Congenital orbital teratoma: a case report with preservation of the globe and 18 years of follow-up

Background Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration. Case presentation We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up. Conclusion Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.

Roles of Surgery in the Treatment of Patients With High-Risk Neuroblastoma in the Children Oncology Group Study: A Systematic Review and Meta-Analysis

Purpose: Neuroblastoma is the most common extracranial solid tumor in children, and most patients are at high risk when they are initially diagnosed. The roles of surgery and induction chemotherapy in patients with high-risk neuroblastoma have been a subject of much controversy and debate. The objective of the current study was to assess the roles of surgery in high-risk neuroblastoma.Method: The review protocol was prospectively registered (PROSPEROID: CRD42021253961). The PubMed, Embase, Cochrane, and CNKI databases were searched from inception to January 2020 with no restrictions on language or publication date. Clinical studies comparing the outcomes of different surgical ranges for the treatment of high-risk neuroblastoma were analyzed. The Mantel–Haenszel method and a random effects model was utilized to calculate the hazard ratio (95% CI).Results: Fourteen studies that assessed 1,915 subjects met the full inclusion criteria. Compared with the gross tumor resection (GTR) group, complete tumor resection (CTR) did not significantly improve the 5-year EFS [p = 1.0; HR = 0.95 (95% CI, 0.87–1.05); I2 = 0%], and the 5-year OS [p = 0.76; HR = 1.08 (95% CI, 0.80–1.46); I2 = 0%] of patients. GTR or CTR resection had significantly better 5-year OS [p = 0.45; HR = 0.56 (95% CI, 0.43–0.72); I2 = 0%] and 5-year EFS [p = 0.15; HR = 0.80 (95% CI, 0.71–0.90); I2 = 31%] than subtotal tumor resection (STR) or biopsy only; however, both CTR or GTR showed a trend for more intra and post-operative complications compared with the STR or biopsy only [p = 0.37; OR = 1.54 (95% CI, 1.08–2.20); I2 = 0%]. The EFS of the patients who underwent GTR or CTR at the time of diagnosis and after induction chemotherapy were similar [p = 0.24; HR = 1.53 (95% CI, 0.84–2.77); I2 = 29%].Conclusion: For patients with high-risk neuroblastoma, complete tumor resection and gross tumor resection of the primary tumor were related to improved survival, with very limited effects on reducing intraoperative and postoperative complications. It is necessary to design strong chemotherapy regimens to improve the survival rate of advanced patients.Systematic Review Registration:https://www.crd.york.ac.uk/PROSPERO/, PROSPEROID [CRD42021253961].

Gnathic (Mandibular) Osteosarcoma in a Middle Adolescent-A rare case report

Gnathic Osteosarcoma (GOS) is a subtype of osteosarcoma (OS) that primarily affects the maxilla and mandible. An accurate diagnosis, usually facilitated by Computed Tomography (CT), Magnetic Resonance Imaging (MRI), and Biopsy, is required to define the stage of the disease and plan adequate treatment. Early diagnosis and complete tumor resection are mandatory to improve the prognosis of GOS. We present a case of GOS in a 17-years-old female based on clinical-radio-pathological correlation.

A pure extradural hemangioblastoma mimicking a dumbbell nerve sheath tumor in cervical spine: illustrative case

BACKGROUND Spinal extradural hemangioblastomas (HBs) are quite uncommon, with most reported cases involving the thoracic and lumbar areas. Therefore, the presence of a dumbbell-shaped pure extradural cervical HB is exceptional, making preoperative diagnosis particularly challenging. OBSERVATIONS The authors report a case of a 27-year-old woman who presented to their outpatient clinic with progressive cervicobrachialgia and numbness in the left arm. Magnetic resonance imaging showed a C5–6 intradural extramedullary lesion, and, despite some atypical features, the diagnosis of a possible neurogenic tumor was made. A multidetector computed tomography scan and angiography confirmed the expansion and remodeling of the left neural foramen as well as the highly vascularized nature of the mass. Preoperative embolization of the lesion was performed. Complete tumor resection was accomplished, followed by a C5–6 posterior fusion. Histopathology and immunohistochemistry revealed an HB. LESSONS HBs should be considered among the differential diagnosis of cervical extradural tumors. Exhaustive preoperative workup and surgical planning are decisive in order to attain gross-total resection with favorable outcomes.

