- Email: [email protected]
MUC2 staining is not useful to predict subsequent development of intestinal metaplasia in oesophageal biopsies
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PATHOLOGY 2012 ABSTRACT SUPPLEMENT
Conclusions: ERG shows a sensitivity of 51.5% and a remarkable specificity of 100% for the diagnosis of prostatic adenocarcinoma versus benign glands. ERG positive PIN is almost always associated with invasive adenocarcinoma in the same core biopsy. Immunohistochemistry for ERG may therefore have significant role in the pathological differential diagnosis of difficult prostate biopsies. CLINICAL UTILITY OF SOLUBLE IL-2 RECEPTOR ALPHA IN HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS Kuang-Chih Hsiao1, Francoise Mechinaud2, Chris Czajko1, Marilyn Clark1, Sharon Choo1 1 Immunology Laboratory, Royal Children’s Hospital, Melbourne, and 2Children’s Cancer Centre, Royal Children’s Hospital, Melbourne, Vic, Australia Haemophagocytic lymphohistiocytosis (HLH) is characterised by lymphocyte and macrophage activation, resulting in fever, hepatosplenomegaly, coagulopathy, cytopenias and other features of inflammation. Diagnosis of HLH can be established when five of eight criteria, one of which is elevated soluble IL2-receptor-alpha (sIL2-Ra), are fulfilled.1 Mortality is high, despite HLH04 chemotherapy, prompting the search for better biomarkers of disease activity. Aims: To assess the utility of sIL2-Ra in diagnosis of patients with suspected HLH and in disease activity monitoring. Method: sIL2-Ra was measured by ELISA (R&D Systems) in stored serum, collected at presentation, from eight patients with known HLH (5 of 7 criteria, excluding sIL2-Ra). In two of the eight patients (Patients A and B), serial sIL2-Ra were measured and compared with serum ferritin and clinical disease activity (CDA), retrospectively assessed by the lead clinician, who was blinded to the sIL2-Ra results. Results: All eight patients had elevated sIL2-Ra levels. Patient A’s sIL2-Ra and ferritin levels correlated with CDA. Serial sIL2-Ra/ ferritin/CDA results were 39518/14987/severe, >50000/6146/ moderate, 13013/2315/mild, 10167/5038/moderate, 8446/2745/ moderate, 5184/1772/inactive. Patient B’s sIL2-Ra levels correlated better with CDA than ferritin levels. Serial sIL2-Ra/ferritin/CDA results were 47021/2948/severe, 18188/1173/moderate, 33870/ 1246/severe, 17147/5277/severe, 9806/20429/severe, 11333/3851/ severe. Conclusion: This study demonstrates the diagnostic sensitivity of sIL2-Ra and its value in complementing traditional disease activity markers in HLH. Reference 1. Henter J-I, Horne A, Arico´ M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124–31.
PERFORMANCE MONITORING IN TRANSFUSION C. Hughes, N. Herrmann RCPA Transfusion QAP, Qld, Australia Aim: In 2012, the RCPA Transfusion QAP will be introducing a pilot for Performance Monitoring in the General Compatibility module. Method: After each survey, the Transfusion Advisory Committee review laboratory results and points are allocated for each assessable element. This allows participants to be placed into a performance level based on the final score obtained.
