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reference to the current literature and discuss the result with the clinicians. References 1. Haque S, Genta RM. Lymphocytic oesophagitis: clinicopathological aspects of an emerging condition. Gut 2012; 61: 1108–14. 2. Cohen S, Saxena A, Waljee AK, et al. Lymphocytic esophagitis: a diagnosis of increasing frequency. J Clin Gastroenterol 2012; 46: 828–32.
CYSTIC LYMPHANGIOMA OF THE ADRENAL GLAND; REPORT OF A RARE CASE AND REVIEW OF LITERATURE Sanaz Sasani and Alar Enno South Western Area Pathology Service, Department of Anatomical Pathology, Liverpool Hospital, NSW, Australia Adrenal lymphangiomas are rare benign lesions of vascular origin with only limited cases discussed in the literature. They most often remain non-functional and asymptomatic, detected as incidental findings on abdominal imaging studies. We report a 28-year-old female with high body mass index and history of hypertension who was incidentally diagnosed with a large cystic mass superior to the right kidney during investigation for irregular menstruation. Imaging assessment suggested a developmental, post-infective, or post-haemorrhagic cystic change of the adrenal as the most likely causes. The patient underwent elective laparoscopic adrenalectomy and a thin walled, centrally septated, and internally calcified cystic lesion originated from the adrenal gland was surgically excised. The procedure and subsequent recovery was uneventful and she stayed stable post-operatively. The histopathological diagnosis of cystic lymphangioma was established and further supported by focal immunohistochemical expression of lymphatic endothelial markers and positive staining for elastic fibres, confirming the vascular nature of the lesion.
A CASE OF DENOSUMAB-TREATED MULTIFOCAL GIANT CELL TUMOUR – PITFALLS AND COMPLICATIONS Laveniya Satgunaseelan1, Richard Boyle2, Wendy Brown3, Stan McCarthy1, Fiona Bonar4, Fiona Maclean4 and Annabelle Mahar1 1Department of Tissue Pathology and Diagnostic Oncology, 2Institute of Rheumatology and Orthopaedics, 3Department of Radiology, Royal Prince Alfred Hospital, Sydney, and 4Department of Histopathology, Douglass Hanly Moir Pathology, Sydney, NSW, Australia Giant cell tumours account for 4–5% of all primary bone tumours and are usually unifocal. Multifocal giant cell tumour (GCT) is a rare entity, accounting for less than 0.1% of all bone tumours. Denosumab, a RANK-L inhibitor, prevents osteoclast-mediated destruction and is currently being trialled as a possible alternative treatment to surgery. We report a case of a 23-year-old female with multifocal GCT treated with denosumab. The pathological and radiological features prior to, during, and following cessation of treatment with denosumab are described and illustrated. The fibroosseous appearance of treated giant cell tumour, often devoid of osteoclast-type giant cells, is a potential pathological pitfall. Denosumab-treated giant cell tumour can be mistaken for other
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neoplasms, including osteosarcoma, emphasising the importance of clinical history and radiological findings in the interpretation of bone pathology. References 1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC Press, 2013. 2. Branstetter DG, Nelson SD, Manivel JC, et al. Denosumab induces tumor reduction and bone formation in patients with giant-cell tumor of bone. Clin Cancer Res 2012; 18: 4415–24. 3. Chawla S, Henshaw R, Seeger L, et al. Safety and efficacy of denosumab for adults and skeletally mature adolescents with giant cell tumour of bone: interim analysis of an open-label, parallel-group, phase 2 study. Lancet Oncol 2012; 14: 901–8.
