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6. Extragenital primary malignant mixed mullerian tumour - A case presentation
S108
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Background: Necrotising fasciitis is a severe, rapidly destructive soft tissue infection characterised by widespread fascial necrosis. Its estimated incidence is 3.5 cases per 100,000 persons, with a case-fatality rate of 24%. Traditional microbiological classification includes polymicrobial infections involving anaerobic bacteria and non-serogroup A streptococci (Type I), and Streptococcus pyogenes with or without Staphylococcus species (Type II). Aims: To determine the morphological and microbiological characteristics of necrotising fasciitis in the Northern Territory. Methods: In the Northern Territory between January 2000 and December 2012 there were 84 cases of necrotising fasciitis. Histological diagnosis was made according to the presence of necrosis of the superficial fascia, with or without neutrophilic infiltrates or bacteria, and oedema of the reticular dermis, subcutaneous fat and superficial fascia. Patient demographics and microbiological results were collated. Results and conclusions: The average age of patients with necrotising fasciitis was 48.9 years. There was a male predominance, and Indigenous patients accounted for 49% of cases. Sites affected, in descending frequency, were lower limbs, upper limbs and torso, head/neck and scrotum/perineum. Despite prompt antimicrobial therapy and surgical intervention, 10% of patients required limb amputation and 25% of patients died from their disease, with almost half being Indigenous. Tissue reaction patterns were similar to those described in the literature, initially involving the superficial fascia followed by extension to both superficial and deep structures with disease progression. Interestingly, our data highlights deviation from the traditional microbiological classification. We demonstrated a high prevalence of pure growths of Staphylococcus (20%) and Pseudomonas (4.7%). Remarkably, fungal isolates were also relatively prevalent. Our findings confirm necrotising fasciitis is a devastating infection associated with high morbidity and mortality. Despite morphological similarities of necrotising fasciitis, data from the Northern Territory portrays unique microbiological variation in its causative organisms.
5. DIVERTICULAR DISEASE, COMPLICATIONS RE-VISITED Showan Balta, Jespal Gill and Reena Ramsaroop Dorevitch Pathology/Ballarat Health Services, Vic, Australia Introduction: Filiform polyposis of the colon is an uncommon entity occasionally encountered in patients with inflammatory bowel disease, characterised by multiple worm-like mucosal projections consisting of sub-mucosal cores lined by normal mucosa. Case study: A 60-year-old man presented with signs and symptoms of large bowel obstruction for 7 days. The patient had a CT scan of the abdomen that showed thickening of the sigmoid wall with suspicion of malignancy. Subsequent colonoscopy showed an exophytictumour extending over 5 cm of the distal sigmoid. The biopsy showed only mucosal ulceration with no evidence of malignancy. The patient then underwent anterior resection of the sigmoid colon. The sigmoid colon was carpeted by numerous finger like projections. On histology this showed mucosal prolapse as a result of extensive diverticular disease. Discussion: We discuss the unusual clinical presentation and gross findings of colonic polyposis related to diverticular disease. Only three similar cases have been reported in the English literature since 2010.
