Impact of MLL::AF9 Gene Rearrangement on Survival of Acute Myeloid Leukaemia Patients: An Insight into Pakistani Population.

OBJECTIVE: To ascertain the frequency of the MLL::AF9 gene rearrangement and its association with survival in Pakistani patients suffering from acute myeloid leukaemia (AML). STUDY DESIGN: Analytical study. Place and Duration of the Study: Department of Haematology, National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi, Pakistan, from 2015 to 2020. METHODOLOGY: Patients without a history of past AML chemotherapy, aged from 10 to 75 years, were included. Individuals with metastatic cancer, chronic myeloid leukaemia, or other haematological conditions were excluded. Identifying the MLL::AF9 gene involved RNA extraction, cDNA synthesis, and Real-time PCR amplification. The Chi-square test was used to examine the relationship between survival and the MLL::AF9 mutation. A Welch two-sample t-test was used to evaluate survival days depending on the MLL::AF9 gene rearrangement, while ANOVA was used to analyse survival days across various death statuses. RESULTS: The mean age of 130 patients was 36.65 ± 13.01 years, with 64.62% being males. The most common leukaemia type was AML-M2 (n = 32, 24.62%). During the study follow-up, 22.31% were still alive, 40.77% died, and the status of 36.92% were unknown. MLL::AF9 gene rearrangement was present in 11.54%. The group with MLL::AF9 gene rearrangement had significantly longer mean 'survival days' (1,542.33 ± 926.07) compared to the group without the gene rearrangement (206.42 ± 359.57, p <0.001). CONCLUSION: MLL-AF9 mutation was present in 11.54%. Age and MLL::AF9 gene rearrangement were significant predictors of survival in leukaemia patients. KEY WORDS: Acute myeloid leukaemia, MLL::AF9, Gene rearrangement, Survival.

As simple as it sounds? The treatment of simple bone cysts in the proximal femur in children and adolescents: Retrospective multicenter EPOS study of 74 patients.

Purpose: Simple bone cysts are among the most prevalent benign cystic tumor-like lesions in children. Proximal femoral simple bone cysts may require specific treatment because of increased fracture risk. With limited literature available on this specific localization, consensus regarding optimal treatment is lacking. We present a large international multicenter retrospective cohort study on proximal femoral simple bone cysts. Methods: All consecutive pediatric patients with proximal femoral simple bone cyst from 10 tertiary referral centers for musculoskeletal oncology were included (2000-2021). Demographics, primary treatment, complications, and re-operations were evaluated. Primary outcomes were time until full weight-bearing and failure-free survival. Results: Overall, 74 simple bone cyst patients were included (median age 9 years (range = 2-16), 56 (76%) male). Median follow-up was 2.9 years (range = 0.5-21). Index procedure was watchful waiting (n = 6), percutaneous procedure (n = 12), open procedure (n = 50), or osteosynthesis alone (n = 6). Median time until full weight-bearing was 8 weeks (95% confidence interval = 0.1-15.9) for watchful waiting, 9.5 (95% confidence interval = 3.7-15.3) for percutaneous procedure, 11 (95% confidence interval = -0.7 to 13.7) for open procedure, and 6.5 (95% confidence interval = 5.9-16.1) for osteosynthesis alone (p = 0.58). Failure rates were 33%, 58%, 29%, and 0%, respectively (p = 0.069). Overall failure-free survival at 1, 2, and 5 years was 77.8% (95% confidence interval = 68.2-87.4), 69.5% (95% confidence interval = 58.5-80.5), and 62.0% (95% confidence interval = 47.9-76.1), respectively. Conclusion: A preferred treatment for proximal femoral simple bone cysts remains unclear, with comparable failure rates and times until full weight-bearing. Watchful waiting may be successful in certain cases. If not feasible, osteosynthesis alone can be considered. Treatment goals should be cyst control, minimizing complications and swift return to normal activities. Therefore, an individualized balance should be made between undertreatment, with potentially higher complication risks versus overtreatment, resulting in possible larger interventions and accompanying complications. Level of evidence: Level IV, retrospective multicentre study.

Spilanthes acmella Extract-Based Natural Oils Loaded Emulgel for Anti-Microbial Action against Dermatitis.

