cases of adenomyoepithelioma of the breast in which pre-operative diagnosis of carcinoma was made on cytology or histology but the radiological findings were those of a benign lesion. Case reports
mobile lump of 3 cm in size in the upper outer quadrant of the left breast. The mammogram showed a well defined, 2.5-cm oval opacity, which was scored R2. Ultrasound revealed the presence of a 2-cm mass with no suspicious features. A fine needle aspiration cytology (FNAC) was performed which was reported as showing numerous malignant epithelial cells (Fig. 1). With a working diagnosis of carcinoma of the left breast the patient was referred to the breast clinic in University Hospital of Wales. The patient was given options for treatment and she chose to have a wide local excision with axillary node clearance. Histology results showed a well-circumscribed lesion composed of tubules lined by epithelial cells surrounded by a striking myoepithelial cell proliferation with clear cells. There was no cytological atypia, mitotic activity or necrosis and the lesion was considered to be a benign adenomyoepithelioma (Fig. 2). The 15 axillary lymph nodes examined were free of tumour. The cytology slides were reviewed in view of the histology results but the appearances were consistent with malignant cells of C5 grading. In 18 months follow-up, there has been no evidence of recurrence.
Case 1 A 63-year-old woman with a left breast lump was seen in a district general hospital. Physical examination disclosed a smooth, firm, Correspondence to: Miss Helen M. Sweetland, Senior Lecturer and Consultant Surgeon, University Hospital of Wales, Heath Park, Cardiff CF4 4XW, UK.
Case 2 A 68-year-old woman was recalled for assessment after a routine screening mammogram showed a 20-mm mass lesion in the lower outer quadrant of right breast, which was scored R3. Physical examination was normal. Ultrasound confirmed the presence of a 17-mm mass in the area of concern. Ultrasound-guided wide bore
317
Case reports
Fig. 1. Cytology from case 1 showing group of cells with large pleomorphic nuclei, prominent nucleoli and some loss of cohesion (Papanicolaou stain; original magnification ×600, reproduced here at 60%). needle biopsy showed a tumour with the histological features of low grade ductal carcinoma in situ with an abnormal architecture and possibly invasive carcinoma (Fig. 3). The patient was given options and she chose to have a wireguided wide local excision of the lesion with axillary node clearance. Histology results showed a tumour composed of regular epithelial cells arranged around hyaline fibrovascular cores. In some areas, this appeared to be biphasic with a mixture of epithelial cells and myoepithelial cells. Immunocytochemistry confirmed the biphasic nature of the tumour (Fig. 4). The tumour was completely excised with a margin of 4 mm. All the 12 axillary lymph nodes were free of tumour. The final diagnosis was adenomyoepithelioma with epithelial hyperplasia but without atypia. In 10 months follow-up, she has had no problems. Discussion Tavassoli proposed a classification system of myoepithelial lesions of the breast. These lesions were divided into three types; myoepitheliosis, adenomyoepithelioma and malignant myoepithelioma. Adenomyoepithelioma is subdivided into four types, spindle cell, tubular, lobulated and carcinoma arising in adenomyoepithelioma.7 These different histological types behave differently both in clinical presentation and follow-up. Local recurrence after excision of the tumour has been reported in some cases6,7 and there may be distant metastases in cases of malignant variety.1,8 In triple assessment, FNAC is the investigation of choice to diagnose breast lumps when an experienced cytologist is available. A diagnostic accuracy of 96.9% and positive predictive value of 98.4% have been reported.9 Wide-bore needle biopsy is performed in case of indefinite cytology results or when there is suspicion of unusual pathology. A varied spectrum of cells is seen on the FNAC
Fig. 2. Histology from case 1 showing well demarcated mass composed of tubular structures with a mixed cell population (Haematoxylin and eosin; original magnification ×30, reproduced here at 60%). of adenomyoepithelioma of the breast and may result in confusion with other lesions. It may reveal different patterns of growth histologically and some additional features may result in diagnostic error.10 In both of these cases a pre-operative diagnosis of cancer was made, by C5 grading of cytology (case 1) and appearance of ductal carcinoma in situ and possible invasive cancer on wide-bore needle biopsy (case 2). The mammograms in both cases showed wellcircumscribed mass lesions with a score of R2 (case 1) and R3 (case 2). Review of the literature has confirmed that benign adenomyoepitheliomas have mammographic findings, which are suggestive of a benign lesion.11 Sonographic appearances vary from a cystic to a solid mass lesion. We therefore recommend that all patients with discordant results from radiology and cytology or histology should be reviewed carefully in multidisciplinary meetings before definitive treatment. If cytology is used for diagnosis, C5 should be considered for definitive treatment only when the lesion is clinically malignant and radiologically has a score of R5. A malignant result from a core biopsy can override a radiology score of less than 5. If all features do not fit together, one should be cautious to avoid unnecessary surgery for benign lesions and the subsequent morbidity of axillary clearance. If there is any doubt, the patients should have wide local excision for the primary lesion and if the subsequent diagnosis is that of cancer, another operation for the treatment of axilla should be acceptable. This practice will avoid unnecessary treatment of the axilla of patients with these types of benign lesions. References 1. Foschini MP, Eusebi V. Carcinomas of the breast showing myoepithelial cell differentiation. A review of the literature. Virchows Archiv 1998; 432: 303–10.
