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A rare case of myxoid soft tissue perineurioma involving perinephric fat
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PATHOLOGY 2012 ABSTRACT SUPPLEMENT
sheets of spindle cells. Immunohistochemistry was positive for CD99 and BCL2 and negative for cytokeratins and S100. Fluorescence in situ hybridisation (FISH) showed t(X; 18) (p11; 911) translocation in all four cases. Diagnosis: Synovial sarcoma. Discussion: Synovia sarcoma is commonly found in soft tissue. Histologically it is classified into four subtypes: biphasic, monophasic, monophasic epithelial and poorly differentiated. Synovial sarcoma occurs predominantly in the extremities. Primary synovial sarcoma arising in lung is rare. Reference 1. Dennison S, Weppler E, Giacoppe G. Primary pulmonary synovial sarcoma: A case report and review of current diagnostic and therapeutic standards. Oncologist 2004; 9: 339–42.
Q FEVER (COXIELLA BURNETTI ): RARE DIAGNOSIS ON LIVER BIOPSY M. Gera, A. Kaufman Royal North Shore Hospital, Sydney, NSW, Australia Case report: A 39-year-old male presented with unexplained fever, hepatosplenomegaly, jaundice and encephalopathy. Liver biopsy was performed as a last diagnostic tool. Macroscopic examination showed 5–15 mm of multiple fragments of liver cores. Microscopically there was inflammatory infiltrate in both liver lobules and portal tracts composed primarily of histiocytic granulomas. The granulomas had a distinct ring form with central clearing and a ring of fibrin. Immunohistochemistry was positive for CD68 and negative for CMV, ZN and fite. Diagnosis: Granulomatous hepatitis with ring granulomas (Q fever). Discussion: Q fever, due to Coxiella burnetti is a ubiquitous zoonosis. In humans, infection results from inhalation of contaminated aerosols from amniotic fluid or placenta or contaminated wool. Diagnosis can be confirmed by serology and/or DNA amplification from blood, cerebrospinal fluid, bone marrow, bone biopsy, liver biopsy and fetal specimens. The immune response results in the formation of granulomatous lesions most commonly involving the lungs, liver, and bone marrow. Prognosis: Q-fever present as acute or illness which can be treated by oral doxycycline. References 1. Fournier P-E, Marrie TJ, Raoult D. The diagnosis of Q fever. J Clin Microbiol 1998; 36: 1823. 2. Parker NR, Barralet JH, Bell AM. Q fever. Lancet 2006; 367: 679–88.
A RARE CASE OF MYXOID SOFT TISSUE PERINEURIOMA INVOLVING PERINEPHRIC FAT M. Gera, B. Atmore PaLMS, Royal North Shore Hospital, Sydney, NSW, Australia Case report: A 38-year-old female presented with an exophytic right kidney mass. Macroscopic examination showed a 25 mm myxoid, nodular lesion within the perinephric fat. Microscopically, thre was a well circumscribed, uniformly mildly cellular, unencapsulated, myxoid spindle cell lesion present in the perinephric fat, immediately adjacent to the renal capsule. There was no involvement of the kidney. Immunohistochemistry was positive for EMA, SMA, Vimentin, BCL2, WT-1 and negative for MDM2, CDK4, claudin, CD34, S100, Desmin, HMB45, melan A, Bcatenin, and AE1/AE3. Fluorescence in situ hybridisation (FISH)
Pathology (2012), 44(S1)
showed a normal signal pattern for probe for FUS/CREB3L2 fusion transcript between chromosomes 7 and 16 (excluding low grade fibromyxoid sarcoma). Diagnosis: Myxoid soft tissue perineurioma. Discussion: Perineuriomas are uncommon benign peripheral nerve sheath tumours. Location in the perinephric fat has not been previously reported in the literature, and therefore it is important to be aware of the possibility of occurrence at this site. The tumours typically show a morphological spectrum ranging from a hypercellular with collagenous stroma to hypocellular with myxoid stroma. In our case, the lesional cells are notable for having delicate elongated nuclei and numerous very attenuated bipolar cytoplasmic processes, which are highlighted by EMA. Prognosis: Generally benign with rare local recurrence. Acknowledgement: Professor CDM Fletcher, Brigham and Women’s Hospital, USA (consultation diagnosis). THE RECTAL TONSIL IN RECTAL ADENOCARCINOMA: A FORTUITOUS ASSOCIATION? S. G. H. Goh, Y. H. Ho, C. Y. P. Chau, W. M. Yap, K. L. Chuah Department of Pathology, Tan Tock Seng Hospital, Singapore The rectal tonsil is a term originally coined to describe a structure