Involvement of the bony orbit in infantile myofibromatosis

Infantile myofibromatosis is a rare disorder of infancy that can provoke osteolytic lesions. A 15-day-old infant presented with three round, firm lesions located on the forehead, shoulder, and back. Excisional biopsy of the forehead lesion revealed that the tumor was composed of spindle cells resembling normal smooth muscle arranged in short fascicles. Immunohistochemical staining was positive for vimentin and actin. Five months later, the child presented with three new lesions, including one in the superolateral aspect of the left orbit. It is important to recognize the multicentric form of infantile myofibromatosis because, despite its aggressive clinical presentation, the disease is benign and usually does not require extensive surgery or chemotherapy.     

Orbital solitary fibrous tumor: radiographic and histopathologic correlations

OBJECTIVE: To correlate the clinicopathologic and radiographic features characteristic of orbital solitary fibrous tumor (SFT). METHODS: The diagnostic features and clinical outcome of seven adults with orbital SFT are retrospectively outlined. Orbital imaging was performed by ultrasonography, computed tomography, or magnetic resonance imaging. Some cases were imaged by multiple modalities. Histopathologic examination of each tumor specimen included standard light and immunohistochemical stains. RESULTS: Heterogeneous internal composition was better appreciated on magnetic resonance imaging than on computed tomography. All cases undergoing magnetic resonance imaging showed T1 isointensity and T2 hypointensity relative to gray matter. Strong, generalized immunohistochemical reactivity to vimentin and CD34 validated the diagnosis of SFT and differentiated the specimens from other spindle cell neoplasms. After complete tumor resection, our patients remain tumor free with postoperative intervals of 15 to 45 months. CONCLUSIONS: Solitary fibrous tumor has now been reported in 26 orbits. No physical finding is pathognomonic, but several imaging traits are highly characteristic. Intralesional image heterogeneity and a predominantly low T2 signal intensity are distinctive of SFT. Complete tumor resection and immunohistologic specimen evaluation are emphasized. Clinicians should consider the diagnosis of SFT when confronted with an adult patient having an orbital soft tissue mass demonstrating the distinctive magnetic resonance imaging findings.     

Solitary infantile myofibroma of the orbital bone

Infantile myofibroma of the orbit is an extremely rare condition. Only a few instances of this condition have ever been reported. A 3-year-old boy visited our clinic with lateral lower eyelid swelling and a palpable mass in the left eye, which had apparently persisted for 2 months. A computed tomography scan revealed a well-circumscribed mass in the inferolateral orbital portion of the zygomatic bone, coupled with erosion of bone and orbital extension with reactive hyperostosis. Immunohistochemical stains proved positive for smooth muscle actin, supporting the diagnosis of solitary infantile myofibroma of the orbital bone (zygoma). Although rarely found in the orbit, solitary infantile myofibroma can display more aggressive or malignant neoplasm. Immunohistochemistry is integral to the differential diagnosis and a systemic evaluation for multicentric myofibroma is essential.     

Salivary duct carcinoma metastatic to eyelid and orbit—a case report

BACKGROUND: Salivary duct carcinoma is a rare, extremely aggressive malignant tumor, demonstrating invasive growth with early regional and distant metastasis. We describe a case of parotid gland salivary duct carcinoma metastatic to the eyelid and orbit, as confirmed by immunohistochemical analysis. To the best of our knowledge, this is the first such case reported in the literature. METHODS: A 43-year-old male had left lower eyelid and left inferior fornix/anterior orbital masses. The patient underwent left lower eyelid and anterior orbital biopsies for histopathological evaluation. Immunohistochemical analysis for markers like androgen receptors, gross cystic disease fluid protein-15, cytokeratins, HER-2/neu, epithelial membrane antigen, S-100 proteins, progesterone receptors, and estrogen receptors were performed to establish diagnosis. RESULTS: Specimens from the eyelid and orbit revealed lobules of tumor cells exhibiting solid, micopapillary, and glandular appearance with central necrosis-comedo patterns. The tumor cells showed immunohistochemical reactivity to androgen receptor proteins, pankeratin, HER-2/neu and epithelial membrane antigen. Focal reactivity to gross cystic disease fluid protein-15 was also present. Immunoreactivity to S-100 proteins, progesterone receptors, and estrogen receptors were negative. Diagnosis was metastatic paratoid duct carcinoma to the left lower eyelid and left inferior orbit. CONCLUSIONS: Although metastatic SDC of the eyelid and orbit is a rare phenomenon, the possibility of this extremely aggressive entity should be included in the differential diagnosis when dealing with a patient with an eyelid and/or orbital tumor.     

