Mesenchymal chondrosarcoma of the orbit: report of a case and review of the literature

AIM: To report a rare case of mesenchymal chondrosarcoma in the orbit and to explore its clinic manifestations, pathologic characters, management and prognosis. METHODS: We report a case of mesenchymal chondrosarcoma of the orbit. The clinical materials, including ophthalmological examination, computed tomography scan of the orbit, histopathology and immunohistochemistry of the biopsy specimen were reported, and its pertinent literatures were reviewed. RESULTS: A 36-year-old female was seen with proptosis and decreased vision. Histopathology demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage. Immunohistochemical studies revealed positivity for vimentin and S-100, which was consistent with the diagnosis of mesenchymal chondrosarcoma. · CONCLUSION: Mesenchymal chondrosarcoma in the orbit is extremely rare malignant tumor. Multi-modality treatments (surgery, chemotherapy and radiotherapy) may lead to long-term survival.     

Mesenchymal Chondrosarcoma of the Orbit

Purpose: To report a case of mesenchymal chondrosarcoma of the orbit and describe its clinical features, radiological findings and management.

Method: Interventional case report.

Result: A 50 year old man presented with right sided proptosis of 3 months duration. CT scan showed well circumscribed lobulated extraconal mass lesion in the inferotemporal qaudrant with specked calcification within. Patient underwent excisional biospsy with excision of mass lesion in toto. Histopathological examination was suggestive of mesenchymal chondrosarcoma of orbit. Post operatively patient was advised radiotherapy.

Conclusion: Orbital mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.     

眼眶间叶性软骨肉瘤——病例报告与文献复习(英文)

目的:报道1例罕见的眼眶间叶性软骨肉瘤,探讨其临床表现、病理特点、治疗以及预后。方法:详细报告1例眼眶间叶性软骨肉瘤病例的临床资料,并检索迄今国内所有该病例报道,结合国外文献进行分析总结。结果:该病例非常罕见,迄今国外报道仅18例,国内报道仅8例。本文报告的1例36岁女性患者,2次手术,1次误诊为血管外皮瘤,2a后因复发行2次手术,术后病理检查:肿瘤组织由未分化的间叶瘤细胞及岛状的软骨细胞组成,免疫组织化学检查:Vimentin(++),S-100(+),病理报告为右眼眶间叶性软骨肉瘤。结论:间叶性软骨肉瘤是发生于有成软骨潜能的原始间叶组织的恶性肿瘤,发生于眼眶者罕见,综合治疗(手术、化学治疗以及放射治疗)可减少复发、提高长期生存率。     

Mesenchymal chondrosarcoma of the orbit

PURPOSE: To describe a patient with mesenchymal chondrosarcoma of the orbit, review clinical and imaging findings, and refine the differential diagnosis from other tumors of the orbit. METHODS: Interventional case report and literature review. RESULTS: A 21-year-old woman with a 6-month history of progressive proptosis presented with left-sided visual loss of recent onset. CT revealed a clearly outlined heterogeneous mass with calcified foci, whereas MRI showed an isointense signal to gray matter on T1- and T2-weighted images. The patient was diagnosed with mesenchymal chondrosarcoma of the orbit only after orbitotomy and subsequent histopathologic study. After surgery, she received adjuvant therapy with irradiation of the orbit. CONCLUSIONS: Mesenchymal chondrosarcoma is a rare malignant tumor of the orbit capable of mimicking several other lesions. Early diagnosis requires a high level of suspicion, particularly with regard to internally calcified lesions. The treatment of choice is complete surgical resection, or, when the tumor is not resectable or residual mass is present after surgery, chemotherapy and radiotherapy. Prognosis for patients with orbital mesenchymal chondrosarcoma is tentative at best, in part because of the rarity of the lesion.     

