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Rhabdomyosarcoma arising in testicular teratoma
S64
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Pathology (2014), 46(S1)
1. Gill A. Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia: Review. Pathology 2012; 44: 285–92. 2. Pham TH, Moir C, Thompson GB, et al. Pheochromocytoma and paragnaglioma in children: A review of medical and surgical management at a tertiary care centre. Pediatrics 2006; 118: 1109–17.
Aim: To develop a process for the production, dissemination and implementation of international evidence-based pathology cancer datasets (IPCDS). Methods: The International Collaboration on Cancer Reporting (ICCR) was established in 2011 between the Pathology Colleges and Associations of the USA, UK, Canada and Australia. Cancer datasets from various organisations are harmonised and updated by internationally recognised pathologists and subjected to evidentiary and worldwide review, followed by publication in peer reviewed journals. Key international cancer organisations endorse and participate in the process. Results and Discussion: Four datasets have been published and posted to the ICCR website to date. The International Agency for Research in Cancer (IARC) has partnered with ICCR to synchronise the publication of subsequent ICCR datasets with future WHO Tumour Classification volumes. The ICCR is engaging with organisations involved in tumour staging including the Union for International Cancer Control (UICC) and the American Joint Commission on Cancer (AJCC). The European Society of Pathology (ESP) joined ICCR as a founding member in 2013, bringing over 68 countries and more than one billion people under a common process. Conclusions: The ICCR has developed an efficient process for the production of standardised and evidence-based IPCDS. Engagement with key international cancer and pathology organisations will foster their adoption worldwide.
RHABDOMYOSARCOMA ARISING IN TESTICULAR TERATOMA
LYMPHOMATOID GRANULOMATOSIS PRESENTING AS GRANULOMATOUS ORCHITIS
Amalika Edirisinghe, Geoff Watson and Renee Chan Anatomical Pathology Department, Concord Hospital and Royal Prince Alfred Hospital, Sydney, NSW, Australia
Georgina England1, Lisa Salter2, Sonja Klebe1 and Douglas Henderson1 1Department of Surgical Pathology, Flinders Medical Centre, SA Pathology, Adelaide, SA, and 2Department of Urology, The Canberra Hospital, ACT, Australia
16% (5/30) prevalence of SDH mutation in a retrospective review of the Mayo clinic involving thirty patients (1975–2005). Aim: Ascertain the prevalence of PHEO-PGL due to SDHB germline mutations in a local paediatric population by using IHC. Method: 14 patients were ascertained from the pathology database with pheochromocytoma and or paraganglioma, SDHA and SDHB IHC was performed on FFPE blocks. Results: Three patients had SDHB germline mutations, three had VHL mutation and eight had no genetic studies performed. Of the 14 patients, four had multiple tumours and of these, three showed loss of SDHB staining. All three patients with loss of SDHB by IHC had genetically confirmed germline mutations of SDHB. A novel observation was that three patients with VHL syndrome showed a characteristic weak granular cytoplasmic staining, a pattern distinct from SDHB loss and normal controls. Discussion: (1) We found the prevalence of SDH germline mutations was 28% in children with PHEO-PGL, which is higher than historically identified. (2) In patients with multiple tumours the prevalence was 75% (3/4 patients). (3) We found a distinctive weak granular positive cytoplasmic SDHB staining pattern in tumours with VHL germline mutations. References
Somatic type malignancy arising de novo from testicular germ cell tumours is a rare occurrence, particularly sarcomatous malignancy. This case report is of a late presentation with disseminated rhabdomyosarcoma that had arisen from a large 110 mm diameter left testicular tumour (ignored by the patient for several months). Rhabdomyosarcoma had extensively overgrown background mature teratomatous elements within the primary, while FNA staging from the cervical nodes yielded just rhabdomyosarcoma. The diagnosis of rhabdomyosarcoma was confirmed with immunochemistry and electron microscopy. It highlights an unusual differential for a testicular tumour.
CANCER REPORTING – FROM CLINICAL NEED TO INTERNATIONAL PARTNERSHIPS AND GLOBAL GOALS D. W. Ellis1, B. Chmara2, J. E. Dahlstrom1, J. Dvorak2, L. Hirschowitz3, M. Judge1, A. Kwiatkowski4, J. Srigley5, M. K. Washington2 and M. Wells3,6 1Royal College of Pathologists of Australasia, Sydney, Australia, 2College of American Pathologists, Northfield, United States, 3Royal College of Pathologists, London, United Kingdom, 4Canadian Partnership Against Cancer, Toronto, Canada, 5Canadian Association of Pathology, Toronto, Canada, and 6European Society of Pathology
Background: Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disorder which involves the lungs, skin, central nervous system, liver, spleen and kidneys. Only a single case of testicular LYG has been reported. LYG is an EBV driven process, with demonstration of EBV positive large B cells required for diagnosis. Case presentation: We report a case of a 51-year-old man with a testicular mass. At orchidectomy, a polymorphous lymphoid infiltrate was present incorporating scattered, large, atypical cells with a vaguely granulomatous appearance. A polyclonal B cell population was demonstrated on flow cytometry. The case was reported as a granulomatous orchitis. Five months later he developed leg ulcers and bilateral pulmonary infiltrates. He was diagnosed with LYG and responded to treatment with R-CHOP. Five years later he developed further nodular infiltrates within the lungs and leg ulceration. A wedge biopsy of the lung showed an angiocentric lymphoid infiltrate with numerous large, atypical, CD20 positive B cells showing EBV positivity. Thus, a histological diagnosis of LYG was made. Conclusion: Lymphomatoid granulomatosis is a rare lymphoproliferative disorder, with only a single case report of testicular involvement. It may be considered in the differential diagnosis of granulomatous orchitis or testicular lymphoma.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Pathology (2014), 46(S1)
1. Gill A. Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia: Review. Pathology 2012; 44: 285–92. 2. Pham TH, Moir C, Thompson GB, et al. Pheochromocytoma and paragnaglioma in children: A review of medical and surgical management at a tertiary care centre. Pediatrics 2006; 118: 1109–17.