Solitary orbital infantile myofibroma: a case report and review of the literature.

Infantile myofibromatosis is a benign condition involving the skin, viscera, or bone that presents rarely in the orbit where rapid growth and bony destruction can mimic malignant tumors. Lesions can be solitary or scattered throughout the body. Growth in viscera such as the lungs can lead to death. Herein, we present a case of a solitary orbital myofibroma in a young child.     

Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with review of the literature

Solitary fibrous tumor of the orbit is a rare spindle cell neoplasm. There are 42 cases of solitary fibrous tumor of the orbit available in the literature. We present six more cases of orbital solitary fibrous tumors, which presented to our institute between 1999 and 2001. We highlight the need for clinical recognition of these tumors as a distinct entity and inclusion of this tumor in the etiological differential diagnosis of well-circumscribed orbital lesions presenting as unilateral proptosis in both children and in adults. The diagnosis may be suspected based on radiological features supported by histopathologic and immunohistochemical study. The strong CD34 immunoreactivity of this tumor supports its diagnosis. Complete surgical resection is the most important prognostic factor of this tumor.     

Congenital orbital and disseminated extrarenal malignant rhabdoid tumor

A 5-week premature infant boy with tumorous malformations underwent biopsy of two truncal masses and exenteration of the left orbit. Specimens were examined histologically. Histologic reports, slides, and clinical photographs were reviewed. A diagnosis of malignant rhabdoid tumor was made. Malignant rhabdoid tumors can present as local or disseminated neoplastic disease involving the orbit and should be considered in the differential diagnosis of rapidly progressing orbital lesions presenting in early infancy. We review the current classification of rhabdoid tumors and the previous literature on orbital rhabdoid tumors.     

Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature

PURPOSE: The real incidence of solitary fibrous tumor (SFT) of the orbit is unknown, but it seems that since it was first described in 1994, orbital SFT has been increasingly recognized. We believe that the orbital SFT is a relatively common tumor and that it should be considered in the differential diagnosis of any orbital tumor. DESIGN: Interventional case series. PARTICIPANTS: Four new cases of orbital SFT. METHODS: Four patients affected by solitary fibrous tumor of the orbit are described. One patient experienced a recurrent SFT shortly after initial surgical excision performed elsewhere. Thirty-eight cases have been reported in the literature in 7 years. RESULTS: The number of orbital SFTs reported has been increasing, reaching an average of more than five tumors reported per year. Since the first orbital SFT was described in 1994, 37 cases have been reported in the literature. We add four new cases in our series, including a recurrent tumor. A total of 42 cases have now been described, eight with recurrences. Malignant transformation occurred in one case. CONCLUSIONS: We believe that before 1994, the diagnosis orbital SFT was confused with other benign orbital tumors, such as fibrous histiocytoma and hemangiopericytoma because of a lack of use of immunohistochemical techniques. This entity should now be considered relatively common and should be included in the differential diagnosis of orbital tumors in any age group. Local recurrences of SFT are possible and usually follow an incomplete initial excision. Recurrent tumors in the orbit have shown the tendency to infiltrate the surrounding tissues and the bone, rendering complete secondary excision more difficult. Recurrent orbital SFT also has the potential for malignant transformation. The treatment of choice of orbital SFT is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.     

