Lecture: Malignant Orbital Tumors of Childhood

During this live webinar, we will discuss the evaluation, diagnosis, and management of malignant orbital tumors in childhood. Rhabdomyosarcoma, metastatic neuroblastoma, secondary retinoblastoma, and others will be discussed. Physicians will learn to recognize clinical signs, perform a focused evaluation, and become familiar with managing these cancers. (Level: Beginner and Intermediate)

Lecturer: Dr. Thomas Johnson, Ophthalmologist, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, USA


>> THOMAS JOHNSON: Good morning. I’m going to be talking about an update on malignant orbital tumors of childhood. I have no financial closures. So proptosis in childhood can be very concerning. It can be due to infectious orbital cellulitis, injuries, but we’re going to concentrate on malignant tumors. Most are benign, a majority are cystic lesions or vascular lesions. When we evaluate a child who has proptosis, we have to take a careful history, look at vital signs, do a careful eye exam, and of course a systemic exam looking for skin lesions, masses, congenital anomalies. So malignant orbital tumors of childhood I’ll discuss will be the following. Rhabdomyosarcoma, metastatic tumors, which I’ve listed, and secondary retinoblastoma. You can see this child has downward displacement which was a rhabdomyosarcoma. It’s very tumor involving the bone, mum, or other connected tissue. Rhabdo means rod-shaped. Myo is muscle. Sarcoma is a type of cancer. So during fetal development, rhabdo myoblasts, this is a child who presented to me with a rhabdomyosarcoma, downward displacement of his right eye. Painless progressive proptosis. And this is the most common malignant orbital tumor of childhood. It comprises about 5 to 8% of childhood cancers. The average age of onset is somewhere between 7 and 8. Patients usually present with a painless progressive proptosis. There’s a predilection for the superior orbit especially in the embryonal subtype. Even though rhabdomyosarcoma is a malignant tumor, the survival rate is over 90% if it’s caught when the tumor has not progressed. So the presentation, there’s rapid onset of progressive painless proptosis, ptosis, chemosis, decreased motility, and can present in the alveolar subtype. This is a patient with a history of slowly progressive proptosis of the right eye, without pain. So the differential diagnosis includes orbital cellulitis, idiopathic orbital inflammation, neuroblastoma, which is often bilateral, ruptured dermoid sift, Langerhans history EEO cytosis and leukemic infiltrate. Some of the other things which can cause orbital tumors include infantile hemangiomas, benign tumors composed of tissue found at the site. They grow rapidly during the first 1 to 2 years of life and spontaneously involute by age 5 to 7 years. This child has multiple hemangiomas but one large lid and orbital mass, as you can see here. This is a CT scan. We usually don’t do CT scans at this point. This is an older patient. But you can see there’s this large lesion involving the orbit extending toward the apex that enhances readily with contrast due to the high vascularity of this hemangioma. Lymphangiomas can also present with the acute onset of proptosis. These are benign ones as well. Again, patients may present with a rapid onset of proptosis and ecchymosis, especially after trauma. These tumors can infiltrate into normal structures. They’re not encapsulated. So they are very difficult to treat and excise. They may grow infections. And they are impossible to completely excise. So we have different treatments for them these days, usually we inject these tumors with sclerosing agents. This patient as a young boy with acute onset of proptosis. You can see bluish discoloration of his left lower lid and on his CT scan he has this multilobulated lesion. These days the choice is to aspirate the fluid and inject a sclerosing agent. So they can present acutely with proptosis, kind of similar to rhabdomyosarcoma. This is a patient that had a known lymphangioma of his right orbit, after he had some trauma playing football, with acute hemorrhage into the lymphangioma with bleed. This can be dangerous because it compresses the optic nerve and it needs to be treated immediately. Plexiform neurofibromas can involve the upper eye lit and anterior orbit. They’re vascular, infiltrate structures. They’re impossible to completely excise due to the infiltrative nature of these lesions. This is a young girl with a large plexiform neurofibroma. She has the absence of the greater wing of sphenoid as well. Optic nerve gliomas can also present with slow progressive proptosis, painless. They may be associated with neurofibromatosis. They often cause strabismus and visual loss. Treatment is surgery when threatening to extend intracranially and they are usually excised. So this is a patient with that tumor. Here is a patient with optic nerve glioma. You can see the proptosis of the left side. And on the scan you can see this tumor of the optic nerve. And this is another patient with an effusive form optic nerve glioma of the right orbit. Eosinophilic granuloma is another tumor that can be mistaken for the tumor, it’s a benign destructive tumor. It