- Email: [email protected]
28. Tonsilar angioleiomyoma: second reported case
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PATHOLOGY 2015 ABSTRACT SUPPLEMENT
diarrhoea. The latest recurrence in the right gluteus muscle showed a chordoma with prominent rhabdoid and chordoid differentiation with prominent nuclear atypia, vascular invasion and positive margins. However there were still no features of dedifferentiation. References 1. Iorgulescu B, Laufer I, Hammed M, et al. Benign notochordal cell tumours of the spine: natural history of 8 patients with histologically confirmed lesions. Neurosurgery 2013; 73: 411–6. 2. Folpe A, Inwards C. Bone and Soft Tissue Pathology. Philadephia: Saunders, 2010; 408–13. 3. Flanagan AM, Yamaguchi T. Chordoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, editors. WHO Classification of Tumours of the Soft Tissue and Bone. Lyon: IARC, 2013; 328–9. 4. Fletcher CDM, editor. Diagnostic Histopathology of Tumours. 4th ed, Vol 2. New York: Churchill Livingstone, 2013; 1910–1.
25. COMPLETE PENOSCROTAL TRANSPOSITION AND BILATERAL RENAL CYSTIC DYSPLASIA Lauren Kalinowski1,2, Admire Matsika1,2, Sheyna Manawwar1 and David Godbolt1 1Department of Anatomical Pathology, The Prince Charles Hospital, and 2Department of Pathology, University of Queensland Introduction: Complete penoscrotal transposition is a rare male genital malformation where the scrotum is located entirely cephalad to the penis. This malformation is most commonly associated with congenital abnormalities involving the genitourinary system but anomalies of the gastrointestinal, cardiovascular and the central nervous systems have also been described. Case report: We present a case of a twenty-four week old foetus with oligohydramnios, bilateral hydronephrosis, enlarged kidneys, thickened nuchal fold and abnormal limbs on antenatal ultrasound. The pregnancy proceeded to termination based on these features being incompatible with life. At autopsy, the foetus was found to have a complete penoscrotal transposition with hypospadias and urethral meatus stenosis. Both kidneys were markedly enlarged and cystic on macroscopic appearance. Microscopic examination confirmed bilateral renal cystic dysplasia with hydronephrosis. No other congenital abnormalities were identified. Discussion: There are only a small number of cases of complete penoscrotal transposition reported in the literature. An association between genital malformations, including penoscrotal transposition, and distal deletions of the long arm of chromosome 13 have been reported, with this region encoding the EFNB2 gene. A brief review of the literature and genetic associations of penoscrotal transposition ensues.
26. PERSISTENT MICROSPHERE-INDUCED GASTRIC ULCERATION AFTER SELECTIVE INTERNAL RADIATION THERAPY: A CASE REPORT Kais Kasem1,2, Li Ma1, Alfred Lam1,2 and Stephen Weinstein1,2 1Gold Coast University Hospital - Queensland, and 2Griffith Health Institute/Griffith University - Queensland Radioactive microsphere-based selective internal radiation therapy has emerged as an effective therapeutic modality for treatment of primary and secondary malignant liver tumours. However, a number of treatment-related complications have been described. This report describes a case of persistent pre-pyloric gastric ulceration in a 65 year old man with a history of recent selective
Pathology (2015), 47(S1)
internal radiation therapy for metastatic appendiceal adenocarcinoma to the liver. The patient was presented with epigastric abdominal pain after the radiation therapy. Two endoscopic examinations over the course of 4 months revealed a persistent gastric ulcer of maximum dimension of 30 mm in the pre-pyloric region. In both examinations, biopsies from the area showed reactive changes, inflammation and ulceration of surface epithelium. In addition, multiple symmetrical, uniform, round, purple, PAS positive spherules within the gastric mucosa and the lamina propria are identified. Their identical regular appearance suggested foreign material, which when correlated with the clinical history confirmed their nature as radiomicrosphere used to treat hepatic malignancy. Previous cases of persistent microsphere-induced gastrointestinal ulceration have been described with potential life-threatening outcomes. Hence, both gastroenterologists and pathologists must be aware of this important complication, given its characteristic endoscopic and pathognomonic microscopic features.