Aim: To develop a process for the production, dissemination and implementation of international evidence-based pathology cancer datasets (IPCDS). Methods: The International Collaboration on Cancer Reporting (ICCR) was established in 2011 between the Pathology Colleges and Associations of the USA, UK, Canada and Australia. Cancer datasets from various organisations are harmonised and updated by internationally recognised pathologists and subjected to evidentiary and worldwide review, followed by publication in peer reviewed journals. Key international cancer organisations endorse and participate in the process. Results and Discussion: Four datasets have been published and posted to the ICCR website to date. The International Agency for Research in Cancer (IARC) has partnered with ICCR to synchronise the publication of subsequent ICCR datasets with future WHO Tumour Classification volumes. The ICCR is engaging with organisations involved in tumour staging including the Union for International Cancer Control (UICC) and the American Joint Commission on Cancer (AJCC). The European Society of Pathology (ESP) joined ICCR as a founding member in 2013, bringing over 68 countries and more than one billion people under a common process. Conclusions: The ICCR has developed an efficient process for the production of standardised and evidence-based IPCDS. Engagement with key international cancer and pathology organisations will foster their adoption worldwide.
RHABDOMYOSARCOMA ARISING IN TESTICULAR TERATOMA
LYMPHOMATOID GRANULOMATOSIS PRESENTING AS GRANULOMATOUS ORCHITIS
Amalika Edirisinghe, Geoff Watson and Renee Chan Anatomical Pathology Department, Concord Hospital and Royal Prince Alfred Hospital, Sydney, NSW, Australia
Georgina England1, Lisa Salter2, Sonja Klebe1 and Douglas Henderson1 1Department of Surgical Pathology, Flinders Medical Centre, SA Pathology, Adelaide, SA, and 2Department of Urology, The Canberra Hospital, ACT, Australia
16% (5/30) prevalence of SDH mutation in a retrospective review of the Mayo clinic involving thirty patients (1975–2005). Aim: Ascertain the prevalence of PHEO-PGL due to SDHB germline mutations in a local paediatric population by using IHC. Method: 14 patients were ascertained from the pathology database with pheochromocytoma and or paraganglioma, SDHA and SDHB IHC was performed on FFPE blocks. Results: Three patients had SDHB germline mutations, three had VHL mutation and eight had no genetic studies performed. Of the 14 patients, four had multiple tumours and of these, three showed loss of SDHB staining. All three patients with loss of SDHB by IHC had genetically confirmed germline mutations of SDHB. A novel observation was that three patients with VHL syndrome showed a characteristic weak granular cytoplasmic staining, a pattern distinct from SDHB loss and normal controls. Discussion: (1) We found the prevalence of SDH germline mutations was 28% in children with PHEO-PGL, which is higher than historically identified. (2) In patients with multiple tumours the prevalence was 75% (3/4 patients). (3) We found a distinctive weak granular positive cytoplasmic SDHB staining pattern in tumours with VHL germline mutations. References
Somatic type malignancy arising de novo from testicular germ cell tumours is a rare occurrence, particularly sarcomatous malignancy. This case report is of a late presentation with disseminated rhabdomyosarcoma that had arisen from a large 110 mm diameter left testicular tumour (ignored by the patient for several months). Rhabdomyosarcoma had extensively overgrown background mature teratomatous elements within the primary, while FNA staging from the cervical nodes yielded just rhabdomyosarcoma. The diagnosis of rhabdomyosarcoma was confirmed with immunochemistry and electron microscopy. It highlights an unusual differential for a testicular tumour.
CANCER REPORTING – FROM CLINICAL NEED TO INTERNATIONAL PARTNERSHIPS AND GLOBAL GOALS D. W. Ellis1, B. Chmara2, J. E. Dahlstrom1, J. Dvorak2, L. Hirschowitz3, M. Judge1, A. Kwiatkowski4, J. Srigley5, M. K. Washington2 and M. Wells3,6 1Royal College of Pathologists of Australasia, Sydney, Australia, 2College of American Pathologists, Northfield, United States, 3Royal College of Pathologists, London, United Kingdom, 4Canadian Partnership Against Cancer, Toronto, Canada, 5Canadian Association of Pathology, Toronto, Canada, and 6European Society of Pathology
Background: Lymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disorder which involves the lungs, skin, central nervous system, liver, spleen and kidneys. Only a single case of testicular LYG has been reported. LYG is an EBV driven process, with demonstration of EBV positive large B cells required for diagnosis. Case presentation: We report a case of a 51-year-old man with a testicular mass. At orchidectomy, a polymorphous lymphoid infiltrate was present incorporating scattered, large, atypical cells with a vaguely granulomatous appearance. A polyclonal B cell population was demonstrated on flow cytometry. The case was reported as a granulomatous orchitis. Five months later he developed leg ulcers and bilateral pulmonary infiltrates. He was diagnosed with LYG and responded to treatment with R-CHOP. Five years later he developed further nodular infiltrates within the lungs and leg ulceration. A wedge biopsy of the lung showed an angiocentric lymphoid infiltrate with numerous large, atypical, CD20 positive B cells showing EBV positivity. Thus, a histological diagnosis of LYG was made. Conclusion: Lymphomatoid granulomatosis is a rare lymphoproliferative disorder, with only a single case report of testicular involvement. It may be considered in the differential diagnosis of granulomatous orchitis or testicular lymphoma.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.