Successful management of aggressive fibromatosis of the neck using wide surgical excision: a case report

<p>Abstract</p> <p>Introduction</p> <p>Aggressive fibromatosis is a benign tumor, thought to arise from deep musculoaponeurotic structures, rarely found in the head or neck. However, when it does occur in the head and neck region, it tends to be more aggressive and associated with significant morbidity, which may be attributed to the vital vascular, neurological or anatomical structures in close proximity.</p> <p>Case presentation</p> <p>We report the case of a 39-year-old Pakistani man who presented with a two-month history of a lump on the right side of his neck. The mass was excised and histopathological analysis revealed a case of aggressive fibromatosis.</p> <p>Conclusion</p> <p>Due to the rarity of the condition no guidelines are available on the indications and extent of each modality. Due to its aggressive behavior and tendency to invade local structures and recur, a multi-modality management strategy is usually employed. On the basis of this case, we suggest that aggressive surgery is a viable management option and may be successfully used as a single modality treatment.</p

Hemolytic uremic syndrome following the infusion of oxaliplatin: case report

BACKGROUND: Oxaliplatin is a platinum derivative, which is used in the treatment of colorectal cancer. A small number of oxaliplatin-related hemolytic and/or thrombocytopenic reactions have been reported. We present a case of hemolytic-uremic syndrome that developed during the 4(th )cycle of combination chemotherapy with oxaliplatin, 5-fluorouracil and leucovorin. CASE PRESENTATION: A 52-year-old-male was administered chemotherapy with oxaliplatin, 5-fluorouracil and leucovorin for a Duke's stage C colorectal carcinoma. Three cycles of chemotherapy had been administered without complications when, at the beginning of the fourth cycle, the patient developed clinical and laboratory abnormalities consistent with the development of the hemolytic-uremic syndrome. Treatment was discontinued; the patient was managed with monitored IV hydration and loop diuretics, high dose corticosteroids and fresh frozen plasma infusions and recovered completely. CONCLUSION: The hemolytic-uremic syndrome may be a rare complication of oxaliplatin-based chemotherapy. Clinicians need to maintain a high index of suspicion to diagnose and treat this life-threatening adverse event

Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review

Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome

Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

Item does not contain fulltextBACKGROUND: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients. METHODS: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids. RESULTS: Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy. CONCLUSION: For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin

IMMUNOHISTOCHEMICAL EXPRESSION OF PLACENTAL ALKALINE-PHOSPHATASE AND VIMENTIN IN EPITHELIAL OVARIAN NEOPLASMS

The immunohistochemical expression of PLAP and vimentin was assessed in 23 benign, 6 borderline malignant and 31 malignant epithelial ovarian neoplasms. PLAP and vimentin were expressed in some benign (3/23 and 5/23 respectively) and borderline malignant (2/6 for both markers) tumours and they were often expressed in malignant tumours (16/31 and 17/31 respectively). There was a significantly increased expression of PLAP and vimentin in serous cystadenomas and serous carcinomas compared to their mucinous counterparts. Although there was no significant correlation between PLAP expression and histologic grade of carcinomas-there was a trend towards increased expression in more differentiated carcinomas. No correlation was found between vimentin expression and degree of differentiation

THE IMMUNOHISTOCHEMICAL EXPRESSION OF PROLIFERATING CELL NUCLEAR ANTIGEN (PCNA CYCLIN) IN MALIGNANT AND BENIGN EPITHELIAL OVARIAN NEOPLASMS AND CORRELATION WITH PROGNOSIS

Proliferating cell nuclear antigen (PCNA)/cyclin is considered to be a marker of cell proliferation. The aim of this study was to evaluate the expression of PCNA/cyclin in epithelial ovarian neoplasms (EON) as well as the possible correlation with degree of differentiation, tumour stage and overall survival. The material consisted of 34 benign and 40 malignant EON. Positive nuclear staining was detected in 2/34 (6%) of benign and 23/39 (59%) malignant EON (P &lt; 0.001). Most cases in the high proliferation group were diagnosed in advanced clinical stages. There was no difference in overall survival between nuclear PCNA positive and negative patients, as well as the high and the low proliferation group. In conclusion, the role of PCNA as a marker of malignant potential and prognosis in EON merits further investigation

Immunohistochemical expression of p53 protein and proliferating cell nuclear antigen in hepatocellular carcinoma

There is an increased prevalence of p53 mutations in hepatocellular carcinomas (HCCs). A total of 62 HCC samples with adjacent liver tissue were analyzed immunohistochemically for the presence of p53 by two different commercial sources of Pab 1801. Polyclonal antibodies anti-HbsAg and anti-HbcAg were employed for the detection of HBV in the adjacent tissue and PC-10 for the defection of proliferating cell nuclear antigen (PCNA). Positive staining for p53 runs identified In 42% and 55% of the HCC cases using each monoclonal antibody. p53 teas found in 42% of the low grade and 43% of the high grade HCC. In 32% of the HCC cases, p53 was found in the adjacent liver tissue. In 52.6% of the cases with evidence of HBV infection, p53 positive expression was observed. PCNA was detected in 56% of the HCC cases (69% low grade, 57% high grade HCC). Eighty-one percent of the p53 positive tumours expressed PCNA, mostly with a high index. p53 and PCNA were not related to histologic grade. A trend for positive correlation was observed between p53 expression and HBV infection. The defection of p53 in non neoplastic tissue and the absence of a significant correlation between p53 expression and degree of differentiation support the hypothesis that the p53 gene mutation is involved in early stages of hepatocellular carcinogenesis