composed of dense lymphoid tissue primarily in rodents. Subsequently, this term is applied to prominent lymphoid tissue located in the rectum in human beings as well. When the lymphoid proliferation becomes exuberant, separation from a lymphoma can be problematic, particularly for those who are not aware of this rather uncommon entity. The simultaneous occurrence of rectal tonsil and adenocarcinoma has not been previously documented to the best of our knowledge. In this report, we detail an instance when an exaggerated proliferation of such lymphoid tissue occurred in association with rectal adenocarcinoma in an 82-year-old lady who underwent a rectal biopsy during sigmoidoscopy for dyspepsia. On histology, there was a marked lymphoid proliferation expanding the lamina propria accompanying malignant glands. Lymphoepithelial complexes were noted. A subsequent anterior resection was performed. The differential diagnoses and criteria for separating benign from malignant lymphoid proliferations as well as the significance of the occurrence of rectal tonsil in association with an adenocarcinoma are presented. IgG4-POSITIVE PLASMA CELLS IN CARCINOMAS OF THE PANCREAS AND AMPULLA OF VATER Goh Chin Hong Ronald1, Avery Khoo1, Steven Messenas2, Damien Tan2, Lim Kiat Hon Tony1 1 Department of Pathology, and 2Department of Gastroenterology, Singapore General Hospital, Singapore Aim: To study the prevalence and distribution of IgG4-positive plasma cells in pancreatic and ampullary carcinoma. Method: 29 cases of primary pancreatic and ampullary carcinoma between 2008 and 2011 were retrieved from archive. IgG4 staining by immunohistochemistry was performed on sections of the tumour with surrounding pancreas and pancreatic ducts. The pattern of distribution and the mean numbers of IgG4-positive plasma cells were obtained. Results: 19 of 29 (66%) cases showed the presence IgG4-positive plasma and eight cases (28%) featured >30 positive cells/HPF. Six cases (21%) showed a periductal distribution of IgG4 positive plasma cell with three cases (10%) displaying >10 positive
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright
PATHOLOGY 2012 ABSTRACT SUPPLEMENT
sheets of spindle cells. Immunohistochemistry was positive for CD99 and BCL2 and negative for cytokeratins and S100. Fluorescence in situ hybridisation (FISH) showed t(X; 18) (p11; 911) translocation in all four cases. Diagnosis: Synovial sarcoma. Discussion: Synovia sarcoma is commonly found in soft tissue. Histologically it is classified into four subtypes: biphasic, monophasic, monophasic epithelial and poorly differentiated. Synovial sarcoma occurs predominantly in the extremities. Primary synovial sarcoma arising in lung is rare. Reference 1. Dennison S, Weppler E, Giacoppe G. Primary pulmonary synovial sarcoma: A case report and review of current diagnostic and therapeutic standards. Oncologist 2004; 9: 339–42.
Q FEVER (COXIELLA BURNETTI ): RARE DIAGNOSIS ON LIVER BIOPSY M. Gera, A. Kaufman Royal North Shore Hospital, Sydney, NSW, Australia Case report: A 39-year-old male presented with unexplained fever, hepatosplenomegaly, jaundice and encephalopathy. Liver biopsy was performed as a last diagnostic tool. Macroscopic examination showed 5–15 mm of multiple fragments of liver cores. Microscopically there was inflammatory infiltrate in both liver lobules and portal tracts composed primarily of histiocytic granulomas. The granulomas had a distinct ring form with central clearing and a ring of fibrin. Immunohistochemistry was positive for CD68 and negative for CMV, ZN and fite. Diagnosis: Granulomatous hepatitis with ring granulomas (Q fever). Discussion: Q fever, due to Coxiella burnetti is a ubiquitous zoonosis. In humans, infection results from inhalation of contaminated aerosols from amniotic fluid or placenta or contaminated wool. Diagnosis can be confirmed by serology and/or DNA amplification from blood, cerebrospinal fluid, bone marrow, bone biopsy, liver biopsy and fetal specimens. The immune response results in the formation of granulomatous lesions most commonly involving the lungs, liver, and bone marrow. Prognosis: Q-fever present as acute or illness which can be treated by oral doxycycline. References 1. Fournier P-E, Marrie TJ, Raoult D. The diagnosis of Q fever. J Clin Microbiol 1998; 36: 1823. 2. Parker NR, Barralet JH, Bell AM. Q fever. Lancet 2006; 367: 679–88.
