- Email: [email protected]
Composite phaeochromocytoma-ganglioneuroma at autopsy
ABSTRACTS
direct maternal deaths were due to amniotic fluid embolism (n ¼ 7) and obstetric haemorrhage (n ¼ 4). Indirect maternal deaths were mainly due to cardiac disease or non-maternal sepsis. The most common co-incidental deaths were due to motor vehicle accidents and suicide. Conclusion: Maternal deaths are uncommon (average 3 per year); direct maternal deaths outnumbered indirect deaths and amniotic fluid embolism was the most common cause of death in this review. BASAL CELL CARCINOMA. . .SQUAMOUS CELL CARCINOMA. . .OR IS IT A BASOSQUAMOUS CARCINOMA? CASE REPORT AND LITERATURE REVIEW Mila Volchek1, Simon Nazaretian1,2 Monash Medical Centre and 2Monash University, Clayton, Vic, Australia 1
In this report we present a rare case of a nasal skin basosquamous carcinoma in a 68-year-old male diagnosed clinically as a basal cell carcinoma. The histology revealed an infiltrating tumour with a mixed basal and spindle cell pattern. Immunohistochemical (IHC) analysis with BerEP4, EMA, p63 and CK5/6 demonstrated a mixed pattern of staining consistent with a diagnosis of a basosquamous carcinoma (BSC), commonly reported in various studies. The diagnosis of the BSC has clinical and prognostic implications. As a variant of a basal cell carcinoma, BSC has a higher recurrence rate and a risk of metastasis. This tumour is not common and larger studies would be desirable to elicit the tumour’s pathogenesis, confirm immunohistochemical pattern of staining and long term patient prognosis. References Weedon D, LeBoit P, Burg G, Sarasin A, editors. World Health Classification of Tumours. Pathology and Genetics: Skin Tumors. Lyon, IARC Press, 2005. Maloney ML. What is basosquamous carcinoma? Dermatol Surg 2000; 26: 505–6. Bowman PH, Ratz JL, Knoepp TG, et al. Basosquamous carcinoma. Dermatol Surg 2003; 29: 830–3. Martin RCG II. Basosquamous carcinoma – analysis of prognostic factors influencing recurrence. Cancer 2000; 88: 1365–9. Beer TW, Shepherd P, Theaker JM. BerEP4 and epithelial membrane antigen aid distinction of basal cell, squamous cell and basosquamous cell carcinomas of the skin. Histopathology 2000; 37: 218-23.
INTRACRANIAL SUPRATENTORIAL ENTEROGENOUS CYST: CASE REPORT Darina Vuong, Scott Campbell, Thomas Robertson Pathology Queensland and Department of Neurosurgery, Royal Brisbane and Women’s Hospital, Herston, Qld, Australia Enterogenous cysts are infrequent developmental lesions of the central nervous system lined by endoderm-derived epithelium. The majority of these lesions occur in the spinal canal. Intracranial enterogenous cysts are uncommon with posterior fossa being the predominant site. Supratentorial enterogenous cysts are distinctly rare. We present a case of a 20-year-old female patient who
S83
presented with a 1 week history of headaches and left temporal hemianopia. MRI revealed a large extra-axial cystic lesion in the right parieto-occipital region measuring 45 mm in maximal dimension. Histological examination showed an enterogenous cyst lined by ciliated respiratory-type epithelium with an underlying basement membrane that stained with periodic acid-Schiff and type IV collagen. The epithelial cells were immunoreactive to cytokeratin and epithelial membrane antigen. Classification, differential diagnosis and histopathological findings of enterogenous cysts will be discussed. COMPOSITE PHAEOCHROMOCYTOMAGANGLIONEUROMA AT AUTOPSY Lorraine Westacott, Belinda Clarke The Prince Charles Hospital, Brisbane, Qld, Australia Phaeochromocytoma is an uncommon tumour which is well known to be associated with several genetic conditions including neurofibromatosis and von Hippel Lindau syndrome. In this report, we present the findings from an autopsy case in which a patient with neurofibromatosis presented with cardiorespiratory collapse and was subsequently found to have a 570 g composite phaeochromocytoma-ganglioneuroma. On review of clinical history, it is likely that this tumour had been present and biologically active for at least several years. We review the literature on the prevalence and clinical manifestations of composite phaeochromocytoma-ganglioneuromas and present the autopsy findings which superbly illustrate the characteristic sequelae in multiple organ systems arising from longstanding unregulated catecholamine release. A CASE REPORT OF PRIMARY CUTANEOUS EWING’S SARCOMA / PRIMITIVE NEUROECTODERMAL TUMOUR (ES/PNET) Jian Zhong, Adrienne Morey, Richard Scolyer, Graham Windrum St Vincents Hospital, and RPAH, Sydney, NSW, Australia Aim: To review the histological features, differential diagnosis, and the role of molecular testing in confirming the diagnosis of primary cutaneous Ewing’s sarcoma / primitive neuroectodermal tumour (ES/PNET). Result: Primary cutaneous ES/PNET is a rare tumour of proliferation of small, undifferentiated, blue cells which are uniformly immunoreactive for the CD99 cell surface glycoprotein in a charcteristic membranous pattern. The nuclear staining of FLI-1 is positive in some cases. Presence of intracytoplasmic glycogen can be demonstrated by PAS staining. Discussion: Primary ES/PNET arising in skin is distinctly rare with the potential of being misdiagnosed if it is not considered as a possible differential diagnosis. Special stains and immunohistochemstry are helpful. Molecular testing for evidence of the EWS rearrangement is useful ancillary investigation for confirming the diagnosis.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
