Basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma: a case report and review of the literature.

BACKGROUND: This paper reports the first case of basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma. CASE PRESENTATION: A 66-year-old Sinhalese male with unilateral hearing impairment and 7th-12th (excluding 11th) cranial nerve palsies was diagnosed radiologically with a head and neck paraganglioma by magnetic resonance imaging of the brain, which revealed a hypointense and hyperintense punctate mass centered at the jugular fossa with intracranial extension. The ascending pharyngeal artery, recognized as the major feeder, was embolized by percutaneous embolization following digital subtraction angiography. Gross total resection of the tumor was followed by an uneventful postoperative recovery. Combined immunohistochemistry and histopathological morphology revealed a basaloid squamous cell carcinoma, following which the patient completed radiotherapy and is at 3-month follow-up currently. CONCLUSION: This case report discusses the diagnostic pitfalls and management challenges of this rare entity on the basis of prior evidence, as well as a literature review and clinical and surgical analysis.

Breast Cancer Survival in Sri Lanka.

PURPOSE: In this study, we report survival data of the largest cohort of patients with breast cancer in Sri Lanka. PATIENTS AND METHODS: All female patients with histologically confirmed breast cancer treated at a single unit at the National Cancer Institute of Sri Lanka between 1994 and 2006 were included in the study. Clinical records were reviewed and data obtained on the following clinical and pathologic factors: age, histology, stage at presentation, grade, and immunohistochemistry profile. Treatment details such as type of surgery and use of systemic chemotherapy, hormonal therapy, trastuzumab, and radiation therapy were also collected. In localized cancer, disease-free survival (DFS) was the primary end point, while in patients who presented with de novo metastases, progression-free survival (PFS) was the primary end point. RESULTS: A significant proportion of patients presented with de novo metastases (14%) and locally advanced disease (18%). While 57% of patients had hormone-sensitive tumors, human epidermal growth factor receptor 2 overexpression was seen in 14%, and 29% had triple-negative tumors. Only 3% of patients with localized disease were treated with breast-conserving surgery, with the rest undergoing modified radical mastectomy. The 5- year DFS rate was 71.6% (95% CI, 69.2 to 74.0) in patients with localized disease. The median PFS in patients with metastatic disease was 20 months (95% CI, 18 to 22 months), while the median overall survival was 30 months (95% CI, 32 to 35 months). On multivariable analysis, immunohistochemical group and stage were prognostic factors in localized disease, while in patients with metastases, immunohistochemical group and tumor grade were associated with PFS. CONCLUSION: More effective screening and early detection programs along with increasing breast-conserving surgery will improve breast cancer outcomes in Sri Lanka.

ATG vs thiotepa with busulfan and cyclophosphamide in matched-related bone marrow transplantation for thalassemia.

Matched-related bone marrow transplantation (BMT) may cure >80% of low-risk children with severe thalassemia (ST). Very long-term follow-up studies have shown how the standard busulfan-cyclophosphamide (BuCy) regimen may be associated with normalization of health-related quality of life, no second malignancies in the absence of chronic graft-versus-host disease, and fertility preservation in many patients. However, because BuCy may be associated with high rejection rates, some centers incorporate thiotepa (Tt) in busulfan- or treosulfan-based regimens, a combination that may increase the risk of permanent infertility. This study retrospectively compares matched-related BMT outcomes in 2 groups of low-risk ST patients conditioned with either Tt or anti-thymocyte globulin (ATG) in addition to BuCy. A total of 81 consecutive first BMTs were performed in 5 collaborating startup BMT centers in the Indian subcontinent between January 2009 and January 2016; 30 patients were transplanted after conditioning with Tt-BuCy between January 2009 and July 2013, whereas between August 2013 and January 2016, 51 patients received ATG-BuCy. All patients were <15 years and had no hepatomegaly (liver ≤2 cm from costal margin). Actuarial overall survival in the Tt-BuCy and ATG-BuCy groups was 87% and 94% and thalassemia-free survival was 80% and 85% at a median follow-up of 37 and 17 months, respectively, with no significant differences by log-rank statistics. Substituting Tt with ATG in the standard BuCy context seems safe and effective and may decrease transplant-related mortality. Higher fertility rates are expected for patients who received ATG-BuCy.