A RARE CASE OF MYXOID SOFT TISSUE PERINEURIOMA INVOLVING PERINEPHRIC FAT M. Gera, B. Atmore PaLMS, Royal North Shore Hospital, Sydney, NSW, Australia Case report: A 38-year-old female presented with an exophytic right kidney mass. Macroscopic examination showed a 25 mm myxoid, nodular lesion within the perinephric fat. Microscopically, thre was a well circumscribed, uniformly mildly cellular, unencapsulated, myxoid spindle cell lesion present in the perinephric fat, immediately adjacent to the renal capsule. There was no involvement of the kidney. Immunohistochemistry was positive for EMA, SMA, Vimentin, BCL2, WT-1 and negative for MDM2, CDK4, claudin, CD34, S100, Desmin, HMB45, melan A, Bcatenin, and AE1/AE3. Fluorescence in situ hybridisation (FISH)
Pathology (2012), 44(S1)
showed a normal signal pattern for probe for FUS/CREB3L2 fusion transcript between chromosomes 7 and 16 (excluding low grade fibromyxoid sarcoma). Diagnosis: Myxoid soft tissue perineurioma. Discussion: Perineuriomas are uncommon benign peripheral nerve sheath tumours. Location in the perinephric fat has not been previously reported in the literature, and therefore it is important to be aware of the possibility of occurrence at this site. The tumours typically show a morphological spectrum ranging from a hypercellular with collagenous stroma to hypocellular with myxoid stroma. In our case, the lesional cells are notable for having delicate elongated nuclei and numerous very attenuated bipolar cytoplasmic processes, which are highlighted by EMA. Prognosis: Generally benign with rare local recurrence. Acknowledgement: Professor CDM Fletcher, Brigham and Women’s Hospital, USA (consultation diagnosis). THE RECTAL TONSIL IN RECTAL ADENOCARCINOMA: A FORTUITOUS ASSOCIATION? S. G. H. Goh, Y. H. Ho, C. Y. P. Chau, W. M. Yap, K. L. Chuah Department of Pathology, Tan Tock Seng Hospital, Singapore The rectal tonsil is a term originally coined to describe a structure composed of dense lymphoid tissue primarily in rodents. Subsequently, this term is applied to prominent lymphoid tissue located in the rectum in human beings as well. When the lymphoid proliferation becomes exuberant, separation from a lymphoma can be problematic, particularly for those who are not aware of this rather uncommon entity. The simultaneous occurrence of rectal tonsil and adenocarcinoma has not been previously documented to the best of our knowledge. In this report, we detail an instance when an exaggerated proliferation of such lymphoid tissue occurred in association with rectal adenocarcinoma in an 82-year-old lady who underwent a rectal biopsy during sigmoidoscopy for dyspepsia. On histology, there was a marked lymphoid proliferation expanding the lamina propria accompanying malignant glands. Lymphoepithelial complexes were noted. A subsequent anterior resection was performed. The differential diagnoses and criteria for separating benign from malignant lymphoid proliferations as well as the significance of the occurrence of rectal tonsil in association with an adenocarcinoma are presented. IgG4-POSITIVE PLASMA CELLS IN CARCINOMAS OF THE PANCREAS AND AMPULLA OF VATER Goh Chin Hong Ronald1, Avery Khoo1, Steven Messenas2, Damien Tan2, Lim Kiat Hon Tony1 1 Department of Pathology, and 2Department of Gastroenterology, Singapore General Hospital, Singapore Aim: To study the prevalence and distribution of IgG4-positive plasma cells in pancreatic and ampullary carcinoma. Method: 29 cases of primary pancreatic and ampullary carcinoma between 2008 and 2011 were retrieved from archive. IgG4 staining by immunohistochemistry was performed on sections of the tumour with surrounding pancreas and pancreatic ducts. The pattern of distribution and the mean numbers of IgG4-positive plasma cells were obtained. Results: 19 of 29 (66%) cases showed the presence IgG4-positive plasma and eight cases (28%) featured >30 positive cells/HPF. Six cases (21%) showed a periductal distribution of IgG4 positive plasma cell with three cases (10%) displaying >10 positive
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited. Copyright