-
Ophthalmic Plastic and Reconstructive...A 3-year-old boy developed proptosis over 3 weeks. CT and MRI disclosed a 3.2 × 1.9 cm soft-tissue mass of the right extraconal and intraconal orbit with sphenoid...
A 3-year-old boy developed proptosis over 3 weeks. CT and MRI disclosed a 3.2 × 1.9 cm soft-tissue mass of the right extraconal and intraconal orbit with sphenoid bone erosion. After debulking through an upper eyelid crease incision, the tumor was diagnosed as a spindle cell/sclerosing rhabdomyosarcoma. DNA sequencing was negative for an L122R mutation in MyoD1 . Spindle cell/sclerosing rhabdomyosarcoma is an uncommon variant of this neoplasm, and only 2 patients with orbital tumors have been reported in 2 case series. Spindle cell/sclerosing rhabdomyosarcomas confined to the orbit are considered to have an excellent prognosis when treated with chemotherapy and radiation therapy. Diagnosis and treatment planning rely on histology, immunohistochemistry, and molecular analysis.
Topics: Male; Humans; Child, Preschool; Orbit; Rhabdomyosarcoma; Orbital Neoplasms; Mutation
PubMed: 35829629
DOI: 10.1097/IOP.0000000000002252 -
Orbital exenteration for sinonasal malignancies: indications, rehabilitation and oncologic outcomes.Current Opinion in Otolaryngology &... Apr 2018Review the recent literature regarding the management of orbital invasion in sinonasal malignant tumors. (Review)
Review
PURPOSE OF REVIEW
Review the recent literature regarding the management of orbital invasion in sinonasal malignant tumors.
RECENT FINDINGS
There is a recent trend in preserving the orbit in cases of minimal invasion of periosteum and limited periorbit involvement, as well as in presence of good response to neoadjuvant chemotherapy, mainly in squamous cell carcinoma and neuroendocrine histologies.
SUMMARY
The decision about orbital exenteration in cases of sinonasal malignancies is facilitated if the patient already has clear clinical signs of intraconal invasion such as visual loss, restriction of ocular mobility or infiltration of the eyeglobe. However, in borderline situations, confirmation of orbital involvement should be performed intraoperatively. In selected cases with minimal orbital invasion without functional compromise, orbit sparing surgery can be done with acceptable oncological outcomes.
Topics: Aged; Disease-Free Survival; Endoscopy; Female; Humans; Male; Middle Aged; Neoplasm Invasiveness; Neoplasm Staging; Orbit Evisceration; Orbital Neoplasms; Paranasal Sinus Neoplasms; Postoperative Care; Prognosis; Risk Assessment; Survival Analysis
PubMed: 29465436
DOI: 10.1097/MOO.0000000000000441 -
Der Ophthalmologe : Zeitschrift Der... Oct 2021Orbital space-occupying lesions can have a significant impact on the quality of life. Depending on the dignity and etiology of the mass, they can even represent a... (Review)
Review
BACKGROUND
Orbital space-occupying lesions can have a significant impact on the quality of life. Depending on the dignity and etiology of the mass, they can even represent a life-threatening process, which must be timely treated in an interdisciplinary cooperation.
OBJECTIVE
To achieve a comprehensive overview about the diagnostics and frequency of malignant and benign masses of the orbit.
MATERIAL AND METHODS
A literature search was carried out in PubMed and Google scholar.
RESULTS AND DISCUSSION
Clinically, orbital lesions can show symptoms, such as exophthalmos with an increase in the vertical lid fissure, visual impairment and motility dysfunction with diplopia. Depending on the extent, an exposure keratopathy can occur due to failure to close the eyelids. For the diagnostics several tests should be carried out. Following a thorough anamnesis and clinical examination, these should include a blood sample to test for various parameters, an ultrasound examination with subsequent computed tomography (CT) in cases of bone involvement or thin slice magnetic resonance imaging (MRI) of the orbit for assessment of soft tissues. In adults an endocrine orbitopathy is the most frequent cause of an inflammatory orbital process, where lymphomas are the most frequent entity among malignant tumors and vascular space-occupying lesions are the most frequent benign tumors. In children the most frequent benign masses are dermoid cysts and rhabdomyosarcoma is the most frequent primary malignant orbital tumor. The multimodal interdisciplinary treatment can include surgical excision and adjuvant radiotherapy or chemotherapy, depending on the entity.
