Collision tumor of the colon – colonic adenocarcinoma and ovarian granulosa cell tumor

<p>Abstract</p> <p>Background</p> <p>Collision tumors of the colon are rare. We report the first case, to our knowledge in the English literature, of a collision tumor composed of a colonic adenocarcinoma arising in a sigmoid diverticulum coexisting with a recurrent ovarian granulosa cell tumor.</p> <p>Case presentation</p> <p>A 64-year old woman presented with small bowel obstruction and a large, heterogenous, solid/cystic serosal based pelvic mass consistent with a gastrointestinal stromal tumor on imaging. Her significant past history 16-years ago included a bilateral salpingo-oophrectomy with hysterectomy. Surgical removal of the mass and pathological examination revealed the presence of a colonic adenocarcinoma arising in a large sigmoid diverticulum coexistent with a second neoplastic tumor phenotype; confirmed to be a delayed recurrent ovarian granulosa cell tumor. Though coexistent, the two tumor phenotypes respected their boundaries with no diffuse intermingling or transition between them. She developed lung metastases from the recurrent ovarian tumor within 6 months and died within a year of follow-up.</p> <p>Conclusion</p> <p>Collision tumors of the colon are rare. This is the first case reported of a collision tumor composed of adenocarcinoma colon and recurrent granulosa cell tumor representing an example of two independent tumors in a unique one-on-another collision. Clinical awareness and recognition of such tumors are important as they will dictate appropriate treatment strategies dependent on the individual biological aggressiveness of each of the tumor components. Our report highlights the need for histopathologists, surgeons, and oncologists to be aware of the rare possibility of collisions tumors. As seen in our case, the delayed recurrence of granulosa cell tumor of the ovary sixteen years after the initial presentation was the key determining factor in tumor recurrence, tumor progression, and tumor metastasis within three months, which ultimately lead to accelerated death within a year of clinical presentation. Thus accurate identification and recognition of the second neoplasm is important as prognosis and survival may be determined by this component as seen in our index case.</p

Pseudomyxoma peritonei – a revisit: report of 2 cases and literature review

BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare, chronic, relapsing, diagnostically challenging and poorly understood disease characterized by disseminated mucinous ascites and peritoneal implants. CASE PRESENTATION: We report two cases of PMP that represent the two biological variants of disseminated peritoneal adenomucinosis (DPAM) – the benign variant and the peritoneal mucinous carcinomatosis (PMCA) – the malignant variant, both of which were characterized by multiple relapses and progression of the disease despite aggressive management. CONCLUSION: Even with a better understanding and recent advances in the management of these cases, PMP remains an enigmatic disease with a protracted clinical course characterized by multiple recurrences despite surgery and/or chemotherapy. Recognition of PMP as a delayed consequence years later should alert all surgeons to be extremely vigilant when treating mucinous neoplasms of the appendix, with special care being directed towards adequate excision and thorough debridement at the initial diagnosis

The functional significance of human connexin40 mutations on atrial physiology and propensity to atrial arrhythmia

Objective: To characterise the phenotype of the human connexin40 mutations P88S, G38A, and A96S in rat hearts and primary cardiomyocyte cultures. Background: Patch clamping of transfected non-cardiomyocytes revealed that P88S, G38A, and A96S, identified in patients with atrial fibrillation (AF), impair gap junction conductance. Methods: Left atria of adult Sprague-Dawley rats were transduced in vivo (lentivirus) with wtCx40, P88S, G38A, A96S, or eGFP (n=6 per group). Electrophysiology studies (EPS) were performed prior to and 7 days after transduction. Atria were assessed for connexin expression, mRNA levels, inflammation and fibrosis. Primary cardiomyocyte cultures were also transduced (n=6 per transgene); conduction velocities (CV) and protein expression were assessed at 96hrs. Results: Day7 P wave and induced AF durations were longer in mutant groups compared to wtCx40 controls (p<0.05). Inflammation, fibrosis and heart/body weight ratios were similar. A96S culture CV’s were slower compared to wtCx40. P88S and wtCx40 CV’s were similar but slower compared to eGFP (p<0.01). G38A cultures fibrillated spontaneously and were non-capturable. Immunofluorescence revealed that P88S and G38A reduced native connexin43 myocyte coupling. A96S and wtCx40 appeared to co-localise with membranous conneixn43. Connexin43 mRNA levels were similar between groups. Conclusion: A96S, G38A, and P88S slow conduction and increase propensity for AF