BACKGROUND: Dermatitis is skin disorder that is complicated by recurrent infections of skin by bacteria, viruses, and fungi. Spilanthol is an active constituent of Spilanthes acmella, which possess strong anti-bacterial properties. The purpose of this study was to develop a herbal emulgel for the treatment of dermal bacterial infections, as microscopic organisms have created solid resistance against anti-microbials. METHODS: Emulgels were prepared and characterized for parameters such as physical examination, rheological studies, spreading coefficient, bio-adhesive strength measurement, extrudability study, antibacterial activity, FTIR analysis, in vitro drug dissolution, and ex vivo permeation studies. RESULT: With a statistically significant p-value = 0.024, 100% antibacterial activity was observed by F4 against Staphylococcus aureus, Pseudomonas aeruginosa, and Escherichia coli (mean ± S.D) (25.33 ± 0.28, 27.33 ± 0.5, and 27 ± 0.5). However, maximum antibacterial effect 100% formulations produced zones of inhibitions against E. colip-value = 0.001. The mean zone of inhibition produced by F4 was greatest among all at 26.44 ± 0.37 mm (mean ± S.D). The F4 formulation produced a maximum percentage dissolution, permeation, and flux of 86.35 ± 0.576, 55.29 ± 0.127%, and 0.5532 ug/cm2/min, respectively. CONCLUSIONS: The present study therefore, suggests the use of S. acmella extract and olive oil containing emulgel for treating bacterial skin infections.

Impact Of Severity Of Covid-19 On Haematological Parameters In Patients Reporting To Saidu Group Of Teaching Hospitals, Swat.

BACKGROUND: In 2019 novel coronavirus was discovered in Wuhan, China and declared pandemic by world health organization. The disease caused by this virus called coronavirus disease 2019 (COVID-19). Among the corona family the actual virus responsible for COVID-19 is Severe Acute Respiratory Syndrome Corona Virus 2 (SARS-CoV-2). Objective of the study was to determine the pattern of blood parameters in corona virus disease (COVID-19) positive cases and the association of these parameters with severity of COVID-19. METHODS: This cross-sectional descriptive study was conducted on 105 participants who were confirmed positive by SARS-CoV-2 through real-time reverse transcriptase PCR, both genders, and Pakistani nationals. The participants who were below 18 years age and missing data were excluded. Haemoglobin (Hb), total leukocyte count (TLC), neutrophil, lymphocyte, monocyte, basophil and eosinophil counts were calculated. Comparison of blood parameters was done among various severity classes of COVID-19 by running one way ANOVA. The level of significance was p≤0.05. RESULTS: The mean age of the participants was 50.6±6.26 years. Males were 78 (74.29%) and females were 27 (25.71%). In critical type COVID-19 the mean haemoglobin was least (10.21±1.07 g/dl) and highest in mild cases (15.76±1.16 g/dl) and these differences were highly statistically significant (p<0.001). TLC was highest in critical COVID cases (15.90±0.51x103 /µl) followed by moderate (12.44±0.65x103/µl). Similarly, neutrophil count was highest in critical (89±2.1) followed by severe (86±1.12). CONCLUSIONS: There is significant decrease in mean haemoglobin level and platelet count but increase in TLC in patients infected from COVID-19.

Selective Neck Dissection for Node-Positive Oral Cavity Squamous Cell Carcinoma: A Retrospective Cohort Study

Abstract Introduction Selective neck dissection inclinically node-negative neckisconsidered the standard of care for oral squamous cell carcinomas (SCCs). Controversy still prevailsinnode-positive disease regarding the extent of neck dissection. In our part of the world, comprehensive neck dissection is mostly considered to be the minimal optimal treatment for palpable neck disease. Objective To compare regional control and disease-specific survival between clinically node-positive and node-negative patients undergoing selective neck dissection for oral SCC. Methods This was a retrospective cohort study conducted in the department of ENT, Head and Neck surgery at a tertiary care hospital. All patients with biopsy-proven oral and lip SCC, with or without nodal disease, who underwent selective neck dissection between April 2006 and July 2015 were included in the study. Results During the study period, 111 patients with oral SCC underwent selective neck dissection, of whom 71 (62%) were clinically node-negative and 40 (38%) patients had clinically positive nodes in the neck. The mean follow-up was 16.62 months (standard deviation [SD]: 17.03). The overall regional control rates were 95 versus 96% for clinical negative versus positive nodes, respectively (p = 0.589). The disease-specific survival was 84.5% in the node negative group versus 82.5% in the node-positive group (p = 0.703). Conclusion Selective neck dissection in node-positive neck oral SCC has similar regional control rates when compared with node-negative neck SCC. The difference in disease-specific survival between the two groups is also not significant.