HASHIMOTO’S THYROIDITIS MANIFESTING AS IGG4 RELATED DISEASE IN A THYROID REMNANT FOLLOWING TOTAL THYROIDECTOMY FOR GRAVE’S DISEASE M. N. Saxena1 and A. A. Thomas1,2 1Department of Anatomical Pathology, PathWest QEII Medical Centre, Nedlands, and 2School of Pathology and Laboratory Medicine, University Of Western Australia, Crawley, Nedlands, WA, Australia IgG4 related disease (IgG4-RD) is now a widely recognised multiorgan system disease and is an emerging entity characterised by a diffuse or mass forming inflammatory reaction. Recent reports show that it can involve various organs including thyroid and that there is a unique subtype of Hashimoto’s thyroiditis, termed IgG4 thyroiditis, which is histopathologically characterised by lymphoplasmacytic infiltration, fibrosis, increased numbers of IgG4positive plasma cells, and may have high serum IgG4 levels. Furthermore, IgG4 thyroiditis and non-IgG4 thyroiditis present different clinical features, with IgG4 thyroiditis being more prevalent in males, closely associated with rapid progress, subclinical hypothyroidism, higher levels of circulating antibodies, and more diffuse low echogenicity. We report a case of a 46-year-old woman with Hashimoto’s thyroiditis with histopathological features of fibrosing variant of Hashimoto’s thyroiditis and IgG4 related disease in a remnant thyroidectomy, 6 years after total thyroidectomy for Grave’s disease. It is not unusual for Grave’s disease and Hashimoto’s thyroiditis to coexist or follow one another. Awareness of clinico-pathological and possible radiological features of IgG4 related subset of Hashimoto’s thyroiditis would help better diagose this entity, rationalise treatment and avoid surgical options to diagnose and treat this variant.
MASSIVE FIBROELASTOSIS OF GALLBLADDER, A RARE ENTITY – REPORT OF A CASE Adam Scarlett1, Chris Dow2 and Swapna Kamal Sengupta1 1Dorevitch Pathology, Ballarat Base Hospital, Ballarat, and 2Dorevitch Pathology, Western Hospital, Footscray, Vic, Australia Fibroelastosis is a rare entity in the gallbladder, not well described in the literature, for which the aetiology remains uncertain. We report here a case of massive fibroelastosis of gallbladder in association with smouldering cholecystitis and cholelithiasis. The gallbladder showed a 13 mm white nodule at the fundus.
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On histology the nodule was composed of degenerate elastin and collagen, demonstrated with elastic-Van Gieson (EVG) and trichrome stains. Literature search reveals only three cases of gallbladder fibroelastosis.1– 3 The origin of elastosis in such an unusual site is not well understood. Bile leakage into interstitium or local tissue response to cytokines liberated by histiocytes may play a role. References 1. Evert M, Roessner A, Rocken C. Synchronous granulomatous cholecystitis and fibroelastosis of the gallbladder. Virchows Arch 2004; 445: 655–8. 2. Eggleston JF, Goldman RL. Neurofibroma and elastosis of the gallbladder. Report of an unusual case. Am J Gastroenterol 1982; 77: 335–7. 3. Furugaki K, Satoh H, Shinohara M, et al. Benign pseudotumours lesion (fibroangiomatous hyperplasia with elastosis) in the gallbladder. J Gastroenterol 2001; 36: 504–7.
EXTRADURAL VASCULAR LESION OF THE SPINE MIMICKING A NERVE SHEATH TUMOUR Gabriel Scripcaru and Siew-Khin Tang Dorevitch Pathology, Heidelberg, Melbourne, Vic, Australia Benign vascular lesions located in the spinal extradural space are rare, representing up to 4% of all spinal neoplasms. They are often misdiagnosed as nerve sheath tumours or meningiomas, based on their clinical and radiological features. In particular, only two cases of angioleiomyoma in the posterior mediastinum have ever been reported in the literature. We present the case of a 60-year-old man with a 12 month history of lower thoracic back pain radiating around the front of his ribcage to his epigastrium. MRI showed a solid, homogenous left paraspinal mass adjacent to the T11 vertebra, with no evidence of destructive changes or bony infiltration. Based on MRI, CT and FDG-PET studies this lesion was provisionally diagnosed as a benign nerve sheath tumour. He underwent successful surgical resection with remission of symptoms. Histological examination corroborated with immunohistochemistry and special