Objective To investigate the clinical features and treatment of anti-myelin oligodendrocyte glycoprotein-IgG associated disorders （MOGAD） and the risk factors for recurrence and poor long-term prognosis. Methods A total of 91 patients who were diagnosed with MOGAD in The First Affiliated Hospital of Zhengzhou University from January 2018 to March 2023 were enrolled，and their clinical features and auxiliary examinations were analyzed，as well as the risk factors for recurrence and long-term prognosis. Results Among the 91 patients，69 experienced the first attack of MOGAD，and there were 39 female patients and 47 children （aged<18 years）. The proportion of patients with acute disseminated encephalomyelitis among children was significantly higher than that among adults （42.6% vs 18.2%，P=0.012），while the proportion of patients with transverse myelitis among adults was significantly higher than that among children （29.5% vs 2.1%，P<0.001）. The proportion of patients receiving hormones combined with immunoglobulins during hospitalization among children was significantly higher than that among adults （36.2% vs 11.4%，P=0.006），and the children had a significantly better Expanded Disability Status Scale （EDSS） score than the adults at discharge [1（0，1） vs 2（0，4.75），P=0.007]. Visual impairment was an independent risk factor for increased recurrence risk （OR=4.215，95%CI 1.236-14.377，P=0.022）. A higher EDSS score at discharge （OR=5.05，95%CI 1.27-20.07，P=0.021） and a higher number of attacks （OR=9.235，95%CI 1.352-63.10，P=0.023） were independent factors for poor long-term prognosis，while a steroid maintenance time of >5 weeks at initial diagnosis （OR=0.001，95%CI 0.00-0.33，P=0.001） was an independent factor for improving long-term prognosis. Conclusion For patients newly diagnosed with MOGAD，especially those with a high EDSS score at discharge and features indicating a high risk of recurrence （such as visual impairment），it is recommended that they receive an appropriate course of steroid maintenance treatment after acute-stage treatment.
Recurrence ; Prognosis

OBJECTIVE: To compare the accuracy and effectiveness of orthopaedic robot-assisted minimally invasive surgery versus open surgery for limb osteoid osteoma. METHODS: A clinical data of 36 patients with limb osteoid osteomas admitted between June 2016 and June 2023 was retrospectively analyzed. Among them, 16 patients underwent orthopaedic robot-assisted minimally invasive surgery (robot-assisted surgery group), and 20 patients underwent tumor resection after lotcated by C-arm X-ray fluoroscopy (open surgery group). There was no significant difference between the two groups in the gender, age, lesion site, tumor nidus diameter, and preoperative pain visual analogue scale (VAS) scores ( P>0.05). The operation time, lesion resection time, intraoperative blood loss, intraoperative fluoroscopy frequency, lesion resection accuracy, and postoperative analgesic use frequency were recorded and compared between the two groups. The VAS scores for pain severity were compared preoperatively and at 3 days and 3 months postoperatively. RESULTS: Compared with the open surgery group, the robot-assisted surgery group had a longer operation time, less intraoperative blood loss, less fluoroscopy frequency, less postoperative analgesic use frequency, and higher lesion resection accuracy ( P<0.05). There was no significant difference in lesion resection time ( P>0.05). All patients were followed up after surgery, with a follow-up period of 3-24 months (median, 12 months) in the two groups. No postoperative complication such as wound infection or fracture occurred in either group during follow-up. No tumor recurrence was observed during follow-up. The VAS scores significantly improved in both groups at 3 days and 3 months after surgery when compared with preoperative value ( P<0.05). The VAS score at 3 days after surgery was significantly lower in robot-assisted surgery group than that in open surgery group ( P<0.05). However, there was no significant difference in VAS scores at 3 months between the two groups ( P>0.05). CONCLUSION Compared with open surgery, robot-assisted resection of limb osteoid osteomas has longer operation time, but the accuracy of lesion resection improve, intraoperative blood loss reduce, and early postoperative pain is lighter. It has the advantages of precision and minimally invasive surgery.
Humans ; Robotics ; Osteoma, Osteoid/surgery* ; Orthopedics ; Blood Loss, Surgical ; Retrospective Studies ; Neoplasm Recurrence, Local ; Minimally Invasive Surgical Procedures ; Bone Neoplasms/surgery* ; Analgesics ; Treatment Outcome

OBJECTIVE

This study aimed to identify the effects of COVID-19 pandemic on radiation treatment delays, interruptions, and cancer outcomes if any, in a Philippine tertiary hospital.

A retrospective observational cohort study was conducted among patients living with cancer who were referred for radiation therapy from March to July 2020. Data on treatment delays, treatment interruptions, and average treatment times were collected and analyzed. An association between radiation treatment interruption and disease failure rate was also evaluated.

The study found the mean radiation treatment time ranged from 50.3 days to 140.6 days from the start of radiation treatment to the end of radiation treatment, and 181.7 days to 217.3 days from the date of CT simulation to the end of radiation treatment. These radiation treatment times and treatment interruptions are beyond any published recommendations for external beam radiation therapy, thus potentially leading to worse oncologic outcomes. However, no association between treatment interruption and disease failure rate was noted. This may be due to limitations of the study such as small sample size and heterogeneous patient characteristics such as different cancer pathologies and different disease stages.