318
Case reports
Fig. 3. Histological appearances of the core biopsy from case 2 showing intraductal proliferation of small dark cells with a cribriform architecture interpreted as DCIS (Haematoxylin and eosin; original magnification ×30, reproduced here at 60%).
2. Ahmed A. The myoepithelium of the human breast carcinoma. J Pathol 1974; 112: 121–35. 3. Sciubba JJ, Brannon RB. Myoepithelioma of salivary glands: report of 23 cases. Cancer 1982; 49: 562–72. 4. Wallis NT, Banerjee SS, Eyden BP, Armstrong GR. Adenomyoepithelioma of the skin: a case report with immunohistochemical and ultrastructural observations. Histopathology 1997; 31: 374–7. 5. Tsuji N, Tateishi R, Ishiguro S, Terao T, Higashiyama M. Adenomyoepithelioma of the lung. Am J Surg Pathol 1995; 19: 956–62. 6. Young RH, Clement PB. Adenomyoepithelioma of the breast. Am J Clin Pathol 1988; 89: 308–14. 7. Tavassoli FA. Myoepithelial lesions of the breast: myoepitheliosis, adenomyoepithelioma, and myoepithelial carcinoma. Am J Surg Pathol 1991; 15: 554–68.
Fig. 4. Histological appearances of the wide local excision (case 2) showing a lobulated well defined mass (a; haematoxylin and eosin; original magnification ×30, reproduced here at 60%) with extension into ducts (arrows) around the main tumour simulating DCIS (inset b; haematoxylin and eosin; original magnification ×100, reproduced here at 60%). Immunocytochemistry for SMA-smooth muscle actin showing two cell populations (inset c; original magnification ×400, reproduced here at 60%). 8. Simpson RH, Cope N, Skalova A, Michal M. Malignant adenomyoepithelioma of the breast with mixed osteogenic, spindle cell, and carcinomatous differentiation. Am J Surg Pathol 1998; 22: 631–6. 9. Cook AIM, Robinson KB. Aspiration cytology of the breast with immediate reporting: initial experience with 1000 patients in a district general hospital. J R Coll Surg Edinb 1991; 36: 289–92. 10. Laforga GB, Aranda FI, Sevilla F. Adenomyoepithelioma of the breast: report of two cases with prominent cystic changes and intranuclear inclusions. Diagn Cytopathol 1998; 19: 55–8. 11. Berna JD, Arcas I, Ballester A, Bas A. Adenomyoepithelioma of the breast in a male. Am J Roentgenol 1997; 169: 917–8.
Case reports
tumour responded very well to the 5-fluorouracil treatment and shrinkage of the pancreatic tumour was confirmed radiologically in just 6 weeks. The reduction in size of the tumour coupled with the slow growing nature of this tumour prompted us to perform a resection. Thus, pre-operative adjuvant chemotherapy plays a significant role in downstaging the endocrine pancreatic tumour. Secondly, although a curative resection may not have been achieved despite radical excision, the patient remained well after 13 years following the Whipple’s procedure. Indeed her recent CT failed to show any evidence of recurrent disease. In conclusion, chemotherapy has a significant role to play in the management of malignant insulinomas. Although non-functioning malignant insulinomas presenting with obstructive features are rare, they may be successfully treated by surgery. If this is not feasible, surgical debulking of the primary and metastatic lesions produce good palliation and increased survival. Due to the slow nature of their growth it is imperative to follow-up these patients for many years.