usually occurs in the superolateral orbital rim. The treatment usually involves curettage and sometimes chemotherapy. This young patient was referred to me with a diagnosis of rhabdomyosarcoma. You can see this patient has proptosis of the right eye. On his CT scan he has this large orbital mass and eroding bone at the orbit. This ended up being an eosinophilic granuloma, treated with curettage and steroids. I’ll just show you some of the classic histopathology of these eosinophilic cells, you can see the eosinophils here and the histiocytes in this area. Fibrous dysplasia is a benign fibro-osseous hamartoma. It’s thought to be due to the interruption of maturation of bone at the woven stage. It can be monostotic or polyostotic. This is a young boy that had a slow, painless proptosis of his left eye that you can see. On his scan here, CT scan, without contrast, you can see this large bony mass involving the skull base and the lateral orbital wall. On the bone window, you can see it has a ground glass appearance which is characteristic of fibrous dysplasia. So let’s get back to rhabdomyosarcoma. We’ve talked about the things that can be mistaken for it. One of the risk factors for developing this most common tumor of childhood, there are thought to be several. Radiation exposure in utero, accelerated fetal growth. There has been a study about parents taking recreational drugs during the pregnancy such as cocaine and marijuana that may be a risk factor. Lower socioeconomic status may be a risk factor as well as antibiotic therapy soon after birth. There are also heritable syndromes associated with rhabdomyosarcoma. You can see there’s multiple syndromes that can be also associated with rhabdomyosarcoma. So the study on the parents’ drug use is right here. This gives a two to five-fold increase of rhabdomyosarcoma when the parents used cocaine and marijuana during pregnancy, during the year preceding the child’s birth. This was published several years ago. So imaging for rhabdomyosarcoma, we can do a CT scan or an MRI scan. These days we mostly do MRI scans just because of the less risk of radiation to the growing tissues in these patients. So MRI scan is preferred. When I first started working, we did CT scans often, because MRI scans were not that available. So some of my slides do have CT scans. But MRI scan is really the imaging modality of choice. On MRI, these tumors are relatively isodense on T1 weighted images and slightly hyperintense on T2. Here you can see T1 and T2 of rhabdomyosarcoma of the right orbit. You can see here it’s iso intense on the T1 weighted and slightly hypertense on T2. This is the CT of an embryonal rhabdomyosarcoma. The thing important to note is the tumor usually in its early stages does not erode bone, it actually interacts very benignly with the bone. There’s no bone erosion and so forth. It does have a mass effect causing proptosis. It conforms to the shape of the bone. It has a predilection for the superior orbit in the most common subtype which is the embryonal subtype. These tumors can enhance readily with contrast. This is a patient we saw recently who had slowly progressive proptosis of his left eye. He had an intraocular pressure of 31. Pupils were normal. He had proptosis. He had lateral displacement of his eye. You can see he has some meld erythema. On the scan of this patient, you can see T1 with and without contrast. Again, a very large medial mass extending deep into the orbit which does enhance with contrast administration. So the management of rhabdomyosarcoma includes first a thorough history and physical examination. We as ophthalmologists perform a prompt biopsy. If we can safely debulk the lesion without damaging the adjacent structures, we can do that. Then we refer the patient as soon as possible to pediatric on college for systemic workup as well as consideration for radiation therapy. When we do the biopsy, usually we find a small blue cell tumor. On this patient we did a lid crease incision, took a biopsy, we see a small blue cell tumor. That can narrow the differential down to rhabdomyosarcoma, neuroblastoma, as well as several other tumors including Ewing’s sarcoma, lymphoma, retinoblastoma. So we usually just make a skin crease incision, do a biopsy, do a frozen section at the time we do a biopsy so we get a good idea that we are intralesional and we have a small blue cell tumor. You have the patient’s pediatric oncology as we’re waiting for the final pathology to arrive. The histopathology is interesting. You see round to oval hyperchromatic nuclei. We use immunostains often to make this diagnosis. These are some slides of the histopathology. You can see these tad pole shaped cells with this loose eosinophilic cytoplasm, and this streaming cytoplasm. You can see on this lower power view, abundant eosinophilic cytoplasm, hyperchromatic nuclei as well. So we use immunohistochemistry often in making the diagnosis on these patients. So the stains we usually use are myogen stain, you can see it’s staining positive on this slide, and desmin stain. These are markers for rhabdomyosarcoma that helps differentiate this from other blue cell tumors of the orbit. Other syndromes or other things can appear to be rhabdomyosarcoma