Synovial sarcoma: a rare neoplasm of paranasal sinus.

Synovial Sarcoma (SS) is a rare soft-tissue malignant tumour. Its presentation in the head and neck region is uncommon. Because of the complex anatomy of the head and neck region, surgery with clear margins is not achievable. In such cases, a multi-modality approach is required as there is no established standard of care. In this report, we share the case of a girl who presented with nasal obstruction. Imaging revealed a mass involving the left nasal cavity, paranasal sinuses without intracranial extension. It was diagnosed as synovial sarcoma. She underwent surgical excision and adjuvant radiation therapy (RT) to the tumour bed, followed by an incomplete course of chemotherapy. Later on, she developed systemic disease. Considering the rarity of this case and lack of guidelines for standard treatment, we report on this case to share our experience with management and treatment outcome.

A Rare Case of High-Grade Spindle Cell Sarcoma of the Breast: A Case Report.

Introduction: Spindle cell sarcomas are rare breast lesions which are difficult to diagnose due to resemblance with other breast lesions. Histopathological examination and immunohistochemical staining are essential for diagnosis. Case Presentation: We present a rare case of a 15-year-old female presenting with high-grade rapidly progressive spindle cell sarcoma of the breast, differentiated as phyllodes tumor, with axillary lymph node involvement. Her lesion, on the left breast, measured 16.9 × 10.1 × 13.7 cm. Histology revealed malignant neoplasm arranged in sheets and individual neoplastic cells with an epithelioid to spindled morphology with scant cytoplasm and irregular nuclear membranes. Immunohistochemistry showed weakly positive focal CD-99, and negative WT-1, Myogenin, Desmin, p63, Cytokeratin, Synaptophysin, and CD-34 markers. She was successfully managed with modified radical mastectomy and discharged with regular follow-up advised. Conclusion: Spindle cell sarcomas have a very aggressive course and prompt diagnosis, and management is mandatory for better patient outcomes. Modified radical mastectomy is the mainstay of treatment.

Active implementation of low disease activity state as a treatment endpoint in childhood-onset systemic lupus erythematosus in routine practice is both feasible and associated with better outcomes.

INTRODUCTION: Treat-to-target (T2T) strategies aim to facilitate tight disease control to improve outcomes. No previous studies evaluated prospectively the feasibility and impact of the T2T strategy in routine practice in childhood-onset SLE (cSLE). METHODS: Adolescents and young adults (AYA) with cSLE were recruited for T2T implementation from a large tertiary centre over a period of 6 months and followed up at least twice over a prospective period of 12 months. RESULTS: During Oct 2022-April 2023, 135/162 (83.3%) AYA with cSLE had disease scores evaluated at their routine appointment to enable inclusion in the study, and 122/135 (91.2%) had their disease assessed, and a suitable treatment target agreed and documented at each routine clinical appointment over the 12 months prospective follow-up. T2T strategy led to improved disease control at 12 months: more AYA with cSLE achieved clinical remission off steroids (4.1% vs. 10.7%, P = 0.048), or minimum childhood-lupus low disease activity (cLLDAS) (81.9% vs. 91.8%, P = 0.022). Achieving minimum cLLDAS for longer than 3 months was associated with reduced damage accrual (HR = 1.7; 95%CI = 1.1-2.5; P < 0.0001) at 12 months. CONCLUSION: T2T strategy implementation was achievable and associated with improved cSLE control. Spending at least 3/12 months in cLLDAS led to less damage accumulation. Key Points • This is the first large prospective study in AYA with cSLE to evaluate the impact of active T2T implementation in routine practice. • T2T strategies were feasible to implement in 122/135 (91.2%) AYA with cSLE in routine practice. • The T2T approach was associated with improved disease control and decreased damage accrual at 12 months.

Compliance and clinical efficacy of vaginal dilator after radiotherapy for cervical and endometrial malignancies.

Objective: To investigate the compliance and clinical efficacy of vaginal dilators (VDs) as an educational intervention in patients receiving pelvic radiation therapy (RT) for endometrial and cervical malignancies. Material and methods: This is a single institution, retrospective chart review. Patients undergoing pelvic RT for endometrial or cervical cancer at our center were educated about the use of a VD starting 1 month after completion of RT. The patients were assessed after 3 months of prescribing VD. The demographic details and physical examination findings were extracted from medical records. Results: We identified 54 female patients at our institution during the 6-month duration. The median mean age of patients was 54 ± 9.9 years. Twenty-four (44.4%) had endometrial cancers and 30 (55.6%) were diagnosed with cervical cancers. All patients received external beam RT, 38 (70.4%) received a dose of 45 Gy, and 16 (29.6%) patients received 50.4 Gy. Brachytherapy was also received by all patients, 28 (51.9%) received 5 Gy × 2 fractions, 4 (7.4%) received 7 Gy × 3 fractions and 22 (40.7%) received 8 Gy × 3 fractions. The compliance with VD use was 36 (66.6%) patients. Twenty-two (40.7%) used 2-3 times a week, 8 (14.8%) used <2 times per week and 6 (11.9%) used only once a month, and 18 (33.3%) did not use the VD post-treatment. Per vaginal (PV) examination findings of the patient's vagina with normal mucosa were evaluated in 32 (59.3%) and adhesions were found in 20 (37.0%) and 2 (3.7%) were unable to examine due to dense adhesions. During examination 12 (22.2%) had bleeding PV, however, the majority of the patients, 42 (77.8%) experienced no bleeding PV. Out of the 36 patients who used a VD, it was found to be efficacious in 29 (80.6%) of patients. Upon stratification of efficacy with a frequency of VD, 72.4% (n = 21) efficacy was seen in patients using frequent VD as prescribed 2-3 times per week. Conclusion: The compliance and efficacy of VD use after radiation to pelvic in cervical and endometrial cancers at 3 months follow-up were found to be 66.6% and 80.6%, respectively. This shows that VD therapy is an effective interventional tool and patients should receive specialist education about vaginal stenosis as toxicity at the outset of treatment.