Pathology (2012), 44(S1)
From the survey levels, a cumulative performance level (CPL) is calculated. Performance levels are weighted and the CPL is calculated from the sum of individual survey performance levels divided by the last six surveys regardless of year/cycle. Performance levels: Reference 100, loss of 0 points, weighting ¼ 1; Reference 99, loss of 1–19 points (minor errors), weighting ¼ 2; Operational, loss of 20–49 points (minor errors), weighting ¼ 3; Review, loss of 50–99 points (1 critical error) weighting ¼ 4; Unsatisfactory, loss of >100 points (2 or more critical errors) weighting ¼ 5. Exceptions: Unsatisfactory survey performance level defaults to unsatisfactory CPL; Review survey performance level defaults to review CPL; 3rd consecutive Reference 100 survey performance level resets the CPL to Reference 100. Conclusions/outcomes: Participants results falling outside the criteria will be referred to the Advisory Committee for review. A letter of notification and committee report will be issued to the participant, supervisor(s) and Director of Pathology as below. Criteria for letter of notification: Letter 1: two critical errors – letter and report sent to participant and supervisor(s); Letter 2: three critical errors – letter and report sent to participant and supervisor(s); Letter 3: four critical errors – letter and report sent to participant, supervisor(s) and Director of Pathology. OVARIAN SERTOLI-LEYDIG CELL TUMOUR OF INTERMEDIATE-DIFFERENTIATION WITH HETEROLOGOUS ELEMENTS COMPRISING OF SEROMUCINOUS EPITHELIAL LINED CYSTS AND IMMATURE NEURAL TISSUE: AN UNUSUAL NEOPLASM Doris Ip Hee Wai, Simon Nazaretian The Royal Women’s Hospital Anatomical Pathology, Melbourne, Vic, Australia Ovarian Sertoli-Leydig cell tumour (SLCT) is a rare sex cord stromal tumour. Some SLCT, usually of intermediate or poor differentiation show heterologous elements such as gastrointestinal type epithelium, immature cartilage or skeletal muscle. However, SLCT with serous epithelium and immature neural tissue as heterologous elements have not been documented. We describe a rare ovarian SLCT with heterologous elements in a 25-year-old woman with post-partum abdominal swelling and pain. Heterologous elements included cystic locules lined by a benign epithelium of serous and mucinous cells and a small foci of immature neural tissue. SCLT with heterologous mucinous elements should be distinguished from pure mucinous cystic ovarian tumours which may appear similar macroscopically and present with virilising symptoms. Heterologous elements may present in a variable proportion from microscopic foci to predominant tumour masking the SLCT. This case highlights the importance of extensive sampling because of common and overlapping morphological features with other ovarian tumours. MUC2 STAINING IS NOT USEFUL TO PREDICT SUBSEQUENT DEVELOPMENT OF INTESTINAL METAPLASIA IN OESOPHAGEAL BIOPSIES P. Irandoost, L. Siosson, A. Clarkson, A. J. Gill Royal North Shore Hospital, Sydney, NSW, Australia Aims: It has recently been postulated that goblet cells in the oesophagus eventually develop from a field of metaplastic
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright
ABSTRACTS
columnar cells with MUC2 positivity. The aim of our study was to assess whether MUC2 positivity in columnar cells predicts subsequent identification of goblet cells. Methods: Twenty-two study cases were found (over 1998–2011) with intestinal metaplasia that had initial negative biopsies. MUC2 staining was performed on a positive and a negative biopsy from every case and compared with 29 control cases that never had goblet cells on multiple subsequent biopsies. Results: All goblet cells and the majority of columnar cells directly in the vicinity of goblet cells (17/22 cases) showed MUC2 staining. This was less significant in the columnar cells away from goblet cells with only 6/22 cases showing positive staining. Fifteen of 22 (68%) cases with intestinal metaplasia showed some positive MUC2 staining on their earlier negative biopsies. Fourteen of 29 (48%) individuals who never had goblet cells on subsequent biopsies showed positive MUC2 staining. Conclusion: Although the rate of MUC2 positivity was slightly higher on initial negative biopsies of individuals with intestinal metaplasia, our findings do not suggest that routine staining for MUC2 will be useful to predict subsequent diagnosis of intestinal metaplasia in columnar lined oesophagus. References 1. McIntire M, Soucy G, Vaughan T, et al. MUC2 is a highly specific marker of goblet cell metaplasia in the distal oesophagus and the gastrooesophageal junction. Am J Surg Pathol 2011; 35: 1007–13. 2. Hahn H, Blount PL,Ayub K, et al. Intestinal differentiation in metaplastic, non-goblet columnar epithelium in the oesophagus. Am J Surg Pathol 2009; 33: 1006–15. 3. Chandrasoma P, Wijetunge S, DeMeester S, et al. Columnar-lined oesophagus without intestinal metaplasia has no proven risk of adenocarcinoma. Am J Surg Pathol 2012; 36: 1–7. 4. Niv Y, Fass R. The role of mucin in GERD and its complications. Nat Rev Gastroenterol Hepatol 2011; 9: 55–9.