Integration of pathology teaching : students and faculty perceptions

Reports on undergraduate medical education in the recent decade clearly point towards a need for greater integration of content in the medical curriculum. The pedagogy of an integrated curriculum embraces many models of integration, representing a continuum where full integration sits at one end and discipline–based teaching at the other, with many intermediate steps between the two extremes. A vertically integrated curriculum seeks to bridge the preclinical and clinical divide in content by teaching the content concurrently rather than sequentially, but still retaining discipline boundaries. A horizontally integrated curriculum seeks to further break down the distinctions between the basic and clinical sciences, with the early years of the program focusing on the basic sciences and introducing clinical features into the program wherever possible as part of a gradual shift to a more continued collaborative clinical focus. At the College of Medicine, University of Saskatchewan, the overall redesigned curricular program will be phased in over the next four years of the curriculum, with a greater emphasis towards an integrated approach of the teaching and learning of human disease. In the first year, this has led to the creation of a patchwork quilt teaching style, where a cross disciplinary functional system incorporates elements of the traditional basic science components of anatomy, physiology, embryology, and histology, and an introduction of core general pathological concepts in a vertical and horizontal integrated fashion. The main objective of this research, detailed in Chapter 1, was to investigate the advantages and disadvantages of the two models of horizontal and vertical integration of the reorganized structural teaching of pathology through an analysis of the perceptions of medical educators and first- and second-year students in the undergraduate curriculum at the College of Medicine, and based contextually within a theoretical framework of the newly designed medical curriculum. In this context, the literature review in Chapter 2 focused on four major areas that are the underpinnings of the pedagogy of pathology teaching in the undergraduate medical curriculum: (a) integration concepts in relation to medical education; (b) the practice of pathology teaching in the past, present, and future; (c) theories of curricular integration; and (d) its effects on the student learning environment. This resulted in the development of the pre-research conceptual framework for this study. The in-service monitoring research design for this study included a triangulation of research methodologies using multiple data sources, multiple subjects, and multiple data collection techniques using comparative qualitative and quantitative research inquiry techniques. Data collected from the semi-structured interviews of the medical faculty provided not only an understanding of the educators’ perceptions towards the integrated curriculum, but also some insight towards their feelings of respect, power, and identity in this new integrative environment. Personal perceptions of fear, apathy, and stress and perceptions regarding accountability and sustainability of this integrative process were also observed as arising from this educational intervention. Quantitative data analysis collected from the first-year student survey questionnaires derived the following grand mean responses with respect to the vertical integration of pathology teachings: student learning satisfaction with integration (3.6); the learning environment (3.8); student engagement (3.3); and student stress (2.9). The grand mean responses to horizontal integration showed a similar trend: student learning satisfaction (3.7); learning environment (4); student engagement (3.5); and student stress (3). Perceptions of the second-year medical students to horizontal integration of pathology teachings were comparable: student learning satisfaction (3.7); learning environment (4.2); student engagement (3.7); and student stress (3.1). A comparison of first- and second-year medical students showed a significant difference (

Solitary Plasmacytoma of the Parotid Gland with Crystalline Inclusions: A Case Report

BACKGROUND: Solitary extramedullary plasmacytoma of the parotid gland is a rare condition. Intracytoplasmic Crystalline inclusions in the tumor are even rarer and have been reported only once in the parotid gland. CASE PRESENTATION: We report here, a case of plasmacytoma of the parotid gland with intracellular crystalline inclusions in a 73-year-old woman CONCLUSION: Solitary extramedullary plasmacytoma of the parotid gland and crystalline inclusions in the tumor is of rare occurrence. The importance of such a finding with relation to tumor progression, clinical course of the disease or prognosis in general remains to be understood

Incidental thyroid papillary carcinoma in a thyroglossal duct cyst – management dilemmas

AbstractINTRODUCTIONBenign thyroglossal duct tract remnants typically thyroglossal duct cysts, (TDCs) are one of the commonest congenital childhood midline neck masses. Clinical presentation of persistent TDCs in adults is uncommon and the occurrence of incidental primary thyroid papillary carcinoma (TPC) in these cysts is rare.PRESENTATION OF CASEWe report the case of a 32-year-old female with an asymptomatic midline neck mass compatible with a TDC that was excised by Sistrunk's procedure. Histopathological examination revealed an incidental primary intraluminal TPC arising from the wall of the TDC.DISCUSSIONManagement dilemmas regarding the roles for total thyroidectomy, regional lymph node dissection, radioactive iodine, and suppressive thyroxine therapy are reviewed in the context of relevant evidence based literature.CONCLUSIONThe occurrence of incidental TPC in a TDC is rare. Though Sistrunk's procedure is adequate treatment for TDC, based on low, moderate, and high risk stratification, recommendations for further management of incidental TPC in TDC is discussed

