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2. Double hit mantle cell lymphoma with blastoid morphology: a case report
ABSTRACTS
1. A STUDY OF DISTRIBUTION OF MAST CELLS IN UTERINE LEIOMYOMAS AND MYOMETRIUM AND THEIR RELATIONSHIP TO MORPHOLOGY OF LEIOMYOMAS AND PATIENTS’ CLINICAL FEATURES N. V. A. Abeyratne, L. D. Santos and L. Y. C. Yong Department of Anatomical Pathology, SSWPS, Liverpool, NSW, Australia Introduction: Mast cells (MCs) effects on uterine leiomyomas (LMs) are still unclear. Haematoxylin and eosin (H&E) stain is suboptimal for MC visualization. Objectives: We compared (1) MC density in LMs with adjacent myometrium (control), morphology of LMs and clinical features and (2) compared MC counts in H&E with Toluidine blue (Tolb), Giemsa, Mast cell tryptase (MCT) and CD117/ckit. Methods: We selected 37 LMs from 27 patients and analysed clinical features. Each section of 37 LMs and controls was stained with H&E, Tolb, Giemsa, MCT and CD117. Two observers independently counted MCs in 50 high power fields (hpfs) and averaged to MCs/ 10hpfs and mean value was calculated. MC counts for 37 LMs and 26 controls were analysed and compared with each stain. Number of MCs/10hpf stained with CD117 was compared with size of LMs, degenerative change of LMs, status of endometrium and with the control. Results: The mean age of 27 patients was 52.8 years (ranged 31– 69). 11 patients presented with fibroids. 25 of 37 LMs were small (40 mm). CD117 showed higher number of MCs/10hpfs. H&E count was generally low. 50% of controls have higher counts (50/ 10hpfs). 72% of LMs with hyalinisation and 72.7% with atrophic endometrium showed low MC counts (<50/10hpfs). Conclusion: 1. Tolb and Giemsa were better than H&E for MC count. MCT showed staining problems. CD117 was the best stain. 2. No linear relationship found between LMs size and MC counts. 3. MC count was low in LMs with hyaline degeneration and atrophic endometrium. Further studies are required to find whether MCs are inhibitors or promoters of LMs.
2. DOUBLE HIT MANTLE CELL LYMPHOMA WITH BLASTOID MORPHOLOGY: A CASE REPORT Chandra Adhikari, Oana Crainic and Wendy Cooper Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney About 40% of all B-cell lymphomas are characterized by the presence of recurrent reciprocal chromosomal translocations. The term ‘double-hit’ lymphoma is mostly used for mature B cell lymphomas with a chromosomal breakpoint affecting the MYC locus along with another recurrent chromosomal breakpoint. These lymphomas are aggressive and usually show high proliferation rate. Mantle cell lymphoma is a mature B-cell lymphoma characterized by t(11;14) resulting in cyclin D1 over expression. Although rare, a MYC and cyclin D1 double-hit phenomenon can occur in mantle cell lymphoma and is associated with an adverse clinical course and blastoid morphology. We report a case of a 58 years old female with a left maxillary mass histologically showing features of blastoid mantle cell lymphoma. Fluorescence in situ hybridization (FISH) was positive for CCND1 and MYC gene rearrangements. Recognising ‘double-hit’ lymphomas, through use of appropriate investigations is important for
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optimal management of patients with these highly aggressive tumours.
3. DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOURLIKE NEOPLASM OF THE SEPTUM PELLUCIDUM: A CASE REPORT B. M. Allanson1, A. Thomas1,2, C. Lind3 and P. Robbins1,2 1Department of Anatomical Pathology PathWest Laboratory Medicine, Queen Elizabeth II Medical Centre, Western Australia, 2School of Pathology and Laboratory Medicine, University of Western Australia, and 3Department of Neurosurgery, Sir Charles Gairdner Hospital, Nedlands, Western Australia Background: Dysembryoplastic Neuroepithelial Tumour-like Neoplasm of the Septum Pellucidum is a rare, low grade CNS neoplasm that may be misdiagnosed as a diffuse glioma. Clinical features: We report a case of a 22 year old woman who presented with a first seizure. She was found to have a 31 mm nonenhancing cystic lesion on the right hand side of the anterior septum pellucidum. Pathological features: The lesion was completely resected and the specimen was received as fragmented pale-white gelatinous pieces of tissue. Histologically the lesion demonstrated a lobular, microcystic, and mucin-rich appearance, and had relatively well defined margins. A population of small uniform cells, often with perinuclear halos, and cytologically virtually indistinguishable from oligodendroglioma predominated. These small ‘oligodendrocyte-like cells’ (OLC) formed somewhat ‘patterned’ architectural arrangements with perivascular pseudorosettes and laminated arrays resembling the specific glioneuronal component of a dysembryoplastic neuroepithelial tumour. Rare individual neurons ‘floating’ in myxoid microcystic spaces were also present. These floating neurons lacked dysplastic features and evidence of satellitosis. Immunohistochemical staining showed mixed glioneuronal differentiation and the MIB1 proliferation index was low (less than 1%). PCR amplification and Sanger direct bidirectional sequencing for IDH1 and IDH2 mutations were negative. Discussion: Less than 20 cases of this rare tumour have been reported to date. DNT-like lesions should be included in differential diagnosis of midline intraventricular tumours in children and young adults. Distinction from diffuse glioma is essential because these tumours appear to behave in a benign fashion.
4. GOBLET CELLS IN NEOPLASTIC BARRETT MUCOSA AND THEIR RELEVANCE IN NEOPLASTIC PROGRESSION: A SEMIQUANTITATIVE ANALYSIS J. Bonavita1, B. M. Allanson1, S. Raftopoulos2 and M. P. Kumarasinghe1 1PathWest Laboratory Medicine, Queen Elizabeth II Medical Centre, and 2Department of Gastroenterology, Sir Charles Gardiner Hospital Background: The trigger for screening of columnar lined mucosa (CLM) of oesophagus is the presence of histologically proven goblet cells (GC). There is some evidence that oesophageal adenocarcinoma (EAC) may arise in CLM without GCs. Pathological data on association of GCs and oesophageal glandular neoplasia is limited.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
1. A STUDY OF DISTRIBUTION OF MAST CELLS IN UTERINE LEIOMYOMAS AND MYOMETRIUM AND THEIR RELATIONSHIP TO MORPHOLOGY OF LEIOMYOMAS AND PATIENTS’ CLINICAL FEATURES N. V. A. Abeyratne, L. D. Santos and L. Y. C. Yong Department of Anatomical Pathology, SSWPS, Liverpool, NSW, Australia Introduction: Mast cells (MCs) effects on uterine leiomyomas (LMs) are still unclear. Haematoxylin and eosin (H&E) stain is suboptimal for MC visualization. Objectives: We compared (1) MC density in LMs with adjacent myometrium (control), morphology of LMs and clinical features and (2) compared MC counts in H&E with Toluidine blue (Tolb), Giemsa, Mast cell tryptase (MCT) and CD117/ckit. Methods: We selected 37 LMs from 27 patients and analysed clinical features. Each section of 37 LMs and controls was stained with H&E, Tolb, Giemsa, MCT and CD117. Two observers independently counted MCs in 50 high power fields (hpfs) and averaged to MCs/ 10hpfs and mean value was calculated. MC counts for 37 LMs and 26 controls were analysed and compared with each stain. Number of MCs/10hpf stained with CD117 was compared with size of LMs, degenerative change of LMs, status of endometrium and with the control. Results: The mean age of 27 patients was 52.8 years (ranged 31– 69). 11 patients presented with fibroids. 25 of 37 LMs were small (40 mm). CD117 showed higher number of MCs/10hpfs. H&E count was generally low. 50% of controls have higher counts (50/ 10hpfs). 72% of LMs with hyalinisation and 72.7% with atrophic endometrium showed low MC counts (<50/10hpfs). Conclusion: 1. Tolb and Giemsa were better than H&E for MC count. MCT showed staining problems. CD117 was the best stain. 2. No linear relationship found between LMs size and MC counts. 3. MC count was low in LMs with hyaline degeneration and atrophic endometrium. Further studies are required to find whether MCs are inhibitors or promoters of LMs.