Two unusual osteogenic orbital tumors: presumed parosteal osteosarcomas of the orbit

OBJECTIVE: To report two cases of suspected parosteal osteosarcoma of the orbit, with dedifferentiation into a high-grade liposarcoma occurring in one patient. DESIGN: Two retrospective case reports. METHODS: The clinical, radiologic, and pathologic records of two patients with suspected orbital parosteal osteosarcoma were retrospectively reviewed. MAIN OUTCOME MEASURES: Histologic evaluation and clinical follow-up were measured. RESULTS: The first patient was a 47-year-old male presenting with a 5-month history of painless right lower lid swelling; excision biopsy suggested a well-differentiated parosteal osteosarcoma of the orbital floor, which recurred 3 years later. Six months after excision of the recurrence, the mass demonstrated accelerated growth, and a lid-sparing exenteration was performed; histologic examination showed a high-grade liposarcoma. The patient remains disease-free at 4 years. The second patient, a 40-year-old male, presented with an 8-year history of proptosis and a right superotemporal orbital mass. The mass was excised completely at lateral orbitotomy; histologic examination suggested a well-differentiated parosteal osteosarcoma. The patient remains well 9 months postoperatively. CONCLUSIONS: Parosteal osteosarcoma is an uncommon tumor, usually affecting long bones, that is extremely rare in the orbit. It is a low-grade sarcoma that tends to recur locally after excision but has a favorable prognosis. Dedifferentiation into a high-grade sarcoma occasionally occurs in parosteal osteosarcoma, but transformation into liposarcoma does not seem to have been previously reported. It is important to recognize dedifferentiated parosteal osteosarcoma, because the prognosis is poor, and radical treatment may be required.     

Orbital solitary fibrous tumor: A clinicopathologic correlation and review of literature

Orbital solitary fibrous tumor (SFT) is a rare tumor originating from the mesenchyme. Initially described in the pleura and subsequently in other mesenchymal structures, orbit continues to be one of the uncommon extrapleural sites. The diagnosis of orbital SFT cannot be made with certainty on clinical or radiological evaluation and requires histologic studies with immunohistochemical confirmation for which CD 34 is the most specific diagnostic test. We describe clinical presentations, radiological and operative findings, and pathological features of a patient with orbital SFT along with a review of literature.     

Mesenchymal chondrosarcoma of the orbit: A case report with 5 years of follow-up

Mesenchymal chondrosarcoma is a rare orbital tumor. Several case reports of this rare tumor have been published in the literature but only 6 cases have documented a follow up of 5 years or more. We report a case of 28 year-old female who presented with left orbital mass. Computed Tomography (CT) revealed a lobulated mass in the superior extraconal space with dense intralesional calcification. Patient underwent complete resection of the mass and histopathology was suggestive of mesenchymal chondrosarcoma. He was given adjuvant radiotherapy and there was no recurrence or metastasis at 5 years of follow-up. The case highlights that a complete resection with adjuvant radiotherapy in cases of orbital mesenchymal chondrosarcoma offers excellent prognosis.     

Orbital leiomyoma: report of a case and review of the literature

A 10-year-old female presented with slowly progressive proptosis of 1 year's duration. Orbital MRI revealed an extraconal oval mass occupying the lateral half of the mid and posterior orbit. The tumor was isointense to the extraocular muscle and cerebral gray matter on T1-weighted images and hyperintense on T2-weighted images. The patient underwent superolateral orbitotomy, and it was felt that the tumor was totally excised in a piecemeal fashion. Histopathologic examination showed that the tumor was composed of spindle cells arranged in a fibrous stroma rich in dilated sinusoidal capillaries. The nuclei of tumor cells were oval with blunted ends. There were no mitotic figures. Immunohistochemically, the tumor stained positive with smooth muscle actin, desmin, and vimentin. Based on the histopathologic and immunohistochemical staining results, a diagnosis of orbital leiomyoma was made. At 34 months follow-up, the patient continues to have 20/20 vision and is free of tumor recurrence clinically. Orbital leiomyoma is an uncommon tumor. Search of the English literature showed only 15 previous cases of orbital leiomyoma that have been published since 1963.     