that aren’t. It can masquerade as a chalazion. She has a mass in this area. We were suspicious that this was not a chalazion, so we did an imaging study. One can see she has a mass extending into the orbit. This ended up being an alveolar rhabdomyosarcoma, the second most common subtype of this tumor. So the two main histological subtypes are the embryonal, the most common at 80%, with a higher incidence in young children, and alveolar, less common at 20%. The survival rate is usually lower. I’ll get into that in a few minutes. The pleomorphic subtype is benign, it’s usually in adults. So one thing we look at these days for prognosis of these studies is fusion genes. The alveolar rhabdomyosarcoma, the cells demonstrate unique chromosomal translocation. So to study this we do an FISH study, fluorescence in situ hybridization. One study found the gene present in 25 cases of alveolar, eight in embryonal, and one of neuroblastoma. So this is, again, the signature genetic change that’s found in alveolar rhabdomyosarcoma. The fusion gene results from this reciprocal translocation of chromosomes 2 and 13. Staging of rhabdomyosarcoma tumor is the following. If it’s a group 1 tumor, that means it’s a localized disease completely resected. So if we had a tumor just in the orbit that we could completely resect, that would be group 1. Group 2, if there’s regional disease, grossly resected. And that could be an orbital mass where we were able to remove the majority of it. But not the whole thing. Group 3 is gross residual disease after surgery. A lot of the orbital rhabdomyosarcoma patients are group 3 patients. Group 4 are those patients with metastatic disease. So the treatment is based on the clinical staging that we just talked about. Group 1 patients may just receive chemotherapy only. Group 2 may also receive chemotherapy and some radiation therapy in a lower dose of 36Gy. Group 3, if there’s gross residual disease, a very common outcome after we biopsy these very large orbital masses, about 45GY. And group 4, with distant metastases present at onset, you have the combination of intensive chemotherapy and radiation therapy. So the chemotherapy regimens vary. There’s a lot of new treatments coming down the line. This is coordinated with the pediatric oncologist. The traditional agents are VAC, newer agents are being used like etoposide/fosfamide, topotecan, and other ones in higher risk/refractory cases. Radiation therapy, usually 4500 to 5,000Gy fractionated doses. Proton beam, when we can use it. Exenteration isn’t used very much anymore. In the ’60s and so forth, patients were on any exenterated with a survival rate very low. These days we only use it for these cases, refractory chemotherapy and radiation therapy. The survival rate can be 71% at three years when the tumor is confined to the orbit, when it’s also confined with chemotherapy. So proton beam therapy is a new type of ionizing radiation using protons. It has very little lateral scatter to the brain tissue, to the pituitary gland. So the tissues deep to the tumor receive very few of the protons, so a very small amount of radiation. So this reduces the risk of secondary malignancy. It tends to be a more expensive type of treatment, there’s only a few centers in the world that have this. And it can be expensive. So the intergroup Rhabdomyosarcoma Study Group defines standard therapy at VIC. High risk groups, again, are receiving these newer agents that we talked about. Bone marrow transplant has failed to improve the prognosis in those patients with distant metastases. So the prognosis, again, is excellent if the tumor is treated while still confined to the orbit. Survival rate is over 93% at five years. Embryonal rate has the best survival rate. And it comprises about 80% of cases. And the five-year survival rate of embryonal is 94%. Alveolar cell has the worst prognosis with a five-year survival rate of about 74%. This is a patient that I took care of that actually had a group 1 disease. This patient had a rhabdomyosarcoma, inferior orbit, yet we were able to completely excise without residual disease. These are rare but they do occur. The treatment side effects are usually due to the radiation therapy. And it’s confined to the orbit. Patients felt dry eye, corneal scarring, cataract, most commonly. Very often these patients need cataract surgery later on. It’s very rare for them to develop radiation retinitis and optic neuropathy with radiation treatments these days. In the past, sometimes radiation would actually result in a blind, painful eye, and the patient would develop neo-vascular glaucoma. But with newer radiation protocols, especially proton beam, it gives less collateral damage, less radiation to the pineal gland, less risk for secondary tumors. So what’s new in rhabdomyosarcoma, newer molecular inject techniques allow us to know which patients will respond to different treatments. Better radiation treatments, including proton beam. Limiting chemotherapy for lower risk tumors. And newer medications, IGF-1 receptor inhibitors, drugs that inhibit the tumor’s ability to make blood vessels, such as Avastin, and targeting the mTOR protein and ALK protein are being tried for treatment of rhabdomyosarcoma. I’m