Pediatric Orbital Tumors Research Articles

Do Minimally Invasive Approaches to Pediatric Orbital Tumors Provide an Advantage on Outcome and Efficiency?

ObjectiveThe present study aims at evaluating whether minimally invasive approaches to orbital lesions could improve surgical, clinical, and aesthetic outcomes compared with more invasive ones. This is the first study specifically addressing this topic in children. MethodsChildren consecutively operated on from January 2010 to January 2020 were analyzed. Thirty patients matched the inclusion criteria and were divided into Group A: 14 cases, treated with traditional surgical approaches; and Group B: 16 cases, managed by minimally invasive approaches. ResultsThere were no significant differences between the two groups in terms of demographic data and extent of tumor resection. Mean surgical time for the approach (40 min vs 70 min, p < 0.0001), surgical complication such as periorbital edema (37% vs 78%, p = 0.02) and dural tear (0 vs 21%, p = 0.05), and procedures cost (p < 0.0001) were significantly reduced in Group B. About clinical outcomes, Group B showed a significant reduction both in terms of post-operative pain (mean score based on visual pain scale was 2.9 vs 4.1 p = 0.003) and mean hospitalization time (4.5 days vs 5.5 days, p = 0.0004). The aesthetic outcome according to Sloan’s classification was significantly better in Group B as well (81% vs 36% class I patients, p = 0.005). ConclusionThe use of mini-invasive approaches to orbital tumor has clear advantages in terms of surgical, clinical, and aesthetic outcomes in comparable patients; therefore they should be preferred whenever feasible. Craniotomic approaches remain necessary for very large tumors.

Primary extragonadal yolk sac tumor of the orbit: A rare case and review of literature

Germ cell tumors usually arise from primordial cells in gonads, mainly testes, and ovaries. Extragonadal involvement of orbit is very unusual. We report one case of primary yolk sac tumor of orbit in a 3-year-old male child presented as a left lateral orbital mass. Its clinical presentation may mimic many common pediatric orbital tumors causing misdiagnosis which affects ocular morbidity and mortality. Awareness about this rare entity to clinicians is helpful in the early diagnosis and proper management of young kids.

Clinical spectrum and clinicopathological correlation of pediatric orbital tumors: 20 years' experience from a tertiary eye care center.

To study the epidemiological profile of various pediatric orbital tumors and determine their clinicopathological correlation over 20 years. A retrospective analysis of all pediatric patients with orbital tumors from the medical records between 1995 and 2015 was done. Tumors were categorized based on their cellular origin. Demographic details, clinical and histopathological diagnoses, frequency, and clinicopathological correlation were studied. A total of 265 pediatric patients with orbital tumors were analyzed, of which 145 (54.7%) patients were males and 120 (45.3%) were females. The mean age was 7 years (median: 6 years; range: 10 days-16 years). The distribution of lesions in each group was as follows: vasculogenic, 76 (28.7%); lipogenic and myxoid, 66 (25%); myogenic, 31 (11.7%); optic nerve and meningeal, 22 (8.3%); lymphoid and lymphoproliferative, 18 (6.8%); secondary orbital tumors, 14 (5.2%); histiocytic, 9 (3.4%); miscellaneous, 9 (3.4%), lacrimal gland, 8 (3%); mesenchymal with neural differentiation, 6 (2%); osseous, 4 (1.5%), and fibrocystic, 2 (0.8%). The most common benign and malignant pediatric orbital tumors were dermoid cysts (65, 24.5%) and rhabdomyosarcoma (31, 11.7%). A statistically significant ( P < 0.0001) clinicopathological correlation was observed in 208 (83.2%) out of 250 biopsy-proven cases. Pediatric orbital neoplasms include a broad spectrum of benign and malignant lesions. Vasculogenic tumors constitute the majority of them, followed by lipogenic and myxoid tumors. A thorough knowledge of the incidence, clinical profile, imaging features, and histopathology of specific orbital tumors aids in accurate diagnosis and their successful management.

