Spindle cell sarcoma is a malignant soft tissue tumor. Its name is derived from the shape of cells that dominate the histological picture.
SCS may develop virtually anywhere, with predilection sites of subtypes as follows:
- Fibrosarcoma: deep soft tissues of limbs, trunk, head and neck 
- Leiomyosarcoma: uterus and kidneys
- Synovial sarcoma: feet, knees, hands, or otherwise in close proximity to the joints of arms and legs
- Malignant peripheral nerve sheath tumor: limbs and trunk wall, less frequently observed in head and neck region 
SCS, not otherwise specified, is very rare and predilection sites can't be reported .
Depending on the site of tumor growth, SCS may or may not be palpable. If so, they are generally of firm consistency. SCS may exert local mass effects and ensuing symptoms may comprise edema, neurological deficits, gastrointestinal or urogenital complaints, pain and reduced motion ranges, among others. Of note, lesions are not necessarily painful .
Entire Body System
Soft Tissue Mass
Lipomas are common, benign, soft tissue masses in adults. Sarcomatous change within a giant lipoma is a very rare event. [ncbi.nlm.nih.gov]
The presentation is most often with a slowly-enlarging soft tissue mass which may have been noted for some years and gives a false impression of a benign process 4. [radiopaedia.org]
A CT scan of the paranasal sinus showed a soft tissue mass in the left nasal cavity, measuring 5.34 cms x 4 cms, causing pressure effect over the surrounding structures and pushing the left lateral nasal wall and nasal septum to the right, obliterating [ijdr.in]
Furthermore, patients suffering from SCS may experience non-specific constitutive symptoms like fatigue, fever, and weight loss. [symptoma.com]
A 45-year-old man presented with episodes of cough and chest discomfort. CT examination showed multiple nodules of bilateral lungs. The pathology of CT-guided biopsy was consistent with spindle cell sarcoma. [ncbi.nlm.nih.gov]
The patient also had ptosis and miosis in the right eye. Right ulnar nerve palsy with atrophy of hand muscles was seen. [ncbi.nlm.nih.gov]
STROMALE ENDOLINFATICA (182.0) 8931.3;MIOSI STROMALE ENDOLINFATICA (C54.1) 8931.3;MIOSI STROMALE NAS (182.0) 8931.3;MIOSI STROMALE NAS (C54.1) 8931.3;SARCOMA STROMA ENDOMETRIALE A BASSO GRADO ( C54.1) 8931.3;STROMATOSI ENDOLINFATICA (182.0) 8931.3;STROMATOSI [salute.regione.emilia-romagna.it]
It becomes symptomatic after one month to several years after onset and could be associated with visual loss, restriction of eyeball movement, palpable mass or blepharoptosis. [eyeplastics.com]
Anterior Knee Pain
We present a 36-year-old woman with right anterior knee pain and a buttock mass. [ncbi.nlm.nih.gov]
Atrophy of Hand Muscle
Right ulnar nerve palsy with atrophy of hand muscles was seen. His chest X-ray showed bilateral pleural effusion with an opacity involving the apex of the right lung along with mediastinal widening. [ncbi.nlm.nih.gov]
Computed tomography scans or magnetic resonance imaging should be carried out to confirm the presence of a tumor and to assess its extension. In images obtained by means of either technique, SCS typically appear as heterogeneous masses with pronounced contrast enhancement . Invasive growth behavior may be observed.
For tumor grading, representative tissue samples should be obtained. An incisional biopsy should be performed if feasible . Otherwise, core needle biopsies are preferred over fine-needle aspirations. As per definition, spindle-shaped cells dominate the histological picture. These cells have an extended body and tapered ends and may contain elongated nuclei. With regards to cell morphology, distinct degrees of pleomorphism may be observed. Necrotic foci may be present. The share of actively proliferating cells, expressed as the mitotic index, should be determined.
Spindle cell sarcoma (SCS) is a malignant neoplasm of mesenchymal origin. The term is used somewhat inconsistently and may refer to not otherwise specified soft tissue tumors (according to "Pathology and Genetics of Tumors of Soft Tissue and Bone" as proposed by the World Health Organization Classification of Tumors ), or to any of the following entities:
- Malignant fibrous histiocytoma
- Synovial sarcoma
- Malignant peripheral nerve sheath tumor
Although the respective hypothesis is not yet universally accepted, recent evidence implies mesenchymal stem cells to undergo malignant transformation and to form undifferentiated SCS . With regards to the given list, the term malignant fibrous histiocytoma should be avoided since these tumors are no longer assumed to be of histiocytic lineage . The remainder of enumerated tumors originate from connective tissue or smooth musculature, or from as-of-yet undefined tissues. In any case, "spindle" refers to the morphology of tumor cells as observed upon in histopathological analysis.
A considerable share of SCS corresponds to radiation-associated sarcomas . Genetic factors play a major role in SCS etiology; patients suffering from the Li-Fraumeni syndrome, neurofibromatosis type I, Rothmund Thomson syndrome, and Werner syndrome are predisposed for sarcomas .
SCS are likely to invade neighboring tissues and metastasize into distant organ systems, e.g., into lungs, liver, and kidneys. While surgery is mostly indicated to treat SCS, chemotherapy and irradiation may also be used in some cases where surgery cannot be done. However, the patient's prognosis worsens rapidly with increasing tumor stages.
- Fletcher C, Bridge J, Hogendoorn P, Mertens F. World Health Organization classification of tumours of soft tissue and bone: pathology and genetics of tumours of soft tissue and bone. 4 ed. Lyon, France: IARC Press; 2013.
- Rodriguez R, Rubio R, Menendez P. Modeling sarcomagenesis using multipotent mesenchymal stem cells. Cell Res. 2012; 22(1):62-77.
- Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: between the past and the present. Arch Pathol Lab Med. 2008; 132(6):1030-1035.
- Fletcher CD. The evolving classification of soft tissue tumours - an update based on the new 2013 WHO classification. Histopathology. 2014; 64(1):2-11.
- Fernebro J, Bladstrom A, Rydholm A, et al. Increased risk of malignancies in a population-based study of 818 soft-tissue sarcoma patients. Br J Cancer. 2006; 95(8):986-990.
- Folpe AL. Fibrosarcoma: a review and update. Histopathology. 2014; 64(1):12-25.
- Le Guellec S, Decouvelaere AV, Filleron T, et al. Malignant Peripheral Nerve Sheath Tumor Is a Challenging Diagnosis: A Systematic Pathology Review, Immunohistochemistry, and Molecular Analysis in 160 Patients From the French Sarcoma Group Database. Am J Surg Pathol. 2016; 40(7):896-908.
- Toro JR, Travis LB, Wu HJ, Zhu K, Fletcher CD, Devesa SS. Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978-2001: An analysis of 26,758 cases. Int J Cancer. 2006; 119(12):2922-2930.
- Nissim L, Mackstaller L, Hooten J, Motamedi K, Graham A, Taljanovic M. Spindle-cell sarcoma of the popliteal fossa mimicking a benign vascular lesion. Radiol Case Rep. 2010; 5(1):340.
- Coindre JM. Grading of soft tissue sarcomas: review and update. Arch Pathol Lab Med. 2006; 130(10):1448-1453.