Mesenchymal chondrosarcoma of the orbit: a report of two cases and review of the literature

Mesenchymal chondrosarcoma, an uncommon lesion in bone and extraskeletal tissue, is extremely rare in the orbit. Two cases of orbital mesenchymal chondrosarcoma in young adults presenting with proptosis and diminution of vision are reported. The diagnosis was established by histopathological examination in both cases, which showed undifferentiated mesenchymal cells with islands of cartilage. Both patients underwent exenteration followed by chemotherapy and radiation therapy and are alive with healthy orbits after two years of follow-up.     

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.     

Spheno-orbital mesenchymal chondrosarcoma. A case report

Mesenchymal chondrosarcoma is a highly malignant and extremely rare tumor of the orbit: only 18 cases have been reported to date. We report a case of spheno-orbital mesenchymal chondrosarcoma in a 36-year-old woman presented with a 4-month history of progressive left exophthalmia and temporal mass. A CT-scan of the orbit and MRI showed a spheno-orbital mass, with temporal fossa extension, fed by the internal maxillary artery visible on cerebral angiography. Surgery via a transcranial, left frontotemporozygomatic approach after selective embolization enabled subtotal removal. Definitive histologic examination revealed mesenchymal chondrosarcoma. Postoperatively, exophthalmia spectacularly regressed. We report our clinical findings and present a review of the literature.     

Mesenchymal chondrosarcoma of the orbit: a clinicopathological study

Extraskeletal mesenchymal chondrosarcoma of the orbit is a rare tumour and generally presents in young females. We report the clinical presentation and histopathological features of a case of orbital mesenchymal chondrosarcoma in a middle-aged man. The prognosis of this tumour is poor and though rare it should be kept in the differential diagnosis of orbital masses.     

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.     

Eyelid Basal cell carcinoma arising on the site of a congenital port wine hemangioma

 Purpose: Only one previous case of eyelid basal cell carcinoma arising in a facial port wine stain without previous local radiotherapy has been reported. We now report a second case. Methods: A 42-year-old female patient with eyelid basal cell carcinoma developing within a facial port wine stain underwent incisional biopsy, surgical excision and repair. Results: The patient had a mass at the inner canthus of the left eye for two years. She had a left facial congenital port wine hemangioma involving the left eyelid, for which no topical treatment had been given. Clinical examination disclosed a 1.5×1.2 cm ulcerated skin mass with irregular borders in the medial canthal region involving the medial aspect of both upper and lower left eyelids. Incisional biopsy revealed basal cell carcinoma. She underwent surgical excision by Mohs’ technique and subsequent reconstructive eyelid surgery. The wound healed well postoperatively. At 2 years of follow up the patient showed no recurrence. Conclusion: Patients with congenital facial port wine stain may develop basal cell carcinoma, and should be regularly monitored.     

Current Orbital Literature

Extraskeletal mesenchymal chondrosarcoma of the orbit is a rare tumour and generally presents in young females. We report the clinical presentation and histopathological features of a case of orbital mesenchymal chondrosarcoma in a middle-aged man. The prognosis of this tumour is poor and though rare it should be kept in the differential diagnosis of orbital masses.     

Orbital chondrosarcoma arising from paranasal sinuses

Orbital chondrosarcoma arising from paranasal sinuses or from the nasal cavity with orbital extension is highly unusual. The aim of this report is to describe our multidisciplinary experience in the treatment of three patients with extensive sino-orbital chondrosarcomas. The patients were aged 43, 75 and 63 years, and the tumors originated from the maxillary, sphenoidal, and ethmoidal sinuses, respectively. Magnetic resonance imaging studies showed homogeneously hypointense lesions on T1-weighted scans, which were hyperintense on T2-weighted images. Intranasal endoscopic surgery alone or in combination with other conventional surgical approaches was the main therapeutic choice. Two patients had recurrences treated with wider surgical removal. Fractionated stereotactic radiotherapy was used in two cases. Two patients had histopathological grade I tumor and one had grade III chondrosarcoma. No patient had regional spread or distant metastasis. All patients were disease-free at 4, 2, and 3 years, respectively, following their last therapeutic interventions. Endoscopic surgery results in lesser morbidity for chondrosarcomas arising from paranasal sinuses and extending into posterior parts of the orbit. However, because control of surgical margins may not be complete, recurrences may occur justifying more radical approaches.     

