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BMJ Case Reports Mar 2017Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic... (Review)
Review
Extraskeletal orbital mesenchymal chondrosarcoma (MC) is an extremely rare and highly aggressive tumour. It has characteristic radiological features and pathognomic histological biphasic pattern. Radical resection with negative margins is the mainstay of treatment; role of adjuvant chemotherapy and radiotherapy is yet not well defined. We report a rare case of 18-year-old man who was diagnosed to have orbital MC. He presented with locally advanced disease with no vision in the affected eye. He underwent right orbital exenteration; a transcranial intradural approach was used to divide the optic nerve, and the temporalis muscle flap was utilised to fill the exenterated orbit. Though optic nerve involvement is rare in orbital MCs, a transcranial approach may be used effectively to avoid traction on optic chiasma and ensure margin-free resection in case of optic nerve involvement up to orbital apex. Unfortunately, prognosis remains dismal in MCs despite treatment.
Topics: Adolescent; Chondrosarcoma, Mesenchymal; Craniotomy; Humans; Male; Orbital Neoplasms; Radiotherapy, Adjuvant; Treatment Outcome
PubMed: 28258179
DOI: 10.1136/bcr-2016-218744 -
Anticancer Research 2004As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction...
BACKGROUND
As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas.
MATERIALS AND METHODS
The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data.
RESULTS
The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p<0.05). This finding could justify the higher sensibility to pharmacological therapy of RMS of the first group. The group of RMS with a worse prognosis (primary tumors and relapses) showed instead p53 overexpression (W&M and mutated), MDR-1 expression and multiploidy, with high 5cEE values and tetraploid peaks. No significant difference was found concerning the expression of bcl-2 among the two groups of RMS (p>0.05).
CONCLUSION
The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.
Topics: ATP Binding Cassette Transporter, Subfamily B, Member 1; Biomarkers, Tumor; DNA, Neoplasm; Female; Humans; Immunohistochemistry; Ki-67 Antigen; Male; Orbital Neoplasms; Ploidies; Proto-Oncogene Proteins c-bcl-2; Rhabdomyosarcoma; Rhabdomyosarcoma, Alveolar; Rhabdomyosarcoma, Embryonal; Tumor Suppressor Protein p53
PubMed: 15015604
DOI: No ID Found -
Brazilian Journal of Otorhinolaryngology 2019Endoscopic orbital surgery is a nascent field and new tools are required to assist with surgical planning and to ascertain the limits of the tumor resectability.
INTRODUCTION
Endoscopic orbital surgery is a nascent field and new tools are required to assist with surgical planning and to ascertain the limits of the tumor resectability.
OBJECTIVE
We purpose to utilize three-dimensional radiographic reconstruction to define the theoretical lateral limit of endoscopic resectability of primary orbital tumors and to apply these boundary conditions to surgical cases.
METHODS
A three-dimensional orbital model was rendered in 4 representative patients presenting with primary orbital tumors using OsiriX open source imaging software. A 2-Dimensional plane was propagated between the contralateral nare and a line tangential to the long axis of the optic nerve reflecting the trajectory of a trans-septal approach. Any tumor volume falling medial to the optic nerve and/or within the space inferior to this plane of resectability was considered theoretically resectable regardless of how far it extended lateral to the optic nerve as nerve retraction would be unnecessary. Actual tumor volumes were then superimposed over this plan and correlated with surgical outcomes.
RESULTS
Among the 4 lesions analyzed, two were fully medial to the optic nerve, one extended lateral to the optic nerve but remained inferior to the plane of resectability, and one extended both lateral to the optic nerve and superior to the plane of resectability. As predicted by the three-dimensional modeling, a complete resection was achieved in all lesions except one that transgressed the plane of resectability. No new diplopia or vision loss was observed in any patient.
CONCLUSION
Three-dimensional reconstruction enhances preoperative planning for endoscopic orbital surgery. Tumors that extend lateral to the optic nerve may still be candidates for a purely endoscopic resection as long as they do not extend above the plane of resectability described herein.
Topics: Humans; Imaging, Three-Dimensional; Magnetic Resonance Imaging; Optic Nerve; Orbital Neoplasms; Preoperative Period; Plastic Surgery Procedures; Software; Tomography, X-Ray Computed; Transanal Endoscopic Surgery; Treatment Outcome; Tumor Burden
PubMed: 29337012
DOI: 10.1016/j.bjorl.2017.11.010 -
Indian Journal of Ophthalmology Sep 2017Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin. The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma. Most... (Review)
Review
Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin. The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma. Most commonly, it involves trunk (62%) followed by extremities (25%) and head and neck (13%). It is a slow growing tumor with locally aggressive behavior. Here, a 50-year-old female diagnosed with orbital dermatofibrosarcoma developed extra-axial component in right frontal region even on chemotherapy. Hence, the bad prognostic factors are yet to be established in dermatofibrosarcoma protuberans.
Topics: Biopsy; Dermatofibrosarcoma; Diagnosis, Differential; Ethmoid Sinus; Female; Frontal Sinus; Humans; Magnetic Resonance Imaging; Middle Aged; Neoplasm Invasiveness; Orbital Neoplasms; Skin Neoplasms
PubMed: 28905843
DOI: 10.4103/ijo.IJO_770_16 -
Cancer Imaging : the Official... Oct 2006Primary malignant lesions in the orbit are relatively uncommon. However, the orbits are frequently involved in haematogeneous metastasis or by direct extension from...
