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The chance of recurrence leaves room for close clinical follow-up and review surgery, but there is no conclusive evidence supporting radiotherapy or chemotherapy for prevention of recurrence.En bloc surgical excision with complete removal of the tumour is the treatment of choice for spindle cell sarcoma of the orbit.Diagnosis is confirmed using histopathologic features and immunohistochemical studies.MRI reveals intermediate to moderate intensity mass on T1-weighted images before and after gadolinium while angiography shows a richly vascular tumour with uniform staining. CT scan shows well-circumscribed mass with or without bony remodelling with moderate enhancement.Low reflectivity with moderated sound attenuation on ultrasonography might indicate the presence of a solitary fibrous tumour. Solitary fibrous tumours of the orbit are usually diagnosed using various imaging modalities such as ultrasonography, Computerized Tomography, Magnetic Resonance Imaging, and Magnetic Resonance Angiography.The growth of orbital solitary fibrous tumour is usually variable, non-tender, and non-pulsatile. It becomes symptomatic after one month to several years after onset and might possibly be associated with visual loss, restriction of eyeball movement, palpable mass or blepharoptosis.Spindle cell sarcoma of the orbit is a slow-growing, mostly benign tumour presenting with gradual unilateral painless progressive proptosis and pressure symptoms in a middle-aged individual.Symptoms and Signs of Spindle Cell Sarcoma Orbit Orbital solitary fibrous tumours stain positive for CD 34 and BCL-2 on immunohistochemistry. 1 Less common primary sites include the hypopharynx, oropharynx, sinuses, and nasal cavity. In the upper aerodigestive tract, similar to conventional SCC, SpCC occurs most commonly in the larynx. The tumour cells have a characteristic spindle-shaped appearance and are arranged in whorls. Spindle cell carcinoma (SpCC) is a unique and challenging variant of squamous cell carcinoma (SCC). They are well-circumscribed, encapsulated tumours with cellular and cystic areas.They are most commonly seen originating from the pleura and are rarely known to affect extrapleural sites like the orbit. Orbital solitary fibrous tumours show mesenchymal and/or fibroblastic differentiation and have a strong and diffuse immunoreactivity to CD 34.Pathophysiology of Spindle Cell Sarcoma Orbit Orbital solitary fibrous tumours (SFT) remains a highly underdiagnosed tumour due to its rare occurrence outside of the pleural sites and its similarity with other spindle cell tumours. Solitary fibrous tumour is a visceral spindle cell tumour originating from the mesenchyme and is rarely seen in the orbit.It commonly occurs in the pleura, lungs, peritoneum, pericardium, liver, kidneys and rarely in the head and neck region. Spindle cell tumour is a type of connective tissue cancer which is characterized by the presence of spindle-shaped cells when seen under the microscope.Introduction to Spindle Cell Sarcoma Orbit Treatment of Spindle Cell Sarcoma Orbit.Diagnosis of Spindle Cell Sarcoma Orbit.Symptoms and Signs of Spindle Cell Sarcoma Orbit.Pathophysiology of Spindle Cell Sarcoma Orbit.Introduction to Spindle Cell Sarcoma Orbit.

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This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License (creativecommons. Indian J Pathol Oncol 2018 5(3):512-515Ĭopyright © 2018 by author(s) and Indian J Pathol Oncol. u, Jayalakshmy P S, Sideeque A, Mucinous tubular and spindle cell carcinoma kidney with sarcomatoid and rhabdoid differentiation- A rare entity. Initially local and superficial, the lesion may later invade and metastasize. carcinoma developed from squamous epithelium, having cuboid cells and characterized by keratinization. Keywords: Mucinous tubular and spindle cell, Sarcomatoid, Rhabdoid. spindle cell carcinoma squamous cell carcinoma marked by development of rapidly proliferating spindle cells. Sarcomatoid change is very rare in mucinous tubular and spindle cell tumor and we could find only 4 similar cases in the published literature.

We report a case of mucinous tubular and spindle cell neoplasm with areas having sarcomatoid and rhabdoid morphology with large areas of necrosis.

Sarcomatoid change is described in many renal cell carcinomas, indicative of a worse clinical course. Mucinous tubular and spindle cell neoplasm is a rare renal tumor with favourable outcome. Mucinous tubular and spindle cell carcinoma kidney with sarcomatoid and rhabdoid differentiation- A rare entityĪuthor Details : Fadiya Zainudeen, Umasankar.