Selective Neck Dissection for Node-Positive Oral Cavity Squamous Cell Carcinoma: A Retrospective Cohort Study.

Introduction Selective neck dissection in clinically node-negative neck is considered the standard of care for oral squamous cell carcinomas (SCCs). Controversy still prevails in node-positive disease regarding the extent of neck dissection. In our part of the world, comprehensive neck dissection is mostly considered to be the minimal optimal treatment for palpable neck disease. Objective To compare regional control and disease-specific survival between clinically node-positive and node-negative patients undergoing selective neck dissection for oral SCC. Methods This was a retrospective cohort study conducted in the department of ENT, Head and Neck surgery at a tertiary care hospital. All patients with biopsy-proven oral and lip SCC, with or without nodal disease, who underwent selective neck dissection between April 2006 and July 2015 were included in the study. Results During the study period, 111 patients with oral SCC underwent selective neck dissection, of whom 71 (62%) were clinically node-negative and 40 (38%) patients had clinically positive nodes in the neck. The mean follow-up was 16.62 months (standard deviation [SD]: 17.03). The overall regional control rates were 95 versus 96% for clinical negative versus positive nodes, respectively ( p = 0.589). The disease-specific survival was 84.5% in the node negative group versus 82.5% in the node-positive group ( p = 0.703). Conclusion Selective neck dissection in node-positive neck oral SCC has similar regional control rates when compared with node-negative neck SCC. The difference in disease-specific survival between the two groups is also not significant.

Our experience of Mega-prosthesis in bone tumours: A retrospective cross-sectional study in a tertiary care hospital.

OBJECTIVE: To evaluate the oncological and functional outcomes of bone tumour patients who underwent reconstruction with mega prosthesis. METHODOLOGY: A retrospective study was conducted in the department of Orthopaedics Aga Khan University Hospital, Karachi. All the paediatric and adult age group patients diagnosed with malignant, benign and metastatic bone tumours and meeting the inclusion criteria were selected and analysed. Retrospective data was collected from January 2008-January 2018. RESULTS: Sixty-two patients, 30 (48.4%) females and 32 (51.6%) males. were included in the study. Of these 57 (92%) cases had involvement of the lower limb. The mean age was 36.95±19.1 years with a range of 9-81 years. The duration of patients follow up was from 1-124 months (mean 32.7±36.43 months). There were 29 (47%) malignant cases. The most commonly occurring tumour site was distal femur and proximal femur. There were 53 (85%) primary surgeries (first time conducted surgeries) while 9(15%) revision surgeries were done. Major complications were encountered in 19 (30.6%) patients and 13 (20.9%) had minor complications. Post-surgery local recurrence occurred in 2 (3.2%) patients while 7 (11.2%) had distant metastasis. In functional outcomes the mean MSTS score of our patients was 72.09±26.43. The survival rate was 69.8% with 45 patients recovered. CONCLUSIONS: With a good patient selection, adherence to the principles of tumour surgery and an adequate applied knowledge of mega prosthesis insertion, a good functional outcome was achieved.

Treatment and outcomes of soft tissue sarcoma of groin, hip and thigh: a retrospective review from a tertiary care hospital.