Primary orbital extraskeletal osteosarcoma

The authors describe a case of orbital extraskeletal osteosarcoma. A 78-year-old man with a history of rheumatoid arthritis on long-term corticosteroids had a left medial canthal basal cell carcinoma excision followed by adjuvant radiotherapy. Twelve months later, he re-presented with a large rapidly-growing calcified mass involving his left medial canthus and orbit. An incisional biopsy demonstrated an infiltrate of atypical cells exhibiting mitotic activity with a rosette arrangement around partially calcified necrotic tissue. The patient underwent orbital exenteration and a partial maxillectomy. Histopathology demonstrated an extraskeletal osteosarcoma. It is extremely rare for this tumor to occur in the orbit. Immunosuppression and adjuvant radiotherapy were possible predisposing factors in the development of this tumor. Extraskeletal osteosarcoma (ESOS) is a malignant tumour that produces osteoid. It develops in soft tissue without continuity to bone or periosteum. It is rare and comprises fewer than 5% of all osteosarcomas. Extraskeletal osteosarcoma primarily affects patients above 50 years of age and has a poor prognosis. In this report, we describe the clinical, radiologic, and pathologic records of a rare case of primary ESOS of the orbit.     

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.     

Primary Orbital Extraskeletal Osteosarcoma

The authors describe a case of orbital extraskeletal osteosarcoma. A 78-year-old man with a history of rheumatoid arthritis on long-term corticosteroids had a left medial canthal basal cell carcinoma excision followed by adjuvant radiotherapy. Twelve months later, he re-presented with a large rapidly-growing calcified mass involving his left medial canthus and orbit. An incisional biopsy demonstrated an infiltrate of atypical cells exhibiting mitotic activity with a rosette arrangement around partially calcified necrotic tissue. The patient underwent orbital exenteration and a partial maxillectomy. Histopathology demonstrated an extraskeletal osteosarcoma. It is extremely rare for this tumor to occur in the orbit. Immunosuppression and adjuvant radiotherapy were possible predisposing factors in the development of this tumor.

Extraskeletal osteosarcoma (ESOS) is a malignant tumour that produces osteoid. It develops in soft tissue without continuity to bone or periosteum. It is rare and comprises fewer than 5% of all osteosarcomas. Extraskeletal osteosarcoma primarily affects patients above 50 years of age and has a poor prognosis. In this report, we describe the clinical, radiologic, and pathologic records of a rare case of primary ESOS of the orbit.     

Primary Ewing sarcoma of the orbit

PURPOSE: To report the clinicopathologic features of a 17-year-old patient with primary Ewing sarcoma of the orbit. METHODS: The patient was evaluated clinically before surgery with computed tomography scans of the orbit. After surgery, the patient was staged with computed tomography scans and bone scan and was treated with systemic chemotherapy and radiation therapy to the orbit. The orbital biopsy was evaluated with conventional light microscopy and immunohistochemistry. RESULTS: Clinical evaluation revealed proptosis and limited upgaze. Computed tomography scans disclosed a mass involving the superior orbit, anterior cranial fossa, and temporal fossa. Microscopic examination revealed small, poorly differentiated cells with medium-sized nuclei containing finely granular chromatin and small nucleoli. The cytoplasmic borders of the cells were indistinct. A PAS stain revealed modest glycogen in many of the tumor cells. The tumor stained positive for O-13 and vimentin and was negative for neural, skeletal, and lymphoid cell markers. Computed tomography scan, bone scan, and blood chemistries revealed no other site of involvement. After treatment, the clinical symptoms and signs resolved, and there has been no evidence of residual orbital tumor or metastasis. CONCLUSIONS: Primary Ewing sarcoma of the orbit should be considered in the differential diagnosis of children or young adults with proptosis, diplopia, or periorbital swelling. Immunohistochemistry is essential to distinguish Ewing sarcoma from other small round cell tumors.     