going to go on to another tumor that is less common but is also one we see in ophthalmology, metastatic neuroblastoma, a tumor that is metastatic tumor from another area. These patients usually present early in life, in the first two years of life. The most common origin of the primary tumor is the adrenal gland. And these tumors can also originate in the retroperitoneal area and the ganglia. Oftentimes these patients have bilateral presentation. This patient had metastatic ganglia neuroblastoma. She presented with proptosis and ecchymosis of the eyelids. These tumors grow rapidly, outgrow their blood supply and cause bleeding and some ecchymosis in the eyelid. You can see on her scan, this tumor is pretty extensive. It metastasized to the bone of the lateral orbit and extends up her skull. This was the biopsy that we took showing these ganglia cells and these neuroblastoma cells intermixed. This was a ganglial neuroblastoma. It’s very common in neuroblastoma to cause bilateral metastases. These patients have eyelid ecchymosis when they present with proptosis. Not all of them have this, of course. Urine catecholamines often present. The prognosis has improved recently due to better chemotherapy and radiation regimens. There may be a chromosome genetic variation associated with this disease. This is an 18-month-old child I saw who came to me with ecchymosis and proptosis of his left eye, brought in by his parents. You can see this patient has a tumor that has a predilection for the superolateral orbit. The bone is increased in density and size. The tumor extends into the orbit as well as intracranially in this one spot. You can see this ecchymosis a little more carefully. Biopsied, it’s small blue cell tumor. Again, when we do a frozen section, you usually get the diagnosis of a small blue cell tumor. Further studies are needed to differentiate this tumor. The most common place of origin of the tumor is the adrenal gland. You can see this is the mass here in the abdomen which was the origin of the metastatic neuroblastoma. So this is another child I saw who had kind of a similar picture. He had a hemorrhage, ecchymosis of his lid on the right side, proptosis. You can see this outward and downward displacement of his right eye. A young child, less than 2 years of age, presented like this with a history of incidental trauma which was of course not related to this. When we look at his scan, again, this is a patient from many years ago so we had a CT scan, we see this patient has bilateral orbital masses, involvement of the skull base, and also temporalis muscle, you can see swelling on this side and the bilaterality of this tumor. This is metastatic neuroblastoma from the abdomen. So the patient was treated with chemotherapy and radiation therapy. And at this stage, usually the prognosis historically has been poor, but it has improved in recent years due to better regimens for chemotherapy and radiation therapy. So very rarely metastatic neuroblastoma can be seen at birth. This is a patient that I was called to the neonatal intensive care unit. This patient was born with this large mass. You can see the eye is completely proptotic, the eye is being compressed by this huge lesion. We actually took this lesion out. It was a metastatic neuroblastoma. This patient had multiple metastases, and unfortunately passed away due to his disease. So the treatment of this tumor is stratified based on the clinical features, age, and staging. These days it’s a combination of keep owe, radiation, surgery, stem cell transplant, immunotherapy. Many chemotherapy agents are used, I’ve listed them here, some of the same ones as in rhabdo. Cyclophosphamide, vincristine. Targeted specific genetic molecular therapy is being used for those patients. The survival right is about 85%. In children greater than one year, 45%. A couple of other tumors that are much less common include the Ewing’s sarcoma group of tumors. These are also included in the family of primitive peripheral neuroectodermal tumors. These include Askin tumors, one of the tumors that commonly affects the orbit. So this is one patient I saw with a peripheral neuroectodermal tumor in a young teenaged girl. Again, this is also systemic metastatic disease. Oftentimes these occur primarily in the long bones of the limbs and metastasize. Again, treated with chemo and radiation therapy. Ewing’s sarcoma can involve the orbit. It’s rare, mainly metastasis from a distant site. Survival rate is about 50% in these patients. Treated with chemotherapy and radiation. An interesting tumor is granulocytic sarcoma. It can be bilateral. More commonly unilateral. It’s a variant of acute myeloid leukemia, AML. It can also be known by the name of chloroma because in the tumor cells there is mieloperoxidase and that imparts a green hue. This tumor can present either before the onset of leukemia, during the treatment of leukemia, or even after the leukemia has been diagnosed. For some reason this is more common in the Middle East and Asia and Africa. So I’ve seen several of these patients. This was a patient who presented to me with slowly progressive proptosis of his right eye. He had no diagnosis before of any systemic disease. You can see his eye is pushed