27. ADRENAL ONCOCYTIC PHEOCHROMOCYTOMA WITH PROBABLE MALIGNANT POTENTIAL: A UNIQUE CASE REPORT Kais Kasem and Alfred Lam Gold Coast University Hospital and Griffith Health Institute, Griffith University - Queensland Oncocytic pheochromocytomas are exceedingly rare tumours. To date, there are 3 reported cases in the literature. We present a case of a 68-year-old man with moderate hypertension and an incidental adrenal mass on radiological investigations. The patient had normal levels of 24-hour urine cortisol, metanephrines and nonmetanephrines. The tumour was subsequently excised. Macroscopic examination revealed a light brown, 45 35 35 mm, 36 g tumour of the adrenal gland. Histologically, the tumour cells showed oncocytic appearance with high-grade nuclear abnormalities and foci of extension into the peri-adrenal fat. Immunohistochemical studies revealed that the tumour was positive for chromogranin, S-100 and p53 and negative for inhibin, HMB45 and EMA. The malignant potential of pheochromocytoma is known to be difficult to predict. Two scoring systems were used; PASS (phaeochromocytoma of adrenal scaled score) by Thompson and a Japanese scoring system by Kimura and colleagues. Both scoring systems predicted the tumour had a malignant potential. However, no recurrence was detected after 5 years of follow-up. In summary, this is the first reported case of oncocytic pheochromocytoma with probable malignant potential.
28. TONSILAR ANGIOLEIOMYOMA: SECOND REPORTED CASE Kais Kasem1,2, Sonu Nigam1,2, David Ryan1 and Leon Kitipornchai1 1Gold Coast University Hospital, and 2Griffith Health Institute Griffith University, Gold Coast A second reported case of an uncommon tumour in a very unusual site is described. Most cases of angioleiomyoma involve the extremities, with a few described in the head and neck region. We report a case of a 33-year-old man who presented with a history of sore throat for 6 months. Throat examination revealed
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
ABSTRACTS
S109
a flesh-coloured mass attached to the lower pole of the right palatine tonsil. The mass presence was then confirmed on CT scan. The surgically excised mass measured 20 mm in largest dimension and had a red-brown spongy cut surface. Histologically, the well-circumscribed mass comprised numerous blood vessels and spindle cell proliferation in a loose stroma with no evidence of atypia or necrosis. The spindle cells have ovoid nuclei with blunt ends and smooth muscle morphology. The cells show diffusely positive reaction for SMA, vimentin and collagen IV, focal positivity for desmin and no reaction with S100, HMB45 and PGP9.5. The overall morphological and immunohistochemical features are consistent with angioleiomyoma. In the literature, there is only one case of angioleiomyoma in the tonsil reported in 2002.
reported ranging from 2% up to 40%. Although it is usually recognized in patients with other systemic involvements, sarcoidosis in the cardiovascular system may occur in isolation as a benign, incidentally discovered condition or a life-threatening disorder causing sudden death. The commonly affected site of the cardiovascular system appears to be myocardium of the left ventricular free wall, followed by the septum, right ventricle, and atria. Aortic sarcoidosis is extremely rare and so far only ten cases have been reported. Here we are presenting a case of sarcoidosis which involved the ascending aorta and resulted in aortic aneurysm. Differential diagnosis of aortic sarcoidosis with other granulomatous aortitis will be discussed.