stains revealed a complex vascular lesion which, according to our knowledge has never been described before. Microscopic examination showed a circumscribed nodular lesion with vascular spaces of varying size. There were large muscular arteries with adjacent bundles of smooth muscle in the stroma. Other areas showed ectatic vascular spaces lacking a smooth muscle layer, lined by flattened endothelial cells, in myxoid stroma. These features clearly indicate the presence of an arteriovenous malformation with an extensive cavernous component, as well as a prominent angioleiomyomatous component. Besides highlighting the importance of histopathology in the diagnosis of these rare mimickers and the pitfalls of the differential diagnosis, we believe this case sheds relevant light on the hamartomatous nature of these rare and poorly understood entities. A RARE CASE OF LEIOMYOSARCOMA ARISING FROM THE DARTOS MUSCLE Q. Y. See and C. H. Y. Teo Department of Pathology, Tan Tock Seng Hospital, Singapore Leiomyosarcoma of the scrotum is a rare malignant tumour. Cutaneous and subcutaneous leiomyosarcomas constitute the two subtypes. To date, about 40 cases are reported in the English
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literature. We report a case of cutaneous leiomyosarcoma arising from the dartos muscle in a 64-year-old male. He presented with a pedunculated right scrotal lesion that was gradually increasing in size over the past year. Excision biopsy revealed a malignant smooth muscle tumour which was positive for desmin and caldesmon. Such tumours are often mistaken for a benign lesion because they are generally painless and slow growing. Cutaneous leiomyosarcomas are best treated with wide excision. Long-term follow-up is essential due to the risk of delayed local recurrence and distant metastasis.
FLUORESCENT IN SITU HYBRIDISATION: A NOVEL APPROACH TO SALIVARY GLAND TUMOURS Christina Selinger1, Peter Luk1, Jared Weston2, Jonathan R. Clark3,4, Sandra O’Toole1,4 and Ruta Gupta1,4 1Department of Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, 2University of Notre Dame, 3Head and Neck Surgery, Sydney Head and Neck Cancer Institute and Chris O’Brien Lifehouse at RPA, and 4University of Sydney, Sydney, NSW, Australia Background: Specific translocations have recently been described in salivary gland adenoid cystic carcinoma (AcCa), mucoepidermoid carcinoma (MEC), and hyalinising clear cell carcinoma (HCCCa), and Her2 amplification in most salivary duct carcinomas (SDCa). The diagnostic sensitivity and specificity of rearrangements of MYB in AcCa and of MAML2 in MEC are under investigation with speculations about their prognostic implications. Aim: To evaluate the diagnostic and prognostic utility of these genetic alterations. Methods: Fluorescent in situ hybridisation (FISH) studies using ZytoLight SPEC MYB Break Apart Probe were performed on AcCa (n ¼ 10), ZytoLight SPEC MAML Break Apart Probe on MEC (n ¼ 10), PathVysion HER-2 DNA Probe on SDCa (n ¼ 4) and Vysis LSI EWSR1 Probe on HCCCa (n ¼ 1). Results: AcCas (n ¼ 10) demonstrating a spectrum of histomorphologic and prognostic features including low grade (n ¼ 4), solid high grade (n ¼ 1), dedifferentiated AcCa (n ¼ 1), metastatic to lymph node (n ¼ 2), metastatic to bone (n ¼ 1) and tracheal AcCa (n ¼ 1) were selected. MYB rearrangement was seen in three (30%) of the cases including a case each of bone metastases and extensive infiltration of the underlying skull bone. All the SDCas (n ¼ 4) demonstrated Her2 amplification and the HCCCa demonstrated EWSR1 rearrangement. Conclusions: FISH studies augment the diagnostic and prognostic accuracy in the appropriate morphologic setting.
MET COPY NUMBER IN TRIPLE NEGATIVE BREAST CANCERS Christina I. Selinger1, Rhiannon Beckers1, Thang Tran1, Wendy Cooper1,2,4, Kate Harvey5, Alexander Swarbrick5 and Sandra O’Toole1,2,5 1Department of Anatomical Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Camperdown, 2School of Medicine, University of Western Sydney, Cambelltown, 3Department of Molecular and Clinical Genetics, Royal Prince Alfred Hospital, Camperdown, 4Sydney Medical School, University of Sydney, Camperdown, and 5The Kinghorn Cancer Centre, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia
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