out and down, and he has quite a bit of proptosis, chemosis. When I did a CT scan you can see he has this very large orbital mass and smooth borders extending down into the apex of the orbit. This was biopsied and found to be a granulocytic sarcoma. These tumor cells have small cytoplasmic granules. Histopathology of this, again, the small blue cell tumor which was a granulocytic sarcoma. You can see the stain positive for these granules in the tumor cells. We just had one similar patient recently at Bascom Palmer, a history of leukemia in a 15-month-old female. She presented with bilateral proptosis, periorbital edema. As you can see in this clinical slide and on her MRI scan, you can see bilateral orbital masses extending back deep into the orbit. Again, a very smooth appearance similar to that last patient we showed. And so AML is a type of leukemia that only represents about 20% of acute pediatric leukemias. ALL is much more common. The survival rate is about 70%. The treatment of this tumor includes chemotherapy, including four to five cycles. These are some of the agents that are used. Stem cell transplant is often used in these patients. And then gene mutation targeted therapies are now being applied based on next generation sequencing. This is that same patient showing a CT scan of this tumor on the right side. So again, there’s oftentimes bilateral involvement in this disease as well. You can see bilateral orbital masses in this patient with acute leukemia. Again, on the scan you can see these large bilateral masses and also involvement of the skull base in this patient. So this patient was treated with chemo and is doing well at this point. Secondary tumors are those tumors that are defined as tumors that extend into the orbit from other structures. They can extend from the perinasal sinuses, from the skin, from the nose. They’re also considered tumors that extend from the eye into the orbit. Secondary retinoblastoma is a type of tumor that starts in the eye and then left untreated, it can erode through the eye and extend to the orbit. And so these are usually neglected cases with delayed presentation. The prognosis is poor. The treatment is mainly palliative. When I worked in Saudi Arabia many years ago, this was the most common malignant tumor in childhood in Saudi Arabia. Also there was a study of Pakistan that showed in that location it was also the most common malignant orbital tumor. This is a young girl that I saw when I was in Saudi Arabia who had a very large retinoblastoma of her left orbit. You can see this tumor extending out quite far. This really destroyed the ocular structures, you can see here a very large orbital mass extending back towards the apex and no real recognizable ocular structures. She also has retinoblastoma of her right eye, pretty extensive, you can see on the CT scan here. She has diffuse calcifications in her right eye. So again, this portends a very poor prognosis. So I’m going to go into my questions now. I have three questions. So the first question is, the most common histological subtype of rhabdomyosarcoma is alveolar, embryonal, or none of the above. I’ll give you a little time to answer that question. Okay. Let’s go on to the second question. Good, most of you good that right. Alveolar is the most common type — I’m sorry, embryonal is the most common type. About 80% of rhabdomyosarcomas are the embryonal subtype. Let’s go on to question 2. Okay. Question 2. Which genetic change is most commonly found in alveolar rhabdomyosarcoma? Application of the MCYM oncogene to the PACS3, FOX1fusion gene? And all of the above. Very good. It’s the PACS 3, FOX 1 fusion gene. When we biopsy these tumors, of course we send the histopathology to pathology and we do the straight H&E study. We look at the immunohistochemistry. There are some other markers we use for rhabdomyosarcoma. Then we send for the FISH study to look for the fusion gene. We’ll go on to the third question now. Okay. This is a little tricky. But not really. Which of the following pediatric orbital tumors is most likely to present with bilateral orbital involvement? You only have these choices. You have metastatic neuroblastoma, granulocytic blastoma. The most common. Correct, metastatic neuroblastoma is the most common. I find that really most of these have bilateral involvement. In AML, it can be bilateral as well. But I think it’s more common in metastatic neuroblastoma. I’ve never seen a bilateral rhabdomyosarcoma. I’m sure they could occur in untreated cases. But I’ve never seen it, and I’ve never seen that happen in lymphangioma as well. The thing to remember about the metastatic neuroblastoma, it’s usually lateral in the orbit. It’s usually a superior lateral tumor that can cause bony changes, bony erosions and so forth. Granulocytic can cause involvement but much less commonly associated with bony changes of the orbit. Rhabdomyosarcoma usually does not present bilaterally. And it doesn’t usually, when we diagnose it, have any bony changes to the orbit. Lymphangioma as well does not usually occur bilaterally and does not have bony changes as well. All right. So we finished a couple of minutes early. So we have time for questions. So I’m happy to answer any