Ossifying fibromyxoid tumor of the orbit

PURPOSE: This report describes the clinical, radiographic, and histopathologic features of the first known case of ossifying fibromyxoid tumor involving the orbit. METHODS: Case report and literature review. RESULTS: An 81-year-old woman presented with a 3-month history of diplopia, pain, and right upper eyelid swelling. CT and MRI of the orbit disclosed a 2.5-cm extra-conal mass superomedially in the right orbit, with displacement of the globe inferiorly and remodeling of the orbital roof. During surgery, the tumor was noted to be firm, gritty, and extra-periosteal in location, and the bone adjacent to the tumor was irregular and abnormal in consistency. The tumor was removed intact without difficulty. Histologic, immunohistochemical, and transmission electron microscopic examination disclosed features consistent with ossifying fibromyxoid tumor. Fourteen months after removal, the tumor recurred and the patient underwent a second excisional biopsy. The tumor recurred a second time 30 months after the first surgery. The patient underwent a third surgery to remove the tumor and received adjuvant radiation therapy. She has been free of disease for more than 18 months. CONCLUSIONS: This is the first known case of ossifying fibromyxoid tumor involving the orbit. Increased cellularity, nuclear atypia, and mitotic activity have been associated with metastatic and recurrent potential. The tumor in this case had moderate cellularity, low nuclear grade, and no mitotic figures. However, the close proximity of the tumor to important structures within the orbit precluded its excision with wide margins and may have predisposed the patient to the two episodes of local recurrence.     

Primitive neuroectodermal tumor of the orbit in an adult: A case report and literature review

ObjectiveThe authors describe the salient clinical, radiologic, and histopathologic features of an orbital primitive neuroectodermal tumor in a 28-year-old man. This is an extremely rare tumor of the orbit, previously reported exclusively in children.DesignCase report.InterventionExcisional biopsy of the tumor en bloc was performed.Main outcome measuresHistopathologic examination was performed by standard techniques and immunohistochemical stains on formaldehyde-fixed, paraffin-embedded tumor tissues.ResultsHistologic examination of the sections of the tumor showed small, blue, round cells with occasional Homer-Wright rosette formations. The tumor cells stained positively with neuron-specific enolase and vimentin.ConclusionsThis newly recognized, highly unusual peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of hypercellular, small, round cell tumor of the orbit in adults.     

Solitary Fibrous Tumor of the Orbit: A Case Series

Purpose: Solitary fibrous tumor (SFT) occurs rarely in the orbit. We present four such cases, representing one of the largest case series reported to date of this rare orbital tumor. Method: The four patients ranged from ages 20 to 50, all of whom presented with lid swelling or bulging in the involved eye. All four patients underwent CT scan to confirm the diagnosis of an orbital mass, which was then excised. Results: The tumors of all four specimens contained spindle-shaped cells with bland nuclei and rare mitotic figures. They all stained positively with CD34 and vimentin. Resection margins were positive in two of the four cases. One of these cases demonstrated residual tumor on follow-up CT scan, which remained unchanged at one-year follow-up. No evidence of residual tumor was found in the other three cases, despite one having malignant pathology. Conclusion: SFT is a rare and generally benign tumor of the orbit. Immunohistochemical testing with CD34 is necessary to confirm the diagnosis. Although en bloc tumor resection is the definitive treatment, residual tumor may remain stable for some time. However, this must be followed closely if complete resection is not carried out.     

Respiratory choristomatous cysts in the temporal orbit.

PURPOSE: To describe the clinical and histopathological findings in two patients with cystic lesions located in the temporal orbit. METHODS: Case reports. RESULTS: Cystic lesions excised from the temporal orbit of two patients were found to be lined with ciliated pseudostratified epithelium, consistent with a choristoma composed of tissue derived from the respiratory system. A review of the literature suggests that this is an uncommon cause of a cystic orbital mass. CONCLUSIONS: Respiratory choristomatous cysts should be considered in the differential diagnosis of cystic lesions of the temporal orbit.     

