Spindle cell sarcoma is a rare cause of large abdominal mass. As with all cancers, spindle cell sarcoma can spread metastasise to other parts of the body. Spindle cell sarcoma is a type o f connective tissue cancer in which the cells are spindle shaped w hen examined under a microscope see image below. Sarcomas are cancers that begin in muscle, fat, fibrous tissue, or other connective or supportive tissue in the body. When there is an infection or injury or any related immune response mechanism, connective tissues take up the responsibility to begin healing through division and hence if chances are that the tissue could be diagnosed as spindle cell sarcoma then there is a higher risk of cancerous development which would later lead to the formation of that tumor.
This cancer is rare and makes up just 25% of all primary bone cancer cases. February 1923 a biopsy at roosevelt hospital revealed a spindlecell sarcoma. Definition of spindle cell sarcoma nci dictionary of. Jun 18, 2019 icd03 seer sitehistology validation list june 18, 2019 7 baseof tongue c019 embryonal rhabdomyosarcoma 891 89103 embryonal rhabdomyosarcoma 89123 spindle cell rhabdomyosarcoma mixed tumor, malignant, nos 894 89403 mixed tumor, malignant, nos.
The following case of highly malignant, large spindlecell sarcoma of five years duration, originating in the fascia of the thigh and metastasizing to the pleurae and lungs, seemed to be of sufficient rarity and interest to warrant its report. Immunohistochemistry was performed and revealed pan cytokeratin negative, introduction malignant tumors of the vagina are extremely rare and accounts for 2% of all gynecological malignancies. Spindle cell tumour presenting as an abdominal mass. A sarcoma is considered stage iv when it has spread to distant parts of the body. Synovial sarcoma ss is a spindle cell sarcoma that may show epithelial differentiation and a specific chromosomal translocation tx. Pdf primary intimal spindle cell sarcoma of the heart. Spindle cell neoplasm are diverse in nature by means of clinicopathologic and tumor biological heterogeneity 1. In october of 2015 he went for a check up and was told there was a mass on his kidney over the next few months he was going back and forth to the hospital for different checks. The cells which make up a spindle cell sarcoma are long and narrow, which is known as a spindlecell shape1. A spindle cell sarcoma is a cancerous tumor that is made up of soft cells that grow from the mesodermal tissue. It begins on the skin, soft tissues, and connective tissues. Spindle cell sarcoma is usually discussed unde r the umbrella of soft tissue sarcoma. Spindle cell sarcoma occurs more often in men than it does in women, and it is known to most commonly affect. The tumors generally begin in layers of connective tissue such as that under the skin, between muscles, and surrounding organs, and will generally start as a small lump with inflammation that grows.
The evolving classification of round cell sarcomas is driven by molecular alterations. Oct 06, 2019 this case is a spindle cell sarcoma with limited puncture tissue, and it was recommended after tumor removal to perform pathology for tumor differentiation. The shape of the cancer cells is spindle and so it is named spindle cell sarcoma. Mar 22, 2018 spindle cell neoplasm are diverse in nature by means of clinicopathologic and tumor biological heterogeneity 1. I am 18 years old and about a week ago i got what i had been told was a small cyst. Some pathologists try to classify this group of tumors by their cells of origin, but this is not always possible. Although there is a predilection for the extremities. Spindle and round cell sarcoma with ewsr1patz1 gene. In cases of neurological tumors, they tend to be malignant peripheral nerve sheath tumors. At the end of 2015 my dad who is only 54 years old was diagnosed with a rare form of kidney cancer called spindle cell sarcoma. Spindle cell sarcoma characterized by translocation tx. Newer diagnostic techniques, like immunohistochemistry, are now available to pick up even submicroscopic diagnosis of many pathological lesions.
Pathologist, city hospital, new york the following case of highly malignant, large spindle cell sarcoma of five years duration, originating in the fascia of the. It starts with just a small lump and inflammation and then the symptoms slowly progress as the cancer grows from one stage to another. Spindle cell sarcoma is a softtissue tumour which can start in the bone. Jo, in head and neck pathology third edition, 2019 synovial sarcoma. Morbidity and mortality 5year and 10year survival in children and adolescents is 83% and 75%, respectively. The only way to cure a soft tissue sarcoma is to remove it with surgery, so surgery is part of the treatment for all soft tissue sarcomas whenever possible. A woman aged thirtythree years was admitted to the. The soft tissues like muscle, fat, fibrous tissues, nerves, deep skin tissues or blood vessels, which are seems as spindle shaped means long, narrow appearance under microscope, affected by sarcoma called spindle cell sarcoma. But some patients may be cured if the main primary tumor and all of the areas of cancer spread metastases can be removed by surgery. Spindle cell sarcoma can spread to the lungs, so we will closely monitor your chest with xrays at each clinic appointment during your followup. Sarcoma termed is used when the tumors are developed in muscle or bone and they are malignant or cancerous in nature.