OBJECTIVE: To study the frequency of the thigh, hip and groin soft tissue sarcomas and retrospectively analyse the management, treatment results, and outcomes of these uncommon malignant tumours, in a tertiary care hospital of the city of Karachi. METHODOLOGY: Data of soft tissue tumours registered from 2017-2018 was retrieved during January 2019 to March 2019 from Aga Khan University Hospital, Karachi bone and soft tissue tumour registry. A retrospective review was performed and all soft tissue tumour cases treated with surgical intervention (with adjuvant /neoadjuvant therapy) or palliative intention were included. RESULTS: Total 119 cases of soft tissue tumours (STS) were identified out of which 85 were malignant cases (sarcomas) while 30 were benign. On presentation 84 (70.6%) were primary cases. On topographical distribution, there were 25 patients who had hip, groin and thigh sarcoma. Of these, 15 were males and 10 were females. As treatment, neo-adjuvant radiation was done in 4 (16%) patients and adjuvant chemo/radio therapy was given to 13 (52%) patients. Wide margin excision was performed in 19 (76%) patients and 4 (16%) had amputation. Reconstruction was offered to 3 (12%) patients. In post-surgical complications, 1 (4%) patient had wound infection. On final surgical histopathology, majority of the sarcomas were liposarcomas, myxofibrosarcoma, synovial sarcoma and Leiomyosarcoma. Post-surgery recurrence occurred in 7 (28%) patients. Overall survival was 76%. CONCLUSIONS: In treatment of soft tissue sarcoma, limb salvage is an achievable option and survival results are also good.

Our experience of treating adult bone lymphoma, A retrospective cross-sectional study in a tertiary care center, Aga Khan University Hospital, Karachi.

OBJECTIVE: To determine the experience at the Aga Khan University Hospital in diagnosing and treating adult patients with primary lymphoma of bone. METHODOLOGY: All patients with Primary lymphoma of bone (PLB) that were diagnosed and/or treated at Aga Khan University Hospital, Karachi from 2005 to 2019 were included as part of this study. RESULTS: There were 17 patients with PLB including 13 (76.5%) males and 4 (23.5%) females with a mean age of 44 ± 16.5 years. Nine patients were between 30-59 years of age at diagnosis. The mean follow-up time of patients was 80±46.7 months. Six patients had tumours of pelvic bone followed by tibia (5) and femur (4). Four patients had a pathological fracture at the time of presentation whereas 2 (11.8%) required surgical fixation of the pathological fracture. The stage of the tumour was based on Ann Arbor classification. Nine (52.9%) cases had Stage 1 disease, 7 (41.2%) had stage IV disease with metastasis in extra nodal tissues. As for treatment, every patient received chemotherapy whereas 5 (29.4%) received adjuvant radiotherapy. Complete remission in the size of the tumour was seen in 11 (64.7%) patients while 6 (35.3%) had partial remission. Post-treatment, 4 (23.5%) patients expired. The mean Overall Survival (OS) time was 80.18 ± 46.71months with a survival rate of 76.5. CONCLUSIONS: Primary lymphoma of the bone can be treated with medical regime and good prophylactic surgeries to avoid pathological fracture such as intramedullary nailing.

Functional outcomes and complications of total hip arthroplasty with dual mobility cup : an audit.

OBJECTIVE: To determine the functional outcomes in total hip arthroplasty with a dual mobility cup, performed in our hospital. METHODS: After receiving an exemption from the Ethics review committee of the hospital, data collection for audit was started in January 2019. Records from July 2016 to June 2018 were included. All patients who underwent total hip arthroplasty with dual mobility prosthesis without any age limit were included. A proforma was prepared to collect the required information. Data was entered and analyzed on SPSS v. 21. RESULTS: Two hundred and ten patients were included, 114 females and 96 males. Of the total, 188 patients underwent unilateral surgery while 22 had bilateral hip arthroplasty. The mean postoperative hospital stay was 5.91±3.9 days. . Mean pre-op Harris score was 33.7±7.6 and the post-op mean score was 75.9± 5.34. Eighty-three (39.5 %) patients had the neck of femur fracture, 31(14.8%) had osteoarthritis while 28(13.3%) had avascular necrosis. Post-surgery complications included, wound infection, surgical site haematoma, NSTEMI, and only one patient reported dislocation after use of dual mobility cup. CONCLUSIONS: The dislocation rate which was the prime concern, has been reduced with the use of dual mobility implant in total hip arthroplasty patients.

Long-term functional outcomes after total scapulectomy with dual suspension reconstruction in children - A case series.

