A single-cell atlas of conventional central chondrosarcoma reveals the role of endoplasmic reticulum stress in malignant transformation.

The transformation of benign lesions to malignant tumours is a crucial aspect of understanding chondrosarcomas, which are malignant cartilage tumours that could develop from benign chondroid lesions. However, the process of malignant transformation for chondroid lesions remains poorly understood, and no reliable markers are available to aid clinical decision-making. To address this issue, we conducted a study analysing 11 primary cartilage tumours and controls using single-cell RNA sequencing. By creating a single-cell atlas, we were able to identify the role of endoplasmic reticulum (ER) stress in the malignant transformation of conventional central chondrosarcomas (CCCS). Our research revealed that lower levels of ER stress promote chondrosarcoma growth in a patient-derived xenograft mouse model, while intensive ER stress reduces primary chondrosarcoma cell viability. Furthermore, we discovered that the NF-κB pathway alleviates ER stress-induced apoptosis during chondrosarcoma progression. Our single-cell signatures and large public data support the use of key ER stress regulators, such as DNA Damage Inducible Transcript 3 (DDIT3; also known as CHOP), as malignant markers for overall patient survival. Ultimately, our study highlights the significant role that ER stress plays in the malignant transformation of cartilaginous tumours and provides a valuable resource for future diagnostic markers and therapeutic strategies.

Assessing the short- and long-term outcomes after resection of benign insulinoma.

BACKGROUND: Insulinoma is a rare functional pancreatic neuroendocrine tumour (NET) believed to have an excellent long-term outcome, but few studies have solely focused on this issue after apparently curative resection. This study aimed to assess post-operative and long-term outcomes after resection of benign insulinomas. METHODS: From 1998 to 2013, 36 consecutive patients with insulinomas underwent surgery. Three patients had multiple endocrine neoplasia type-1 (MEN-1). Demographics, operative findings, tumour grade (2010 World Health Organization (WHO) NET classification), post-operative pancreatic fistula (POPF) grade (International Study Group of Pancreatic Fistula (ISGPF)), complications and recurrence were analysed. RESULTS: Eighteen (50%) had enucleation while the rest underwent pancreatic resection. The majority (86.1%) of insulinomas belonged to WHO NET grade G1. POPF occurred in 58.3% of patients while clinical fistula (ISGPF grades B and C) occurred in 19.4%. One (2.8%) patient required reoperation. The occurrence of POPF was not related to type of resection or surgical approach. There was no perioperative mortality. After a mean follow-up of 83.6 months, two patients (5.7%) developed disease recurrence at 34.4 and 131.9 months after initial surgery. No patients developed distant metastasis. The 10- and 15-year disease-free rates were 95.6 and 85.4%, respectively. CONCLUSION: POPF occurred frequently and posed a significant morbidity after resection of insulinoma. However, it occurred independently of type of resection or surgical approach. Although the immediate cure rate after resection was high (100%), long-term disease recurrence in sporadic (non-MEN-1) cases was not insignificant. Regular long-term follow-up is recommended.

Giant cell tumor of the humeral head treated by denosumab: Implication to shoulder surgeons.

Giant cell tumor is a benign bone tumor that is commonly encountered. The optimal treatment of a giant cell tumor which causes extensive bony destruction is controversial. Recent studies on the receptor activator of nuclear factor κB ligand antagonist denosumab may offer a new treatment option for these patients. We presented a patient with giant cell tumor of the humeral head. He was initially treated with denosumab and subsequently with the operation. The shoulder joint was successfully salvaged. But there are potential difficulties that surgeons may face in patients treated with denosumab.

Unusual presentations of osteoarticular tuberculosis in two paediatric patients.

Tuberculosis is a universal mimicker and thus could be a differential diagnosis of any osteolytic lesion. Bone biopsy is crucial in these cases for culture and histological proof of tuberculous infection. This is a case report of two paediatric patients with unusual presentations of tuberculosis. One patient presented with knee pain and had imaged findings of an osteolytic lesion at the epiphysis. Interval scan showed spread of the lesion through the physis to the metaphyseal region. The second patient presented with hip pain and an osteolytic lesion of the acetabulum. He was subsequently found to have involvement of the brain and spine as well. Both patients were diagnosed with tuberculosis by bone biopsy for culture and pathological examination. They were treated successfully with antituberculous medications without chronic sequelae. These two patients showed that early recognition and prompt treatment are critical for management of tuberculosis to avoid chronic sequelae.

Parotid acinar cell carcinoma in a long-term survivor of childhood acute lymphoblastic leukemia.

Secondary malignancies are an important cause of morbidity and mortality in childhood cancer survivors. Salivary gland tumors account for about 6% of the second cancers. The majority of these are mucoepidermoid carcinomas (MEC) of the parotid gland. We report the clinical and pathological features of a rarer histological type, acinic cell carcinoma (ACC), in a childhood acute lymphoblastic leukemia (ALL) survivor. The behavior of secondary ACC appears similar to primary tumor and similar treatment may be adopted. Early recognition and complete resection is important for achieving a good outcome. Careful monitoring for recurrence or a third malignancy is needed.

First-trimester diagnosis of hydrolethalus syndrome in a Chinese family.

We report a case resembling hydrolethalus syndrome in a Chinese family. Fetal polydactyly, syndactyly, encephalocele and cardiac malformation were detected on ultrasound examination at 12 weeks' gestation. Termination of pregnancy was performed, and postmortem examination confirmed the findings. This is the first report of a first-trimester prenatal diagnosis of hydrolethalus syndrome in the Chinese population.

Thermocoagulation of fetal sacrococcygeal teratoma.

Fetal sacrococcygeal teratoma can lead to a high output cardiac failure resulting in hydrops fetalis. One of the prenatal therapeutic options is to occlude the feeding vessels by radiofrequency ablation. We present a case of fetal sacrococcygeal teratoma diagnosed at 13 weeks of gestation. The tumour increased in size more than 100 fold over 5 weeks causing polyhydramnios and cardiac and placental enlargement. Thermocoagulation was performed at 18 weeks' gestation by passing an insulated electric wire through an 18 gauge needle placed close to the feeding vessels of the tumour at its neck. Blood supply to the tumour was successfully reduced. However, fetal death was diagnosed 2 days after the procedure. We speculate that it may be safer to limit the extent of coagulation in one attempt but to repeat the procedure at a later stage when necessary.