This study showed that the COVID-19 pandemic had a significant impact on radiation treatment interruptions and delays, potentially leading to worse oncologic outcomes. The lack of association between treatment interruption and disease failure rate needs further investigation with a larger sample size, more homogeneous patient characteristics, and longer follow-up. The findings highlight the importance of maintaining cancer care services during pandemics and implementing measures to minimize treatment interruptions and delays to improve cancer outcomes.

OBJECTIVE

This study aims to describe the epidemiologic profile and determine the clinical outcomes of patients with pheochromocytoma at the University of the Philippines Philippine General Hospital (UP-PGH).

We reviewed the medical records of 30 patients with histopathology-proven, clinical, and biochemical diagnosis of pheochromocytoma. Demographic, clinical characteristics, and clinical outcomes were collected for each patient.

The median age at diagnosis of pheochromocytoma was 37.5 years (IQR 28-55) and the most common metabolic comorbidities were glucose intolerance (60%) and hypertriglyceridemia (23.3%). Majority of the patients were hypertensive (90%). Two third of the patients presented with classic features of pheochromocytoma while the remaining third presented as adrenal incidentaloma. Recurrence was found in 17% of subjects, who were significantly younger (25 years vs 46.5 years P = 0.0229), and had higher rates of bilateral pheochromocytoma (0 vs 75%), p = 0.002). Metastatic pheochromocytoma was found in 10% of the subjects.

Our study demonstrated that patients with pheochromocytoma in our setting exhibit great variability in terms of clinical behavior. Although majority of the patients presented with symptoms related to catecholamine excess, almost one-third of the patients were only incidentally discovered. Incidence of pheochromocytoma recurrence and metastasis in our setting are comparable with current available foreign studies.

Objective: This study described the clinical profile and outcomes of patients with ocular dermoid cysts at a subspecialty Ophthalmology clinic of the Philippine General Hospital (PGH). Methods: Medical records of 38 patients seen at the External Disease & Cornea Clinic from January 2012 to June 2023 were reviewed. Demographics, best-corrected visual acuity (BCVA), refraction, other associated malformations, and dermoid characteristics were collected. For those that underwent surgery, the procedures performed and histopathology reports were reviewed. Most recent refraction, BCVA, presence of amblyopia, recurrence, and complications were recorded. Results: Mean age at presentation was 8.33 years old with equal male:female distribution (52% vs 48%). Thirtyfour (34) participants (89.5%) had unilateral dermoids and 4 participants (10.5%) had bilateral dermoids, for a total of 42 eyes. Thirty-five (35) or 83.3% of the dermoids were located at the inferotemporal quadrant of the cornea. There were 42.1% participants who had associated congenital anomalies, with dermolipoma and preauricular tags being the most common ocular and craniofacial findings, respectively. Twenty-seven (27) eyes (64.2%) underwent surgery and the most common procedure was excision with limbal conjunctival autograft (55.6%). In the 8 eyes that underwent simple excision, recurrence of the dermoid was seen in 1 patient and development of pseudopterygium in 5 patients. Dermoid was the histopathological diagnosis in 96.3% of the excised tissues. Twenty-four (24) patients (63.2%) presented with ambylopia with BCVA <20/30, and regardless of type of intervention done, only 7 participants (23.3%) had a visual acuity better than 20/30 during their latest consult. Conclusion Ocular dermoid is a common congential anomaly usually presenting as an isolated, unilateral mass at the inferotemporal limbal area. Aside from the obvious effect on the cosmetic appearance of the eye, a more pressing concern is the high incidence of amblyopia. Timely excision with limbal conjunctival autograft is highly recommended coupled with aggressive amblyopia therapy.
Recurrence ; Amblyopia

Sinonasal inverted papilloma（SNIP） is a kind of benign tumor originating from the nasal cavity and paranasal sinuses, accounting for 70% of papillomas. The incidence of the disease is more common in males, with an average age of 50-60 years. It is most likely to occur in unilateral maxillary sinus and ethmoid sinus, followed by sphenoid sinus and frontal sinus.It has the characteristics of local invasion, high recurrence rate and malignant tendency, and most malignant transformation into squamous cell carcinoma. Endoscopic nasal resection and appropriate adjuvant therapy can help to reduce the recurrence rate and inhibit further deterioration. We report the results of a 10-year follow-up of a SNIP patient, including the clinical manifestations, recurrence course and treatment plan during the 10 years. The patient underwent multiple nasal endoscopic surgeries, and had a recurrence of multiple focal attachment pattern, and finally had direct invasion and distant metastasis. Tumor recurrence and further deterioration persisted despite the use of a comprehensive treatment.
Male ; Humans ; Middle Aged ; Papilloma, Inverted ; Follow-Up Studies ; Neoplasm Recurrence, Local ; Head and Neck Neoplasms ; Frontal Sinus

