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Malignant fibrous histiocytoma of salivary gland
ANATOMICAI. PATHOLOGY
MULTlFOCAL GASlRlCAND Sh4ALL lNlESllNAL MUCOSAL AsSOClAlED LYMPHOMA IN A PATIENTwml AIDS. *P. Kostos. H. Preston. R Nonk,Laboratory Medicine Services, CauMeld General Medical Centre, 260 Kooyong Road, Caulfield Vic. 3162. Non-Hodgkln's lymphoma is a common terminal manifestation in AIDS sufferers. Involvement of the gastro-Intestinaltract and liver by tymphoma is relatively uncommon withln this group: 6% of all AIDS patients and up to 16% of the non-Hodgkin's lymphoma group. In May 1991a 47 year old male with AIDS underwent post mortem following a long hlstory of Illness. Terminally the patlent had suffered from intractable " diarrhoea requlrlng total parenteral nutrition. At post mortem there were 25 discrete mucosal tumours of the small intestine and stomach varying In size up to lOmm In diameter. Histology showed these tumours to represent diffuse large cell non-Hodgkin's lymphoma lmmunohlstochemistryshowed features of Bcell lymphoma The liver showed extensive replacement by large nodules of tymphoma, some tumour measuring up to 40mm in diameter. Mesenterlc lymph nodes were also found to be involved by lymphoma. The large Intestine was unramarkable. There was no evidence of lymphoma elsewhere. Presenter: Dr.Phil Kostos, Assistant Pathologist Caulfield General Medical Centre.
5 MALIGNANT FIBROUS HlSnOCYFOMAOF SAWARY GLAND
rHmw 8 . F R C P A and Or.Janell~omwon. MBBS M R C P a Caumeld Oeneral Medlcal Centre, 260 Kooyong Road, Caulfield Wctorla 3162 0
Soft tlssue tumours of all solld organs are uncommon. in the salivary gland soft tlssue tumoure comprlse less than 5% of all prlmary neoplasms with malignant neoplasms representing less than 20% of these (1). The maJOrfty of soft tissue tumoura of the salivary gland are haemangiomas,lymphanglomas, neurllemmomas, neuroflbroma and Ilpomaa The vest majority d mallgnant neoplasms are lymphomas h o k i n g usually the intra-parotid lymph nodes. Malignant soft tissue turnours are extremely rare. A 78 year old male presented to CauMeld General Medlcai Centre with peripheral vascular disease and en passant was noted to have a a painless 20mm diameter swelllng at the lower pole of the rlgM parotid gland. Exclsional biopsy showed a poorly defined hard white tumour m a s replaclng the parotid tissue. Histological examination showed a mallgnant splndie cell tumour with bizarre fom.The tumour was posithe for vlmenth and alpha-I -antitrypsin. Electron microscopy showed features conslstent with cells or ribroblastic and hlstlocytlc orlgln. Malignant fibrous histlocytoma erislng prlmarlly in the salivary gland has been described on 7 prevlous occasions, wlth 6 prevlous cases In the parotid and one In the submandibular gland. The tumour has a marked male predominance (7M:lQ.The leslon occurs between 25 and 78 yeam ol age with a median age of 51 years. The tumour was more common on the right side. Six of the elght patlents have survived more than 2 years since dlagnosls with the longest sunriving patlent alive with skin recurrence at 6 years post-operathre. Presenter:
.
MYOSlTlS OSSlFlCANS IN CHILDHOOD
ck. RW Bvard. Depts of Hirtopathology, Radiology and Surgery, Adelaide Medical Centre for Women end Children, North Adelaide, South Australia, 5006. CL
Myositis ossificans (MO)is a benign, localized, ossifying lesion of soft tissues which may be mistaken for extraskeletal osteosarcoma. Usual locations include the musculature of the upper arm, upper leg, buttocks and hands. The occurrence of this lesion in young children is rarely reported, with the bulk of childhood bone-forming lesions of soft tissue being due to MO progressiva. Following a recent case, a 28 yr search of surgical pathology files from 1964-91 was conducted to determine the prevalence of MO amongst biopsies received at the AMCWC. Only 1 further case was found. The clinicopathological features are detailed below: 1) A 7yo boy presented with a non-tender anterior chest wall mass that had been painlessly enlarging over 6 weeks. Radiological investigations demonstrated a soft tissue lesion with focal ossification of uncertain nature. Histologic examination of the excised 2Ox35x40mm mass revealed a zonal pattern typical of maturing MO with a central loosely cellular region composed of spindled cells surrounded by a rim of well formed woven bone trabeculae lined by osteoblasts. 2) A 7.5 yo boy presented with a tender right quadriceps mass that had been enlarging over 3 wks. Histologic examination of a small wedge biopsy revealed loose fibroblastic stroma with foci of osteoblasts surrounding osteoid and woven bone; compatible with the early stages of MO. No significant atypia was present. Within 1 2 days of presentation X-rays showed the typical features of MO; and followup over 3yrs showed no evidence of further growth or malignancy. There were no other lesions or digital malformations present in either patient to suggest MO progressiva. Neither patient had a history of trauma. These patients demonstrate that M O should be considered in the differential diagnosis of ossifying soft tissue masses in young children. Both radiological and pathological diagnosis may be difficult, particularly in early lesionr where the characteristic zoning pattern has not developed. Although MO progressiva always should be excluded, the most significant differential diagnoses are extraosseous and juxtacortical oswosarcomas.