Pathology (2014), 46(S1)
6. EXTRAGENITAL PRIMARY MALIGNANT MIXED MULLERIAN TUMOUR – A CASE PRESENTATION J. Bennett and S. Nigam Pathology Queensland – Gold Coast Hospital Laboratory, Qld, Australia Background: Malignant mixed mullerian tumour (MMMT) is an uncommon, often aggressive malignancy of the female reproductive system, typically arising from the uterus. Histologically, this neoplasm exhibits a biphasic tissue pattern with sarcomatous and carcinomatous elements. Whilst this tumour is known to metastasise outside of the reproductive tract, case reports of its occurrence as an extragenital primary malignancy are exceedingly rare. Case: The following case was seen in 2013 at the Gold Coast Hospital, Queensland. A 67-year-old woman presented to the surgical outpatient clinic complaining of several months of increasing abdominal discomfort. She reported a past history of ER/PR negative breast cancer, which was treated with surgery (unilateral mastectomy) and chemotherapy. She had also undergone a total hysterectomy and bilateral salpingo-oophorectomy many years earlier for abnormal per-vaginal bleeding. After further investigation, she was found to have a large intraabdominal mass within her right iliac fossa, adhered to the anterior abdominal wall. The 2319 cm mass was surgically removed. Macroscopic examination revealed a blood-filled cystic lesion with a thickened wall and necrotic internal lining. Microscopic examination showed predominantly sarcomatous tissue composed of spindle cells, with interspersed nests and trabeculae of malignant epithelial cells, together suggestive of carcinosarcoma. Further immunohistochemical staining confirmed this diagnosis. Discussion: Given this woman’s history of a hysterectomy and bilateral salpingo-oophorectomy more than 30 years prior to this presentation, the favoured diagnosis was an extragenital primary MMMT (as opposed to metastatic disease), potentially arising from pre-existing endometriosis, or from mullerian remnants. There are very few case reports of this particular diagnosis, and as such the subsequent paucity of effective treatment options means this malignancy continues to carry an overall poor prognosis.
7. THE GRENZ ZONE IN DERMATOFIBROMA – AN INCONSISTENT TRUTH. A HISTOPATHOLOGICAL STUDY OF 86 CASES J. F. Cernelc, N. T. Harvey and B. A. Wood Division of Tissue Pathology, PathWest Laboratory Medicine, Sir Charles Gairdner Hospital and Faculty of Pathology and Laboratory Medicine, the University of Western Australia, WA, Australia Background: Dermatofibroma is a benign dermal fibrohistocytic lesion with a number of typical diagnostic features, including the presence of an uninvolved zone of the papillary dermis, referred to as a grenz zone. We identified a number of cases in consultation practice in which the absence of a grenz zone led the pathologist to question the diagnosis of dermatofibroma in otherwise typical cases. Aim: To examine the frequency of the presence of a grenz zone in dermatofibroma and to explore the extent to which the presence of this feature can be explained by ‘off-centre’ sectioning of a spheroid proliferation.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
PATHOLOGY 2014 ABSTRACT SUPPLEMENT
Background: Necrotising fasciitis is a severe, rapidly destructive soft tissue infection characterised by widespread fascial necrosis. Its estimated incidence is 3.5 cases per 100,000 persons, with a case-fatality rate of 24%. Traditional microbiological classification includes polymicrobial infections involving anaerobic bacteria and non-serogroup A streptococci (Type I), and Streptococcus pyogenes with or without Staphylococcus species (Type II). Aims: To determine the morphological and microbiological characteristics of necrotising fasciitis in the Northern Territory. Methods: In the Northern Territory between January 2000 and December 2012 there were 84 cases of necrotising fasciitis. Histological diagnosis was made according to the presence of necrosis of the superficial fascia, with or without neutrophilic infiltrates or bacteria, and oedema of the reticular dermis, subcutaneous fat and superficial fascia. Patient demographics and microbiological results were collated. Results and conclusions: The average age of patients with necrotising fasciitis was 48.9 years. There was a male predominance, and Indigenous patients accounted for 49% of cases. Sites affected, in descending frequency, were lower limbs, upper limbs and torso, head/neck and scrotum/perineum. Despite prompt antimicrobial therapy and surgical intervention, 10% of patients required limb amputation and 25% of patients died from their disease, with almost half being Indigenous. Tissue reaction patterns were similar to those described in the literature, initially involving the superficial fascia followed by extension to both superficial and deep structures with disease progression. Interestingly, our data highlights deviation from the traditional microbiological classification. We demonstrated a high prevalence of pure growths of Staphylococcus (20%) and Pseudomonas (4.7%). Remarkably, fungal isolates were also relatively prevalent. Our findings confirm necrotising fasciitis is a devastating infection associated with high morbidity and mortality. Despite morphological similarities of necrotising fasciitis, data from the Northern Territory portrays unique microbiological variation in its causative organisms.