2. DOUBLE HIT MANTLE CELL LYMPHOMA WITH BLASTOID MORPHOLOGY: A CASE REPORT Chandra Adhikari, Oana Crainic and Wendy Cooper Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney About 40% of all B-cell lymphomas are characterized by the presence of recurrent reciprocal chromosomal translocations. The term ‘double-hit’ lymphoma is mostly used for mature B cell lymphomas with a chromosomal breakpoint affecting the MYC locus along with another recurrent chromosomal breakpoint. These lymphomas are aggressive and usually show high proliferation rate. Mantle cell lymphoma is a mature B-cell lymphoma characterized by t(11;14) resulting in cyclin D1 over expression. Although rare, a MYC and cyclin D1 double-hit phenomenon can occur in mantle cell lymphoma and is associated with an adverse clinical course and blastoid morphology. We report a case of a 58 years old female with a left maxillary mass histologically showing features of blastoid mantle cell lymphoma. Fluorescence in situ hybridization (FISH) was positive for CCND1 and MYC gene rearrangements. Recognising ‘double-hit’ lymphomas, through use of appropriate investigations is important for
S101
optimal management of patients with these highly aggressive tumours.
3. DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOURLIKE NEOPLASM OF THE SEPTUM PELLUCIDUM: A CASE REPORT B. M. Allanson1, A. Thomas1,2, C. Lind3 and P. Robbins1,2 1Department of Anatomical Pathology PathWest Laboratory Medicine, Queen Elizabeth II Medical Centre, Western Australia, 2School of Pathology and Laboratory Medicine, University of Western Australia, and 3Department of Neurosurgery, Sir Charles Gairdner Hospital, Nedlands, Western Australia Background: Dysembryoplastic Neuroepithelial Tumour-like Neoplasm of the Septum Pellucidum is a rare, low grade CNS neoplasm that may be misdiagnosed as a diffuse glioma. Clinical features: We report a case of a 22 year old woman who presented with a first seizure. She was found to have a 31 mm nonenhancing cystic lesion on the right hand side of the anterior septum pellucidum. Pathological features: The lesion was completely resected and the specimen was received as fragmented pale-white gelatinous pieces of tissue. Histologically the lesion demonstrated a lobular, microcystic, and mucin-rich appearance, and had relatively well defined margins. A population of small uniform cells, often with perinuclear halos, and cytologically virtually indistinguishable from oligodendroglioma predominated. These small ‘oligodendrocyte-like cells’ (OLC) formed somewhat ‘patterned’ architectural arrangements with perivascular pseudorosettes and laminated arrays resembling the specific glioneuronal component of a dysembryoplastic neuroepithelial tumour. Rare individual neurons ‘floating’ in myxoid microcystic spaces were also present. These floating neurons lacked dysplastic features and evidence of satellitosis. Immunohistochemical staining showed mixed glioneuronal differentiation and the MIB1 proliferation index was low (less than 1%). PCR amplification and Sanger direct bidirectional sequencing for IDH1 and IDH2 mutations were negative. Discussion: Less than 20 cases of this rare tumour have been reported to date. DNT-like lesions should be included in differential diagnosis of midline intraventricular tumours in children and young adults. Distinction from diffuse glioma is essential because these tumours appear to behave in a benign fashion.
4. GOBLET CELLS IN NEOPLASTIC BARRETT MUCOSA AND THEIR RELEVANCE IN NEOPLASTIC PROGRESSION: A SEMIQUANTITATIVE ANALYSIS J. Bonavita1, B. M. Allanson1, S. Raftopoulos2 and M. P. Kumarasinghe1 1PathWest Laboratory Medicine, Queen Elizabeth II Medical Centre, and 2Department of Gastroenterology, Sir Charles Gardiner Hospital Background: The trigger for screening of columnar lined mucosa (CLM) of oesophagus is the presence of histologically proven goblet cells (GC). There is some evidence that oesophageal adenocarcinoma (EAC) may arise in CLM without GCs. Pathological data on association of GCs and oesophageal glandular neoplasia is limited.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.