References 1. Service FJ, McMahon MM, O’Brien PC, Ballard DJ. Functioning insulinoma: incidence, recurrence and long term survival of patients: a 60 year study. Mayo Clin Proc 1991; 66: 711–9. 2. Moldrow RE, Connelly RR. Epidemiology of pancreatic endocrine tumours of the pancreas. Analysis of autopsy cases. Dig Dis Sci 1968; 36: 933–42. 3. Bliss RD, Carter PB, Lennard TWJ. Insulinoma: a review of current management. Surg Oncol 1997; 6: 49–59. 4. Lo CY, Lam KY, Kung AW, Lam KS, Tung PH, Fan ST. Pancreatic insulinomas. A 15 year experience. Arch Surg 1997; 132: 926–30. 5. Grant CS. Surgical management of malignant islet cell tumours. World J Surg 1993; 17: 498–503. 6. Van Heerden J. Pancreatic resection for carcinoma of the pancreas: Whipples versus total pancreatectomy—an institutional perspective. World J Surg 1994; 8: 880. 7. Doppman JL, Miller DL, Chang R. Intraarterial calcium stimulation test for detection of insulinomas. World J Surg 1993; 17: 439–43.
doi:10.1053/ejso.1999.0890, available online at http://www.idealibrary.com on
Adenomyoepithelioma of the breast: a diagnostic dilemma T. Singh Gill, D. Clarke, A. G. Douglas-Jones∗, H. M. Sweetland and R. E. Mansel University Departments of Surgery and Pathology∗, University Hospital of Wales, Heath Park, Cardiff CF4 4XW, UK
Introduction Myoepithelial cells are normally present in the breast and are located between the epithelial cells and the basal lamina of secretory elements of the mammary duct system.1,2 Tumours derived from these cells have been described in skin, salivary glands, breast and lungs.3–5 Mammary tumours with predominant myoepithelial elements are extremely uncommon. The most common lesion with predominance of myoepithelial cells is sclerosing adenosis. Adenomyoepithelioma of the breast is an uncommon tumour characterized by the presence of both epithelial and myoepithelial cells.6 We describe two cases of adenomyoepithelioma of the breast in which pre-operative diagnosis of carcinoma was made on cytology or histology but the radiological findings were those of a benign lesion. Case reports
mobile lump of 3 cm in size in the upper outer quadrant of the left breast. The mammogram showed a well defined, 2.5-cm oval opacity, which was scored R2. Ultrasound revealed the presence of a 2-cm mass with no suspicious features. A fine needle aspiration cytology (FNAC) was performed which was reported as showing numerous malignant epithelial cells (Fig. 1). With a working diagnosis of carcinoma of the left breast the patient was referred to the breast clinic in University Hospital of Wales. The patient was given options for treatment and she chose to have a wide local excision with axillary node clearance. Histology results showed a well-circumscribed lesion composed of tubules lined by epithelial cells surrounded by a striking myoepithelial cell proliferation with clear cells. There was no cytological atypia, mitotic activity or necrosis and the lesion was considered to be a benign adenomyoepithelioma (Fig. 2). The 15 axillary lymph nodes examined were free of tumour. The cytology slides were reviewed in view of the histology results but the appearances were consistent with malignant cells of C5 grading. In 18 months follow-up, there has been no evidence of recurrence.
Case 1 A 63-year-old woman with a left breast lump was seen in a district general hospital. Physical examination disclosed a smooth, firm, Correspondence to: Miss Helen M. Sweetland, Senior Lecturer and Consultant Surgeon, University Hospital of Wales, Heath Park, Cardiff CF4 4XW, UK.
Case 2 A 68-year-old woman was recalled for assessment after a routine screening mammogram showed a 20-mm mass lesion in the lower outer quadrant of right breast, which was scored R3. Physical examination was normal. Ultrasound confirmed the presence of a 17-mm mass in the area of concern. Ultrasound-guided wide bore
317
Case reports
Fig. 1. Cytology from case 1 showing group of cells with large pleomorphic nuclei, prominent nucleoli and some loss of cohesion (Papanicolaou stain; original magnification ×600, reproduced here at 60%). needle biopsy showed a tumour with the histological features of low grade ductal carcinoma in situ with an abnormal architecture and possibly invasive carcinoma (Fig. 3). The patient was given options and she chose to have a wireguided wide local excision of the lesion with axillary node clearance. Histology results showed a tumour composed of regular epithelial cells arranged around hyaline fibrovascular cores. In some areas, this appeared to be biphasic with a mixture of epithelial cells and myoepithelial cells. Immunocytochemistry confirmed the biphasic nature of the tumour (Fig. 4). The tumour was completely excised with a margin of 4 mm. All the 12 axillary lymph nodes were free of tumour. The final diagnosis was adenomyoepithelioma with epithelial hyperplasia but without atypia. In 10 months follow-up, she has had no problems. Discussion Tavassoli proposed a classification system of myoepithelial lesions of the breast. These lesions were divided into three