A Rare Case of Primitive Epithelioid Leiomyosarcoma of the Conjunctiva

Guerriero, Silvana; Sborgia, Alessandra; Giancipoli, Giovanni; Fiore, Maria; Ross, Roberta; Piscitelli, Domenico

doi:10.3109/01676830.2011.574771

Cited by 14 publications

(22 citation statements)

References 6 publications

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“…[ 1 8 9 10 ] According to the literature, three of the cases of conjunctival leiomyosarcoma underwent orbital exenteration. [ 4 5 7 ] In our case, and in the case reported by Kenawy et al .,[ 6 ] a more conservative approach was performed (tumor excision with clear margins). Nevertheless, due to its aggressive course and tendency to recur, an orbital exenteration or extended enucleation should be considered as the treatment of choice.…”

Section: Discussionsupporting

confidence: 79%

“…in their report described a case of a 56-year-old female with a fast growing mass, originating in a previous conjunctival lesion that she had for years. [ 5 ] An examination using electron microscopy showed irregular cells and often grooved nuclei with prominent nucleoli, moderate amounts of cytoplasmic thin filaments with focal densities, immature cell junctions, and discontinuous basal lamina material along the tumoral cell surface. A CT scan showed adenopathies, which were probably of a metastatic origin.…”

Section: Discussionmentioning

confidence: 99%

“…[ 7 ] Due to the differential diagnosis with other types of tumors, such as spindle cell lymphoma, large cell lymphoma, melanoma, and undifferentiated sarcoma, immunohistochemical staining plays an important role in the correct identification of leiomyosarcomas. [ 5 ] Due to the hematogenous spread of leiomyosarcomas, a positron emission tomography-CT scan is also required.…”

Section: Discussionmentioning

confidence: 99%

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Conjunctival leiomyosarcoma: A case report and review of literature

Montes¹,

Agra

Alonso

et al. 2017

Indian J Ophthalmol

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Leiomyosarcoma is a malignant mesenchymal tumor that is very uncommon in the conjunctiva. Nevertheless, we describe here the clinical manifestations, management, and prognosis of a rare case of leiomyosarcoma in this location. An 81-year-old male presented at a tertiary hospital with a rapidly growing mass. After performing biopsy, histopathological examination revealed the existence of a conjunctival leiomyosarcoma. On this diagnosis, a thorough metastatic screening was performed showing no enlarged lymph nodes or metastatic deposits anywhere in the body. To treat the condition, we performed an evisceration with clear margins and subsequent radiotherapy.

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Conjunctival leiomyosarcoma: A case report and review of literature

Montes¹,

Agra

Alonso

et al. 2017

Indian J Ophthalmol

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“…1 Most primary cases of leiomyosarcoma occur in the posterior orbit; however, anterior, lacrimal gland, and even conjunctival cases have been described. [2][3][4] The primary orbital tumors are thought to originate in primitive replicating cells associated with blood vessels in the posterior orbit and possibly Mueller's muscle when present in the anterior orbit. In metastatic lesions, the primary tumors are often pelvic, cutaneous, or gastrointestinal in origin.…”

Section: Discussionmentioning

confidence: 99%

Orbital leiomyosarcoma metastasis presenting prior to diagnosis of the primary tumor