CASE REPORT OF SCLEROSING MESENTERITIS P. Irandoost, K. De Silva Royal North Shore Hospital, Sydney, NSW, Australia Case presentation: An 85-year-old female presented with intestinal obstruction. Imaging revealed a mesenteric mass suspicious for a neuroendocrine tumour. Macroscopic examination showed a convoluted segment of small bowel with a 50 mm mesenteric mass causing adhesion of adjacent bowel loops at multiple points. The cut surface was solid yellow with areas of calcification. Microscopically there was a variable combination of fat necrosis, fibrosis, sclerosis, chronic inflammation and focal calcification. There was no cytological atypia or mitotic activity. Immunohistochemistry was negative for b-catenin, Alk1 and CD34 and positive for SMA. There was no increase in IgG4 positive plasma cells. Diagnosis: Sclerosing mesenteritis. Discussion: This is a rare benign disease of unknown aetiology, characterised by tumour-like mass composed of chronic inflammation, fat necrosis and fibrosis. Patients may present with abdominal pain, obstruction, fever, chylous ascites or changed bowel habbits. Although various causes have been suggested including infection, trauma or ischaemia, the exact aetiology cannot be affirmed. Diagnosis is hard to make clinically and radiologically and needs pathological assessment of the excision biopsy. Treatment and prognosis: It has a favourable prognosis and may be self-limiting. Asymptomatic cases may be observed. Some suggest immunosuppressive therapy for cases with mild symptoms to prevent progression. Surgical resection is reserved for cases with complications (bowel obstruction or perforation).
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References 1. Okumu W, Schmidt CM, LeBlanc JK. Fat necrosis, fibrosis, chronic inflammation, abdominal pain, and a small bowel mass in a 65 year old man. Practical Gastroenterol 2008; July: 44–46. 2. Guo-Li G, Shi-Lin W, Xue-Ming W, et al. Sclerosing mesenteritis as a rare casue of abdominal pain and intraabdominal mass; a case report. Cases Journal 2008; 1: 242. 3. Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis; clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol 2007; 5: 589–96. 4. Lim CS, Singh Tanger G, Tibrewal S, et al. Sclerosing mesenteritis presenting with small bowel obstruction and subsequent retroperitoneal fibrosis. Eur J Gasteroenterol Hepatol 2006; 18: 1285–7.
CASE REPORT OF SUPERFICIAL ACRAL FIBROMYXOMA P. Irandoost, R. Eckstein, S. McCarthy, R. Scolyer Royal North Shore Hospital, and Royal Prince Alfred Hospital, Sydney, NSW, Australia Case presentation: A 38-year-old female presented with a soft tissue lesion beneath the nail bed of the right middle finger. Imaging showed a lucent lesion involving the medial aspect of the shaft of distal phalanx with some bone erosion. Macroscopic examination showed a 12 mm transparent myxoid nodule. Microscopically there was a uniformly moderately cellular myxoid lesion with a pushing margin, composed of spindled and stellate cells. No cytological atypia, mitosis or necrosis was observed. Thin-walled, curvilinear blood vessels and scattered mast cells were also present throughout. Immunohistochemistry was negative for NF, S100, EMA and SMA, and positive for CD34. Fluorescence in situ hybridisation (FISH) was negative for FUS translocation, specific for low-grade fibromyxoid sarcoma. Diagnosis: Superficial acral fibromyxoma. Discussion: These lesions are typically located in the dermis or subcutis of fingers and toes, with a tendency to involve the nail bed. They are composed of spindled and stellate cells in a myxoid background with accentuated vasculartiy and increased mast cells. They are typically CD34, EMA and CD99 positive and need to be differentiated from low-grade fibromyxoid sarcoma (MUC4–, CD34–, FISH showing FUS translocation), myxoid neurofibroma (S100þ), superficial angiomyxoma (CD34–) and myxoid DFSP [also CD34þ but has subcutaneous fat infiltration and typically shows t(17;22)]. Prognosis: Generally benign with 10% recurrence rate and therefore needing complete excision and follow-up. References 1. Al-Daraji W, Miettinen M. Superficial acral fibromyxoma: a clinicopathological analysis of 32 tumors including 4 in the heel. J Cutan Pathol 2007; 35: 1020–6. 2. Fetsch JF, Laskin WB, Miettinen M. Superficial acral fibromyxoma: a clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes. Hum Pathol 2001; 32: 704 3. Abou-Nukta F, Fiedler P, Parkash V, et al. Superficial acral fibromyxoma of the distal phalanx of the thumb. J Hand Surg (Br) 2006; 31: 619. 4. Quaba O, Evans A, Al-Nafussi AA, et al. Superficial acral fibromyxoma. Br J Plast Surg 2005; 58: 561.
