It has been suggested that SpCC may develop after radiation exposure; however, some authors believe that this is not a major etiologic factor. It is a very aggressive form of cancer, and patients who are diagnosed generally do not live more than five years. and down-regulation of microRNAs implicated in the induction of epithelial-mesenchymal transition. Similar to conventional SCC, SpCC has been etiologically related to cigarette smoking and alcohol consumption. Microscopic examination exhibits a proliferation of invasive spindle cells, typically accompanied by a squamous epithelial component (consisting of dysplastic surface epithelium, carcinoma in situ, or conventional invasive squamous cell carcinoma). Polypoid glottic tumors appeared to have the most favorable prognosis (90% 3-year survival rate), whereas patients with supraglottic, hypopharyngeal, sinonasal, and oral SpCCs did poorly regardless of their tumor's gross appearance. Spindle cell sarcoma is a rare malignant (cancerous) tumour which can develop in the bone or soft tissue. Cytokeratin expression can be demonstrated in spindle cells in 40%–85% of cases. Therefore, they have to be considered a variant of SCC in which the pleomorphic component originates through dedifferentiation of the SCC component.353–360 Molecular pathology has recently provided convincing proof of an evolution of the sarcomatoid component from the conventional one.360 Metastasis is to the cervical lymph nodes; the deposits may exhibit conventional SCC, SpCC, or both together.355,357,361,362, An SpCC typically occurs in the oral cavity and the larynx355,362; less frequently, it may arise in the sinonasal area and pharynx. Spindle cells always express vimentin and often other mesenchymal filaments, such as myogenic markers (smooth muscle actin, muscle specific actin, desmin). In a clinicopathologic review of 78 cases of pleomorphic carcinoma of the lung, 18% of patients had incidental findings on chest X-ray and spindle cell carcinoma was most frequently associated with giant cell carcinoma [1] . (a) Polypoid and ulcerated tumor consisting of sheets of malignant spindle cells with moderate amounts of eosinophilic cytoplasm. For example, any mitotic activity in a smooth muscle neoplasm of deep soft tissue or in a neurofibroma is usually indicative of a malignant diagnosis, whereas this is not true for myofibroblastic or “fibrohistiocytic” lesions. Whether or not the entire mass can be removed will depend on where the tumor is located. In spindle cell tumors the important morphologic features to evaluate on hematoxylin and eosin–stained sections are similar to those for other mesenchymal neoplasms, including the following: Architectural arrangement of the tumor cells (growth pattern): long or short fascicles, whorls, sheets, or haphazard architecture, Interface between tumor and adjacent tissues: pushing/expansile or infiltrative borders, Amount and type of extracellular matrix: prominent, scant, or inconspicuous; collagenous, hyalinized, or myxoid, Intratumoral vascularity: well-developed or inconspicuous; muscular thick-walled or thin-walled vessels, hyalinized vessel walls, branching (hemangiopericytoma [HPC]-like) vessels, Cytomorphology: long or short spindle cells, uniformity or pleomorphism, amount and quality of the cytoplasm, nuclear features, degree of atypia. Spindle cells with a vague storiform pattern of DLBCL are not familiar to pathologists. The spindle cell component usually forms the bulk of the tumor. Microscopically, the SCC component may be well-, moderately- or poorly differentiated, keratinizing or nonkeratinizing, and transition between the two components may be abrupt or gradual. This type of carcinoma can cause a large mass, or tumor, which is generally surgically removed during the initial stages of treatment. Oscar Lopez‐Nunez, Lea F. Surrey, Rita Alaggio, Karen J. Fritchie, Ivy John, Novel PPP1CB‐ALK fusion in spindle cell tumor defined by S100 and CD34 coexpression and distinctive stromal and perivascular hyalinization, Genes, Chromosomes and Cancer, 10.1002/gcc.22844, 59, 8, (495-499), (2020). Spindle cell sarcoma is a type of cancer which occurs on the connective tissues of the body. Spindle cell carcinoma is a type of cancer which usually originates in the connective tissues of the body. How well they work will depend on whether or not the disease has moved out of the initial area. The World Health Organization (WHO) recognizes SpCC as a variant of SCC, and includes the term sarcomatoid squamous cell carcinoma . Keywords: Spindle-cell carcinoma, Second primary tumor, Synchronous cancer I. Squamous differentiation may be present including keratinization and intercellular bridges. Lymphocytes and plasma cells may be present. SAUL SUSTER, CESAR A. MORAN, in Modern Surgical