Implant reconstruction following scapulectomy in children is a challenging task. Dual suspension reconstruction may offer an alternative but there is a dearth of literature on functional outcomes following this procedure for malignant tumours in children. A retrospective study was conducted at the Aga Khan University Hospital, a tertiary care centre in Karachi, Pakistan. Children with malignant tumours of the scapula who underwent total scapulectomy with dual suspension reconstruction (n=5) between Jan 2009 and June 2015 were included. Mean follow up was 50±13.39 months. There were four boys and one girl having mean age of 11±3.57 years. All patients were Enneking Stage IIB with 4 patients diagnosed as Ewing's Sarcoma and 1 as osteosarcoma. The MSTS scores ranged from 20-25 points, with a median of 23. One patient developed postoperative surgical site infection requiring surgical debridement whereas all patients remained disease-free till last follow up. Our findings suggest that scapulectomy with dual suspension reconstruction achieves satisfactory functional results with low rate of complications.

Hip replacement for femur neck fracture in the elderly, effects of delayed surgical intervention on morbidity and mortality, a retrospective comparative study.

Hip fracture is one of the most common injuries in the elderly population. Delay in operating on patients with hip fracture is associated with greater mortality and morbidity. A retrospective review of medical charts of patients who underwent primary total hip replacement (THR) for neck of femur fractures at our tertiary care level 1 trauma was carried out. Data was collected from the patients' charts and analysed for 30-day mortality and morbidity. A total of 96 patients were included in the study. Out of the 36 patients in the delayed THR group, mortality within 30 days was observed in 4 (11.1%) patients while none was noted in the early THR group. The difference was statistically significant with a P-value of 0.008. With regards to post-operative complications, significantly higher percentage of patients were noted to have developed electrolyte imbalances (P = 0.003), postoperative psychosis (P = 0.02), and acute kidney injury (AKI) (P = 0.02) in the delayed THR group compared to the early THR group. Delay in surgery for neck of femur fracture is associated with increased 30-day mortality and postoperative complications.

Pistacia integerrima alleviated Bisphenol A induced toxicity through Ubc13/p53 signalling.

Exposure to environmental toxicants such as Bisphenol A (BPA) has raised serious health issues globally particularly in developing countries. It is ubiquitously used in the manufacturing of canned food and feeding bottles. BPA generated reactive oxygen species can lead to several diseases including cardiotoxicity. However, the endpoints stimulated in BPA cardiotoxicity yet need to be investigated. The current study was aimed to investigate the underlying molecular pathways which may contribute in revealing the protective effects of Pistacia integerrima against BPA induced oxidative stress. The dose of 100 µg/kg BW of BPA, 200 mg/kg BW P. integerrima, and 4 mg/kg BW melatonin was administered to Sprague Dawley rats. Present results of western blotting and qRT-PCR showed the increased expression of p53, PUMA and Drp1, while downregulation of Ubc13 in heart tissues of BPA treated group whereas the levels were reversed upon treatment with P. integerrima. The role of BPA in heart tissue apoptosis was further confirmed by the increased level of P-p53, cytochrome C and disrupted cellular architecture whereas the P. integerrima has shown its ameliorative potential by mitigating the adverse effects of BPA. Moreover, the oxidant, antioxidant, lipid, and liver markers profile has also revealed the therapeutic potential of P. integerrima by maintaining the levels in the normal range. However, melatonin has also manifested the normalized expression of apoptotic markers, biochemical markers, and tissue architecture. Conclusively, the data suggest that P. integerrima may be a potential candidate for the treatment of BPA induced toxicity by neutralizing the oxidative stress through Ubc13/p53 pathway.

Spectrum of histological features of Denosumab treated Giant Cell Tumor of Bone; potential pitfalls and diagnostic challenges for pathologists.