MIXED HIGH GRADE NEUROENDOCRINE CARCINOMA AND ADENOCARCINOMA OF THE AMPULLA OF VATER ASSOCIATED WITH INTESTINAL ADENOMA Kambin Jafari Nejad Prince of Wales Hospital, Sydney, NSW, Australia
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright
PATHOLOGY 2012 ABSTRACT SUPPLEMENT
Conclusions: ERG shows a sensitivity of 51.5% and a remarkable specificity of 100% for the diagnosis of prostatic adenocarcinoma versus benign glands. ERG positive PIN is almost always associated with invasive adenocarcinoma in the same core biopsy. Immunohistochemistry for ERG may therefore have significant role in the pathological differential diagnosis of difficult prostate biopsies. CLINICAL UTILITY OF SOLUBLE IL-2 RECEPTOR ALPHA IN HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS Kuang-Chih Hsiao1, Francoise Mechinaud2, Chris Czajko1, Marilyn Clark1, Sharon Choo1 1 Immunology Laboratory, Royal Children’s Hospital, Melbourne, and 2Children’s Cancer Centre, Royal Children’s Hospital, Melbourne, Vic, Australia Haemophagocytic lymphohistiocytosis (HLH) is characterised by lymphocyte and macrophage activation, resulting in fever, hepatosplenomegaly, coagulopathy, cytopenias and other features of inflammation. Diagnosis of HLH can be established when five of eight criteria, one of which is elevated soluble IL2-receptor-alpha (sIL2-Ra), are fulfilled.1 Mortality is high, despite HLH04 chemotherapy, prompting the search for better biomarkers of disease activity. Aims: To assess the utility of sIL2-Ra in diagnosis of patients with suspected HLH and in disease activity monitoring. Method: sIL2-Ra was measured by ELISA (R&D Systems) in stored serum, collected at presentation, from eight patients with known HLH (5 of 7 criteria, excluding sIL2-Ra). In two of the eight patients (Patients A and B), serial sIL2-Ra were measured and compared with serum ferritin and clinical disease activity (CDA), retrospectively assessed by the lead clinician, who was blinded to the sIL2-Ra results. Results: All eight patients had elevated sIL2-Ra levels. Patient A’s sIL2-Ra and ferritin levels correlated with CDA. Serial sIL2-Ra/ ferritin/CDA results were 39518/14987/severe, >50000/6146/ moderate, 13013/2315/mild, 10167/5038/moderate, 8446/2745/ moderate, 5184/1772/inactive. Patient B’s sIL2-Ra levels correlated better with CDA than ferritin levels. Serial sIL2-Ra/ferritin/CDA results were 47021/2948/severe, 18188/1173/moderate, 33870/ 1246/severe, 17147/5277/severe, 9806/20429/severe, 11333/3851/ severe. Conclusion: This study demonstrates the diagnostic sensitivity of sIL2-Ra and its value in complementing traditional disease activity markers in HLH. Reference 1. Henter J-I, Horne A, Arico´ M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124–31.
PERFORMANCE MONITORING IN TRANSFUSION C. Hughes, N. Herrmann RCPA Transfusion QAP, Qld, Australia Aim: In 2012, the RCPA Transfusion QAP will be introducing a pilot for Performance Monitoring in the General Compatibility module. Method: After each survey, the Transfusion Advisory Committee review laboratory results and points are allocated for each assessable element. This allows participants to be placed into a performance level based on the final score obtained.