Difficulty in Management of Advanced Pediatric Orbital Tumor

Introduction: Tertiary care centers often manage pediatric orbital tumors, especially in advanced lesions and complex management. We report a case of a young boy with a malignant orbital tumor involving intracranial infiltration. Case presentation: A four-year-old boy had proptosis on the left eye for two months accompanied with blurred vision, pain, and oftenly bleeds with ipsilateral nasal congestion. There was no history of trauma, eye disorders, systemic diseases, or growth disorders. The physical examination showed stable vital signs, however a weak general condition and no enlarged regional lymph nodes. Visual acuity of the left eye was no light perception. There was a mass with protrusion of the left eye, swelling of the eyelid with an irregular surface, and a tendency to bleeds. The cornea was hazy with partly scarring, so we could not evaluate the posterior segment. MRI of the head presented a malignant soft tissue mass of the left orbital region with intracranial infiltration. The patient was given adjuvant chemotherapy; however, he could not survive due to systemic complications. Conclusions: The definitive diagnosis for the orbital tumor is obtained by histopathological examination. The investigation with CT scan or MRI imaging could be considered if not possible. However, since the definitive diagnosis still not assessed, the management can be affected. It is essential to have a definitive diagnosis to provide adequate treatment for the patients. Delayed and inadequate management can make malignant orbital tumors potentially life-threatening.

Craniofacial Approaches to Pediatric Orbital Tumors.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. A total of 9 patients were identified between the years 2008 to 2017. Pathologies included rhabdomyosarcoma, juvenile ossifying fibroma, optic nerve glioma, and retinoblastoma. Surgical approaches to the orbit included supraorbital bar osteotomy (transcranial) or lateral orbitotomy (extracranial). Surgical team members included neurosurgery, craniofacial surgery, and ophthalmology. This study aims to review the role of surgery in management as well as the specific indications for performing transcranial or extracranial osteotomies. It also highlights the excellent access achieved with the use of these osteotomies in certain cases, especially when compared with transconjunctival or transcutaneous approaches.

Paediatric orbital tumours in Upper Egypt: A 3-year retrospective analysis at a university hospital.

Purpose: To evaluate the relative frequencies, clinical profile, and treatment outcomes of paediatric orbital tumours in Upper Egypt. Materials and Methods: In a retrospective, non-comparative, case series study, the authors reviewed the medical records of all children evaluated at Sohag University Hospital, from September 2014 till August 2017, with the diagnosis of orbital tumours and tumour-like lesions. The final diagnosis in each case was established by a combination of history, ophthalmic examination findings, imaging studies, and histopathologic examination if biopsy was performed. Results: A total of 92 orbital lesions were included in this study, which were classified into the following major categories: Cystic (28 cases, 30.4%), vascular (13 cases, 14.1%), Lipomatous (10 cases, 10.9%), inflammatory (9 cases, 9.8%), neurogenic (7 cases, 7.6%), lymphoid/leukemic (6 cases, 6.5%), myogenic (5 cases, 5.4%), lacrimal gland (5 cases, 5.4%), secondary (4 cases, 4.3%), metastatic (3 cases, 3.3%), and histiocytic (2 cases, 2.2%) orbital lesions in order of frequency. The clinical features and treatment outcomes of these lesions will be discussed. Conclusion: A variety of benign and malignant paediatric orbital tumours may be seen in clinical practice. Management of these tumours requires a multidisciplinary approach. Early diagnosis and prompt treatment provide good cosmetic and functional results, and prevent visual loss in the majority of those children.

Pediatric Primary Orbital Tumors in Dr.M. Djamil General Hospital Padang

&#x0D; &#x0D; &#x0D; Introduction : Primary orbital tumors of childhood are different from tumors that occur in adults. Many are congenital with early presentation. In pediatric population, a delay in diagnosis of orbital tumors , even benign tumor can lead to vision loss and deformity. Objective : To report pediatric primary orbital tumors in Dr.M.Djamil Hospital Padang. &#x0D; Method : A retrospective study of orbital tumors in pediatric patients admitted to DR.M.Djamil Hospital between 2003- 2016. Data were obtained from medical records which described patients characteristics based on type, sex,presentation, and management. Result : There were 36 patients of pediatric primary orbital tumors which consist of 20 boys and 16 girls, diagnosis based onclinical sympoms, CT-Scan and pathological examination. The most common tumors in pediatric was Rhabdomyosarcoma in 11 patients (30,55%), Glioma of the Optic nerve in 8 patients (22.22%), Limphoma Malignum in 5 patients (13.88%), Capillary Hemangiomas in 2 patients (5.55%), Cavernous Hemangioma in 2 patients (5.55%), AV Malformation in 2 patients (5.55%), Meningioma in 1 patient (2.77%), Neurofibromatosis in 1 patient (2.77%), Neurilemomoa in 1 patient (2.77%), Limfohemangioma in 1 patient (2.77%), osteoma in 1 patient (2.77%) and Adenoid Cystic Carcinoma in 1 patient (2,94%). Surgery had been performed in 11 patients (30.55%), chemotherapy in 7 patients (19.44%), radiotherapy in 1 patient (2,77%), Combination therapy in 2 patient (5.55%), medical therapy in 1 patient (2.77%) and the remains 14 patients (38.89%) were observed. &#x0D; Conclusion : There were 36 cases pediatric orbital tumors during 14 years. The most common tumor was Rhabdomyosarcoma. Management of pediatric orbital tumors consists of surgery, chemotherapy, radiotherapy and combination therapy. &#x0D; &#x0D; &#x0D;