Pathology (Second Edition), 2009. Foci of osteosarcomatous, chondrosarcomatous or rhabdosarcomatous differentiation may be present, particularly in patients who had previously been treated by radiotherapy (Fig. 8). Spindle cell carcinomas occur at every head and neck subsite but have a predilection for the oral cavity and larynx. Infiltrative borders, tumor necrosis, atypical or hyperchromatic nuclei, and mitotic activity may or may not be indicative of malignancy, and they should be interpreted according to the line of differentiation and other features of the lesion. Typically, spindle cell carcinoma can occur in any connective tissue in the body, although it is more common in some areas than others. The former component may be very scant or limited to noninvasive areas of epithelial dysplasia or carcinoma in situ located at the surface of the tumor, and its identification may require extensive sampling for histologic examination. Combined chemoradiation therapy frequently shows similar findings but with a greater degree of mucosal and submucosal glandular atypia and distortion. Spindle cell sarcoma is a soft-tissue tumour which can start in the bone. Any large mass, ulcer, sore, or discolored areas of skin should be checked by a trained medical professional. Sixty percent of 53 patients with tumors of the oral cavity died within 1 month to 6 years, 77% of 13 patients with tumors of the sinonasal tract died within 6 months to 2.5 years, and 34% of 65 patients with tumors of the larynx died within 4 months to 2 years. The only known cause of … Spindle cell sarcomas of the bone are often found in the arms, legs and pelvis. LewisJr., R.D. 2-35). Certain activities or behaviors may make you more prone to spindle cell carcinoma. The most sensitive and reliable epithelial markers to be used for demonstration of the epithelial phenotype are keratin (AE1/AE3), K1, K18, and epithelial membrane antigen.359 Moreover, double-labeling has indicated keratin and vimentin in individual spindle cells,356 thereby illustrating the versatility of the intermediate filament phenotype. Because these tumors demonstrate prominent exophytic growth with less extension into the esophageal wall, they have been associated with relatively good survival rates. Spindle cell carcinoma represents a rare variant of squamous cell carcinoma, characterized by spindled tumor cells that simulate a sarcoma but are epithelial in nature. Branching cystic glands can occasionally be identified, lined by mucinous or ciliated respiratory cells. Storiform or giant cell areas may also be present. The most sensitive/reliable epithelial marker for SpCC seems to be keratin (AE1/AE3, K1) K1, K18 and EMA. This latter feature may be helpful in making the appropriate diagnosis. After radiation exposure, bizarre granulation tissue–containing pleomorphic spindle cells and atypical mitotic figures may develop that should not be misinterpreted as tumor recurrences displaying the histomorphology of SpCC277; this was discussed previously in the section on laryngeal SCC. Spindle cell carcinomas are characterized by a proliferation of spindle cells with varying degrees of atypia and mitotic activity, often exhibiting transitions with areas displaying the features of conventional spindle cell thymoma (Fig. The elongated nuclei have a syncytial pattern with delicate nuclear chromatin and small nucleoli (Figure 24-87) The epithelial cells are more polygonal and are arranged in glands, tubules, trabeculae, cords, papillae, glomeruloid structures, and sheets (Figure 24-87). Spindle Cell Carcinoma Spindle cell carcinoma (SpCC), also referred to as sarcomatoid carcinoma, is a biphasic tumor composed of conventional SCC and malignant spindle cells. Oral spindle cell carcinomas usually are treated by surgical resection, alone or combined with radiotherapy. 2-30). They most commonly arise in patients over the age of 40 and are extremely rare - making up just 2-5% of all primary bone cancer cases. Spindle Cell Squamous Cell Carcinoma (SCC) of Skin is a malignant tumor of skin that is typically seen with a higher frequency in immunosuppressed individuals. The epithelial origin of the spindle cells has been recently confirmed by both immunohistochemical and electron micrographic studies.1, 20 The mesenchymal component may show liposarcoma, rhabdomyosarcoma, leiomyosarcoma, or even chondro- or osteosarcoma differentiation. The differential diagnosis of spindle cell lymphoma of the uterine cervix includes chronic cervicitis, soft tissue sarcoma, spindle cell carcinoma, spindle cell melanoma and dendritic cell tumour. (B) Spindle cell carcinoma with abundant edematous, myxoid stroma. The epithelioid cells have a resemblance to thymomas or thymic carcinomas with epithelioid to spindle-shaped cells arranged