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 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

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.

We explored clinicopathological features and treatment strategies for thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT). Thoracic SMARCA4-UT is a new entity recently acknowledged in the 2021 edition of World Health Organization Classification of Thoracic Tumors, and doctors are relatively unfamiliar with its diagnosis, treatment, and prognosis. Taking a case of SMARCA4-UT treated in Peking University First Hospital as an example, this multi-disciplinary discussion covered several hot issues on diagnosing and treating thoracic SMARCA4-UT, including histological features, immu- nohistochemical and molecular phenotype, immune checkpoint inhibitor (ICI) therapy, and pathological assessment of neoadjuvant therapy response. The patient was an older man with a long history of smoking and was admitted due to a rapidly progressing solid tumor in the lower lobe of the right lung. Histologically, tumor cells were epithelioid, undifferentiated, diffusely positive for CD34, and partially positive for SALL4.The expression of BRG1 protein encoded by SMARCA4 gene was lost in all of tumor cells, and next-generation sequencing(NGS)confirmed SMARCA4 gene mutation (c.2196T>G, p.Y732Ter). The pathological diagnosis reached as thoracic SMARCA4-UT, and the preoperative TNM stage was T1N2M0 (ⅢA). Tumor proportion score (TPS) detected by immunohistochemistry of programmed cell death 1-ligand 1 (PD-L1, clone SP263) was 2%. Tumor mutation burden (TMB) detected by NGS of 1 021 genes was 16. 3/Mb. Microsatellite detection showed the tumor was microsatellite stable (MSS). Neo-adjuvant therapy was implemented with the combined regimen of chemotherapy and ICI. Right lower lobectomy was performed through thoracoscopy after the two weeks' neoadjuvant. The pathologic assessment of lung tumor specimens after neoadjuvant therapy revealed a complete pathological response (CPR). The post-neoadjuvant tumor TNM stage was ypT0N0M0. Then, five cycles of adjuvant therapy were completed. Until October 2022, neither tumor recurrence nor metastasis was detected, and minimal residual disease (MRD) detection was negative. At present, it is believed that if BRG1 immunohistochemical staining is negative, regardless of whether SMARCA4 gene mutation is detected, it should be classified as SMARCA4-deficient tumors. SMARCA4-deficient tumors include a variety of carcinomas and sarcomas. The essential criteria for diagnosing SMARCA4-UT includes loss of BRG1 expression, speci-fic histological morphology, and exclude other common thoracic malignant tumors with SMARCA4-deficiency, such as squamous cell carcinoma, adenocarcinoma and large cell carcinoma. SMARCA4-UT is a very aggressive malignant tumor with a poor prognosis. It has almost no targeted therapy mutations, and little response to chemotherapy, but ICI is currently the only effective drug. The successful diagnosis and treatment for this case of SMARCA4-UT should enlighten significance for various kinds of SMARCA4-deficient tumors.
Humans ; Immune Checkpoint Inhibitors ; Neoplasm Recurrence, Local ; Lung Neoplasms/genetics* ; Thoracic Neoplasms/pathology* ; Adenocarcinoma ; DNA Helicases ; Nuclear Proteins ; Transcription Factors

Objective: To evaluate the efficacy of supraclavicular fasciocutaneous island flap (SIF) for repairing the defect of parotid or auricle regions after tumor resection. Methods: From February 2019 to June 2021, 12 patients (11 males and 1 female, aged 54-77 years old), of whom 4 with parotid adenoid cystic carcinoma and 8 with auricular basal cell carcinoma underwent reconstruction surgery for postoperative defects in the parotid gland area and auricular area with SIF in the Department of Otorhinolaryngology Head and Neck Surgery, the Second Xiangya Hospital of Central South University and their clinical data were retrospectively analyzed. Size of the SIF, time for harvesting SIF, neck lymph node dissection and postoperative complications were recorded. Results: The flap areas were (6-9) cm × (8-13) cm, and the harvesting time for SIF ranged from 40 to 80 min, averaging 51.7 min. The donor sites were directly closed. All patients underwent ipsilateral levels Ⅰ-Ⅲ neck dissection, with 4 cases undergoing additional level Ⅳ neck dissection and 2 cases undergoing level Ⅳ-Ⅴ neck dissection. Of the 12 SIF, 10 were completely survival and 2 had flap arterial crisis with partial flap necrosis, in addition, 1 had donor site wound dehiscence. With follow-up of 10-42 months, there were no tumor recurrences in 10 patients, 1 patient was lost to follow-up at 10 months postoperatively, and 1 patient experienced local tumor recurrence at 11 months after surgery and died 15 months later. Conclusion: SIF is an easily harvested flap with good skin features matching the skin in parotid and auricle regions and less damage to donor site, and this flap has no need for microvascular anastomosis technique. SIF is feasible and effective for repairing defects in parotid and auricle area.
Male ; Humans ; Female ; Middle Aged ; Aged ; Plastic Surgery Procedures ; Parotid Gland/surgery* ; Retrospective Studies ; Neoplasm Recurrence, Local ; Surgical Flaps/blood supply* ; Skin Transplantation/methods* ; Postoperative Complications ; Treatment Outcome

Humans ; Nasal Cavity ; Nasopharyngeal Carcinoma/pathology* ; Neoplasm Recurrence, Local/therapy* ; Paranasal Sinuses ; Nose Neoplasms/therapy* ; Nasopharyngeal Neoplasms/pathology* ; Paranasal Sinus Neoplasms/pathology*

Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.
Humans ; Male ; Female ; Young Adult ; Adult ; Middle Aged ; Facial Paralysis/etiology* ; Diagnosis, Differential ; Jugular Foramina ; Retrospective Studies ; Cough ; Hoarseness ; Neoplasm Recurrence, Local ; Chondrosarcoma/surgery*

INTRODUCTION: The superiority of laparoscopic repair over open repair of incisional hernias (IHs) in the elective setting is still controversial. Our study aimed to compare the postoperative outcomes of laparoscopic and open elective IH repair in an Asian population. METHODS: This retrospective study was conducted in an acute general hospital in Singapore between 2010 and 2015. Inclusion criteria were IH repair in an elective setting, IHs with diameter of 3-15 cm, and location at the ventral abdominal wall. We excluded patients who underwent emergency repair, had recurrent hernias or had loss of abdominal wall domain (i.e. hernia sac containing more than 30% of abdominal contents or any solid organs). Postoperative outcomes within a year such as recurrence, pain, infection, haematoma and seroma formation were compared between the two groups. RESULTS: There were 174 eligible patients. The majority were elderly Chinese women who were overweight. Open repair was performed in 49.4% of patients, while 50.6% underwent laparoscopic repair. The mean operation time for open repair was 116 minutes (116 ± 60.6 minutes) and 139 minutes (136 ± 64.1 minutes) for laparoscopic repair (P = 0.079). Within a year after open repair, postoperative wound infection occurred in 15.1% of the patients in the open repair group compared to 1.1% in the laparoscopic group (P = 0.0007). Postoperative pain, recurrence and haematoma/seroma formation were comparable. CONCLUSION Elective laparoscopic IH repair has comparable outcomes with open repair and may offer the advantage of reduced postoperative wound infection rates.
Humans ; Female ; Aged ; Incisional Hernia/surgery* ; Surgical Wound Infection/epidemiology* ; Retrospective Studies ; Seroma/surgery* ; Herniorrhaphy/adverse effects* ; Surgical Mesh ; Recurrence ; Hernia, Ventral/surgery* ; Laparoscopy/adverse effects* ; Postoperative Complications/surgery*