Topics: Adult; Child; Exophthalmos; Humans; Orbit; Orbital Neoplasms; Quality of Life; Tomography, X-Ray Computed
PubMed: 34196748
DOI: 10.1007/s00347-021-01429-x -
Survey of Ophthalmology 2016Lymphoid hyperplasia (LH) is a benign lymphoproliferative disorder that, in a minority of cases, may be associated with concurrent or metachronous non-Hodgkin lymphoma.... (Review)
Review
Lymphoid hyperplasia (LH) is a benign lymphoproliferative disorder that, in a minority of cases, may be associated with concurrent or metachronous non-Hodgkin lymphoma. LH cases are further subdivided into "reactive" and "atypical" categories based on the presence or absence of unequivocal malignant features. With improving molecular diagnostic technologies, "reactive" LH is by far the most common category of LH, with atypical LH accounting for only a small minority of specimens. Similarly, lesions previously diagnosed as LH are now being revised as low-grade B-cell non-Hodgkin lymphoma or diagnosed as newly described benign conditions such as IgG4-related disease. Additional differential diagnoses include specific and nonspecific orbital inflammations, infiltrative processes, and depositions. Hence, there are emerging changes in the patterns and proportions of entities that fall within the spectrum of lymphoproliferative disorders of the orbit and ocular adnexa. Reactive LH and low-grade malignant lymphoproliferative disorders in the orbit and ocular adnexa are clinically and radiologically indistinguishable from each other, requiring tissue biopsy in all cases. The prognosis of ocular adnexal LH is generally favorable, but the small risk of non-Hodgkin lymphoma mandates follow-up for at least 5 years. We summarize the current state of knowledge on LH occurring in the orbit and ocular adnexa.
Topics: Biopsy; Conjunctival Neoplasms; Diagnosis, Differential; Diagnostic Techniques, Otological; Eyelid Neoplasms; Humans; Immunohistochemistry; Orbital Neoplasms; Prognosis; Pseudolymphoma
PubMed: 27127077
DOI: 10.1016/j.survophthal.2016.04.004 -
Tierarztliche Praxis. Ausgabe K,... Dec 2018A 10-year-old Rottweiler presented with right-sided moderately painful exophthalmia, blindness, absence of dazzle and pupillary light reflexes, a swollen optic nerve...
A 10-year-old Rottweiler presented with right-sided moderately painful exophthalmia, blindness, absence of dazzle and pupillary light reflexes, a swollen optic nerve head and ventrolateral indentation of the globe. On magnetic resonance imaging, a 3 x 2 x 2 cm mass with a fluid filled center and contrast-enhancing periphery was noted posteriolateral of the globe. Orbital ultrasound was used for a guided fine needle aspirate of the mass. Cytology revealed moderate numbers of polygonal cells with lightly basophilic cytoplasm. Several cells showed nuclear pseudoinclusions. Histopathology following exenteration of the orbit revealed an infiltrative, extradural neoplasm surrounding the optic nerve. Cells were arranged in packets. Neoplastic cells were immunopositive for neuron specific enolase, synaptophysin and chromogranin A and immunonegative for cytokeratin. Findings were consistent with an extra-adrenal paraganglioma (neuroendocrine tumour). Although complete excision could not be confirmed on histopathology, the owners reported no apparent tumour recurrence 25 months after surgery. In conclusion a paraganglioma should be considered as a differential diagnosis of an orbital mass.
Topics: Animals; Diagnosis, Differential; Dog Diseases; Dogs; Female; Orbital Neoplasms; Paraganglioma, Extra-Adrenal
PubMed: 30658369
DOI: 10.1055/s-0038-1677406 -
Orbit (Amsterdam, Netherlands) Apr 2023To describe the modified vertical lid split (VLS) technique for anterior orbitotomy and report surgical outcomes in patients with intraconal lesion removal using this... (Review)
Review
PURPOSE
To describe the modified vertical lid split (VLS) technique for anterior orbitotomy and report surgical outcomes in patients with intraconal lesion removal using this surgical technique.
METHOD
Retrospective review of medical records and orbital images.
RESULTS
A total of four (female = 3) patients with intraconal orbital lesions underwent modified VLS orbitotomy from January 1, 2019 to June 30, 2021 in Hong Kong East Cluster. The average age was 49.3 years old (range: 35-65). Complete excision of intraconal orbital lesion was performed in all four cases. All cases were cavernous haemangioma histologically. All patients were satisfied with their cosmesis postoperatively and most had satisfactory postoperative extraocular movement.
CONCLUSION
Modified VLS approach is simple and provides good intraoperative surgical exposure for intraconal lesions, giving the additional advantage of better scar camouflage.
Topics: Humans; Female; Middle Aged; Orbital Neoplasms; Orbit; Surgical Flaps; Retrospective Studies; Hemangioma, Cavernous
PubMed: 35657059
DOI: 10.1080/01676830.2022.2083185 -
Ophthalmic Plastic and Reconstructive...Sebaceous carcinoma is characterized by its aggressive local tumor behavior and ability to metastasize. Small periocular sebaceous carcinoma are typically treated by...