Clinical findings in a patient with spontaneous arteriovenous fistulas of the orbit

PURPOSE: To report clinical and radiologic findings of a patient with spontaneous arteriovenous fistulas of the orbit. METHOD: Case report. RESULTS: A 73-year-old woman was initially examined with a 1-year history of mild proptosis of the right eye. She had no history of trauma. Neuro-ophthalmologic examination disclosed dilatation of conjunctival vessels, increased intraocular pressure, mild proptosis and bruit in the right eye, and ocular signs suggestive of carotid-cavernous sinus fistulas or orbital arteriovenous malformations. The patient exhibited dilation of the superior ophthalmic vein in enhanced computed tomography of the orbit. Selective cerebral angiography disclosed communications between branches of both ophthalmic and facial arteries and the superior ophthalmic vein in the orbit. CONCLUSION: Arteriovenous fistulas of the orbit must be considered in the differential diagnosis of carotid-cavernous sinus fistulas and arteriovenous malformations, although they are quite rare.     

Rhabdomyomatous mesenchymal hamartoma of the eyelid: report of a case and literature review

PURPOSE: To report a rare case of rhabdomyomatous mesenchymal hamartoma and to compare its features with those cases previously reported. DESIGN: Interventional case report and literature review. INTERVENTION: Complete ophthalmologic and systemic examinations followed by excisional biopsy and histopathologic examination. MAIN OUTCOME MEASURES: Clinical examination features and histopathologic findings. RESULTS: A 6-month-old Latino male presented with a congenital, elevated, smooth, flesh-colored right lower eyelid lesion. An ipsilateral right limbal dermoid and an upper eyelid coloboma were also present. Excisional biopsy of the eyelid lesion revealed randomly oriented mature striated muscle tissue with associated adipose tissue, blood vessels, pilosebaceous units, and peripheral nerves, findings consistent with rhabdomyomatous mesenchymal hamartoma. Of the 24 reported cases (including the current case), eight had associated congenital anomalies. CONCLUSIONS: Although rhabdomyomatous mesenchymal hamartomas are rare and benign, they may be associated with other congenital anomalies and anomaly syndromes. As a result, we recommend systemic evaluation of patients diagnosed with this entity.     

Congenital smooth muscle hamartoma of the conjunctival fornix

PURPOSE: Congenital smooth muscle hamartomas are benign tumors composed of proliferating smooth muscle cells. They are usually seen as abnormal patches of skin. Ocular involvement of congenital smooth muscle hamartomas is unusual, with rare reports of patients with external eyelid involvement or proptosis resulting from orbital tumors. We describe a patient with a congenital smooth muscle hamartoma that involved the tarsal conjunctival fornix. METHODS: Review of the patient's medical records, including the results of ophthalmologic, radiologic, and histologic examinations. RESULTS: A healthy 2-year-old boy was initially seen with a conjunctival mass. He had a discrete, gray, cystic-appearing lesion in the inferior fornix of the left eye. A magnetic resonance imaging study revealed no signs of extension of the lesion into the orbit. The lesion was surgically excised. Histologic sections showed large bundles of smooth muscle with a fibrotic background and interdigitating fat, consistent with a diagnosis of a congenital smooth muscle hamartoma. CONCLUSION: To our knowledge, this is the first report of a patient with a congenital smooth muscle hamartoma arising from the conjunctival fornix. It presumably originated from either the smooth muscle of the vascular endothelium or from the capsulopapebral muscle. Congenital smooth muscle hamartoma should be considered in the differential diagnosis of cystic-appearing conjunctival fornix lesions.