Dr. H.S.Preston, Chler of Laboratory Mediclne Selvices, CauMeld Oeneral Medical Centre.
PATHOLOGICAL FEATURES OF TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE (TAPVD) IN FOUR CASES OF PAEDIATRIC SUDDEN DEATH. R.W. Byard, L. Moore*, Department of Histopathology, Adelaide Medical Centre for Women & Children, North Adelaide, S.A. 5006. Significant congenital cardiovascular anomalies usually present soon after birth with cyanosis, respiratory distress and failure to thrive. Certain malformations, however, such as TAPVD, may remain undetected until unexpected cardiac arrest occurs. Four cases of sudden collapse and death in the neonatal period and early infancy are presented : 1 ) A 30 hr old girl noted to have mild tachypnoea, which remained unchanged until unexpected cardiac arrest and death occurred 6 hrs later. At autopsy right ventricular hypertrophy (RVH), aortic and left ventricular hypoplasia and a patent ductus arteriosus (PDA) were associated with TAPVD into the left innominate vein. 2) A 5 wk old boy found dead in his cot. At autopsy, RVH, a patent foramen ovale (PFO) and PDA were associated with TAPVD into the superior vena cava. 3) A 7 wk old girl who suddenly collapsed and died after feeding, had a tachycardia 2 days prior to death. At autopsy, RVH and PFO were associated with TAPVD into the hepatic vein. 4) An 8 wk old girl found dead in her car seat had been feeding poorly for 2 weeks. At autopsy, RVH and PFO were associated with TAPVD into the inferior vena cava. These cases demonstrate a potential cause of paediatric sudden death that may be found at autopsy in apparently healthy infants. While those mechanisms responsible for sudden infant death syndrome cannot, on occasion, be definitely excluded, the finding of a vascular malformation with RVH strongly suggests a causal relationship. Clinical symptoms, when present, probably related to the anomaly, but were non-specific in nature and were of no assistance in suggesting the diagnosis prior to autopsy. The importance of careful in sifu dissection of the heart and vessels in all cases of paediatric sudden death is stressed, even in the absence of significant antemortem symptoms and signs. This is particularly so in cases of TAPVD where the range of drainage sites may necessitate dissection above and below the diaphragm.
MULTlFOCAL GASlRlCAND Sh4ALL lNlESllNAL MUCOSAL AsSOClAlED LYMPHOMA IN A PATIENTwml AIDS. *P. Kostos. H. Preston. R Nonk,Laboratory Medicine Services, CauMeld General Medical Centre, 260 Kooyong Road, Caulfield Vic. 3162. Non-Hodgkln's lymphoma is a common terminal manifestation in AIDS sufferers. Involvement of the gastro-Intestinaltract and liver by tymphoma is relatively uncommon withln this group: 6% of all AIDS patients and up to 16% of the non-Hodgkin's lymphoma group. In May 1991a 47 year old male with AIDS underwent post mortem following a long hlstory of Illness. Terminally the patlent had suffered from intractable " diarrhoea requlrlng total parenteral nutrition. At post mortem there were 25 discrete mucosal tumours of the small intestine and stomach varying In size up to lOmm In diameter. Histology showed these tumours to represent diffuse large cell non-Hodgkin's lymphoma lmmunohlstochemistryshowed features of Bcell lymphoma The liver showed extensive replacement by large nodules of tymphoma, some tumour measuring up to 40mm in diameter. Mesenterlc lymph nodes were also found to be involved by lymphoma. The large Intestine was unramarkable. There was no evidence of lymphoma elsewhere. Presenter: Dr.Phil Kostos, Assistant Pathologist Caulfield General Medical Centre.
5 MALIGNANT FIBROUS HlSnOCYFOMAOF SAWARY GLAND
rHmw 8 . F R C P A and Or.Janell~omwon. MBBS M R C P a Caumeld Oeneral Medlcal Centre, 260 Kooyong Road, Caulfield Wctorla 3162 0
Soft tlssue tumours of all solld organs are uncommon. in the salivary gland soft tlssue tumoure comprlse less than 5% of all prlmary neoplasms with malignant neoplasms representing less than 20% of these (1). The maJOrfty of soft tissue tumoura of the salivary gland are haemangiomas,lymphanglomas, neurllemmomas, neuroflbroma and Ilpomaa The vest majority d mallgnant neoplasms are lymphomas h o k i n g usually the intra-parotid lymph nodes. Malignant soft tissue turnours are extremely rare. A 78 year old male presented to CauMeld General Medlcai Centre with peripheral vascular disease and en passant was noted to have a a painless 20mm diameter swelllng at the lower pole of the rlgM parotid gland. Exclsional biopsy showed a poorly defined hard white tumour m a s replaclng the parotid tissue. Histological examination showed a mallgnant splndie cell tumour with bizarre fom.The tumour was posithe for vlmenth and alpha-I -antitrypsin. Electron microscopy showed features conslstent with cells or ribroblastic and hlstlocytlc orlgln. Malignant fibrous histlocytoma erislng prlmarlly in the salivary gland has been described on 7 prevlous occasions, wlth 6 prevlous cases In the parotid and one In the submandibular gland. The tumour has a marked male predominance (7M:lQ.The leslon occurs between 25 and 78 yeam ol age with a median age of 51 years. The tumour was more common on the right side. Six of the elght patlents have survived more than 2 years since dlagnosls with the longest sunriving patlent alive with skin recurrence at 6 years post-operathre. Presenter:
.