direct maternal deaths were due to amniotic fluid embolism (n ¼ 7) and obstetric haemorrhage (n ¼ 4). Indirect maternal deaths were mainly due to cardiac disease or non-maternal sepsis. The most common co-incidental deaths were due to motor vehicle accidents and suicide. Conclusion: Maternal deaths are uncommon (average 3 per year); direct maternal deaths outnumbered indirect deaths and amniotic fluid embolism was the most common cause of death in this review. BASAL CELL CARCINOMA. . .SQUAMOUS CELL CARCINOMA. . .OR IS IT A BASOSQUAMOUS CARCINOMA? CASE REPORT AND LITERATURE REVIEW Mila Volchek1, Simon Nazaretian1,2 Monash Medical Centre and 2Monash University, Clayton, Vic, Australia 1
In this report we present a rare case of a nasal skin basosquamous carcinoma in a 68-year-old male diagnosed clinically as a basal cell carcinoma. The histology revealed an infiltrating tumour with a mixed basal and spindle cell pattern. Immunohistochemical (IHC) analysis with BerEP4, EMA, p63 and CK5/6 demonstrated a mixed pattern of staining consistent with a diagnosis of a basosquamous carcinoma (BSC), commonly reported in various studies. The diagnosis of the BSC has clinical and prognostic implications. As a variant of a basal cell carcinoma, BSC has a higher recurrence rate and a risk of metastasis. This tumour is not common and larger studies would be desirable to elicit the tumour’s pathogenesis, confirm immunohistochemical pattern of staining and long term patient prognosis. References Weedon D, LeBoit P, Burg G, Sarasin A, editors. World Health Classification of Tumours. Pathology and Genetics: Skin Tumors. Lyon, IARC Press, 2005. Maloney ML. What is basosquamous carcinoma? Dermatol Surg 2000; 26: 505–6. Bowman PH, Ratz JL, Knoepp TG, et al. Basosquamous carcinoma. Dermatol Surg 2003; 29: 830–3. Martin RCG II. Basosquamous carcinoma – analysis of prognostic factors influencing recurrence. Cancer 2000; 88: 1365–9. Beer TW, Shepherd P, Theaker JM. BerEP4 and epithelial membrane antigen aid distinction of basal cell, squamous cell and basosquamous cell carcinomas of the skin. Histopathology 2000; 37: 218-23.
INTRACRANIAL SUPRATENTORIAL ENTEROGENOUS CYST: CASE REPORT Darina Vuong, Scott Campbell, Thomas Robertson Pathology Queensland and Department of Neurosurgery, Royal Brisbane and Women’s Hospital, Herston, Qld, Australia Enterogenous cysts are infrequent developmental lesions of the central nervous system lined by endoderm-derived epithelium. The majority of these lesions occur in the spinal canal. Intracranial enterogenous cysts are uncommon with posterior fossa being the predominant site. Supratentorial enterogenous cysts are distinctly rare. We present a case of a 20-year-old female patient who
S83
presented with a 1 week history of headaches and left temporal hemianopia. MRI revealed a large extra-axial cystic lesion in the right parieto-occipital region measuring 45 mm in maximal dimension. Histological examination showed an enterogenous cyst lined by ciliated respiratory-type epithelium with an underlying basement membrane that stained with periodic acid-Schiff and type IV collagen. The epithelial cells were immunoreactive to cytokeratin and epithelial membrane antigen. Classification, differential diagnosis and histopathological findings of enterogenous cysts will be discussed. COMPOSITE PHAEOCHROMOCYTOMAGANGLIONEUROMA AT AUTOPSY Lorraine Westacott, Belinda Clarke The Prince Charles Hospital, Brisbane, Qld, Australia Phaeochromocytoma is an uncommon tumour which is well known to be associated with several genetic conditions including neurofibromatosis and von Hippel Lindau syndrome. In this report, we present the findings from an autopsy case in which a patient with neurofibromatosis presented with cardiorespiratory collapse and was subsequently found to have a 570 g composite phaeochromocytoma-ganglioneuroma. On review of clinical history, it is likely that this tumour had been present and biologically active for at least several years. We review the literature on the prevalence and clinical manifestations of composite phaeochromocytoma-ganglioneuromas and present the autopsy findings which superbly illustrate the characteristic sequelae in multiple organ systems arising from longstanding unregulated catecholamine release. A CASE REPORT OF PRIMARY CUTANEOUS EWING’S SARCOMA / PRIMITIVE NEUROECTODERMAL TUMOUR (ES/PNET) Jian Zhong, Adrienne Morey, Richard Scolyer, Graham Windrum St Vincents Hospital, and RPAH, Sydney, NSW, Australia Aim: To review the histological features, differential diagnosis, and the role of molecular testing in confirming the diagnosis of primary cutaneous Ewing’s sarcoma / primitive neuroectodermal tumour (ES/PNET). Result: Primary cutaneous ES/PNET is a rare tumour of proliferation of small, undifferentiated, blue cells which are uniformly immunoreactive for the CD99 cell surface glycoprotein in a charcteristic membranous pattern. The nuclear staining of FLI-1 is positive in some cases. Presence of intracytoplasmic glycogen can be demonstrated by PAS staining. Discussion: Primary ES/PNET arising in skin is distinctly rare with the potential of being misdiagnosed if it is not considered as a possible differential diagnosis. Special stains and immunohistochemstry are helpful. Molecular testing for evidence of the EWS rearrangement is useful ancillary investigation for confirming the diagnosis.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.