in sheets and nests (Figure 24-89). Currently, there is ample evidence that SCC cells can exhibit differentiation toward cells with a mesenchymal phenotype. (b) Higher-power view of the spindle cell (sarcomatoid) component showing poorly formed fascicles of pleomorphic malignant spindle cells (20 ×). An SpCC should not be confused with the so-called teratocarcinosarcoma.368,369 This neoplasm typically occurs in the nasal cavity and the paranasal sinuses, sites at which SpCC rarely occurs, and is characterized by an extremely diverse histologic pattern with mature and immature glands, benign squamous and malignant poorly differentiated epithelia, and rhabdomyosarcomatous, chondrosarcomatous, and neuroepithelial differentiation (Fig. Several other terminologies have also been used including polypoid squamous cell carcinoma, … Fig. 8. Spindle cell (sarcomatoid) carcinoma of the breast is a rare variant of breast cancer that has been classified under the broad rubric of metaplastic carcinoma. Spindle cell carcinoma is an unusual variant of SCC where part, or all, of the tumor resembles a sarcoma (Figure 28). Smoking or chewing tobacco, sun exposure, alcohol use, and exposure to certain chemicals may raise the risk of developing this and other types of cancer. 2-33). When cells from this type of cancer are viewed under a microscope, they appear spindle-shaped. Spindle cell cancer is strongly associated with cigarette smoking, as our patient had a significant smoking history as well . Most cancers made up of spindle cells are called sarcomas. Treatment of SpCC is the same as for conventional SCC. Although reported survival rates vary, one recent study based on epidemiologic data from the United States reported 5-year disease-specific survival of 39% for spindle cell carcinoma of the oral cavity. The type of tumour depends on the location in the body and the way the cells look when examined under the microscope. The converse situation, granulation tissue mimicking SpCC, may occur after ionizing-radiation exposure. It is more common in areas that have been exposed to the sun, although this is not always the case. There is a component of SCC in situ at the base of the polypoid mass (arrow) confirming this to be a carcinoma and not a true sarcoma (2 ×). Various factors result in spindle cell carcinoma, including genetic predisposition, injury and inflammation. You can barely notice it unless check under the microscope. (B) Apico-basal orientation. In fact, many previously reported presumed primary soft tissue sarcomas of the esophagus, in retrospect, probably represent spindle cell carcinomas with a predominant undifferentiated soft tissue sarcoma component. Immunohistochemically, tumor cells in SpCC often express epithelial and mesenchymal markers. Risk factors for spindle cell carcinoma of the upper aerodigestive tract include tobacco use, alcohol consumption, and previous radiotherapy. A diagnosis of a SpCC is based on demonstration of an invasive or in situ SCC and a malignant spindle cell component. Calcifications can be seen in the stroma. Therefore, discerning this lesion from SpCC should not be too problematic. SETTLE: This is a highly cellular biphasic tumor showing primitive thymus histology, characterized by an admixture of spindle-shaped cells that merge with epithelial cells (Figure 24-86). There are really no clinical symptoms that would distinguish this particular cancer from other types of breast cancers. The cause of the cancer is generally unknown, but it is influenced by … (A) Small islands and cords of squamous cell carcinoma and dense proliferation of neoplastic spindle cells. These pale staining cells are cuboidal to columnar. It can arise in any part of the body but is most common in the limbs (arms and legs). Recently, p63 has been reported as a useful marker for SpCC.364, As SpCCs may, in their spindle cell component, exhibit not only vimentin expression but also other mesenchymal filaments, especially myogenic markers, positivity for this marker does not rule out a diagnosis of SpCC.359 Even the absence of keratin positivity cannot be considered evidence against a diagnosis of SpCC, as this may be due to loss of reactivity for antikeratin antibodies due to fixation or embedding procedures or to a phenotypic change of the tumor cells. There is often dense, desmoplastic fibrosis separating the bands of neoplastic cells into vague lobules and smooth-bordered islands. When cells from this type of cancer are viewed under a microscope, they appear spindle-shaped. When keeping this possibility in mind, immunohistochemistry with the appropriate antibodies (e.g., S-100, HMB45) serves to confirm or rule out this diagnosis if melanin is not found in the primary tumor. 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