types; myoepitheliosis, adenomyoepithelioma and malignant myoepithelioma. Adenomyoepithelioma is subdivided into four types, spindle cell, tubular, lobulated and carcinoma arising in adenomyoepithelioma.7 These different histological types behave differently both in clinical presentation and follow-up. Local recurrence after excision of the tumour has been reported in some cases6,7 and there may be distant metastases in cases of malignant variety.1,8 In triple assessment, FNAC is the investigation of choice to diagnose breast lumps when an experienced cytologist is available. A diagnostic accuracy of 96.9% and positive predictive value of 98.4% have been reported.9 Wide-bore needle biopsy is performed in case of indefinite cytology results or when there is suspicion of unusual pathology. A varied spectrum of cells is seen on the FNAC
Fig. 2. Histology from case 1 showing well demarcated mass composed of tubular structures with a mixed cell population (Haematoxylin and eosin; original magnification ×30, reproduced here at 60%). of adenomyoepithelioma of the breast and may result in confusion with other lesions. It may reveal different patterns of growth histologically and some additional features may result in diagnostic error.10 In both of these cases a pre-operative diagnosis of cancer was made, by C5 grading of cytology (case 1) and appearance of ductal carcinoma in situ and possible invasive cancer on wide-bore needle biopsy (case 2). The mammograms in both cases showed wellcircumscribed mass lesions with a score of R2 (case 1) and R3 (case 2). Review of the literature has confirmed that benign adenomyoepitheliomas have mammographic findings, which are suggestive of a benign lesion.11 Sonographic appearances vary from a cystic to a solid mass lesion. We therefore recommend that all patients with discordant results from radiology and cytology or histology should be reviewed carefully in multidisciplinary meetings before definitive treatment. If cytology is used for diagnosis, C5 should be considered for definitive treatment only when the lesion is clinically malignant and radiologically has a score of R5. A malignant result from a core biopsy can override a radiology score of less than 5. If all features do not fit together, one should be cautious to avoid unnecessary surgery for benign lesions and the subsequent morbidity of axillary clearance. If there is any doubt, the patients should have wide local excision for the primary lesion and if the subsequent diagnosis is that of cancer, another operation for the treatment of axilla should be acceptable. This practice will avoid unnecessary treatment of the axilla of patients with these types of benign lesions. References 1. Foschini MP, Eusebi V. Carcinomas of the breast showing myoepithelial cell differentiation. A review of the literature. Virchows Archiv 1998; 432: 303–10.
318
Case reports
Fig. 3. Histological appearances of the core biopsy from case 2 showing intraductal proliferation of small dark cells with a cribriform architecture interpreted as DCIS (Haematoxylin and eosin; original magnification ×30, reproduced here at 60%).
2. Ahmed A. The myoepithelium of the human breast carcinoma. J Pathol 1974; 112: 121–35. 3. Sciubba JJ, Brannon RB. Myoepithelioma of salivary glands: report of 23 cases. Cancer 1982; 49: 562–72. 4. Wallis NT, Banerjee SS, Eyden BP, Armstrong GR. Adenomyoepithelioma of the skin: a case report with immunohistochemical and ultrastructural observations. Histopathology 1997; 31: 374–7. 5. Tsuji N, Tateishi R, Ishiguro S, Terao T, Higashiyama M. Adenomyoepithelioma of the lung. Am J Surg Pathol 1995; 19: 956–62. 6. Young RH, Clement PB. Adenomyoepithelioma of the breast. Am J Clin Pathol 1988; 89: 308–14. 7. Tavassoli FA. Myoepithelial lesions of the breast: myoepitheliosis, adenomyoepithelioma, and myoepithelial carcinoma. Am J Surg Pathol 1991; 15: 554–68.
Fig. 4. Histological appearances of the wide local excision (case 2) showing a lobulated well defined mass (a; haematoxylin and eosin; original magnification ×30, reproduced here at 60%) with extension into ducts (arrows) around the main tumour simulating DCIS (inset b; haematoxylin and eosin; original magnification ×100, reproduced here at 60%). Immunocytochemistry for SMA-smooth muscle actin showing two cell populations (inset c; original magnification ×400, reproduced here at 60%). 8. Simpson RH, Cope N, Skalova A, Michal M. Malignant adenomyoepithelioma of the breast with mixed osteogenic, spindle cell, and carcinomatous differentiation. Am J Surg Pathol 1998; 22: 631–6. 9. Cook AIM, Robinson KB. Aspiration cytology of the breast with immediate reporting: initial experience with 1000 patients in a district general hospital. J R Coll Surg Edinb 1991; 36: 289–92. 10. Laforga GB, Aranda FI, Sevilla F. Adenomyoepithelioma of the breast: report of two cases with prominent cystic changes and intranuclear inclusions. Diagn Cytopathol 1998; 19: 55–8. 11. Berna JD, Arcas I, Ballester A, Bas A. Adenomyoepithelioma of the breast in a male. Am J Roentgenol 1997; 169: 917–8.