A case series of Paget's disease of bone in Chinese.

OBJECTIVE. To report a series of patients with Paget's disease of bone that is rarely diagnosed in the Chinese, and to describe their presentations and clinical characteristics. DESIGN. A retrospective case series and literature review. SETTING. A regional public hospital in Hong Kong. PATIENTS. Patients with a diagnosis of Paget's disease of bone (or osteitis deformans) documented in the Clinical Management System of the Hospital Authority and being followed up in the medical endocrine clinic of the Pamela Youde Nethersole Eastern Hospital were identified in July 2011. This was performed using the Clinical Data Analysis and Reporting System of the Hospital Authority. Corresponding case notes and radiological imaging data were retrieved and reviewed. Patients with diagnostic X-ray or computed tomography findings of Paget's disease of bone were included in this series. The demographic data, clinical features, and investigation results of the cases were retrieved, recorded, and analysed. RESULTS. Seven Chinese patients (5 men and 2 women; mean age, 66 years) diagnosed to have Paget's disease of bone from 2000 to 2010 were identified. All but one were asymptomatic and presented as an incidental finding (isolated raised serum alkaline phosphatase level or abnormal X-ray). The most commonly involved sites were the skull and pelvis. The majority (71%) of the patients had polyostotic disease. During follow-up, there were no disease-related complications, nor was malignant transformation identified. None reported positive family history. CONCLUSION. In this series of seven Chinese patients with Paget's disease, most were asymptomatic and presented with an isolated raised serum alkaline phosphatase level during routine testing. The disease was predominantly found in males and the elderly, and commonly involved the skull and pelvis.

Ovarian sex cord stromal tumor with annular tubules in a 7-year-old child: A case report.

A seven-year-old girl who presented with precocious puberty was diagnosed with an estrogen-secreting right ovarian tumor. Right salpingo-oophorectomy with staging was performed by gynecologic oncologists. Intraoperative frozen section reported sex cord stromal tumor. Histopathological report confirmed sex cord stromal tumor with annular tubules with brisk mitotic counts (17 per 10 high-power fields). The post-operative course was uneventful. She remained asymptomatic with normalisation of serum estradiol upon six-month follow-up.

Schneiderian papillomas and carcinomas: a retrospective study with special reference to p53 and p16 tumor suppressor gene expression and association with HPV.

Schneiderian papillomas are uncommon benign tumors of the sinonasal area. They are prone to local aggressiveness and recurrence, and some undergo malignant progression. We analyzed specimens obtained from 67 Chinese patients who had presented to the ENT department of a regional hospital with biopsy-proven schneiderian papilloma. Seven of these patients had either synchronous or metachronous carcinoma, 1 of whom had pure carcinoma in situ. For each case, we documented the morphology, immunohistochemical expression of tumor suppressor genes p53 and p16, and any association with human papillomavirus (HPV) infection as detected by either polymerase chain reaction or in situ hybridization techniques. We found that severe dysplasia and p53 positivity were strongly associated with malignant progression. Association with HPV was demonstrated in 22 of the 67 patients (33%); the association was strongest among patients with exophytic papillomas and carcinomas. The effect of HPV in papilloma oncogenesis probably begins during the early phase, while other factors are responsible for progression to carcinoma. We conclude that p53-positive, dysplastic schneiderian papillomas warrant aggressive surgical treatment.