Goblet cell carcinoid of the appendix

BACKGROUND: Goblet cell carcinoid (GCC) of the appendix is a rare neoplasm that share histological features of both adenocarcinoma and carcinoid tumor. While its malignant potential remains unclear, GCC's are more aggressive than conventional carcinoid. The clinical presentations of this neoplasm are also varied. This review summarizes the published literature on GCC of the appendix. The focus is on its diagnosis, histopathological aspects, clinical manifestations, and management. METHODS: Published studies in the English language between 1966 to 2004 were identified through Medline keyword search utilizing terms "goblet cell carcinoid," "adenocarcinoid", "mucinous carcinoid" and "crypt cell carcinoma" of the appendix. RESULTS: Based on the review of 57 published papers encompassing nearly 600 diagnosed patients, the mean age of presentation for GCC of the appendix was 58.89 years with equal representation in both males and females. Accurate diagnosis of this neoplasm requires astute observations within an acutely inflamed appendix as this neoplasm has a prominent pattern of submucosal growth and usually lacks the formation of a well-defined tumor mass. The mesoappendix was involved in 21.64% followed by perineural involvement in 2.06%. The most common clinical presentations in order of frequency were acute appendicitis in 22.5%; asymptomatic in 5.4%; non-localized abdominal pain in 5.15% and an appendicular mass in 3.09%. The most common surgical treatment of choice was appendectomy with right hemicolectomy in 34.70% followed by simple appendectomy in 24.57%. Concomitant distant metastasis at diagnosis was present in 11.16% of patients with the ovaries being the most common site in 3.60% followed by disseminated abdominal carcinomatosis in 1.03%. Local lymph node involvement was seen in 8.76% of patients at the time of diagnosis. The reported 5-year survival ranges from 60 % to 84%. GCC's of the appendix remains a neoplasm of unpredictable biological behavior and thus warrants lifelong surveillance for recurrence of the disease upon diagnosis and successful surgical extirpation. CONCLUSION: GCC of the appendix is a rare neoplasm. Due to its wide range of presentation, this tumor should be considered as a possible diagnosis in many varied situations leading to abdominal surgery. Histopathological features such as increased number of Paneth cells, increased amount of mucin secretion and presence of pancreatic polypeptide may predict a more aggressive behavior. The advocated plan of management recommended for patients with tumors that involve the adjacent caecum or with high-grade tumors with histological features such as an increased mitotic rate involve initial appendectomy with completion right hemicolectomy due to the high possibility of local recurrence with intraperitoneal seeding prior to lymph node involvement and a 20% risk of metastatic behavior. In female patients with GCC of the appendix regardless of age, bilateral salpingo-oophorectomy is advocated. In cases with obvious spread of the disease chemotherapy, mostly with 5-FU and leucovorin is advised. Cytoreductive surgery with adjuvant intraperitoneal chemotherapy can offer improved survival in cases with advanced peritoneal dissemination

Uterine Carcinosarcomas (Malignant Mixed Müllerian Tumours): A Review with Special Emphasis on the Controversies in Management

Uterine carcinosarcomas (MMMT—malignant mixed Müllerian tumours) are highly aggressive, rare, biphasic tumours composed of epithelial and mesenchymal elements believed to arise from a monoclonal origin. While hysterectomy with bilateral salpingo-oophorectomy remains the mainstay treatment, high rates of recurrence and metastases suggest a need for lymphadenectomy and postoperative adjuvant treatment. There are no established consensus guidelines for therapeutic patient management. Though well recognized that it improves locoregional control, the role of radiation in improving overall survival outcomes remains undecided. Although various combinations of chemotherapy have been explored, an optimal therapeutic modality is yet to be determined. As overall survival rates have not improved in thirty years, it is suggested that targeted chemotherapy and/or a multimodality approach may yield better outcomes. This paper provides a summary of the aetiopathogenesis of carcinosarcomas (MMMT) limited to the uterus with special emphasis on the controversies in the management of these patients