Pathology (2012), 44(S1)
From the survey levels, a cumulative performance level (CPL) is calculated. Performance levels are weighted and the CPL is calculated from the sum of individual survey performance levels divided by the last six surveys regardless of year/cycle. Performance levels: Reference 100, loss of 0 points, weighting ¼ 1; Reference 99, loss of 1–19 points (minor errors), weighting ¼ 2; Operational, loss of 20–49 points (minor errors), weighting ¼ 3; Review, loss of 50–99 points (1 critical error) weighting ¼ 4; Unsatisfactory, loss of >100 points (2 or more critical errors) weighting ¼ 5. Exceptions: Unsatisfactory survey performance level defaults to unsatisfactory CPL; Review survey performance level defaults to review CPL; 3rd consecutive Reference 100 survey performance level resets the CPL to Reference 100. Conclusions/outcomes: Participants results falling outside the criteria will be referred to the Advisory Committee for review. A letter of notification and committee report will be issued to the participant, supervisor(s) and Director of Pathology as below. Criteria for letter of notification: Letter 1: two critical errors – letter and report sent to participant and supervisor(s); Letter 2: three critical errors – letter and report sent to participant and supervisor(s); Letter 3: four critical errors – letter and report sent to participant, supervisor(s) and Director of Pathology. OVARIAN SERTOLI-LEYDIG CELL TUMOUR OF INTERMEDIATE-DIFFERENTIATION WITH HETEROLOGOUS ELEMENTS COMPRISING OF SEROMUCINOUS EPITHELIAL LINED CYSTS AND IMMATURE NEURAL TISSUE: AN UNUSUAL NEOPLASM Doris Ip Hee Wai, Simon Nazaretian The Royal Women’s Hospital Anatomical Pathology, Melbourne, Vic, Australia Ovarian Sertoli-Leydig cell tumour (SLCT) is a rare sex cord stromal tumour. Some SLCT, usually of intermediate or poor differentiation show heterologous elements such as gastrointestinal type epithelium, immature cartilage or skeletal muscle. However, SLCT with serous epithelium and immature neural tissue as heterologous elements have not been documented. We describe a rare ovarian SLCT with heterologous elements in a 25-year-old woman with post-partum abdominal swelling and pain. Heterologous elements included cystic locules lined by a benign epithelium of serous and mucinous cells and a small foci of immature neural tissue. SCLT with heterologous mucinous elements should be distinguished from pure mucinous cystic ovarian tumours which may appear similar macroscopically and present with virilising symptoms. Heterologous elements may present in a variable proportion from microscopic foci to predominant tumour masking the SLCT. This case highlights the importance of extensive sampling because of common and overlapping morphological features with other ovarian tumours. MUC2 STAINING IS NOT USEFUL TO PREDICT SUBSEQUENT DEVELOPMENT OF INTESTINAL METAPLASIA IN OESOPHAGEAL BIOPSIES P. Irandoost, L. Siosson, A. Clarkson, A. J. Gill Royal North Shore Hospital, Sydney, NSW, Australia Aims: It has recently been postulated that goblet cells in the oesophagus eventually develop from a field of metaplastic
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright
ABSTRACTS
columnar cells with MUC2 positivity. The aim of our study was to assess whether MUC2 positivity in columnar cells predicts subsequent identification of goblet cells. Methods: Twenty-two study cases were found (over 1998–2011) with intestinal metaplasia that had initial negative biopsies. MUC2 staining was performed on a positive and a negative biopsy from every case and compared with 29 control cases that never had goblet cells on multiple subsequent biopsies. Results: All goblet cells and the majority of columnar cells directly in the vicinity of goblet cells (17/22 cases) showed MUC2 staining. This was less significant in the columnar cells away from goblet cells with only 6/22 cases showing positive staining. Fifteen of 22 (68%) cases with intestinal metaplasia showed some positive MUC2 staining on their earlier negative biopsies. Fourteen of 29 (48%) individuals who never had goblet cells on subsequent biopsies showed positive MUC2 staining. Conclusion: Although the rate of MUC2 positivity was slightly higher on initial negative biopsies of individuals with intestinal metaplasia, our findings do not suggest that routine staining for MUC2 will be useful to predict subsequent diagnosis of intestinal metaplasia in columnar lined oesophagus. References 1. McIntire M, Soucy G, Vaughan T, et al. MUC2 is a highly specific marker of goblet cell metaplasia in the distal oesophagus and the gastrooesophageal junction. Am J Surg Pathol 2011; 35: 1007–13. 2. Hahn H, Blount PL,Ayub K, et al. Intestinal differentiation in metaplastic, non-goblet columnar epithelium in the oesophagus. Am J Surg Pathol 2009; 33: 1006–15. 3. Chandrasoma P, Wijetunge S, DeMeester S, et al. Columnar-lined oesophagus without intestinal metaplasia has no proven risk of adenocarcinoma. Am J Surg Pathol 2012; 36: 1–7. 4. Niv Y, Fass R. The role of mucin in GERD and its complications. Nat Rev Gastroenterol Hepatol 2011; 9: 55–9.