29. SPINDLE CELL UTERINE LIPOMA: A CASE REPORT
31. GIANT CELL EPENDYMOMA OF THE THIRD VENTRICLE: A CASE REPORT
Lauren Kim and Raghwa Sharma Sydpath, St Vincent’s Hospital, Darlinghurst, Sydney, ICPMR Westmead Hospital, Sydney Lipomas are benign tumours composed of mature white adipocytes. One of the subtypes, spindle cell lipoma are most commonly found in subcutaneous tissue of shoulder, back and posterior neck and most often occurs in men. Although lipomas are the most common mesenchymal and soft tissue tumours, to date, less than 80 cases of uterine lipomas have been reported in the literature. The only case of spindle cell lipoma in the female genital tract have been in the vulva and cervix.1 There has been only one case of uterine lipoma reported in Australia in 1946.2 To the best of our knowledge, this is the first case of spindle cell uterine lipoma reported. We present a case of an 87 year old female presenting with post menopausal bleeding and large bulky uterus. The uterus showed 70 mm yellow intramural mass. Histologically the mass consisted of adipose tissue with variably sized adipocytes with interweaving collagenous fibrous tissue containing pleomorphic spindle cells consistent with features of spindle cell lipoma. Immunohistochemistry for CD34 showed positivity of the spindle cells supporting the diagnosis of spindle cell lipoma. References 1. Zahn CM, Kendall BS, Liang CY. Spindle cell lipoma of the female genital tract. A report of two cases. J Reprod Med 2001; 46: 762–72. 2. Hicks JD. Lipomata of the uterus, with report of a case. Med J Aust 1946; 1: 184.
30. AORTIC SARCOIDOSIS: A CASE REPORT AND LITERATURE REVIEW Lauren Kim and Min Ru Qiu SydPath, St Vincent’s Hospital, Darlinghurst, Sydney NSW, Australia Sarcoidosis is a systemic granulomatous disorder of unclear aetiology. The incidence varies between 3 to 20 per 100,000 in Caucasians and 35.5 to 80 per 100,000 in African-Americans. It is characterized by presence of non-caseating well formed granulomas. The most commonly affected organ is the lung with mediastinal lymph nodes. Approximately up to 30 percent of patients also present with extrathoracic manifestations. The most common sites of extrapulmonary organs include cutaneous, musculoskeletal system, and central nervous system. Cardiovascular system can also be involved, and the incidence of cardiac sarcoidosis has been
Boomi Kwon1 and Clinton Turner1,2 1Anatomical Pathology, LabPlus, Auckland City Hospital, and 2Centre for Brain Research, The University of Auckland, Auckland; Department of Anatomy with Radiology, The University of Auckland, Auckland Ependymomas are central nervous system (CNS) glial tumours thought to be derived from ependymal lining cells in the ventricles or spinal cord. The 2007 WHO classification of CNS tumours recognises three grades of ependymoma and several subtypes.1 Giant cell ependymoma is a rare variant of ependymoma characterised by bizarre tumour giant cells with a variable component of more typical ependymoma. It has been described largely as isolated case reports and small series since its first recognition in 1996.2 Giant cell ependymoma may be either WHO grade II or grade III. It has been described in variety of sites, including: spinal cord, cerebellum, the suprasellar region and cerebral hemispheres. We describe a case of giant cell ependymoma of the third ventricle occurring in a previously healthy 7 year old girl. The morphological features, immunohistochemical findings and ultrastructural features are discussed. As this ependymal subtype is not widely known, it may be confused with several other tumours including: pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, giant cell glioblastoma and atypical teratoid/rhabdoid tumour (ATRT). The differential diagnosis is discussed as confusion with these other entities may have significant treatment and prognostic implications making recognition of this rare ependymoma subtype vital. References 1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. Lyon: IARC, 2007. 2. Zec N, De Girolami U, Schofield DE, et al. Giant cell ependymoma of the filum terminale. A report of two cases. Am J Surg Pathol 1996; 20: 1091– 101.
