Carcinosarcoma of the Orbit: Report of Two Cases and Review of the Literature

Abstract

Objective-To report the clinicopathologic features of 2 patients with carcinosarcoma of the orbit. Design-Case reports.Participants-Two patients with orbital carcinosarcoma were identified. Methods-Retrospective chart review with clinicopathologic correlation and literature review.Main Outcome Measures-Clinical examination, imaging studies, and histopathologic findings.Results-Two patients, a 56-year-old woman and a 91-year-old woman, with orbital carcinosarcoma were identified. Both tumors contained sarcomatous and carcinomatous components and invaded periorbital structures.Conclusions-Carcinosarcoma may arise in the orbit or extend into the orbit from the paranasal sinuses. This malignant neoplasm should be aggressively treated with a combination of surgical resection, chemotherapy, and radiation therapy.Carcinosarcoma (true malignant mixed tumor) is a biphasic tumor consisting of intermixed malignant epithelial (carcinomatous) and mesenchymal (sarcomatous) components. Carcinosarcoma has been described to arise in many tissues, including the female genital tract, 1, 2 lung,3 kidney,4 bladder, 5 esophagus, 6 biliary tract, 7 and skin. 8 There are 3 reports of carcinosarcoma arising in the orbit 9-11 and an additional case of parotid gland carcinosarcoma that metastasized to the orbit. 12 Although this is a rare orbital tumor, ophthalmologists and pathologists should be aware of the clinicopathologic features for aggressive management. We report 2 patients with carcinosarcoma of the orbit and briefly review the literature. NIH Public Access Author ManuscriptOphthalmology. Author manuscript; available in PMC 2010 November 30. Case ReportsCase 1-A 56-year-old African-American woman with hypothyroidism, but otherwise healthy, was evaluated for a 3 to 4-month history of right epiphora and a tender right orbital mass. The best-corrected vision was 20/40 − in her right eye and 20/30 − in her left eye. Examination showed a firm, tender 1×1×1-cm nodule between the nasal bone and the right medial canthus (Fig 1). There was mild edema and ecchymosis overlying the mass. Her intraocular pressures were 22 mmHg in both eyes. There was a slight limitation of the right upgaze and 2 mm of right proptosis. The remainder of the ophthalmic examination results was normal. An otolaryngologic examination performed 1 week earlier showed on direct visualization a submucosal bulge anterior to the attachment of the middle turbinate consistent with the lacrimal sac area. CT showed a 2.3×2.0×2.9-cm mass arising from the right nasofrontal recess and eroding into the ethmoid sinus, medial orbital wall, and inner table of the right frontal sinus (Fig 2). A right medial orbitotomy was performed, and the tumor was found to be posterior to the globe and adherent to the periorbita and skin. An incisional biopsy was performed, and a frozen section of the biopsy was diagnosed as spindle cell neoplasm. After examination of permanent sections, the tumor was diagnosed as carcinosarcoma. The treatment plan was extensive surgery, including orbi...

Carcinosarcoma de laringe: Reporte de dos casos

“…El carcinosarcoma es un tumor bifásico maligno ra ro con componentes carcinomatoso y sarcomatoso 7 . Respecto a su histogénesis se han postulado varias teorías, dentro de las cuales se le ha atribuido un origen secundario a una proliferación mesenquimática reactiva a un carcinoma, también como un tumor de colisión en el cual el componente carcinomatoso y sarcomatoso se desarrollarían independientemente, pero de manera simultánea, y como un tumor que podría surgir desde restos embriológicos epiteliales y mesenquimáticos.…”

“…Los términos carcinoma sarcomatoide, pseudosarcoma y carcinoma de células fusiformes hacen referencia a un carcinoma de células escamosas con células fusiformes que simulan un sarcoma, en los cuales el componente epitelial es positivo para citoqueratina y el componente sarcomatoso es positivo para citoqueratina y ocasionalmente para vimentina. En contraste, la denominación carcinosarcoma corresponde a un tumor bifásico con 2 componentes malignos distinguibles, en el cual solo el componente carcinomatoso es positivo para citoqueratina y el componente mesenquimático para vimentina y otros marcadores que dependen de la diferenciación expresada por el tumor 7,15 . En cuanto a la laringe, los términos carcinosarcoma y tumor maligno mixto utilizado en la literatura puede hacer referencia al mismo tipo de tumor en cuestión 16 .…”

“…Su histogénesis es controversial existiendo distintas teorías al respecto 3,7 . A pesar de los primeros reportes en donde se postuló al carcinosarcoma como un tumor de colisión, mucha evidencia se ha acumulado a favor de que éste tenga un origen monoclonal 8 .…”

Corneal carcinosarcoma in a patient with Fanconi anaemia

“…Corneal carcinosarcoma in a patient with Fanconi anaemia Carcinosarcoma is a rare tumour that may originate from several tissues, for example the female genital tract, lungs, kidney, bladder, oesophagus, bladder and skin (Prakalapakorn et al 2008). In the eye region, a carcinosarcoma most often originates in the lacrimal gland (Takahira et al 2005).…”

“…Carcinosarcoma is a rare tumour that may originate from several tissues, for example the female genital tract, lungs, kidney, bladder, oesophagus, bladder and skin (Prakalapakorn et al. 2008).…”