Objective:The aim of this retrospective study is to evaluate the safety and efficacy of tislelizumab in patients with recurrent/metastatic head and neck squamous cell carcinoma. Methods:Six patients with recurrent/metastatic head and neck squamous cell carcinoma who received tislelizumab monotherapy in our hospital from 2018 to 2020 were retrospectively analyzed. The information of sex, age, TNM stage, efficacy, and adverse reactions were collected. All patients were recruited from the RATIONALE 102 study. The primary end point was the objective response rate, and other end points included progression-free survival and overall survival. We performed tumor immune-related gene sequencing and transcriptome sequencing analysis on the tumor tissues of the patient, and used bioinformatics methods to enrich immune cells and analyze signaling pathways. All analyses were performed using R 4.1. 0 software, SPSS Statistics 24.0 software and GraphPad Prism 8. Results:As of May 31, 2020, the median follow-up time was 26.35 months. The objective response rate with tislelizumab was 50.0%, the median progression-free survival was 6.44 months, and the estimated median survival was 20.07 months. The incidence of grade 3 or higher adverse reactions was 66.7%, including hyponatremia, hypokalemia, hypercalcemia, etc. The expression of macrophage, Treg and neutrophil-related genes are higher in immune-sensitive patients, and the signaling pathways of the intestinal immune network for IgA production, graft versus host disease and autoimmune thyroid disease are significantly activated. Conclusion:Tislelizumab was found to be controllable and tolerable in patients with recurrent/metastatic head and neck squamous cell carcinoma. The response to tislelizumab is related to immune cell infiltration and activation of immune-related signaling pathways.
Humans ; Squamous Cell Carcinoma of Head and Neck/etiology* ; Retrospective Studies ; Carcinoma, Squamous Cell/pathology* ; Neoplasm Recurrence, Local/pathology* ; Head and Neck Neoplasms ; Antineoplastic Combined Chemotherapy Protocols

Adenoid cystic carcinoma usually occurs in the salivary glands of the head and neck. It is a malignant tumor with a high degree of malignancy, resistance to radiotherapy and chemotherapy and poor prognosis. The clinical course of adenoid cystic carcinoma is slow and easy to be misdiagnosed. The main diagnosis and treatment means are individualized and precise treatment under the multi-disciplinary consultation mode, that is, surgical treatment and radiotherapy and chemotherapy. Adenoid cystic carcinoma is prone to relapse and hematologic metastasis, and the traditional radiotherapy and chemotherapy based therapies have not achieved satisfactory efficacy in the past three decades. How to detect, diagnose and treat early is an urgent task faced by clinicians.
Humans ; Carcinoma, Adenoid Cystic/pathology* ; Neoplasm Recurrence, Local ; Neck/pathology* ; Oropharynx/pathology* ; Diagnostic Errors

Objective:To investigate the clinical manifestations and treatment of laryngopharynx hamartoma in children. Methods:The clinical data of a child with piriform sinus hamartoma treated in our hospital were analyzed retrospectively. The age, gender, clinical manifestations, auxiliary examination, location of the tumor and surgical methods were analyzed. Results:The patient had a good prognosis after surgery, and no tumor recurrence was found after 1 year of follow-up. Conclusion:Laryngopharynx hamartoma is rare in children. It should be considered in children with laryngeal dysfunction and upper airway obstruction. Complete resection of the tumor is the key to postoperative recurrence.
Child ; Humans ; Hamartoma/surgery* ; Larynx/pathology* ; Neoplasm Recurrence, Local/pathology* ; Pyriform Sinus/pathology* ; Retrospective Studies ; Male ; Female

Objective:To explore the clinical manifestations,the type of pathology, treatment and prognosis of laryngeal rhabdomyosarcoma, and to enhance the understanding of the clinical characteristics of the disease, while improving the diagnosis rateand reducing the misdiagnosis rate, in order to explore effective diagnosis and treatment methods. Methods:A retrospective analysis was conducted on the clinical data of 5 cases of laryngeal rhabdomyosarcoma treated in the First Affiliated Hospital of Zhengzhou University from May 2015 to May 2021. Results:All 5 cases of laryngeal rhabdomyosarcoma were misdiagnosed in the early stage. with tumors mostly occurring in the vocal cords and appearing as smooth mass. The clinical symptoms were mostly hoarseness. According to pathological classification, three cases were embryonic type, one case was polymorphic type, and one case was spindle type.Three patients died due to tumor recurrence, one patient had multiple systemic metastases, and another patient who underwent surgical resection in the early stage and supplemented with postoperative radiotherapy and chemotherapyhas been followed up to date without recurrence. Conclusion:Laryngeal rhabdomyosarcoma has low incidence rate, high malignancy degree and poor prognosis. It is easy to be misdiagnosed as a benign mass. Extensive surgical resection combined with radiotherapy and chemotherapy should be performed as soon as possible after diagnosis.
Adult ; Humans ; Retrospective Studies ; Neoplasm Recurrence, Local/therapy* ; Larynx/pathology* ; Rhabdomyosarcoma/therapy* ; Vocal Cords/pathology*