Thymomas are rare tumours which generally account for only 0.2 – 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities. A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient’s motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness. The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the importance of multidisciplinary management involving oncologists, surgeons, and neurologists.
Thymoma ; Myasthenia Gravis ; Drug Therapy ; Thymectomy

PURPOSE

Wilms tumor (WT) management has evolved into a multimodality paradigm that includes radiotherapy (RT), usually as an adjuvant or consolidative modality. Protocols are refined to maximize cure and compliance while minimizing acute toxicity and long-term effects. RT technique and timing are two factors that could improve these outcomes. We reviewed the evidence on survival and toxicity outcomes among WT patients with conventional versus advanced RT techniques and early versus delayed RT to inform a Department of Health (DOH) commissioned guideline.

We systematically searched PubMed, EuropePMC, EBSCOHost, HERDIN, systematic review and clinical trial registries and official websites of scientific societies for relevant publications and grey literature. Eligibility screening, risk-of-bias assessment and data extraction were performed using a single-reviewer approach. Given the study and data heterogeneity, only a qualitative synthesis was performed. Certainty of evidence assessment was done using the GRADE approach.

We screened 314 studies and included seven in the review, including a phase 1/2 trial and six retrospective studies, all from first-world countries (US, France, Netherlands), except one from a newly industrialized country (Brazil). The certainty of evidence on the survival and toxicity outcomes with advanced RT techniques was very low. Moderate-certainty evidence supports that giving RT >14 days after surgery leads to increased mortality.

Current evidence does not support the routine use of advanced RT techniques; proper contextualization is necessary. Tertiary centers managing WT should strive to administer RT within 14 days after surgery whenever possible.

Nephroblastoma is an uncommon renal malignancy primarily observed in the pediatric population, with its occurrence in adults being exceedingly infrequent. We describe an extremely rare case of a malignant embryonal tumor presenting in an adult patient with right renal mass. Final histopathologic diagnosis was nephroblastoma with favorable histology. Use of immunohistochemistry studies is generally unnecessary but its rarity in the adult population raises uncertainty in diagnosing this malignancy by histomorphology alone.

Humans ; Soft Tissue Neoplasms ; Mesenchymoma/diagnostic imaging* ; Paraneoplastic Syndromes/pathology*

Objective: To investigate the clinical, radiological, histological and molecular features and the differential diagnosis of fibrocartilaginous mesenchymoma (FM). Methods: Four cases of FM diagnosed in the Department of Pathology, the Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine from 2020 to 2022 were analyzed. Related literature was also reviewed. Results: Case 1 was a 10-year-old girl with bone destruction in the sacrum and L5 articular processes revealed by CT scan. Case 2 was a 7-year-old girl with an aggressive lesion in her right distal ulna. Case 3 was an 11-year-old boy with a lesion in the metaphysis of his left proximal tibia. Case 4 was an 11-year-old boy with bone destruction in the distal portion of a radius. Microscopically, the four tumors all consisted of numerous spindle cells, hyaline cartilage nodules, and bone trabeculae. The hypocellular to moderately cellular spindle cell component contained elongated cells with slightly hyperchromatic, mildly atypical nuclei arranged in bundles or intersecting fascicles. Benign-appearing cartilaginous nodules of various sizes and shapes were scattered throughout the tumors. There were areas mimicking epiphyseal growth-plate characterized by chondrocytes arranged in parallel columns and areas of enchondral ossification. The stroma was rich in mucus in case 1. Mutation of GNAS and IDH1/IDH2 and amplification of MDM2 gene were not found in any of the three tested cases. Conclusions: FM is very rare and tends to affect young patients. It most frequently occurs in the metaphysis of long tubular bones, followed by the iliac-pubic bones and vertebrae. FM is characterized by a mixed population of spindle cells, hyaline cartilage nodules and trabeculae of bone, without specific immunophenotypes and molecular alternations. As a borderline, locally aggressive neoplasm, surgical removal with a wide margin is generally the treatment of choice for FM.
Humans ; Male ; Female ; Child ; Mesenchymoma/pathology* ; China ; Osteogenesis ; Cartilage/pathology* ; Tomography, X-Ray Computed

Humans ; Thymoma/pathology* ; Thymus Neoplasms/pathology*

OBJECTIVES: To study the correlation between 25-hydroxyvitamin D [25-(OH)D] and nephroblastoma in children and its value in assessing the prognosis of the disease. METHODS: A total of 50 children with nephroblastoma who were admitted from January 2018 to December 2022 were included as the nephroblastoma group, and according to the postoperative pathological type, they were divided into a good prognosis group with 38 children and a poor prognosis group with 12 children. A total of 50 healthy children who underwent physical examination during the same period of time served as the healthy control group. The above groups were compared in terms of serum creatinine and 25-(OH)D level. A Spearman correlation analysis was used to investigate the correlation between serum 25-(OH)D level and therapeutic effect reaction. A multivariate logistic regression analysis was used to identify the risk factors affecting the prognosis of nephroblastoma in children. RESULTS: The nephroblastoma group had significantly lower levels of serum creatinine and 25-(OH)D than the healthy control group (P<0.05). Compared with the good prognosis group, the poor prognosis group had a significantly larger tumor diameter, a significantly higher proportion of children with stage III-IV tumors, a significantly higher rate of tumor metastasis, and significantly lower serum levels of creatinine and 25-(OH)D (P<0.05). The Spearman correlation analysis showed that serum 25-(OH)D level was negatively correlated with therapeutic effect reaction (r_s=-0.685, P<0.001). The multivariate logistic regression analysis showed that tumor diameter ≥10 cm, stage III-IV tumors, presence of tumor metastasis, and 25-(OH)D <19 ng/mL were closely associated with the poor prognosis of nephroblastoma in children (P<0.05). Serum 25-(OH)D level had an area under the curve of 0.805 (95%CI: 0.706-0.903, P<0.001) in evaluating the prognosis of nephroblastoma in children, with a Youden index of 0.512, a sensitivity of 0.938, and a specificity of 0.575 at the optimal cut-off value of 1.764 ng/mL. CONCLUSIONS There is a significant correlation between 25-(OH)D level and the prognosis of nephroblastoma in children, and 25-(OH)D can be used for prognosis prediction.
Humans ; Child ; Creatinine ; Vitamin D Deficiency/complications* ; Vitamin D ; Calcifediol ; Prognosis ; Wilms Tumor ; Kidney Neoplasms/complications*

Myoepithelioma, also known as malignant myoepithelioma, is a rare malignant tumor originating from myoepithelial cell. This article reports a patient with a huge tumor in the neck and left elbow who underwent fine needle aspiration under local anesthesia. The pathological diagnosis was a myoepithelioma. Under general anesthesia, giant tumors in the lower neck, posterior cranial fossa, neck, and left elbow were removed, and postoperative pathology showed that they were all myoepithelial tumors. Immunohistochemistry showed AE1/AE3 (+), P63 (+), CK7 (+), CK5 (+), and CD138 (+). The clinical characteristics and diagnosis and treatment process of this case are reported and relevant literature is reviewed.
Humans ; Myoepithelioma/pathology* ; Immunohistochemistry ; Epithelial Cells ; Neck/pathology* ; Carcinoma

Humans ; Adenoma, Pleomorphic/genetics* ; Adenoma ; Salivary Gland Neoplasms/genetics* ; Adaptor Proteins, Signal Transducing

Humans ; Thymoma/surgery* ; Thyroid Neoplasms ; Thymus Neoplasms/surgery* ; Neoplasms, Glandular and Epithelial