Sebaceous carcinoma is characterized by its aggressive local tumor behavior and ability to metastasize. Small periocular sebaceous carcinoma are typically treated by excision with cryotherapy. Larger tumors often require adjuvant external beam radiotherapy (EBRT) and/or exenteration surgery. When used alone, EBRT techniques typically exceed the tolerance of critical normal ocular structures. The interstitial orbital brachytherapy-boost technique permits dose escalation to the tumor bed, while minimizing radiation dose to critical normal ocular structures. Here, we present a case of orbital sebaceous carcinoma treated with excision, cryotherapy, and super-thick amniotic membrane fornix reconstruction. Then, after 3 weeks of healing, adjuvant-combined electron interstitial high-dose rate brachytherapy-boost was added to electron-beam radiotherapy to optimize the orbital radiation dose distribution, increase dose to inferonasal orbit, and allow relative sparing of orbital tissues. At 1-year follow-up, there was no evidence of orbital tumor, no significant eye lash loss, normal ocular motility, no radiation retinopathy, optic neuropathy and a visual acuity of 20/20.
Topics: Adenocarcinoma, Sebaceous; Brachytherapy; Humans; Orbital Neoplasms; Radiation Injuries; Radiotherapy Dosage; Sebaceous Gland Neoplasms
PubMed: 34314398
DOI: 10.1097/IOP.0000000000002031 -
Journal Francais D'ophtalmologie Dec 2015Dermoid cysts are benign congenital orbital tumors, they are classified as choristomas. Clinical manifestations vary according to location and size. (Review)
Review
INTRODUCTION
Dermoid cysts are benign congenital orbital tumors, they are classified as choristomas. Clinical manifestations vary according to location and size.
MATERIALS AND METHODS
We retrospectively analyzed 43 cases of dermoid cysts surgically treated between July 1998 and March 2009.
RESULTS
Mean patient age was 18.64 years. Among patients, 48.83% were male. Preseptal location was most common (41 cases). One of the two cases of intraorbital location required orbitotomy. We observed no complications or recurrence.
DISCUSSION
Dermoid cysts are most often seen in young patients. The mean age of our patients was relatively high. The majority of dermoid cysts are periorbital in location. Superficial orbital dermoid cysts are more frequent, present earlier in life and are more easily identified than deep dermoid cysts. Imaging plays a major role in the management of deep orbital tumors. Complete surgical excision has remained the mainstay of treatment.
CONCLUSION
Dermoid cysts are benign orbital tumors. Superficial dermoid cysts are the most common type. Deeper cysts require a more challenging diagnostic and therapeutic approach.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Dermoid Cyst; Female; Humans; Infant; Male; Middle Aged; Orbital Neoplasms; Rupture, Spontaneous; Sebum; Tomography, X-Ray Computed; Treatment Outcome; Young Adult
PubMed: 26563840
DOI: 10.1016/j.jfo.2015.02.012 -
The Journal of Laryngology and Otology Apr 2023The transnasal endoscopic approach may provide better visualisation and a safer approach to the orbital apex. This study presents a case series of orbital apex lesions... (Review)
Review
OBJECTIVE
The transnasal endoscopic approach may provide better visualisation and a safer approach to the orbital apex. This study presents a case series of orbital apex lesions managed by this approach.
METHOD
This study was an eight-year retrospective analysis of seven patients who were operated on for orbital apex lesions in two tertiary medical centres.
RESULTS
Complete tumour removal was performed in three patients and partial removal was performed in four patients. Visual acuity improved in three patients, remained stable in one patient and decreased in the other two patients. The visual field improved in four patients and did not change in two patients. Complications included worse vision and visual fields in 28.6 per cent of patients and late enophthalmos (of -1.25 ± 4.6 mm) in 2 patients.
CONCLUSION
The transnasal approach to orbital apex lesions in selected cases may provide a rational alternative to transorbital surgery. Complete tumour removal should be weighed against the risk of damage to the optic nerve.
Topics: Humans; Orbital Neoplasms; Retrospective Studies; Endoscopy; Visual Acuity
PubMed: 35678382
DOI: 10.1017/S0022215122001323 -
Klinische Monatsblatter Fur... Jan 2019A 30-year-old male patient presented with a painful globe subluxation of the left eye since two weeks. Painless left proptosis had been noted at least 2 years earlier....
A 30-year-old male patient presented with a painful globe subluxation of the left eye since two weeks. Painless left proptosis had been noted at least 2 years earlier. An MRI performed one year after onset of symptoms showed a retrobulbar mass, which on biopsy was diagnosed as spindle cell lipoma. Surgical debulking via a transconjunctival approach successfully reduced proptosis; repeated histology with extensive immunohistochemical analysis now led to the diagnosis of a well-differentiated orbital liposarcoma. The patient underwent orbital exenteration and received adjuvant radiotherapy. Genetic testing showed a heterozygote mutation of the ATM-gene on chromosome 11.
Topics: Adult; Exophthalmos; Humans; Liposarcoma; Magnetic Resonance Imaging; Male; Orbital Neoplasms; Radiotherapy, Adjuvant
PubMed: 30567006
DOI: 10.1055/a-0759-2162