OBJECTIVES: Denosumab is Receptor Activator of Nuclear factor Kappa-B Ligand (RANKL) inhibitor which is being used in the treatment of locally advanced, recurrent and metastatic Giant Cell Tumor of Bone (GCTB). It causes reduction in monocyte recruitment and Osteoclast-Like Giant Cell (OLGC) formation which limits bone destruction. After Denosumab treatment, GCTB exhibit diverse morphological features which can pose diagnostic challenge. Our aim was to study the spectrum of histologic features seen in Denosumab treated GCTB which could be helpful in establishing correct diagnosis. METHODS: We retrieved and reviewed H&E stained microscopic glass slides of 38 GCTB cases who received Denosumab as neoadjuvant treatment. These cases were treated at different institutes and diagnosed at our institute between January 2017 and October 2019. Morphologic features such as presence of residual OLGC, appearances of mononuclear stromal and bony components were assessed along with other non-specific features. RESULTS: Patients' median age was 29 years. Male to female ratio was 1.53:1. Femur was the most commonly involved bone. Microscopically, peripheral shell of reactive bone was observed in all cases. In 20 (52.6%) cases, there was complete elimination of OLGC. Mononuclear stromal cells were predominantly bland spindle shaped and arranged in fascicular and storiform patterns. Focal atypia was noted in 3 cases. Bony component manifested as trabeculae of woven bone with osteoblastic rimming and immature trabeculae of unmineralized osteoid with haphazardly present osteoblasts. Spectrum of stromal changes included cystic spaces, foamy macrophages, inflammatory infiltrate, hemangiopericytoma-like (HPC-like) vessels, hyalinization, edematous areas and hemosiderin pigment. The tumors showed areas which resembled other bony and soft tissue lesions such non-ossifying fibroma, fibrous dysplasia, osteoblastoma, sclerosing epithelioid fibrosarcoma and osteosarcoma. CONCLUSION: Denosumab treatment induces a variety of changes in GCTB. Clinical history and knowledge of these features are necessary for excluding differential diagnoses and avoiding misdiagnosis.

Ossifying fibromyxoid tumor: a rare case.

Ossifying fibromyxoid tumour (OFMT) is a rarely occurring soft tissue neoplasm of mesenchymal origin. It is a rarely found tumour with intermediate behaviour and differentiation. Although it is mostly benign, malignant variants also exist. We are presenting a case of 32-year-old man presented in clinic with complaints of painless swelling in left distal thigh. After reviewing his X-ray, a diagnostic biopsy was planned which came out to be suspicious of solitary fibrous tumour. Other radiological workup was done and the patient was planned to undergo wide margin excision. The final histopathology showed a diagnosis of OFMT of soft tissue, atypical variant. The patient is under follow-up and is disease free. This type of tumour possesses potential of local recurrence and metastases; therefore, it is important to keep a long-term follow-up of patient.

Malignant distal femur tumors management in children, low cost innovations with affordable care in a tertiary care hospital, a cross sectional study.

INTRODUCTION: Malignant musculoskeletal tumors in children are mostly comprised of Osteosarcoma and Ewing's sarcoma and distal femur is the most common site for primary bone tumors. This study examined the surgical management outcomes of pediatric patients (<18 years) presented in our setup with malignant distal femur tumors. METHODOLOGY: We retrospectively reviewed the medical records of patients diagnosed with malignant sarcomas of distal femur and younger than 18 years of age who underwent limb salvage surgery during June 2009-June2017. RESULTS: There were 31 pediatric patients who had distal femur malignant tumors and out of them 20 patients were selected who underwent limb salvage as a primary surgery. The mean age (range) of patients was 11.90 (6-17) years. Seventeen had osteosarcoma and three had Ewing's sarcoma. In surgery all patients were offered reconstruction. Post-operative complications were observed in (50%) patients while other 50% patients had no complications. Wound infection, flap necrosis, broken plates with non-union and recurrence of disease were the reported complications. Revision surgery was performed in 10 patients out of 20 patients. CONCLUSION: Salvage was the primary option in our surgery and revision surgery was performed in the cases due to complications. Small sample size was the limitation of the study.

Assessment of Factors Affecting Quality of Life in Oral Squamous Cell Carcinoma Patients Using University of Washington Quality of Life Questionnaire.