The Frequency of Rh Phenotype and Its Probable Genotype.

AIMS AND OBJECTIVES: Our goal is to disseminate data on the distribution pattern of Rh antigen, its phenotypes, and the likely genotypes of these genetic variants in the Pakistani population. METHODOLOGY: This study was a cross-sectional research project. Patients' demographic statistics, such as age and gender, were gathered from their medical information. Blood group, disease, RhD, and other antigen frequency, phenotype, and probable genotype were considered variables. All blood samples were phenotyped for Rhesus antigens (D, C, c, E, and e), and the test was carried out using the tubing technique. RESULTS: According to gender distribution, most of the patients were males, with 131 frequencies (57.7%), while females had 42.35%. The most common phenotype was DCCee, with its probable genotype DCe/DCe (R1 R1) (34%), followed by DCcee, with probable genotype DCe/ce (R1 r) (29.1%); the least common phenotype was ddCcee, with its probable genotype Ce/ce (r ' r) (0.4%). CONCLUSION: It is concluded that the DCCee phenotype was the most common with its probable genotype DCe/DCe, while the least common phenotype was ddCcee with its probable genotype Ce/ce.

Acute dermatitis in adult female patients receiving hypofractionated radiotherapy for breast cancer: experience from a low- and middle-income country.

Radiotherapy (RT) is an important component of treatment in the management of breast cancer patients. The radiation treatment paradigm has been shifted towards hypofractionated RT. This study aims to determine the severity of acute dermatitis in patients receiving hypofractionated RT for breast cancer at a tertiary care university hospital in Pakistan. Patients with biopsy-proven invasive breast carcinoma or DCIS who were referred for radical radiotherapy after discussion in the breast tumour board were retrospectively reviewed. Physical assessment of the patients for evaluation of the severity of radiation dermatitis will be carried out in the first week, last week and on the first follow-up after 1 month of completion of RT, according to the Radiation Therapy Oncology Group/European Organisation For Research And Treatment Of Cancer (RTOG/EORTC) criteria. We identified 92 female patients in 6 months at Aga Khan University Hospital, with a mean age of 53.1 years. Most of the treated patients had clinical stage 3 (64%) cancer, while others were stage 2 (42%), stage 1 (2%) and stage 0 (2%). The surgeries performed were mastectomy in 59 patients and breast-conserving surgery in 33 patients. Histology was Intra Ductal Carcinoma (IDC) (95%), DCIS (3%) and Invasive Lobular Carcinoma (ILC) (2%). Most of the patients received chemotherapy (96%). Radiotherapy dose was 4256 cGy in 16 fractions, followed by a boost of 10 Gy. The radiation techniques used were intensity-modulated radiotherapy (47.8%) and three-dimensional conformal radiotherapy (52.2%). Most of the patients experienced no toxicity (59%), while grade I toxicity was observed in 29% of the patients and grade II toxicity was observed in 11%. Only 1% of the patients experienced grade III skin toxicity. Hypofractionated radiation therapy is beneficial because of the shorter overall treatment time which reduces the socio-economic burden, not only for patients but also for radiotherapeutic institutions. However, extended follow-up is to be reported for long-term toxicity and other consequences.

Fertility preservation is an imperative goal in the clinical practice of radiation oncology: a narrative review.

With reduced cancer mortality in recent years, increased efforts must be put into safeguarding cancer survivors' long-term quality of life (QOL). Fertility preservation is recognised as a key component of QOL in survivorship. Concerns about fertility have been seen to significantly impact cancer patients' emotional and mental health as, generally, both malignancy and its treatment may cause a temporary or permanent reduction in infertility. This article reviews the primary effects of radiation therapy on male and female gonads and has further highlighted procedures through which the functioning of these organs can be protected before or during radiation treatment. We have also emphasised the importance of the establishment of multidisciplinary tumour boards and patient education regarding future reproductive function which is an important component of the care of individuals with cancer. This article highlights that infertility is a persistent and major concern that can add to long-term stress in cancer survivors, and education about fertility preservation before the initiation of any treatment is especially important.