Primary malignant lesions in the orbit are relatively uncommon. However, the orbits are frequently involved in haematogeneous metastasis or by direct extension from malignancies originating from the adjacent nasal cavity or paranasal sinuses. This paper focuses on the more commonly encountered primary orbital malignancies and the mapping of tumour spread into the orbits.
Topics: Adenoma; Carcinoma, Adenoid Cystic; Child; Cranial Nerve Neoplasms; Female; Humans; Lacrimal Apparatus Diseases; Lymphoma; Magnetic Resonance Imaging; Male; Meningioma; Middle Aged; Neoplasm Invasiveness; Optic Nerve Glioma; Optic Nerve Neoplasms; Orbit; Orbital Diseases; Orbital Neoplasms; Osteolysis; Radiography
PubMed: 17114076
DOI: 10.1102/1470-7330.2006.9003 -
Turkish Journal of Haematology :... May 2019
Topics: Child; Disease-Free Survival; Female; Humans; Male; Orbital Neoplasms; Sarcoma, Myeloid; Survival Rate
PubMed: 30650963
DOI: 10.4274/tjh.galenos.2019.2019.0002 -
Middle East African Journal of... 2015Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the...
Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments.
Topics: Adult; Aged; Biopsy; Carcinoma, Squamous Cell; Eyelid Neoplasms; Female; Hemangioma, Cavernous; Humans; Magnetic Resonance Imaging; Male; Neoplasm Invasiveness; Neurilemmoma; Orbital Neoplasms; Orbital Pseudotumor; Prednisolone; Prospective Studies; Tomography, X-Ray Computed
PubMed: 26692710
DOI: 10.4103/0974-9233.167823 -
Acta Medica Iranica 2015Ocular involvement is seen in approximately 25% of patients with sarcoidosis. Uveitis is the most common ocular manifestation, but sarcoidosis may involve any part of...
Ocular involvement is seen in approximately 25% of patients with sarcoidosis. Uveitis is the most common ocular manifestation, but sarcoidosis may involve any part of the eye. Orbital manifestations of sarcoidosis are uncommon with few series in the literature. A 65-year-old woman presented with redness of the right eye and painless, unilateral eyelid swelling. Orbital scanning revealed mass infiltrating the soft tissue of the inferior right orbital quadrant. Biopsy results showed nodular, noncaseating granulomas consistent with sarcoidosis. The complete systemic workup revealed systemic manifestations of sarcoidosis at the time of examination with hilar and mediastinal lymphadenopathies noted on CT scan. The orbital surgical treatment was followed by systemic prednisone therapy with good response. Although rare, orbital sarcoidosis must be considered in the evaluation of orbital tumors in elderly patients. A search for systemic findings should be undertaken and appropriate therapy should be instituted.
Topics: Aged; Biopsy; Female; Humans; Orbital Neoplasms; Sarcoidosis; Tomography, X-Ray Computed
PubMed: 25796029
DOI: No ID Found -
Survey of Ophthalmology 2017Pituitary adenoma invasion into the orbit is a rare phenomenon with only 22 cases, including the present case, in the literature. Our case is a 31-year-old man who... (Review)
Review
Pituitary adenoma invasion into the orbit is a rare phenomenon with only 22 cases, including the present case, in the literature. Our case is a 31-year-old man who presented with biopsy-proven atypical pituitary adenoma invading the right orbit after a prior resection. We compare his clinical course with previous cases and discuss clinical features, radiological features, management considerations, histologic features, and prognosis. Cases are organized by specific pituitary tumor type to aid in determining appropriate management. Early surgical intervention is the key, especially in the setting of pathologic features indicating aggressive tumor behavior or worsening visual function but is generally not indicated in prolactin-secreting adenomas that may respond to medical therapy. The role of radiation therapy is not fully established; however, it should be strongly considered in conjunction with or after surgery, especially in cases where complete resection is not achieved or histological and molecular analyses indicate a high likelihood of recurrence. More uniform and comprehensive data about management and outcomes are needed to determine the optimal treatment approach for this rare entity.
Topics: Adenoma; Adult; Antineoplastic Agents; Humans; Male; Neoplasm Invasiveness; Ophthalmologic Surgical Procedures; Orbital Neoplasms; Pituitary Neoplasms
PubMed: 28131872
DOI: 10.1016/j.survophthal.2017.01.005 -
Neurologia Medico-chirurgica Sep 1997A 43-year-old male presented with swelling involving the right eye. T1-weighted magnetic resonance imaging demonstrated a round tumor in the lateral region of the right...
A 43-year-old male presented with swelling involving the right eye. T1-weighted magnetic resonance imaging demonstrated a round tumor in the lateral region of the right orbit, which was isointense relative to the cerebral gray matter and homogeneously enhanced by gadolinium-diethylenetriaminepenta-acetic acid with a flow-void signal area in the mass. The tumor was totally resected through the transcranial and fronto-orbitotemporal approach after embolization of feeding arteries arising from the external carotid artery. The histological findings were characteristic of hemangiopericytoma. No radiation therapy was administered. The transcranial and fronto-orbitotemporal approach provides a wide operative field with excellent exposure of the highly vascular orbital tumor.
Topics: Cerebral Angiography; Combined Modality Therapy; Craniotomy; Embolization, Therapeutic; Hemangiopericytoma; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Orbit; Orbital Neoplasms
PubMed: 9330534
DOI: 10.2176/nmc.37.688