Predicting short-term survival after total resection in glioblastomas by machine learning-based radiomic analysis of preoperative MRI

Background Radiomics, in combination with artificial intelligence, has emerged as a powerful tool for the development of predictive models in neuro-oncology. Our study aims to find an answer to a clinically relevant question: is there a radiomic profile that can identify glioblastoma (GBM) patients with short-term survival after complete tumor resection?

A Rare Case of a Congenital Nasopharyngeal Ganglioglioma With Dyspnea in a 1-Month-Old Male Infant: A Case Report

Background: A ganglioglioma (GG), a tumor with both neuronal and astrocytic components, rarely occurs outside the central nervous system.Case Summary: We present the first reported case of a 1-month-old male with a congenital nasopharyngeal GG, nasal congestion, and dyspnea; we include the operative video. Magnetic resonance imaging was used to explore whether the tumor communicated with the intracranial space. We used an endoscopic plasma technique to ensure complete tumor resection. This afforded a good visual field, endoscopic magnification, and good hemostasis.Conclusions: We report a rare case of a nasopharyngeal GG triggering nasal congestion and dyspnea in a 1-month-old male, and report our experience with the treatment of nasopharyngeal GG and similar diseases.

Giant mucinous carcinoma originating from the appendix: A case report

Introduction Appendiceal mucinous neoplasms can involve peritoneal pseudomyxoma or invasion of adjacent organs. This report describes a rare case in which a giant appendiceal mucinous carcinoma expansively developed in the retroperitoneum without perforating the abdominal cavity. Case presentation The patient was a 55-year-old woman with no relevant history who was admitted to our hospital after imaging examinations revealed a retroperitoneal tumor. The clinical diagnosis was a retroperitoneal tumor involving the hedge mucin. The patient underwent right hemicolectomy and partial resection of the duodenum and right abdominal wall to ensure complete tumor resection. Histopathological findings suggested that the tumor was a mucinous carcinoma arising from the appendix. The postoperative course was uneventful. Although adjuvant chemotherapy was performed for 6 months, peritoneal recurrence developed 7 years and 4 months postoperatively. Nine years have passed after surgery and the patient is alive under receiving chemotherapy. Conclusion Detailed pathological examinations revealed that the tumor originated from the appendix. The characteristics of mucinous carcinoma contributed to the extensive growth of the tumor.

Case report: surgical resection of right ventricular cardiac fibroma in an adult patient

Background Cardiac fibromas are rare benign cardiac neoplasms, most frequently occurring in the pediatric population; with very rare cases identified in adults. The tumors are comprised of spindled cells with myofibroblastic ultrastructural features embedded in generally collagenous and elastic stroma. The tumors are intramural in the ventricles, most commonly the left ventricle. Clinical symptoms vary by location and size of tumor and some are asymptomatic. Surgical resection is curative, but rare cases require cardiac transplantation. Case presentation We report an asymptomatic, large, right ventricular fibroma in a 64-year-old woman. The patient underwent open incisional tumor biopsy via lower hemi-sternotomy, followed by complete tumor resection via full sternotomy a week later after confirming the tumor is benign. The tumor was resected using cardiopulmonary bypass, and the defect of right ventricular free wall was repaired using a prosthetic double-patch technique. The postoperative course was uneventful. The patient was discharged to home on day 4 post-complete tumor resection. Conclusion This report expands the existing literature for better comprehension and detection of cardiac fibroma patients and also highlights the various imaging modalities, surgical management, and histological analysis.

Solitary fibrous tumor of the pleura with Doege-Potter syndrome.

Solitary fibrous tumor of pleura (SFTP) is a rare tumor. Complete tumor resection is the curative treatment and the key to preventing recurrence. We present here the case of a 65-year-old female diagnosed with a Doege-Potter syndrome who underwent resection of pleural solitary fibrous tumor.

Management of a primary cardiac leiomyosarcoma in a young woman

Cardiac leiomyosarcoma is an extremely rare tumor with a poor prognosis. An 18-year-old female patient was admitted to our clinic with a left atrial leiomyosarcoma extending to the right lower pulmonary veins. We performed complete tumor excision by the right anterolateral mini-thoracotomy approach using minimally invasive techniques. After pathological confirmation of the tumor, right lower lobectomy was performed with the same incision one week later to prevent recurrence. Although no tumor remnant was found in the lobectomy specimen, adjuvant chemotherapy was started. No recurrence was detected during the 12-month follow-up. In conclusion, the right submammarian minithoracotomy approach has the advantages of its less invasive nature and suitability for complete tumor resection with lobectomy.