Introduction Post-treatment Quality of Life (QOL) is considered an important outcome in cancer patients. A number of questionnaire tools have been designed for its assessment. University of Washington Quality of Life (UW QOL) questionnaire version four is a reliable tool for assessment of post-treatment QOL in oral squamous cell carcinoma (OSCC) patients. Our aim was to identify the post-treatment problems faced by OSCC patients and to assess the impact of clinical factors affecting post-treatment QOL by using UW QOL (version four) questionnaire. Methods The study was conducted on 59 patients with OSCC who were treated with curative intent at Patel Hospital, Karachi from August 2015 to September 2015. Patients were asked to fill the UW QOL questionnaire (version four) on their follow-up visit. Results Overall mean composite QOL score was 66.59 ± 16.98. Chewing and saliva (dryness of mouth) had the lowest scores (38.98 ± 37.2 and 56.78 ± 41.4, respectively) among all domains while pain and anxiety had the highest scores (80.93 ± 20.4 and 79.66 ± 29.8, respectively). Patients having tumors of the tongue, late stage (III and IV) tumors, and restricted mouth opening had significantly lower mean composite QOL scores. Patients with tongue tumors revealed significantly lower scores for pain, swallowing, mood, and anxiety. Patients with late-stage tumors showed significantly lower scores for chewing, swallowing, taste, saliva, appearance, anxiety, and recreation. Patients with restricted mouth opening had significantly lower scores for pain, speech, appearance, recreation, and anxiety domains. Conclusion Different clinical features have different impacts on QOL in terms of problems faced by the patients. Features having a significant effect should be identified, and measures focused on most relevant problems should be employed in order to improve the post-treatment QOL.

Management Outcomes Of Extra Abdominal Fibromatosis Among Adult Patients Treated At A Tertiary Care Hospital.

BACKGROUND: Aggressive fibromatosis or desmoid fibromatosis is a soft tissue neoplasm which is non-metastatic in nature. Among all soft tissue tumours, it comprises of 3% cases and is more common in females as compared to males. Objective of our study was to evaluate the treatment outcomes of extra abdominal fibromatosis in patients who were treated in our setup and determine the recurrence patterns. METHODS: It is retrospective cohort of 15 patients that were treated in section of Orthopaedics, department of surgery, Aga Khan University hospital, Karachi from January 1990 to December 2015. It included all adult patients of age >18 years with biopsy proven extra abdominal aggressive fibromatosis. Data was analysed on SPSS 22 version.. RESULTS: Out of 15 patients, there were 7 males (46.7%) and 8 females (53.3%). Median age was 22 years. Majority of patients [10 (66.6%)] had upper limb lesion. On initial biopsy we had 11 (73.3%) cases of primary fibromatosis while 2 (13.3%) were recurrent and 2 (13.3%) were spindle cell carcinoma. The median (IQR) follow-up time of the participants was 3 (2-3) months. Complications occurred in 8 (53.3%) patients. A significant difference was observed in the haemoglobin levels before and after surgery with a mean difference of 2.74 (p-value<0.001). Recurrence of disease occurred in 4 (26.7%) patients and all of these patients who had recurrence underwent 2nd surgery versus 1 of the participants who had second surgery without recurrence and this was a significant difference (p value <0.004). CONCLUSION: Extra abdominal fibromatosis is commonly found among younger age groups, affecting females more as compared to males. Less than half of the patients had recurrence of disease in our study and intra-operative and postoperative complications are common surgical outcomes and our study results are compatible with previously reported literature.

Aggressive fibromatosis in pediatric population-A case series.

INTRODUCTION: Aggressive fibromatosis or Desmoid fibromatosis is a benign tumor which originated form mesenchymal tissues of the body. This tumor has strong potential of recurrence and infiltration but it does not metastasize to other organs of the body. This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. METHODOLOGY: It is a retrospective case series conducted on 7 patients presented to section of Orthopedics, department of surgery of our institute in Karachi. We included all the cases of pediatric patients from 1 to 16 years, with biopsy proven fibromatosis from January 2000 to December 2015. PRESENTATION OF CASES: Out of 7 pediatric patients, there were 6 (85.7%) males and 1 (14.3%) female patient. The median age was 6 years IQR (5-11) years. Gluteal region was the most common site of disease. Four patients (57.1%) had positive tumor margins while three (42.9%) had negative margins. Out of 7 patients, 4 patients (57.1%) had recurrent disease and they had positive margins. The median Disease Free survival time was 14 months and there was no expiry of patients. CONCLUSION & RECOMMENDATION: The conclusion of our study was that aggressive fibromatosis is more prevalent in children below 15 years of age and disease burden is higher in male gender. Positive margins after surgery indicate a high risk for disease recurrence therefore; primary surgery with negative margins is the treatment of choice for children with AF. However, we recommend that multicenter trials should be conducted in the future to clarify the role of adjuvant treatment for patients with pediatric AF.