MYOSlTlS OSSlFlCANS IN CHILDHOOD
ck. RW Bvard. Depts of Hirtopathology, Radiology and Surgery, Adelaide Medical Centre for Women end Children, North Adelaide, South Australia, 5006. CL
Myositis ossificans (MO)is a benign, localized, ossifying lesion of soft tissues which may be mistaken for extraskeletal osteosarcoma. Usual locations include the musculature of the upper arm, upper leg, buttocks and hands. The occurrence of this lesion in young children is rarely reported, with the bulk of childhood bone-forming lesions of soft tissue being due to MO progressiva. Following a recent case, a 28 yr search of surgical pathology files from 1964-91 was conducted to determine the prevalence of MO amongst biopsies received at the AMCWC. Only 1 further case was found. The clinicopathological features are detailed below: 1) A 7yo boy presented with a non-tender anterior chest wall mass that had been painlessly enlarging over 6 weeks. Radiological investigations demonstrated a soft tissue lesion with focal ossification of uncertain nature. Histologic examination of the excised 2Ox35x40mm mass revealed a zonal pattern typical of maturing MO with a central loosely cellular region composed of spindled cells surrounded by a rim of well formed woven bone trabeculae lined by osteoblasts. 2) A 7.5 yo boy presented with a tender right quadriceps mass that had been enlarging over 3 wks. Histologic examination of a small wedge biopsy revealed loose fibroblastic stroma with foci of osteoblasts surrounding osteoid and woven bone; compatible with the early stages of MO. No significant atypia was present. Within 1 2 days of presentation X-rays showed the typical features of MO; and followup over 3yrs showed no evidence of further growth or malignancy. There were no other lesions or digital malformations present in either patient to suggest MO progressiva. Neither patient had a history of trauma. These patients demonstrate that M O should be considered in the differential diagnosis of ossifying soft tissue masses in young children. Both radiological and pathological diagnosis may be difficult, particularly in early lesionr where the characteristic zoning pattern has not developed. Although MO progressiva always should be excluded, the most significant differential diagnoses are extraosseous and juxtacortical oswosarcomas.
Dr. H.S.Preston, Chler of Laboratory Mediclne Selvices, CauMeld Oeneral Medical Centre.
PATHOLOGICAL FEATURES OF TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE (TAPVD) IN FOUR CASES OF PAEDIATRIC SUDDEN DEATH. R.W. Byard, L. Moore*, Department of Histopathology, Adelaide Medical Centre for Women & Children, North Adelaide, S.A. 5006. Significant congenital cardiovascular anomalies usually present soon after birth with cyanosis, respiratory distress and failure to thrive. Certain malformations, however, such as TAPVD, may remain undetected until unexpected cardiac arrest occurs. Four cases of sudden collapse and death in the neonatal period and early infancy are presented : 1 ) A 30 hr old girl noted to have mild tachypnoea, which remained unchanged until unexpected cardiac arrest and death occurred 6 hrs later. At autopsy right ventricular hypertrophy (RVH), aortic and left ventricular hypoplasia and a patent ductus arteriosus (PDA) were associated with TAPVD into the left innominate vein. 2) A 5 wk old boy found dead in his cot. At autopsy, RVH, a patent foramen ovale (PFO) and PDA were associated with TAPVD into the superior vena cava. 3) A 7 wk old girl who suddenly collapsed and died after feeding, had a tachycardia 2 days prior to death. At autopsy, RVH and PFO were associated with TAPVD into the hepatic vein. 4) An 8 wk old girl found dead in her car seat had been feeding poorly for 2 weeks. At autopsy, RVH and PFO were associated with TAPVD into the inferior vena cava. These cases demonstrate a potential cause of paediatric sudden death that may be found at autopsy in apparently healthy infants. While those mechanisms responsible for sudden infant death syndrome cannot, on occasion, be definitely excluded, the finding of a vascular malformation with RVH strongly suggests a causal relationship. Clinical symptoms, when present, probably related to the anomaly, but were non-specific in nature and were of no assistance in suggesting the diagnosis prior to autopsy. The importance of careful in sifu dissection of the heart and vessels in all cases of paediatric sudden death is stressed, even in the absence of significant antemortem symptoms and signs. This is particularly so in cases of TAPVD where the range of drainage sites may necessitate dissection above and below the diaphragm.