Secretory breast carcinoma (SBC) is a rare type of breast neoplasia that was originally described in children. SBC is an indolent breast tumor with good clinical outcome and rare systemic involvement. Since, majority of studies concerning pediatric SBC have been case reports, it has been difficult to clearly elucidate the characteristics and optimal treatment strategies for SBC in children. Although treatment recommendations vary, surgical excision is the primary mode of treatment. Also, necessity of axillary and/or sentinel lymph node dissection is another matter of discussion in children. We report a 6-year-old girl who was diagnosed as SBC was reported to discuss the use of mastectomy with sentinel lymph node dissection in the treatment of this rare tumor in children.
Burkitt lymphoma manifesting as an intracardiac mass is a rare entity. This report describes the case of a 10-year-old boy who presented with an intracardiac mass and tumor thrombosis in the anterior mediastinum that proved to be Burkitt lymphoma. The LMB-96 chemotherapy protocol was given and at the end of the treatment there was still residual mass. A biopsy was performed and the pathology revealed thymus tissue. The patient has been in complete remission for 3 months. Burkitt lymphoma has a short doubling time and an intracardiac lesion can become life threatening. Early recognition and prompt treatment are crucial in achieving optimal outcomes.
Osteosarcoma is the most common primary malignant tumor of the bone. The most common sites of osteosarcoma in children are the metaphysis of long bones, especially the distal femur, proximal tibia, and proximal humerus. It occurs very rarely in flat bones. Here we report a 14-year-old adolescent boy with primary osteosarcoma of the fifth rib and a review of literature.
Although response to colchicine has been proposed as one of the diagnostic criteria in patients with Familial Mediterranean fever (FMF), the validity of this response has not been validated. The aim of this study was to assess the efficacy of the response to colchicine and to evaluate the extent of the effect of placebo. A double-blind randomized placebo-controlled trial with a cross-over design was conducted. The frequency of FMF attacks, the disease score, physical examination, and acute phase reactants were assessed at 0, 3, and 6 months. Blood samples were collected for complete blood count (CBC), erythrocyte sedimentation rate (ESR), levels of serum C-reactive protein (CRP) and serum amyloid A (SAA), and MEFV mutation analysis in 79 patients with a preliminary diagnosis of FMF. Patients were randomly allocated to receive either drug A or drug B in a double-blind fashion. The designated drug was switched at 3 months. Patients taking colchicine had less frequent FMF attacks (median 0) and lower FMF disease score (median 0) when compared to those on placebo (median 1 and 3, respectively) (p = 0.002 and p = 0.007, respectively). In genetically confirmed FMF patients, median attack number and median disease score was 0 under colchicine treatment, whereas these parameters were significantly higher in the placebo group (median 2 and 8, respectively) (p = 0.007 and p = 0.02, respectively) suggesting that colchicine is more effective than placebo in reducing attacks and disease score. Positive and negative predictive values were 70.2 and 37.5 %, respectively. During the placebo period, patients had less FMF attacks when compared to that of the pre-study period (median 2 vs 6, respectively) (p < 0.001). The high false positive rate raises concerns for considering the colchicine response test as diagnostic for FMF. The role of placebo on the attacks of periodic fever syndromes needs to be further investigated.
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