5. DIVERTICULAR DISEASE, COMPLICATIONS RE-VISITED Showan Balta, Jespal Gill and Reena Ramsaroop Dorevitch Pathology/Ballarat Health Services, Vic, Australia Introduction: Filiform polyposis of the colon is an uncommon entity occasionally encountered in patients with inflammatory bowel disease, characterised by multiple worm-like mucosal projections consisting of sub-mucosal cores lined by normal mucosa. Case study: A 60-year-old man presented with signs and symptoms of large bowel obstruction for 7 days. The patient had a CT scan of the abdomen that showed thickening of the sigmoid wall with suspicion of malignancy. Subsequent colonoscopy showed an exophytictumour extending over 5 cm of the distal sigmoid. The biopsy showed only mucosal ulceration with no evidence of malignancy. The patient then underwent anterior resection of the sigmoid colon. The sigmoid colon was carpeted by numerous finger like projections. On histology this showed mucosal prolapse as a result of extensive diverticular disease. Discussion: We discuss the unusual clinical presentation and gross findings of colonic polyposis related to diverticular disease. Only three similar cases have been reported in the English literature since 2010.
Pathology (2014), 46(S1)
6. EXTRAGENITAL PRIMARY MALIGNANT MIXED MULLERIAN TUMOUR – A CASE PRESENTATION J. Bennett and S. Nigam Pathology Queensland – Gold Coast Hospital Laboratory, Qld, Australia Background: Malignant mixed mullerian tumour (MMMT) is an uncommon, often aggressive malignancy of the female reproductive system, typically arising from the uterus. Histologically, this neoplasm exhibits a biphasic tissue pattern with sarcomatous and carcinomatous elements. Whilst this tumour is known to metastasise outside of the reproductive tract, case reports of its occurrence as an extragenital primary malignancy are exceedingly rare. Case: The following case was seen in 2013 at the Gold Coast Hospital, Queensland. A 67-year-old woman presented to the surgical outpatient clinic complaining of several months of increasing abdominal discomfort. She reported a past history of ER/PR negative breast cancer, which was treated with surgery (unilateral mastectomy) and chemotherapy. She had also undergone a total hysterectomy and bilateral salpingo-oophorectomy many years earlier for abnormal per-vaginal bleeding. After further investigation, she was found to have a large intraabdominal mass within her right iliac fossa, adhered to the anterior abdominal wall. The 2319 cm mass was surgically removed. Macroscopic examination revealed a blood-filled cystic lesion with a thickened wall and necrotic internal lining. Microscopic examination showed predominantly sarcomatous tissue composed of spindle cells, with interspersed nests and trabeculae of malignant epithelial cells, together suggestive of carcinosarcoma. Further immunohistochemical staining confirmed this diagnosis. Discussion: Given this woman’s history of a hysterectomy and bilateral salpingo-oophorectomy more than 30 years prior to this presentation, the favoured diagnosis was an extragenital primary MMMT (as opposed to metastatic disease), potentially arising from pre-existing endometriosis, or from mullerian remnants. There are very few case reports of this particular diagnosis, and as such the subsequent paucity of effective treatment options means this malignancy continues to carry an overall poor prognosis.
7. THE GRENZ ZONE IN DERMATOFIBROMA – AN INCONSISTENT TRUTH. A HISTOPATHOLOGICAL STUDY OF 86 CASES J. F. Cernelc, N. T. Harvey and B. A. Wood Division of Tissue Pathology, PathWest Laboratory Medicine, Sir Charles Gairdner Hospital and Faculty of Pathology and Laboratory Medicine, the University of Western Australia, WA, Australia Background: Dermatofibroma is a benign dermal fibrohistocytic lesion with a number of typical diagnostic features, including the presence of an uninvolved zone of the papillary dermis, referred to as a grenz zone. We identified a number of cases in consultation practice in which the absence of a grenz zone led the pathologist to question the diagnosis of dermatofibroma in otherwise typical cases. Aim: To examine the frequency of the presence of a grenz zone in dermatofibroma and to explore the extent to which the presence of this feature can be explained by ‘off-centre’ sectioning of a spheroid proliferation.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.