questions. Let me look at the Q&A here. Okay. Here are the questions. Hi, there’s a long list of blue cell tumors, differential diagnoses, we [indiscernible] narrow down with such report and what do we request pathologists to help us specifically. Okay. So there is a long list. You know, and so it can be very confusing. When I first do a biopsy, I want to make sure [indiscernible] especially if the tumor is in a difficult location, I want to make sure I have the tumor. Since I’ve taken the patient to surgery. So to narrow down the differential, clinical findings, you know, we do a whole eye examination on the patient. I usually palpate the abdomen. If there’s anything that looks, you know, suspicious for metastatic disease and so forth, you know, you may scan the abdomen and so forth. Urine catecholamines are used to look for metastatic neuroblastoma. I do rely on my pathologist to do a lot of stains, like immunohistochemistry. They can do genetic study to the tumor to narrow down the differential diagnosis and find out what is going on. So, you know, I rely a lot on pathology, systemic evaluation, especially patients with AML, you know, may have blood or bone marrow signs of acute myeloid leukemia, but they may not at that point. So we look at our special stains and immunohistochemistry to help with diagnosis. Sometimes it can be difficult if you don’t have those techniques. I think probably the easiest ones are immunostains to look for different markers like in rhabdomyosarcoma, you can look at desmin and so forth, that will help narrow down differential diagnoses. That can be very tough. So VAC stands for [indiscernible]. It’s the kind of characteristic classic chemotherapy for rhabdomyosarcoma. Is there any role of visual acuity in staging of disease in rhabdomyosarcoma if there is visual acuity present, is it just — okay. [Indiscernible] visual acuity in staging. You know, I don’t think it’s really necessary to do exenteration. I think patients, you know, benefit from chemotherapy and chemotherapy regimens. If it’s a large tumor that has not been able to be grossly resected, radiation therapy. I only use exenteration when they’ve failed. Radiation therapy continues to grow. I do this in conjunction with a pediatric oncologist. We usually present the patient at tumor board, we go over all our options. I’ve only had a handful of patients where I’ve actually had to exenterate patients with rhabdomyosarcoma. When I do, the patients are still followed by oncology. They get chemotherapy after the exenteration. They usually do fairly well. I had another patient who came to me from another country, she had failed radiation therapy, we presented her to tumor board, we did exenteranion and gave her chemotherapy but unfortunately she passed away from the disease. So it’s very rarely used. Again, I’ve only done — I’ve seen a lot of cases of rhabdomyosarcoma. I’ve only exenerated a few of them. Do you look for ganglia cells to decide treatment or [indiscernible] visual fields. So with — I think you’re talking about neuroblastoma. You know, I’m not sure what is meant by the OPG. Okay. Can eye tumors be treated any advanced technology. Yes, eye tumors can be treated. I mean, we treat all of them, basically, we try to tailor our treatment to the specific tumor such as rhabdo may have a different tumor from metastatic neuroblastoma, especially from Ewing’s sarcoma, they all have different treatments. There’s different treatment protocols based on the tumor type and based on the age of the patient and based on the staging of the tumor. So we usually are instrumental in making the diagnosis of the tumor. Patients usually present to us first, and they come to us with proptosis or just an orbital mass and so forth. So we work them up initially. We do the complete eye exam. We do imaging studies. We do a prompt biopsy. We do the histopathology. And we try to find out as much as possible what is going on. Then almost all these patients with malignant tumors will need to see pediatric oncology. They have a lot of new targeted molecular genetic techniques. Usually monoclonal antibodies against different, you know, molecular genetic changes that can really improve the prognosis. There’s a lot of research being performed. The Rhabdomyosarcoma Study Group has been going on for years and years, and they’re always looking at new agents, newer treatments with less side effects, giving better prognosis. So it’s always a work in progress. And we follow the patients along after the treatment starts, because a lot of times patients get radiation side effects or so forth. They get cataracts, they can get dry eye. They need to be followed by us. We follow them for any recurrence in the orbit. If they do have a possible recurrence, we oftentimes rebiopsy them to see if that is active tumor or just necrose tumor that still shows up on imaging scans. So risk factors of Ewing’s sarcoma. That’s a good question. It’s a rare tumor. I think, you know, genetic risk factors, there are genetic, you know, diseases, this group of tumors are associated with different gene mutations. So it’s probably mostly genetic. Could be from, you know, intrauterine radiation and so forth. But it’s such a rare tumor, I don’t know if I