A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed.

Magnetic resonance imaging in the analysis of pediatric orbital tumors: Utility of diffusion-weighted imaging

To identify common radiographic features of pediatric orbital tumors by the use of magnetic resonance imaging (MRI) techniques, diffusion-weighted imaging (DWI), fat saturated T2, and pre- and postgadolinium T1. DWI is hypothesized to help identify and predict the malignancy of specific brain tumors. To our knowledge, a similar analysis in which the authors have used this combination of MRI techniques has not been performed with orbital tumors. We performed a retrospective chart review of all patients younger than 18 years of age, each diagnosed with an orbital mass lesion, imaged by MRI from 2005 to 2008. The MR images were analyzed by use of the aforementioned techniques. Mass lesions identified in the chart review included rhabdomyosarcoma (n = 4), myofibroma (n = 2), hemangioma (n = 4), lymphangioma (n = 2), neurofibroma (n = 4), Langerhans histiocytosis (n = 2), and one of each of the following: giant cell tumor, meningioma, lymphoid hyperplasia of the lacrimal gland (chronic sclerosing sialadenitis), optic nerve glioma, lipodermoid, and dermoid. DWI was used to differentiate tumors into those with increased diffusion, restricted diffusion, and a mixed diffusion pattern. Capillary hemangiomas and rhabdomyosarcomas 2 tumors with potentially overlapping appearances with traditional MRI techniques had contrasting appearances with DNI. DWI can help to distinguish among certain pediatric orbital tumors when combined with traditional MRI techniques. This technique may thus be considered an additional tool to help, refine the differential diagnosis of orbital tumors in children.

Malignant solitary fibrous tumour of the orbit

We aimed to report a case of orbital solitary fibrous tumour (SFT) in a child and to review the relevant literature. We describe an SFT in a 13-year-old boy with a 1-month history of painless proptosis in the left eye. Magnetic resonance imaging revealed a well circumscribed mass filling most of the left intraconal orbit. The lesion was excised and histopathological examination revealed a malignant SFT. Postoperative follow-up for 18 months was uneventful. Malignant SFT of the orbit should be included in the differential diagnosis of paediatric orbital tumours. Complete surgical excision remains the preferred method of management and the longterm prognosis is guarded.

Urgences tumorales orbitaires pédiatriques

Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.

Laser Accelerated Radiotherapy: Is It On Its Way to the Clinic?

radiation. Loma Linda (Calif.) University Medical Center oncologists are using protons to treat early stage lung cancers, pituitary adenomas, pediatric orbital and ocular tumors, acoustic neuromas, benign meningiomas, and prostate cancer. To target therapy and minimize tissue damage, radiation oncologists exploit a proton beam characteristic called the Bragg peak. A Bragg curve plots how ionizing radiation loses energy as it travels through matter, in this case, tissue. For accelerated protons, the energy curve peaks sharply as the particle stops, releasing its power like a well-placed punch. This so-called Bragg peak effect provides “normal tissue sparing superior to other radiotherapies,” said Albert Einstein’s Kalnicki.

025: Diffusion weighted imaging in the analysis of pediatric orbital tumors

Purpose: To identify common radiographic features of pediatric orbital tumors using magnetic resonance imaging (MRI) techniques, particularly diffusion weighted imaging (DWI), fat saturated FSE T2 and pre- and post-gadolinium T1. DWI is hypothesized to help identify and predict the malignancy of specific brain tumors. To our knowledge similar analysis has not been performed with pediatric orbital tumors.