32. THE SPECTRUM OF PAEDIATRIC LIVER DISEASE SEEN IN A CHILDREN’S HOSPITAL M. Y. Leong KK Women’s and Children’s Hospital, Singapore Background: The spectrum of hepatobiliary disease in infancy and childhood has not been reported in our population. Ours is an 830-bed hospital and academic healthcare institution for women and children in the country and the region.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
PATHOLOGY 2015 ABSTRACT SUPPLEMENT
diarrhoea. The latest recurrence in the right gluteus muscle showed a chordoma with prominent rhabdoid and chordoid differentiation with prominent nuclear atypia, vascular invasion and positive margins. However there were still no features of dedifferentiation. References 1. Iorgulescu B, Laufer I, Hammed M, et al. Benign notochordal cell tumours of the spine: natural history of 8 patients with histologically confirmed lesions. Neurosurgery 2013; 73: 411–6. 2. Folpe A, Inwards C. Bone and Soft Tissue Pathology. Philadephia: Saunders, 2010; 408–13. 3. Flanagan AM, Yamaguchi T. Chordoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, editors. WHO Classification of Tumours of the Soft Tissue and Bone. Lyon: IARC, 2013; 328–9. 4. Fletcher CDM, editor. Diagnostic Histopathology of Tumours. 4th ed, Vol 2. New York: Churchill Livingstone, 2013; 1910–1.
25. COMPLETE PENOSCROTAL TRANSPOSITION AND BILATERAL RENAL CYSTIC DYSPLASIA Lauren Kalinowski1,2, Admire Matsika1,2, Sheyna Manawwar1 and David Godbolt1 1Department of Anatomical Pathology, The Prince Charles Hospital, and 2Department of Pathology, University of Queensland Introduction: Complete penoscrotal transposition is a rare male genital malformation where the scrotum is located entirely cephalad to the penis. This malformation is most commonly associated with congenital abnormalities involving the genitourinary system but anomalies of the gastrointestinal, cardiovascular and the central nervous systems have also been described. Case report: We present a case of a twenty-four week old foetus with oligohydramnios, bilateral hydronephrosis, enlarged kidneys, thickened nuchal fold and abnormal limbs on antenatal ultrasound. The pregnancy proceeded to termination based on these features being incompatible with life. At autopsy, the foetus was found to have a complete penoscrotal transposition with hypospadias and urethral meatus stenosis. Both kidneys were markedly enlarged and cystic on macroscopic appearance. Microscopic examination confirmed bilateral renal cystic dysplasia with hydronephrosis. No other congenital abnormalities were identified. Discussion: There are only a small number of cases of complete penoscrotal transposition reported in the literature. An association between genital malformations, including penoscrotal transposition, and distal deletions of the long arm of chromosome 13 have been reported, with this region encoding the EFNB2 gene. A brief review of the literature and genetic associations of penoscrotal transposition ensues.
26. PERSISTENT MICROSPHERE-INDUCED GASTRIC ULCERATION AFTER SELECTIVE INTERNAL RADIATION THERAPY: A CASE REPORT Kais Kasem1,2, Li Ma1, Alfred Lam1,2 and Stephen Weinstein1,2 1Gold Coast University Hospital - Queensland, and 2Griffith Health Institute/Griffith University - Queensland Radioactive microsphere-based selective internal radiation therapy has emerged as an effective therapeutic modality for treatment of primary and secondary malignant liver tumours. However, a number of treatment-related complications have been described. This report describes a case of persistent pre-pyloric gastric ulceration in a 65 year old man with a history of recent selective
Pathology (2015), 47(S1)
internal radiation therapy for metastatic appendiceal adenocarcinoma to the liver. The patient was presented with epigastric abdominal pain after the radiation therapy. Two endoscopic examinations over the course of 4 months revealed a persistent gastric ulcer of maximum dimension of 30 mm in the pre-pyloric region. In both examinations, biopsies from the area showed reactive changes, inflammation and ulceration of surface epithelium. In addition, multiple symmetrical, uniform, round, purple, PAS positive spherules within the gastric mucosa and the lamina propria are identified. Their identical regular appearance suggested foreign material, which when correlated with the clinical history confirmed their nature as radiomicrosphere used to treat hepatic malignancy. Previous cases of persistent microsphere-induced gastrointestinal ulceration have been described with potential life-threatening outcomes. Hence, both gastroenterologists and pathologists must be aware of this important complication, given its characteristic endoscopic and pathognomonic microscopic features.