can specifically tell you other environmental risk factors that are associated with that. Can we join the Rhabdomyosarcoma Study Group, if yes, how? That’s a good question. I think it’s a group of scientists who get together and actually study rhabdomyosarcoma. And mostly oncologists. I’m not a member of the Rhabdomyosarcoma Study Group. I’ve read a lot of their papers, a lot of their works. I don’t know how one becomes a member. I don’t know if it’s by invitation. You know, I think you probably could. I would probably look them up online, contact them and see what the requirements are to become a member. Okay. This is another one. Great presentation, thank you, sir. I would like to know your opinion about a choice of neural imaging for orbital tumors. Good question. I think MRI should be done to reduce radiation exposure [indiscernible] CT scan was done. Yeah, I mean, a lot of those cases where I had the CT scan, I worked in Saudi Arabia from 1986 to ’92, and at that time when I worked there, we only had CT scan available. We didn’t have MRI at that time. So that’s why MRI scans were the imaging modality used. These days at Bascom Palmer, I usually use MRI scans for the reasons you outline. There’s less of a risk of radiation therapy in secondary tumors because MRI does not involve radiation. So I definitely prefer that. Oftentimes, since we’re a referral center, we are referred patients in from the outside, and the patients will bring their own scans. So sometimes a patient will have had a CT scan done on the outside, and then if the CT scan shows me everything I need to know, I can use that. But if they’ve already had it done and they come with it where then we use that scan. But I agree with you 100%, MRI scan, if at all possible, it reduces the risk of radiation and secondary tumors. Okay. How do we deal with a patient with astrocytoma, I assume you mean an orbital astrocytoma. It depends on the location, the size of the tumor. I would try to excise it. I just had a patient recently that had a massive orbital astrocytoma, and we ended up — the patient had no light reception and the tumor would constantly bleed. We ended up exenterating the patient. The patient was being treated with chemotherapy as well, and I exenterated the orbit. It depends on the size and malignancy of the tumor, how that would be treated. Okay. Any final examination tip for primary care — primary eye care provider to detect these tumors early? That’s a great question. Sometimes it’s hard to detect them early because patients may not have any pain. Sometimes it’s a very slow onset, painless proptosis, and usually patients can come in when the tumor is noticeable to the parents, or it’s causing any type of problem. So, you know, when you do a complete eye examine, you can look at the projection of the eyes, see if there’s any abnormal projection of one eye versus the other. Some of these tumors may actually cause an adduced hyperopia causing shortening of the eye and an adduced hyperopia. You see a patient that has more hyperopia on one side than the other, you might want to scan the patient to see if there’s something pressing up against the eye or swelling of the optic disk or of the retinal vessels, could also be another sign that something is in the orbit pressing the eye forward. Any decrease of motility, you know, you could also suspect something behind the eye. And if you’re suspicious, you know, if you’re suspicious there may be a tumor behind the eye, an MRI scan. How can we choroidal nerve tumors treat [indiscernible] — I think we’re talking about choroidal tumors here. With choroidal tumors, it could be due to a metastatic disease. And in my practice, those are usually seen by [indiscernible] doctors to see what type of choroidal tumors they have. If it can be associated with choroidal metastases, the patient will get a whole workup look for intraocular tumor. Is there any [indiscernible] research? I mean, here we have the American society of [indiscernible] surgeon, we have a forum there. We also have the national association of orbital — academic orbital surgeons. We also have a forum there. I think there are several forums around the world with orbital doctors who treat these problems. Those are the two that I participate in. Oh, there’s some question on intracarotid chemotherapy, what’s your experience for retinal blastoma? It’s done quite commonly at Bascom Palmer, it is effective. That’s usually performed by the retina oncology service. But I’ve been peripherally associated with some of these patients. And they do quite well. So I think that’s a very viable treatment for retinal blastoma. We use actually intracarotid chemotherapy often for lacrimal gland tumors, we find it’s a very helpful in adenoid carcinoma of the lacrimal gland. [Indiscernible] low income countries? You know, I usually don’t do a lot of MRIs. Usually if I see a treatment response from the chemotherapy, radiation [indiscernible] we’ll do an MRI scan in three months ago, if everything looks good, maybe in a year. I don’t use it very often unless I suspect there is not a good treatment response. I’m working with an oncologist, sometimes they do scan the patient. And I follow the MRI scan there. But I know MRIs