Orbitaerkrankungen im Kindesalter

Orbital diseases in childhood are rare and differ from orbital lesions occurring in adults. Most paediatric orbital tumours are benign, but rapid tumour growth can nevertheless threaten vision or lives. Retrospectively, we analyzed data of 41 consecutive patients with orbital disease, treated in our hospital between 2000 and 2004. The data was evaluated using descriptive statistics and case reports. All children (from neonatals up to 16 years of age) with orbital disease were included in the study. The most common benign disease of the entire collective was an epidermal cyst (n = 18), followed by orbital capillary haemangioma (n = 4) in children under 3 years, and preseptal cellulitis in children older than 3 years. 5 patients were treated for malignant tumours, of which one patient under 3 years had an orbital metastasis of an abdominal neuroblastoma. Awareness of orbital diseases in childhood, as a rare entity, is crucial for timely diagnosis and appropriate treatment, to save the vision and lives of affected children.

Pediatric Tumors of the Orbit and Optic Pathway

We present an overview of the treatment and clinical outcome of 30 pediatric patients with orbital and optic pathway tumors cared for in our center from 1991 to 2002. Follow-ups were available for 48 months on average. Eight of 14 gliomas of the optic nerve or pathway were subtotally resected via a pterional approach. Two were totally resected with transection of the optic nerve, 2 were only biopsied and 2 were observed. One of 4 optic nerve sheath meningiomas in patients with good visual acuity was subtotally resected with decompression of the optic canal. Two were totally resected with transection of the prechiasmal optic nerve and 1 was only biopsied. Four infectious intra-/extraconal lesions were biopsied (transconjunctival approach) and treated with antibiotics. Two vascular intra-/extraconal tumors (hemangiomas) were removed via a transconjunctival approach and a lateral orbitotomy. The following histologies only occurred once: dermoid cyst, aneurysmal bone cyst, eosinophilic granuloma, fibrous dysplasia, osteopetrosis and rhabdomyosarcoma. The spectrum of pathology in pediatric orbital tumors is wide and requires multidisciplinary treatment.

Recurrent orbital solitary fibrous tumor in a 14-year-old girl

PURPOSE: To report a case of orbital solitary fibrous tumor in a pediatric patient. METHODS: Case report and review of the literature. RESULTS: A 14-year-old girl presented with a 5-month history of painless proptosis of the left eye. Magnetic resonance imaging revealed a well-circumscribed mass in the anterior superomedial left orbit. The lesion was excised, and histopathologic examination revealed a solitary fibrous tumor. The lesion recurred in the orbit 4 months postoperatively, and histologic examination of the new lesion was consistent with solitary fibrous tumor. CONCLUSION: Based on this case report of orbital solitary fibrous tumor in a pediatric patient, solitary fibrous tumor should be included in the differential diagnosis of pediatric orbital tumors.

PEDIATRIC ORBITAL TUMORS

A child presenting to the ophthalmologist with proptosis can be a frightening experience for all involved. The child may be uncomfortable and uncooperative, the parents anxious, and the clinician aware that the differential diagnosis includes several life-threatening diseases. Differing substantially from the adult patient, the pediatric patient with an orbital tumor has a greater incidence of congenital lesions, higher frequency of infection, and unique benign and malignant tumors that involve the orbit. Armed with familiarity with all these disease processes and aided by careful physical exam and ancillary studies, the clinician must provide a prompt diagnosis and initiate treatment planning. This article reviews pediatric orbital tumors, their presentation, characteristics, and treatment approaches.

Pediatric orbital tumors in Turkey.

Pediatric orbital tumors in Turkey.

Pediatric orbital tumors in Turkey.

A retrospective review of 376 pediatric orbital tumor cases seen at the Ankara University Eye Clinic between 1963 and 1993 was undertaken. The diagnosis was made histologically in every case. Secondary tumors accounted for 127 cases (33.8%), cystic lesions for 82 cases (21.8%), rhabdomyosarcomas for 23 cases (6.1%), vascular lesions for 21 cases (5.6%), inflammatory lesions for 21 cases (5.6%), lymphoma and leukemias for 18 cases (4.8%), other mesenchymal tumors for 11 cases (2.9%), metastatic tumors for 5 cases (1.3%), traumatic foreign bodies for 2 cases (0.5%), and lacrimal fossa lesions for 1 case (0.3%). The most common benign orbital tumors were the cystic lesions. The most common primary malignant tumor was rhabdomyosarcoma. Overall, the most frequent orbital lesion was the secondary orbital invasion of retinoblastoma.

Pediatric orbital tumors.

Pediatric orbital tumors.