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Spindle Cell Sarcoma

Sarcomas Spindle Cell

Spindle cell sarcoma is a malignant soft tissue tumor. Its name is derived from the shape of cells that dominate the histological picture.


Presentation

SCS may develop virtually anywhere, with predilection sites of subtypes as follows:

  • Fibrosarcoma: deep soft tissues of limbs, trunk, head and neck [6]
  • 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 [7]

SCS, not otherwise specified, is very rare and predilection sites can't be reported [8].

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 [9].

Furthermore, patients suffering from SCS may experience non-specific constitutive symptoms like fatigue, fever, and weight loss.

Mediastinal Lymphadenopathy
  • The patient's CTscan of chest strongly suspected a malignant mass in right upper lobe with extensive mediastinal lymphadenopathy, pleural metastases and pericardial involvement. He was started on oxygen inhalation, dexamethasone, and clopidogrel.[ncbi.nlm.nih.gov]
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]
Leg Swelling
  • He sought medical attention after experiencing shortness of breath, cough, chest pain, abdominal pain and bilateral leg swelling for about 6 weeks.[ncbi.nlm.nih.gov]
Constitutional Symptom
  • Furthermore, patients suffering from SCS may experience non-specific constitutive symptoms like fatigue, fever, and weight loss.[symptoma.com]
Chest Discomfort
  • 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]
Cough
  • 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]
  • He sought medical attention after experiencing shortness of breath, cough, chest pain, abdominal pain and bilateral leg swelling for about 6 weeks.[cardiothoracicsurgery.biomedcentral.com]
Dyspnea
  • We report on a 42-year-old man, who presented to the emergency department with dyspnea and decreased exercise tolerance. A computed tomography chest scan showed a large mass in the left atrium.[ncbi.nlm.nih.gov]
  • The complications of Spindle Cell Carcinoma of Lung can include: Dyspnea (shortness of breath): If the cancerous tumor expands to block important, large, or small airways of the chest or lung Hemoptysis (coughing-up blood): Individuals may cough-up blood[dovemed.com]
Abdominal Mass
  • A 73-year-old man presented with a huge intra-abdominal mass. Initially a gastrointestinal stromal tumour (GIST) was diagnosed, but his tumour was subsequently classified as a spindle cell sarcoma.[ncbi.nlm.nih.gov]
  • It can present as a large abdominal mass. We report the case of a 65 year old female who presented with abdominal distention. CT scan showed a mass occupying the whole of the abdomen and pelvis.[casereports.in]
Heart Failure
  • Right ventricular sarcoma is a very rare cause of right sided heart failure, a very aggressive tumour whose only chance of successful treatment is complete surgical excision.[ncbi.nlm.nih.gov]
  • Precautions must be taken for kidney failure, heart failure, lung failure, large fluid and electrolyte loss, diabetes mellitus and liver failure.[oncolex.org]
Miosis
  • 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]
Blepharoptosis
  • 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.[eyeplastics.com]
Ptosis
  • 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]
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]
Knee Pain
  • We present a 36-year-old woman with right anterior knee pain and a buttock mass.[ncbi.nlm.nih.gov]
Scrotal Mass
  • They usually manifest themselves as a scrotal mass that should be differentiated from testicular tumors. There are problems regarding their pathologic diagnosis and treatment.[ncbi.nlm.nih.gov]

Workup

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 [9]. Invasive growth behavior may be observed.

For tumor grading, representative tissue samples should be obtained. An incisional biopsy should be performed if feasible [10]. 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.

Pericardial Effusion
Mediastinal Mass
  • Following magnetic resonance imaging and CT-guided biopsy of the mediastinal mass, spindle cell sarcoma of the pericardium was diagnosed. The tumor was deemed unresectable due to invasion of vital mediastinal structures.[ncbi.nlm.nih.gov]
Hepatocellular Carcinoma
  • MLL4 was previously reported to be similarly rearranged in hepatocellular carcinomas, notably those positive for hepatitis B virus.[ncbi.nlm.nih.gov]
  • carcinoma (a type of liver cancer) in certain patients who have already been treated with sorafenib.[cancer.gov]

Treatment

  • Optimal treatment is to obtain complete surgical clearance of the tumour.[ncbi.nlm.nih.gov]

Prognosis

  • Given the poor prognosis and rapid progression of disease, early diagnosis is crucial.[ncbi.nlm.nih.gov]

Etiology

  • 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.[symptoma.com]
  • Approximately 80% of chondrosarcomas are primary lesions arising in healthy tissue, with no know etiologic factor.[emedicine.medscape.com]
  • (Etiology) The exact cause of Spindle Cell Carcinoma of Lung is unknown.[dovemed.com]
  • Evidence for hormonal etiology in pseudoangiomatous hyperplasia of mammary stroma. Am J Surg Pathol 15:145–149 PubMed Google Scholar [6] Arbabi L, Warhol MJ (1982) Pleomorphic liposarcoma following radiotherapy for breast carcinoma.[springerlink.com]

Epidemiology

  • Epidemiology The true incidence of prostatic stromal sarcomas has not been established because of the rarity of this tumor. [1, 2, 3, 4, 13, 14, 15, 16, 17, 18, 19] In the largest series, patients' ages ranged from 25 to 86 years, and at least one half[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology of Spindle Cell Sarcoma Orbit Orbital solitary fibrous tumours show mesenchymal and/or fibroblastic differentiation and have a strong and diffuse immunoreactivity to CD 34.[eyeplastics.com]

Prevention

  • I have been looking for alternative medicines which can help prevent any further recurrence. I am getting in touch with an ayurvedic doctor who has been treating a lot of cancer patients, especially Leukemia.[inspire.com]
  • 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.[eyeplastics.com]
  • Aloxi listen (uh-LOK-see) A drug used to prevent nausea and vomiting caused by chemotherapy. It is also used to prevent nausea and vomiting after surgery.[cancer.gov]
  • Occasionally ghost cells in the center and multinucleated giant cell may present, grading is suggested in soft tissue sarcoma in order to improve the management, prognosis and to prevent the recurrence, based on nuclear atypia, nuclear overlap, mitotic[casereports.in]

Summary

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 [1]), or to any of the following entities:

Although the respective hypothesis is not yet universally accepted, recent evidence implies mesenchymal stem cells to undergo malignant transformation and to form undifferentiated SCS [2]. 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 [3]. 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 [4]. 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 [5].

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.

References

Article

  1. 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.
  2. Rodriguez R, Rubio R, Menendez P. Modeling sarcomagenesis using multipotent mesenchymal stem cells. Cell Res. 2012; 22(1):62-77.
  3. Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: between the past and the present. Arch Pathol Lab Med. 2008; 132(6):1030-1035.
  4. Fletcher CD. The evolving classification of soft tissue tumours - an update based on the new 2013 WHO classification. Histopathology. 2014; 64(1):2-11.
  5. 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.
  6. Folpe AL. Fibrosarcoma: a review and update. Histopathology. 2014; 64(1):12-25.
  7. 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.
  8. 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.
  9. 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.
  10. Coindre JM. Grading of soft tissue sarcomas: review and update. Arch Pathol Lab Med. 2006; 130(10):1448-1453.

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Last updated: 2019-06-28 11:09