Herein, we report two ewsr1patz1 fusion positive spindle and round cell sarcomas with overlapping histological features and polyphenotypic differentiation. Spindle cell hemangioendothelioma mdedge dermatology. Intimal spindle cell sarcomas of the left atrium are extremely rare primary cardiac tumours with three cases reported li et al. Spindle and round cell sarcoma with ewsr1patz1 gene fusion. We report a case of 60 year old man who presented with 2 months history of rapidly growing thyroid enlargement with no obstructive features and preliminary diagnosis of spindle cell lesion of thyroid was made with cytological examination. The biphasic type contains epithelial and spindle cell. This type of cancer is called connective tissue cancer. A rare case of epididymal spindle cell sarcoma in a 68yearold man is described. Therefore, a malignant spindle cell sarcoma may only be diagnosed during surgery, after histological examination. Whats the difference between carcinoma and sarcoma.
When you take the cancer cells, and view them under a microscope, they look like little spindles. The intraabdominal tumors affected female patients, 31 and 53. Its important that your surgeon and other doctors are experienced in the treatment of sarcomas. Spindle cell sarcoma can spread to the lungs, so we will closely monitor your chest. Soft tissue sarcomas epidemiology 2015 estimated new cases of soft tissue cancers 11,930 0.
The spindle cell sarcomas may be removed through surgery, but only if they are located in a single area of the body and the cells have not affected. In the early stages surgery is the best route, but when the cancer has reached to its upper stage, only chemotherapy is the option. The unique immunohistochemical profile and data from expression profiling support ewrs1patz1 spindle and round cell sarcomas as an entity distinct from ewing sarcoma. Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindle shaped when examined under a microscope. Pdf fact sheet on spindle cell sarcoma researchgate. Softtissue sarcomas are rare in adults, accounting for less than 1 percent of all new cases of cancer. Understand the rationale for the use of preoperative radiation in extremity and retroperitoneal soft tissue sarcomas understand the set up and clinical tumor volumes for treatment of extremity soft tissue sarcomas. Three subgroups of spindle cell tumorssarcomas can be identified. Characterized by both collagenized and myxoid zones with bland spindled cells, this tumor has a whirling growth pattern and demonstrates arcades of curvilinear blood vessels. Genetic testing and nf1 gene analysis found a pathogenic variant in exon 32 p. Sarcomas of the esophagus, including carcinosarcoma, are rare neoplasm cases and comprise 0. If the cancer has reached to its upper stage it becomes quite tough for the physician.
We report a case of spindle cell sarcoma which was successfully treated with neoadjuvant chemotherapy due to large size. Incidence and location represents approximately 10% of all soft tissue sarcomas approximately 10% occur in the head and neck. The cells are spindled, simulate a sarcoma but stain for cytokeratin, and usually are more aggressive than nonspindle cell carcinomas. Tenosynovial giant cell tumors also called nodular tenosynovitis. Spindle cell sarcoma is a form of sarcoma that is referred to this way due to the appearance of its tumour cells when they are looked at under a microscope. Ss accounts for approximately 10% of all soft tissue sarcomas. Spindle cell sarcomas of bone represent between 2% and 5% of primary bone malignancies and include a variety of diagnostic groups including. He refered me to a surgical oncologist and told me that the. Spindle cell sarcoma of the bone bone cancer research trust. Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindleshaped when examined microscopically spindle cell sarcoma is a rare cause of large abdominal mass. The most prominent locations for spindle cell sarcoma to develop are in the limbs, but it can form in any part of the body. Lowgrade fibromyxoid sarcoma an overview sciencedirect. Spindle cell lesions of the adult prostate modern pathology.
Ewsr1patz1 fusion positive spindle and round cell sarcoma is one such new tumor entity. Lowgrade fibromyxoid sarcoma lgfms, also called hyalinizing spindle cell tumor with giant rosettes, or evans tumor, is another important diagnostic consideration. In the early stages surgery is the best route, but when the cancer has reached to its. Assistant visiting plysician, city hospital, new york and james r. Spindle cell lipoma is a benign tumor composed of bland spindle cells admixed with mature adipose tissue in a background of thick and ropey collagen. Spindle cell sarcoma treatment can be done in a better way if it is detected in the first stage. Spindle cell sarcoma an overview sciencedirect topics. The best success rate is when it has spread only to the lungs. Understand the set up and clinical tumor volumes for treatment of retroperitoneal soft tissue sarcomas. We present a 69yearold man who first came to medical attention. Spindle cell sarcoma is a rare malignant cancerous tumour which can develop in the bone or soft tissue. Diagnostic approach to soft tissue tumors pdf elsevier health. The patient was admitted to our hospital with a painless, left intrascrotal mass that had been present for 3 months.
A woman aged thirtythree years was admitted to the surgical service of the city hospital in july 1923. Hemangiopericytomas canine spindle cell sarcoma, canine malignant fibrous histiocytoma, canine neurofibrosarcoma, canine perineuroma are common in dogs and rare in cats if they occur at all. It can arise in any part of the body but is most common. Today the surgeon told me it was a malignant spindle cell tumor but only about 2. Spindle cell sarcoma is one type of soft tissue sarcoma. Spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma that has a predilection for young males and most commonly involves the paratesticular region followed by head and neck. Spindle cell sarcoma is a type of cancer that occurs in the soft tissue, it appears like fibrous tissue of the tendons and ligaments. Spindle cell sarcoma of the kidney cancer survivors network. In the oral cavity, the origin of the spindle cell neoplasms may be traced to epithelial, mesenchymal and odontogenic components. While mdm2 overexpression is found in spindle cell sarcomas, it may also be found in other tumours as well, and requires both amplification and molecular abnormalities in others to confirm the diagnosis. Immunotherapy offers new strategy for treating sarcoma. Spindle cell sarcoma treatment what are the options. The tumor is diverse in appearance and several different cell lines produce tumors of similar appearance. The presence of spindle cells on histology is a nonspecific finding, and requires molecular cytogenetics in order to confirm the diagnosis.
It starts with just a small lump and inflammation and then the symptoms slowly progress as the. More than half of sarco mas begin in an arm or leg. This tumor was initially named because it was thought to be derived from fibroblastic cells that surround small vessels. It can arise in any part of the body but is most common in the limbs arms and legs. Spindle cell sarcoma definition of spindle cell sarcoma by. Remarkably, 80% of patients with spindle cell sarcoma have metastases at the time of diagnosis. There is no definite known cause for this type of tumour, but it can sometimes occur as a result of previous radiotherapy treatment. The pleomorphic lipoma is a morphologic continuum of this tumor, exhibiting multinucleated and. Spindle cell soft tissue sarcoma symptoms, treatments. Histopathology demonstrates elongated spindle cells with fusiform to cigarshaped nuclei and indistinct eosinophilic cytoplasm. The rarity of spindle cell sarcoma and its similarities to benign cardiac myxoma and thrombi on echocardiography and cmr imaging present a diagnostic challenge when evaluating patients preoperatively.
In spindle cell sarcoma, cells present in the connective tissue beneath the skin, adjacent areas of the organs, and the muscles begin to create tiny bumps leading to progression of tumor. Primary spindle cell sarcoma scs is an extremely rare entity and one of the least. Spindle cell sarcoma is an uncommon cancerous lesion or tumor that forms in an individuals soft tissues or bone. Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindleshaped when examined under a microscope. Type i neurofibromatosis with spindle cell sarcoma. I am 18 years old and about a week ago i got what i had been told was a small cyst removed from my left shoulder. The main approaches involve checkpoint inhibitors and adoptive t cell therapy, which have shown effectiveness against several. Initially a gastrointestinal stromal tumour gist was diag nosed, but his tumour was subsequently classified as a spindle cell sarcoma. The mesodermal tissue helps in the formation of connective tissue, blood and muscles. These tumours arise in a similar age group to chondrosar. Spindle cell neoplasms can affect the oral cavity and it is often very difficult for the oral pathologists to differentiate it from other similar microscopic simulates.
In october of 2015 he went for a check up and was told there was a mass on his kidney over the next few months he was. Spindle cell sarcoma of thyroid represents a rare entity that may arise primarily from thyroid gland as a variant of thyroid cancer or as metastatic. Soft tissue sarcoma, a diverse group of cancers that arise in the bodys connective tissue, is difficult to treat after it has spread. Spindle cell sarcoma symptoms, prognosis, survival rate. Guidelines ukguidelinesforthemanagementofbonesarcomas. Under a microscope, spindle cells look long and slender. Fibrosarcomas and spindle cell tumors originate from the connective tissue of, or beneath, the skin. Prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. A subset of these lesions arises only within the prostate and generally. The spindle cell sarcomas may be removed through surgery, but only if they are located in a single area of the body and the cells have not affected internal organs. Dec 14, 2006 prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. Primary cardiac sarcoma is rare spindle cell sarcoma is conducted by multiple tests, such as xray, mri scan, bone scan and biopsy.
A sarcoma grows in the bodys connective tissue cells, which include fat, blood vessels, nerves, bones, muscles, deep skin tissues and cartilage. Definition of spindle cell sarcoma nci dictionary of cancer. We will check for this very quickly after your diagnosis by taking ct or pet ct scans. This case is a spindle cell sarcoma with limited puncture tissue, and it was recommended after tumor removal to perform pathology for tumor differentiation. Spindle cell sarcoma of the bone download bone cancer. This type of sarcoma is usually found in the leg, arm, or the torso. Spindle cell sarcoma definition of spindle cell sarcoma. An autopsy confirmed a diagnosis of primary cardiac highgrade spindle cell sarcoma with direct thoracic extension.
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