CASE REPORT OF SCLEROSING MESENTERITIS P. Irandoost, K. De Silva Royal North Shore Hospital, Sydney, NSW, Australia Case presentation: An 85-year-old female presented with intestinal obstruction. Imaging revealed a mesenteric mass suspicious for a neuroendocrine tumour. Macroscopic examination showed a convoluted segment of small bowel with a 50 mm mesenteric mass causing adhesion of adjacent bowel loops at multiple points. The cut surface was solid yellow with areas of calcification. Microscopically there was a variable combination of fat necrosis, fibrosis, sclerosis, chronic inflammation and focal calcification. There was no cytological atypia or mitotic activity. Immunohistochemistry was negative for b-catenin, Alk1 and CD34 and positive for SMA. There was no increase in IgG4 positive plasma cells. Diagnosis: Sclerosing mesenteritis. Discussion: This is a rare benign disease of unknown aetiology, characterised by tumour-like mass composed of chronic inflammation, fat necrosis and fibrosis. Patients may present with abdominal pain, obstruction, fever, chylous ascites or changed bowel habbits. Although various causes have been suggested including infection, trauma or ischaemia, the exact aetiology cannot be affirmed. Diagnosis is hard to make clinically and radiologically and needs pathological assessment of the excision biopsy. Treatment and prognosis: It has a favourable prognosis and may be self-limiting. Asymptomatic cases may be observed. Some suggest immunosuppressive therapy for cases with mild symptoms to prevent progression. Surgical resection is reserved for cases with complications (bowel obstruction or perforation).
S83
References 1. Okumu W, Schmidt CM, LeBlanc JK. Fat necrosis, fibrosis, chronic inflammation, abdominal pain, and a small bowel mass in a 65 year old man. Practical Gastroenterol 2008; July: 44–46. 2. Guo-Li G, Shi-Lin W, Xue-Ming W, et al. Sclerosing mesenteritis as a rare casue of abdominal pain and intraabdominal mass; a case report. Cases Journal 2008; 1: 242. 3. Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis; clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol 2007; 5: 589–96. 4. Lim CS, Singh Tanger G, Tibrewal S, et al. Sclerosing mesenteritis presenting with small bowel obstruction and subsequent retroperitoneal fibrosis. Eur J Gasteroenterol Hepatol 2006; 18: 1285–7.
CASE REPORT OF SUPERFICIAL ACRAL FIBROMYXOMA P. Irandoost, R. Eckstein, S. McCarthy, R. Scolyer Royal North Shore Hospital, and Royal Prince Alfred Hospital, Sydney, NSW, Australia Case presentation: A 38-year-old female presented with a soft tissue lesion beneath the nail bed of the right middle finger. Imaging showed a lucent lesion involving the medial aspect of the shaft of distal phalanx with some bone erosion. Macroscopic examination showed a 12 mm transparent myxoid nodule. Microscopically there was a uniformly moderately cellular myxoid lesion with a pushing margin, composed of spindled and stellate cells. No cytological atypia, mitosis or necrosis was observed. Thin-walled, curvilinear blood vessels and scattered mast cells were also present throughout. Immunohistochemistry was negative for NF, S100, EMA and SMA, and positive for CD34. Fluorescence in situ hybridisation (FISH) was negative for FUS translocation, specific for low-grade fibromyxoid sarcoma. Diagnosis: Superficial acral fibromyxoma. Discussion: These lesions are typically located in the dermis or subcutis of fingers and toes, with a tendency to involve the nail bed. They are composed of spindled and stellate cells in a myxoid background with accentuated vasculartiy and increased mast cells. They are typically CD34, EMA and CD99 positive and need to be differentiated from low-grade fibromyxoid sarcoma (MUC4–, CD34–, FISH showing FUS translocation), myxoid neurofibroma (S100þ), superficial angiomyxoma (CD34–) and myxoid DFSP [also CD34þ but has subcutaneous fat infiltration and typically shows t(17;22)]. Prognosis: Generally benign with 10% recurrence rate and therefore needing complete excision and follow-up. References 1. Al-Daraji W, Miettinen M. Superficial acral fibromyxoma: a clinicopathological analysis of 32 tumors including 4 in the heel. J Cutan Pathol 2007; 35: 1020–6. 2. Fetsch JF, Laskin WB, Miettinen M. Superficial acral fibromyxoma: a clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes. Hum Pathol 2001; 32: 704 3. Abou-Nukta F, Fiedler P, Parkash V, et al. Superficial acral fibromyxoma of the distal phalanx of the thumb. J Hand Surg (Br) 2006; 31: 619. 4. Quaba O, Evans A, Al-Nafussi AA, et al. Superficial acral fibromyxoma. Br J Plast Surg 2005; 58: 561.
MIXED HIGH GRADE NEUROENDOCRINE CARCINOMA AND ADENOCARCINOMA OF THE AMPULLA OF VATER ASSOCIATED WITH INTESTINAL ADENOMA Kambin Jafari Nejad Prince of Wales Hospital, Sydney, NSW, Australia
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright