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Germinoma of the pineal region - a case report and literature review
ABSTRACTS
the appropriate immunohistochemical stains a diagnosis of angiomyofibroblastoma was made. Angiomyofibroblastoma is a benign soft tissue neoplasm of mesenchymal origin, usually occurring in the female genital tract. Cervical presentation is extremely rare. The histogenesis of angiomyofibroblastoma is unknown and it has been postulated that the neoplastic cells most likely arise from mesenchymal cells in the subepithelial myxoid stromal zone. Some authors have suggested that tamoxifen may be causally related to angiomyofibroblastoma. However, further extensive studies are needed to establish the causal association between tamoxifen and angiomyofibroblastoma.
CUTANEOUS PROTOTHECOSIS C. Bura and S. Chou Department of Tissue Pathology and Diagnostic Oncology ICPMR, Westmead Hospital, Sydney, NSW, Australia Cutaneous protothecosis is a rare indolent condition occurring mostly in immunocompromised patient, caused by members of the genus Prototheca. These organisms are considered to be achlorophyllic algae which are ubiquitous in nature. Risk factors include prolonged steroid use, acquired immunodeficiency syndrome, diabetes mellitus and underlying malignancy. Microscopic findings on tissue biopsy and culture of the skin tissue are required for diagnosis. The incidence of protothecosis is likely to increase, given the ever increasing number of immunocompromised patients. The case is that of a 64-year-old male who presented with 1 week history of superficial ulcers with pinpoint purpura on his fingertips. He had a background history of insulin dependent diabetes mellitus and dermatomyositis, and was on regular prednisolone therapy. Histopathology of the cutaneous biopsy revealed moderate acute inflammation within the dermis and subcutaneous fat. Numerous morula-like structures were apparent in the dermis, singly and in clusters. Characteristic septations were seen with formation of endospores. Periodic acid-Schiff and silver stains highlighted the organisms. With microbiological correlation, diagnosis of protothecosis was made.
OVARIAN MASS AS INITIAL PRESENTATION OF A GASTRIC NEUROENDOCRINE TUMOUR Jeanie Chui1, Jennifer Wang2, Bob Li3, Eugeni Mihaylov4, Nick Pavlakis3 and Anita Mani1 1Anatomical Pathology, Pathology New England, Tamworth Base Hospital, Tamworth, 2Anatomical Pathology, Pacific Laboratory Medical Services, Royal North Shore Hospital, St Leonards, 3Medical Oncology, Royal North Shore Hospital, St Leonards, and 4Gynaecology, Armidale Hospital, Armidale, NSW, Australia A 33-year-old woman presented with lower abdominal discomfort and a left adnexal mass on CT scan. Gross examination of the left ovary revealed an 80 mm solid yellow lobulated tumour with overlying serosal puckering. Histologically, the tumour consists of tubules and nests of epithelioid cells with oval nuclei, fine chromatin and 1 mitosis/10 HPF. A well-differentiated neuroendocrine tumour (NET) of the ovary was the favoured diagnosis since the tumour was positive for CK7, chromogranin A and negative for inhibin and CD99 which excluded the differentials of sex cord stromal and Wolffian tumours.
S61
Subsequent Gallium 68 octreotate PET imaging revealed tumours within the abdomen, pelvis and left breast. Total gastrectomy, partial pancreatectomy, splenectomy, hysterectomy, right oophorectomy and left breast lumpectomy were performed. A 72 mm grade 2 NET was found in the stomach with involvement of the pancreas, 10 of 25 lymph nodes, diaphragm, peritoneum, uterus and breast. The gastric tumour was positive for neuroendocrine markers, negative for CDX2, TTF-1 and pancreatic hormones, with 1 mitosis/10 HPF and Ki-67 index of 14%. The extent of extraovarian peritoneal disease favours a NET of gastric rather than ovarian origin. This case illustrates the challenge in distinguishing between primary and metastatic tumours of the ovary with unusual histology. In cases of NET, further investigations including medical imaging is essential to the work up of the patient.
DISSEMINATED PERITONEAL LEIOMYOMATOSIS DETECTED INCIDENTALLY AT CAESARIAN SECTION Colleen D’Arcy and Frances Petry Department of Anatomical Pathology, The Royal Women’s Hospital, Melbourne, Vic, Australia Disseminated peritoneal leiomyomatosis is a rare condition characterised by scattered benign smooth muscle tumour nodules over the peritoneal surfaces of the abdomen or pelvis. This condition is histologically and clinically benign, however it can macroscopically mimic peritoneal carcinomatosis. Disseminated peritoneal leiomyomatosis is associated with pregnancy and other hyperoestrogenic states, previous laparoscopic surgery and uterine leiomyomata. Disseminated peritoneal leiomyomatosis commonly regresses after pregnancy, but rarely it can undergo malignancy transformation, regress or recur. We describe a case of disseminated peritoneal leiomyomatosis detected incidentally at emergency lower uterine segment caesarian section delivery in a woman with a history of uterine leiomyomata. A partial omentectomy was performed and histological analysis confirmed the diagnosis of disseminated peritoneal leiomyomatosis. Although the exact aetiology of disseminated peritoneal leiomyomatosis is unknown, this report of disseminated peritoneal leiomyomatosis further supports the theory that disseminated peritoneal leiomyomatosis occurs secondary to pregnancy and uterine leiomyomata. Benign leiomyomatosis is rare and should be considered in a pregnant patient with multiple peritoneal nodules and a history of uterine leiomyomata.
GERMINOMA OF THE PINEAL REGION – A CASE REPORT AND LITERATURE REVIEW Morgan R. Davidson and Touraj Taheri Department of Anatomical Pathology, Royal Brisbane and Women’s Hospital, Qld, Australia Intracranial germ cell tumours (GCTs) are uncommon primary CNS tumours which resemble those originating in the ovaries or testes and predominantly arise in the first two decades. This report presents a case of a primary intracranial germinoma arising in the pineal region of a 28-year-old female. The patient presented with a 5 week history of headache and progressive neurological symptoms secondary to obstructive hydrocephalus. MRI demonstrated a large enhancing mass lesion in the region of the pineal gland with
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
S62
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Pathology (2014), 46(S1)
radiological features suggestive of a GCT (germinoma). Histologically, the tumour showed classical features of a pineal germinoma, with medium sized, round, uniform, poorly cohesive tumour cells amongst a fibrotic stroma with infiltrating lymphocytes. Immunohistochemical stains confirmed the histological diagnosis, with positive staining for C-Kit, OCT4 and PLAP. Negative staining was observed for CD30, synaptophysin, AFP, HCG and S100. Cerebrospinal fluid (CSF) cytology demonstrated no evidence of malignancy, with negative CSF and serum tumour markers (AFP and HCG). In summary, this case report presents a pineal germinoma arising in a young adult, use of immunohistochemistry for diagnosis and exclusion of differential diagnoses, with review and discussion of the literature.
Conclusion: Our pathological handling of RSL specimens, developed during the pilot study, are safe and effective and now routine. These techniques will continue in the randomised control study phase.
PATHOLOGICAL HANDLING OF RADIOACTIVE SEED LOCALISATION (RSL) BREAST SPECIMENS: THE WESTERN AUSTRALIAN EXPERIENCE
Introduction: Breast implant-associated anaplastic large cell lymphoma is a histologically malignant neoplasm of CD30 positive T-cells that is classically ALK negative. It arises in association with the fibrous capsule or effusion around the implant. Case reports are increasing and knowledge of its behaviour, prognosis and significance is now becoming clearer. Case report: A bilateral capsulectomy specimen was received from a 56-year-old woman. Notably, the inner surface of the left capsule showed a focal fibrinoid exudate on gross inspection. Histologically it consisted of a hyalinised collagenous substance lined by fibrinoid material containing loose aggregates of anaplastic large cells; some with immunoblastic morphology and others with reniform nuclei and basophilic cytoplasm. The abnormal cells expressed CD4, CD43, CD30, EMA and showed a Ki-67 proliferative index of >60%. They were negative for CD3, CD20, S100 and AE1/AE3. The overall features were diagnostic of implantassociated anaplastic large cell lymphoma. Conclusion: This rare case is presented with a review of what has been elucidated so far in order to raise awareness among working pathologists of its existence, histological features, prognostic factors and recommendations of what to look for and report if it is ever encountered.
B. F. Dessauvagie1, F. Frost1, G. Sterrett1,2, M. Hardie2,3, J. Parry2,3, B. Latham3,4, A. Bourke5, D. Taylor6, R. Kamyab7 and C. Saunders8,9 1Department of Anatomical Pathology, PathWest Laboratory Medicine, QEII Medical Centre, Nedlands, 2School of Pathology and Laboratory Medicine, University of Western Australia, Crawley, 3Department of Anatomical Pathology, PathWest Laboratory Medicine, Royal Perth Hospital, Perth, 4School of Medicine, Notre Dame University, Fremantle, 5Breast Centre, Department of Radiology, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, 6Department of Radiology, Royal Perth Hospital, Perth, 7Department of Surgery, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, 8Department of Surgery, Royal Perth Hospital, Perth, and 9School of Surgery, University of Western Australia, Crawley, WA, Australia Introduction: Mammographic screening has resulted in detection of increased numbers of impalpable in situ and invasive carcinomas that require localisation for surgical excision. Radioactive seed localisation (RSL), locally referred to as radio-guided occult lesion localisation using iodine 125 seeds (ROLLIS), is a novel alternative to hookwire guided techniques, with excellent complete excision rates, that has been the subject of a recent pilot study in Western Australia. Aim: To develop safe and effective procedures for the pathological handling of RSL specimens including seed tracking and retrieval. Methods: The pilot study involved 116 patients (62 at QEII and 54 at RPH). Robust protocols for pathological handling of specimens were developed to minimise risk of seed loss. Radioactive hazard stickers were mandatory for specimen containers and request forms. Seed retrieval was conducted by either immediate retrieval from fresh specimens (QEII) or following overnight formalin fixation (RPH). Once retrieved, seeds were placed into lead containers labelled with radioactive hazard stickers, that were collected from the laboratory by medical physics. All involved personnel received training in radiation safety. Results: Both immediate and delayed retrieval techniques resulted in acquisition of all seeds. Immediate retrieval necessitated re-approximation of a specimen incision to prevent distortion of margins. Delayed retrieval meant the radioactive seed remained within the department for longer. Both techniques have been incorporated into the day-to-day running of the laboratories.
BREAST IMPLANT-ASSOCIATED ANAPLASTIC LARGE CELL LYMPHOMA: A CASE REPORT, LITERATURE REVIEW AND GENERAL ADVICE FOR THE EVERYDAY REPORTING PATHOLOGIST R. Devadas Histology Department, Melbourne Pathology, Collingwood, Vic, Australia
GASTRIC NEUROENDOCRINE TUMOUR OCCURRING SECONDARILY TO A DUODENAL GASTRINOMA IN A PATIENT WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE 1: A CASE EXAMPLE OF TYPE 2 GASTRIC NEUROENDOCRINE TUMOURS R. Devadas Histology Department, Melbourne Pathology, Collingwood, Vic, Australia Introduction: Neuroendocrine tumours (NETs) of the stomach comprise 6% of all gastrointestinal NETs. They can be divided into four subtypes based on the clinical setting in which they arise. Type 1 and 2 NETs arise on a background of hypergastrinaemia and are derived from enterochromaffin-like (ECL) cells. Type 3 and 4 arise in patients with normal serum gastrin levels. Case report: A 68-year-old man with multiple endocrine neoplasia type 1 (MEN-1) underwent an endoscopy for altered bowel habit. Biopsies of a gastric ‘polyp’ were taken along with a ‘lump’ seen in D1. On microscopy the gastric ‘polyp’ showed a 0.8 mm well-differentiated NET within the lamina propria comprised of epithelioid cells with central round nuclei and vesicular chromatin, arranged in small trabeculae and acini. The tumour showed
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
the appropriate immunohistochemical stains a diagnosis of angiomyofibroblastoma was made. Angiomyofibroblastoma is a benign soft tissue neoplasm of mesenchymal origin, usually occurring in the female genital tract. Cervical presentation is extremely rare. The histogenesis of angiomyofibroblastoma is unknown and it has been postulated that the neoplastic cells most likely arise from mesenchymal cells in the subepithelial myxoid stromal zone. Some authors have suggested that tamoxifen may be causally related to angiomyofibroblastoma. However, further extensive studies are needed to establish the causal association between tamoxifen and angiomyofibroblastoma.
CUTANEOUS PROTOTHECOSIS C. Bura and S. Chou Department of Tissue Pathology and Diagnostic Oncology ICPMR, Westmead Hospital, Sydney, NSW, Australia Cutaneous protothecosis is a rare indolent condition occurring mostly in immunocompromised patient, caused by members of the genus Prototheca. These organisms are considered to be achlorophyllic algae which are ubiquitous in nature. Risk factors include prolonged steroid use, acquired immunodeficiency syndrome, diabetes mellitus and underlying malignancy. Microscopic findings on tissue biopsy and culture of the skin tissue are required for diagnosis. The incidence of protothecosis is likely to increase, given the ever increasing number of immunocompromised patients. The case is that of a 64-year-old male who presented with 1 week history of superficial ulcers with pinpoint purpura on his fingertips. He had a background history of insulin dependent diabetes mellitus and dermatomyositis, and was on regular prednisolone therapy. Histopathology of the cutaneous biopsy revealed moderate acute inflammation within the dermis and subcutaneous fat. Numerous morula-like structures were apparent in the dermis, singly and in clusters. Characteristic septations were seen with formation of endospores. Periodic acid-Schiff and silver stains highlighted the organisms. With microbiological correlation, diagnosis of protothecosis was made.
OVARIAN MASS AS INITIAL PRESENTATION OF A GASTRIC NEUROENDOCRINE TUMOUR Jeanie Chui1, Jennifer Wang2, Bob Li3, Eugeni Mihaylov4, Nick Pavlakis3 and Anita Mani1 1Anatomical Pathology, Pathology New England, Tamworth Base Hospital, Tamworth, 2Anatomical Pathology, Pacific Laboratory Medical Services, Royal North Shore Hospital, St Leonards, 3Medical Oncology, Royal North Shore Hospital, St Leonards, and 4Gynaecology, Armidale Hospital, Armidale, NSW, Australia A 33-year-old woman presented with lower abdominal discomfort and a left adnexal mass on CT scan. Gross examination of the left ovary revealed an 80 mm solid yellow lobulated tumour with overlying serosal puckering. Histologically, the tumour consists of tubules and nests of epithelioid cells with oval nuclei, fine chromatin and 1 mitosis/10 HPF. A well-differentiated neuroendocrine tumour (NET) of the ovary was the favoured diagnosis since the tumour was positive for CK7, chromogranin A and negative for inhibin and CD99 which excluded the differentials of sex cord stromal and Wolffian tumours.
S61
Subsequent Gallium 68 octreotate PET imaging revealed tumours within the abdomen, pelvis and left breast. Total gastrectomy, partial pancreatectomy, splenectomy, hysterectomy, right oophorectomy and left breast lumpectomy were performed. A 72 mm grade 2 NET was found in the stomach with involvement of the pancreas, 10 of 25 lymph nodes, diaphragm, peritoneum, uterus and breast. The gastric tumour was positive for neuroendocrine markers, negative for CDX2, TTF-1 and pancreatic hormones, with 1 mitosis/10 HPF and Ki-67 index of 14%. The extent of extraovarian peritoneal disease favours a NET of gastric rather than ovarian origin. This case illustrates the challenge in distinguishing between primary and metastatic tumours of the ovary with unusual histology. In cases of NET, further investigations including medical imaging is essential to the work up of the patient.
DISSEMINATED PERITONEAL LEIOMYOMATOSIS DETECTED INCIDENTALLY AT CAESARIAN SECTION Colleen D’Arcy and Frances Petry Department of Anatomical Pathology, The Royal Women’s Hospital, Melbourne, Vic, Australia Disseminated peritoneal leiomyomatosis is a rare condition characterised by scattered benign smooth muscle tumour nodules over the peritoneal surfaces of the abdomen or pelvis. This condition is histologically and clinically benign, however it can macroscopically mimic peritoneal carcinomatosis. Disseminated peritoneal leiomyomatosis is associated with pregnancy and other hyperoestrogenic states, previous laparoscopic surgery and uterine leiomyomata. Disseminated peritoneal leiomyomatosis commonly regresses after pregnancy, but rarely it can undergo malignancy transformation, regress or recur. We describe a case of disseminated peritoneal leiomyomatosis detected incidentally at emergency lower uterine segment caesarian section delivery in a woman with a history of uterine leiomyomata. A partial omentectomy was performed and histological analysis confirmed the diagnosis of disseminated peritoneal leiomyomatosis. Although the exact aetiology of disseminated peritoneal leiomyomatosis is unknown, this report of disseminated peritoneal leiomyomatosis further supports the theory that disseminated peritoneal leiomyomatosis occurs secondary to pregnancy and uterine leiomyomata. Benign leiomyomatosis is rare and should be considered in a pregnant patient with multiple peritoneal nodules and a history of uterine leiomyomata.
GERMINOMA OF THE PINEAL REGION – A CASE REPORT AND LITERATURE REVIEW Morgan R. Davidson and Touraj Taheri Department of Anatomical Pathology, Royal Brisbane and Women’s Hospital, Qld, Australia Intracranial germ cell tumours (GCTs) are uncommon primary CNS tumours which resemble those originating in the ovaries or testes and predominantly arise in the first two decades. This report presents a case of a primary intracranial germinoma arising in the pineal region of a 28-year-old female. The patient presented with a 5 week history of headache and progressive neurological symptoms secondary to obstructive hydrocephalus. MRI demonstrated a large enhancing mass lesion in the region of the pineal gland with
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
S62
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Pathology (2014), 46(S1)
radiological features suggestive of a GCT (germinoma). Histologically, the tumour showed classical features of a pineal germinoma, with medium sized, round, uniform, poorly cohesive tumour cells amongst a fibrotic stroma with infiltrating lymphocytes. Immunohistochemical stains confirmed the histological diagnosis, with positive staining for C-Kit, OCT4 and PLAP. Negative staining was observed for CD30, synaptophysin, AFP, HCG and S100. Cerebrospinal fluid (CSF) cytology demonstrated no evidence of malignancy, with negative CSF and serum tumour markers (AFP and HCG). In summary, this case report presents a pineal germinoma arising in a young adult, use of immunohistochemistry for diagnosis and exclusion of differential diagnoses, with review and discussion of the literature.
Conclusion: Our pathological handling of RSL specimens, developed during the pilot study, are safe and effective and now routine. These techniques will continue in the randomised control study phase.
PATHOLOGICAL HANDLING OF RADIOACTIVE SEED LOCALISATION (RSL) BREAST SPECIMENS: THE WESTERN AUSTRALIAN EXPERIENCE
Introduction: Breast implant-associated anaplastic large cell lymphoma is a histologically malignant neoplasm of CD30 positive T-cells that is classically ALK negative. It arises in association with the fibrous capsule or effusion around the implant. Case reports are increasing and knowledge of its behaviour, prognosis and significance is now becoming clearer. Case report: A bilateral capsulectomy specimen was received from a 56-year-old woman. Notably, the inner surface of the left capsule showed a focal fibrinoid exudate on gross inspection. Histologically it consisted of a hyalinised collagenous substance lined by fibrinoid material containing loose aggregates of anaplastic large cells; some with immunoblastic morphology and others with reniform nuclei and basophilic cytoplasm. The abnormal cells expressed CD4, CD43, CD30, EMA and showed a Ki-67 proliferative index of >60%. They were negative for CD3, CD20, S100 and AE1/AE3. The overall features were diagnostic of implantassociated anaplastic large cell lymphoma. Conclusion: This rare case is presented with a review of what has been elucidated so far in order to raise awareness among working pathologists of its existence, histological features, prognostic factors and recommendations of what to look for and report if it is ever encountered.
B. F. Dessauvagie1, F. Frost1, G. Sterrett1,2, M. Hardie2,3, J. Parry2,3, B. Latham3,4, A. Bourke5, D. Taylor6, R. Kamyab7 and C. Saunders8,9 1Department of Anatomical Pathology, PathWest Laboratory Medicine, QEII Medical Centre, Nedlands, 2School of Pathology and Laboratory Medicine, University of Western Australia, Crawley, 3Department of Anatomical Pathology, PathWest Laboratory Medicine, Royal Perth Hospital, Perth, 4School of Medicine, Notre Dame University, Fremantle, 5Breast Centre, Department of Radiology, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, 6Department of Radiology, Royal Perth Hospital, Perth, 7Department of Surgery, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, 8Department of Surgery, Royal Perth Hospital, Perth, and 9School of Surgery, University of Western Australia, Crawley, WA, Australia Introduction: Mammographic screening has resulted in detection of increased numbers of impalpable in situ and invasive carcinomas that require localisation for surgical excision. Radioactive seed localisation (RSL), locally referred to as radio-guided occult lesion localisation using iodine 125 seeds (ROLLIS), is a novel alternative to hookwire guided techniques, with excellent complete excision rates, that has been the subject of a recent pilot study in Western Australia. Aim: To develop safe and effective procedures for the pathological handling of RSL specimens including seed tracking and retrieval. Methods: The pilot study involved 116 patients (62 at QEII and 54 at RPH). Robust protocols for pathological handling of specimens were developed to minimise risk of seed loss. Radioactive hazard stickers were mandatory for specimen containers and request forms. Seed retrieval was conducted by either immediate retrieval from fresh specimens (QEII) or following overnight formalin fixation (RPH). Once retrieved, seeds were placed into lead containers labelled with radioactive hazard stickers, that were collected from the laboratory by medical physics. All involved personnel received training in radiation safety. Results: Both immediate and delayed retrieval techniques resulted in acquisition of all seeds. Immediate retrieval necessitated re-approximation of a specimen incision to prevent distortion of margins. Delayed retrieval meant the radioactive seed remained within the department for longer. Both techniques have been incorporated into the day-to-day running of the laboratories.
BREAST IMPLANT-ASSOCIATED ANAPLASTIC LARGE CELL LYMPHOMA: A CASE REPORT, LITERATURE REVIEW AND GENERAL ADVICE FOR THE EVERYDAY REPORTING PATHOLOGIST R. Devadas Histology Department, Melbourne Pathology, Collingwood, Vic, Australia
GASTRIC NEUROENDOCRINE TUMOUR OCCURRING SECONDARILY TO A DUODENAL GASTRINOMA IN A PATIENT WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE 1: A CASE EXAMPLE OF TYPE 2 GASTRIC NEUROENDOCRINE TUMOURS R. Devadas Histology Department, Melbourne Pathology, Collingwood, Vic, Australia Introduction: Neuroendocrine tumours (NETs) of the stomach comprise 6% of all gastrointestinal NETs. They can be divided into four subtypes based on the clinical setting in which they arise. Type 1 and 2 NETs arise on a background of hypergastrinaemia and are derived from enterochromaffin-like (ECL) cells. Type 3 and 4 arise in patients with normal serum gastrin levels. Case report: A 68-year-old man with multiple endocrine neoplasia type 1 (MEN-1) underwent an endoscopy for altered bowel habit. Biopsies of a gastric ‘polyp’ were taken along with a ‘lump’ seen in D1. On microscopy the gastric ‘polyp’ showed a 0.8 mm well-differentiated NET within the lamina propria comprised of epithelioid cells with central round nuclei and vesicular chromatin, arranged in small trabeculae and acini. The tumour showed
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.