27. ADRENAL ONCOCYTIC PHEOCHROMOCYTOMA WITH PROBABLE MALIGNANT POTENTIAL: A UNIQUE CASE REPORT Kais Kasem and Alfred Lam Gold Coast University Hospital and Griffith Health Institute, Griffith University - Queensland Oncocytic pheochromocytomas are exceedingly rare tumours. To date, there are 3 reported cases in the literature. We present a case of a 68-year-old man with moderate hypertension and an incidental adrenal mass on radiological investigations. The patient had normal levels of 24-hour urine cortisol, metanephrines and nonmetanephrines. The tumour was subsequently excised. Macroscopic examination revealed a light brown, 45 35 35 mm, 36 g tumour of the adrenal gland. Histologically, the tumour cells showed oncocytic appearance with high-grade nuclear abnormalities and foci of extension into the peri-adrenal fat. Immunohistochemical studies revealed that the tumour was positive for chromogranin, S-100 and p53 and negative for inhibin, HMB45 and EMA. The malignant potential of pheochromocytoma is known to be difficult to predict. Two scoring systems were used; PASS (phaeochromocytoma of adrenal scaled score) by Thompson and a Japanese scoring system by Kimura and colleagues. Both scoring systems predicted the tumour had a malignant potential. However, no recurrence was detected after 5 years of follow-up. In summary, this is the first reported case of oncocytic pheochromocytoma with probable malignant potential.
28. TONSILAR ANGIOLEIOMYOMA: SECOND REPORTED CASE Kais Kasem1,2, Sonu Nigam1,2, David Ryan1 and Leon Kitipornchai1 1Gold Coast University Hospital, and 2Griffith Health Institute Griffith University, Gold Coast A second reported case of an uncommon tumour in a very unusual site is described. Most cases of angioleiomyoma involve the extremities, with a few described in the head and neck region. We report a case of a 33-year-old man who presented with a history of sore throat for 6 months. Throat examination revealed
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
ABSTRACTS
S109
a flesh-coloured mass attached to the lower pole of the right palatine tonsil. The mass presence was then confirmed on CT scan. The surgically excised mass measured 20 mm in largest dimension and had a red-brown spongy cut surface. Histologically, the well-circumscribed mass comprised numerous blood vessels and spindle cell proliferation in a loose stroma with no evidence of atypia or necrosis. The spindle cells have ovoid nuclei with blunt ends and smooth muscle morphology. The cells show diffusely positive reaction for SMA, vimentin and collagen IV, focal positivity for desmin and no reaction with S100, HMB45 and PGP9.5. The overall morphological and immunohistochemical features are consistent with angioleiomyoma. In the literature, there is only one case of angioleiomyoma in the tonsil reported in 2002.
reported ranging from 2% up to 40%. Although it is usually recognized in patients with other systemic involvements, sarcoidosis in the cardiovascular system may occur in isolation as a benign, incidentally discovered condition or a life-threatening disorder causing sudden death. The commonly affected site of the cardiovascular system appears to be myocardium of the left ventricular free wall, followed by the septum, right ventricle, and atria. Aortic sarcoidosis is extremely rare and so far only ten cases have been reported. Here we are presenting a case of sarcoidosis which involved the ascending aorta and resulted in aortic aneurysm. Differential diagnosis of aortic sarcoidosis with other granulomatous aortitis will be discussed.