Malignant peripheral nerve sheath tumor of the orbit in a 15-month-old child. Nine-year survival after local excision.

BACKGROUND: Malignant peripheral nerve sheath tumors are extremely rare orbital tumors that carry a poor prognosis despite wide excision with disfiguring surgery, chemotherapy, or radiotherapy. The authors present the youngest reported case, a 15-month-old boy who underwent an orbitotomy to excise a bilobed tumor from the right orbit. FINDINGS: Histologic examination revealed a malignant peripheral nerve sheath tumor; standard treatment options, including orbital exenteration, chemotherapy, and radiotherapy, were denied and instead the patient was followed with serial computed tomography scans and magnetic resonance imaging. RESULTS: Nine years later, the patient remains without evidence of recurrent tumor and visual acuity is 20/20. CONCLUSION: This patient's course suggests that orbital malignant peripheral nerve sheath tumors, if believed to be completely excised at the time of surgery, might be followed with careful neuroimaging studies for signs of recurrence.     

Orbital solitary fibrous tumor: A report of two cases

Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm that can be found in the orbit. Here, we report two cases affected by orbital SFT. Both patients were female, aged 52 years and 59 years, respectively, and had experienced a painless unilateral orbital lesion. Computed tomography (CT) imaging revealed a well-circumscribed and contrast-enhanced soft tissue mass simultaneously. The tumors were located in the laterotemporal extraconal space of the right orbit and the inferior portion of the left orbit, respectively. Both patients underwent complete resection of their tumors. The histological findings showed alternating hypercellular and hypocellular areas composed of bland spindle cells with a fibrous stroma. The strong immunoreactivity for CD34 supported the diagnosis of orbital SFT. There was no recurrence at the 2-year and 3-year follow-up visits, respectively. SFTs should be considered in the differential diagnosis of an orbital tumor. The combination of a CT scan, histologic findings, and immunohistochemical staining will provide an accurate diagnosis. En bloc excision of the tumor is the mainstay of treatment in order to avoid recurrence.     

Orbital Ewing's sarcoma of the orbit

PURPOSE: Ewing's tumor is a primary tumor of bone in childhood that only rarely involves the orbit. Most such cases are metastatic from distant sites. This tumor may be confused with other small round cell malignancies of childhood, and immunohistochemical studies are essential in making the diagnosis. METHODS: We present two cases of Ewing's tumor of the orbit. One was in a 22-year-old boy with an occult primary tumor in the frontal bone that became symptomatic after forehead trauma. The other example was in a 7-year-old boy with a known Ewing's primary of the clavicle. The clinical manifestations and diagnostic criteria are discussed. RESULTS: In most cases with orbital involvement, ophthalmic symptoms consist of proptosis, pain, and occasionally visual loss and motility restriction. The diagnosis is typically unsuspected before histologic evaluation. Electron microscopic and immunohistochemical analyses are essential in making the diagnosis and are necessary for all such small round cell tumors. CONCLUSIONS: Ewing's sarcoma is a rare orbital tumor of bone mainly affecting children. Local treatment relying on surgical extirpation and radiotherapy alone has proven inadequate, with 5-year survival rates of <10%. The addition of chemotherapy has improved survival rates significantly to approximately 50%.     

Intracranial paraganglioma (glomus tumor) with orbital extension.

PURPOSE: To describe the clinical features of an intracranial glomus tumor with orbital extension. METHODS: Case report. RESULTS: A 15-year-old girl was examined for a large, vascular intracranial mass extending into the orbit. Attempted orbital biopsy was aborted for fear of uncontrollable hemorrhage. Tumor embolization and partial resection was performed. Histology was consistent with paraganglioma (glomus tumor). CONCLUSION: The orbital surgeon should include glomus tumor in the differential diagnosis of vascular orbital lesions. Their vascularity and hemorrhagic potential should be considered when approaching these lesions surgically.