et al. 2017

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SummaryLeiomyosarcomas, neoplasms of smooth muscle, are rarely found within the orbit. Orbital leiomyosarcoma may be primary, metastatic, or secondary to radiation. When they are metastatic, patients almost exclusively have a history of a primary leiomyosarcoma, often occurring in the spermatic cord, skin, gastrointestinal tract, or the uterus. We present the case of 48-year-old woman who presented with a metastatic orbital leiomyosarcoma, which was identified before the primary tumor. Case ReportA 48-year-old woman with a history of primary openangle glaucoma presented at Massachusetts Eye and Ear with binocular, horizontal diplopia, intermittent left retrobulbar pain, and mild discomfort of the left eye with eye movement. The patient complained of a 4-month history of hypoesthesia of the left cheek, which she associated to apparent nerve injury from ipsilateral injection of a local anesthetic for a root canal performed just prior to onset of symptoms. The patient's visual acuity was 20/15 in each eye, with slight dyschromatopsia in both eyes. She had a superior arcuate visual field defect in the left eye secondary to glaucoma ( Figure 1A). There was 1 mm of proptosis and limitation of abduction of the left eye resulting in an incomitant esotropia larger in left gaze. Magnetic resonance imaging (MRI) demonstrated a well circumscribed lesion in the left orbital apex that appeared inseparable from the left lateral rectus muscle; the lesion enhanced slightly less than muscle, with more prominent enhancement of the rim of the lesion (Figure 2).Given that the lesion appeared intrinsic to the extraocular muscle, a common site to harbor an orbital metastasis, the patient underwent a systemic work-up searching for an occult primary malignancy. An 18-fluorodeoxyglucose positron emission tomography (18-FDG PET) scan showed a right lower quadrant mass that was lobulated and heterogeneously enhancing with 18-FDG avidity in the pelvis demonstrating its hypermetabolic nature (Figure 3).A radiologically guided core needle biopsy was performed of the mass. The pathology demonstrated eosinophilic spindle-shaped cells with elongated nuclei, scattered mitotic figures, and areas of ischemic degeneration. The lesion stained positive for both desmin and smooth muscle actin, in keeping with a malignant smooth muscle neoplasm, specifically a leiomyosarcoma (Figure 4). Systemic chemotherapy was being considered for management of her metastatic disease when the patient developed worsening retrobulbar pain (2 months after initial presentation). On examination, her visual acuity was reduced to 20/25, and there was notable worsening of the dyschromatopsia and visual field defect in the left eye ( Figure 1B). In addition, she had further limitation of abduction of the left eye. Repeat MRI scan demonstrated enlargement of the lesion, with compression of the left optic nerve laterally ( Figure 5). In order

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“…Conjunctival Leiomyosarcoma has also been reported, which may originate from previous undiagnosed leiomyoma. [9] This lays emphasis on the fact that these benign lesions may turn malignant; hence follow-up of patients with conjunctival leiomyoma is mandatory.…”

Section: Discussionmentioning

confidence: 99%

Conjunctival leiomyoma; a rare tumor of the ocular surface

Afghani¹,

Khan²,

Mansoor³

2016

J Ophthalmic Vis Res

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Purpose:To report a case of conjuctival leiomyoma.Case Report:An 18-year-old female patient presented with a conjunctival growth in the left eye for a 2-year-period with no change in size. She merely presented for cosmetic reasons. Visual acuity was 20/20 in each eye. The lesion had prominent vessels and was not adherent to the sclera. The growth resembled a benign lesion of the conjunctiva. The tough and vascular tissue was excised and sent for histopathology. The defect was covered by an end to end conjunctival suturing. Histopathology showed the lesion to be conjunctival leiomyoma. Trichome was used to highlight the spindle shaped cells and immuno-histochemistry using anti-smooth muscle actin antibody and Vimentin was applied to confirm the diagnosis.Conclusion:This case adds to the total number of cases of conjuctival leiomyoma reported in the literature to date. An ophthalmologist needs to think beyond a common mass when encountering a conjunctival growth extending onto the cornea.

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A Rare Case of Primitive Epithelioid Leiomyosarcoma of the Conjunctiva

Abstract: primary involvement of the orbit by a leiomyosarcoma is rare, but this eventuality should be considered in the differential diagnosis of rapidly growing orbital and conjunctival masses.

Cited by 14 publications

References 6 publications

Conjunctival leiomyosarcoma: A case report and review of literature

Conjunctival leiomyosarcoma: A case report and review of literature

Orbital leiomyosarcoma metastasis presenting prior to diagnosis of the primary tumor

Conjunctival leiomyoma; a rare tumor of the ocular surface

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