29. SPINDLE CELL UTERINE LIPOMA: A CASE REPORT
31. GIANT CELL EPENDYMOMA OF THE THIRD VENTRICLE: A CASE REPORT
Lauren Kim and Raghwa Sharma Sydpath, St Vincent’s Hospital, Darlinghurst, Sydney, ICPMR Westmead Hospital, Sydney Lipomas are benign tumours composed of mature white adipocytes. One of the subtypes, spindle cell lipoma are most commonly found in subcutaneous tissue of shoulder, back and posterior neck and most often occurs in men. Although lipomas are the most common mesenchymal and soft tissue tumours, to date, less than 80 cases of uterine lipomas have been reported in the literature. The only case of spindle cell lipoma in the female genital tract have been in the vulva and cervix.1 There has been only one case of uterine lipoma reported in Australia in 1946.2 To the best of our knowledge, this is the first case of spindle cell uterine lipoma reported. We present a case of an 87 year old female presenting with post menopausal bleeding and large bulky uterus. The uterus showed 70 mm yellow intramural mass. Histologically the mass consisted of adipose tissue with variably sized adipocytes with interweaving collagenous fibrous tissue containing pleomorphic spindle cells consistent with features of spindle cell lipoma. Immunohistochemistry for CD34 showed positivity of the spindle cells supporting the diagnosis of spindle cell lipoma. References 1. Zahn CM, Kendall BS, Liang CY. Spindle cell lipoma of the female genital tract. A report of two cases. J Reprod Med 2001; 46: 762–72. 2. Hicks JD. Lipomata of the uterus, with report of a case. Med J Aust 1946; 1: 184.
30. AORTIC SARCOIDOSIS: A CASE REPORT AND LITERATURE REVIEW Lauren Kim and Min Ru Qiu SydPath, St Vincent’s Hospital, Darlinghurst, Sydney NSW, Australia Sarcoidosis is a systemic granulomatous disorder of unclear aetiology. The incidence varies between 3 to 20 per 100,000 in Caucasians and 35.5 to 80 per 100,000 in African-Americans. It is characterized by presence of non-caseating well formed granulomas. The most commonly affected organ is the lung with mediastinal lymph nodes. Approximately up to 30 percent of patients also present with extrathoracic manifestations. The most common sites of extrapulmonary organs include cutaneous, musculoskeletal system, and central nervous system. Cardiovascular system can also be involved, and the incidence of cardiac sarcoidosis has been
Boomi Kwon1 and Clinton Turner1,2 1Anatomical Pathology, LabPlus, Auckland City Hospital, and 2Centre for Brain Research, The University of Auckland, Auckland; Department of Anatomy with Radiology, The University of Auckland, Auckland Ependymomas are central nervous system (CNS) glial tumours thought to be derived from ependymal lining cells in the ventricles or spinal cord. The 2007 WHO classification of CNS tumours recognises three grades of ependymoma and several subtypes.1 Giant cell ependymoma is a rare variant of ependymoma characterised by bizarre tumour giant cells with a variable component of more typical ependymoma. It has been described largely as isolated case reports and small series since its first recognition in 1996.2 Giant cell ependymoma may be either WHO grade II or grade III. It has been described in variety of sites, including: spinal cord, cerebellum, the suprasellar region and cerebral hemispheres. We describe a case of giant cell ependymoma of the third ventricle occurring in a previously healthy 7 year old girl. The morphological features, immunohistochemical findings and ultrastructural features are discussed. As this ependymal subtype is not widely known, it may be confused with several other tumours including: pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, giant cell glioblastoma and atypical teratoid/rhabdoid tumour (ATRT). The differential diagnosis is discussed as confusion with these other entities may have significant treatment and prognostic implications making recognition of this rare ependymoma subtype vital. References 1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. Lyon: IARC, 2007. 2. Zec N, De Girolami U, Schofield DE, et al. Giant cell ependymoma of the filum terminale. A report of two cases. Am J Surg Pathol 1996; 20: 1091– 101.
32. THE SPECTRUM OF PAEDIATRIC LIVER DISEASE SEEN IN A CHILDREN’S HOSPITAL M. Y. Leong KK Women’s and Children’s Hospital, Singapore Background: The spectrum of hepatobiliary disease in infancy and childhood has not been reported in our population. Ours is an 830-bed hospital and academic healthcare institution for women and children in the country and the region.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.