are expensive. So I think, you know, if you’re seeing a good clinical response, I would probably — possibly do one MRI several months after the treatment has been given, and then if all looks good, maybe once a year. Regarding abduction blindness or glaze of [indiscernible] visual loss which tumor in practice is responsible for gaze induced [indiscernible] loss. I mean, it could be a lot of tumors like abduction deficits, you know, can be due to any tumor that impinges on the [indiscernible] muscle, which could be rhabdomyosarcoma, could be granulocytic sarcoma and also neuroblastoma. I don’t find in these pediatric patients they usually get gaze induced visual loss. But I guess it could be possible. Most of our patients with rhabdomyosarcoma come with stage 3. They initially respond very well to chemo but stagnate at the fourth or fifth cycle. We use [indiscernible] from there it’s just downhill. We usually switch [indiscernible] poor. [Indiscernible] most of our patients. Is there anything you can suggest that can improve our outcomes. Radiation has not helped these patients either. Well, that’s unusual. You know, I think radiation therapy would be definitely helpful. I mean, that’s the exact right treatment. Some of those newer agents that I listed can also be tried, you know, for these patients that are refractory. I’m not really sure why you’re losing your patients. I think given the radiation simultaneously with the chemotherapy, I don’t know if you’re doing that, but, you know, we give radiation therapy at the same time as chemotherapy, again, most of our patients are also stage 3. And so we can biopsy the tumor, we can’t take it out, there’s usually gross tumor left in the orbit. We start them on chemotherapy. And yeah, usually they respond pretty well combined with radiation therapy. But, I mean, I would be sure it is rhabdomyosarcoma. I would be sure that we’re not missing, you know, another lesion that may have a different treatment. And maybe try some of the other agents that we talked about as well. Let’s see. Did I miss anything here? What is the progression of Ewing’s sarcoma with mild metastasis in the liver? I think the prognosis is guarded. A patient would definitely need chemotherapy. You know, with mild metastasis to the liver, the prognosis with newer agents has improved quite a bit, probably 50% or greater. And Ewing’s sarcoma is usually a metastatic tumor at the discovery, when we see it in the orbit, it’s metastatic from somewhere else. It’s very uncommon for it to be primary in the orbit. So usually statistically I think the studies I’ve read, there’s a really good study by Jonathan Dutton in OPRS, where he talks about a very comprehensive review of Ewing’s sarcoma. It’s from several years ago. I think I actually quoted it here. Let me see if I can find that quote for you. But it’s a really nice study on Ewing’s sarcoma. Let me just see if I can find that real quick. Let’s see. Oh, here it is. Okay. This is it. Jonathan Dutton, OPRS, 16.4, 292-300. That was done in the year 2000. That’s a really good study of Ewing’s sarcoma. Thank you for your presentation. We give chemotherapy separately and follow radiation therapy. We’ll try to give both simultaneously, okay, that’s good. Proton center in the USA, please. There’s one in Jacksonville, Florida, that’s usually the one we use, there’s one in Boston. There’s several around the country. We usually use the one in Florida because I’m in Florida. Rhabdomyosarcoma is most common in boys, what is the age? I find the usual median age is 7 to 8 years of age. [Indiscernible] glioma treatment, the problem with pathway gliomas is if you remove them at an early stage, the eye is blind. So usually we follow them along and if they are not encroaching on the optic canal or extending into the optic canal or threatening to involve the chiasm or optic nerve, we usually watch them carefully. If the eye is blind, if there is a risk of extending in, we usually work with neurosurgery and do a neurosurgical approach to completely resect the optic nerve from the canal to the back of the eye. We do that with the neurosurgeon. I think we got through all the questions. And I think we’re at 10:03, so if you have any other questions, you can email me. The email, I’m going to put it in the chat, so you can just email me. I’ll put my email in the chat. Whoops. Typed it wrong. Okay. So my email address is in the chat. If you have any other questions, please feel free to email me. I hope you all got something out of the webinar. Have a wonderful day. Thank you.

6 thoughts on “Lecture: Malignant Orbital Tumors of Childhood”

  1. I’m highly impressed and enriched with this detailed and comprehensive later. I thank Dr Johnson and indeed Cybersight for this lecture, looking forward to more of such lectures in future meetings. Thank you.

  2. I watched the video, and is very attractive and helpful to me, and I got the Pdf, in what causes are you offering certificate please

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  3. I was on duty on the stated time and was busy to attend it.
    I can further read it and would you attavh me any document on the topic?
    Thank you!

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