benign spindle cell tumor

This lesion tends to occur in the hands and feet of children, with an age range of infancy to adolescence. HISTOPATHOLOGY The histology of Gardner fibroma is nonspecific. Nodules of inclusion body fibromatosis tend to reach about 2 cm in size. Fibromatosis encompasses both superficial and deep (desmoid fibromatosis) forms of lesions. Relatively immature lesions often have foci of chondroid metaplasia associated with giant cells and foci of small granular-type calcifications. FIBROMA OF TENDON SHEATH /* ]]> */ Individuals with NF1 may have multiple neurofibromas that need excision for functional purposes as well as to exclude the possibility of malignant transformation. The appearance of this lesion has also been compared to a “bag of worms.” FIGURE 6.1.13    Masson’s stain is helpful in highlighting the small intracytoplasmic inclusion bodies that are characteristic of this inclusion body fibromatosis. Spindle cell neoplasms are dened as neoplasms that consist of spindle-shaped cells in the histopathology. This example is extensively hyalinized and sclerotic to the point that it almost resembles a keloid. FIGURE 6.1.6    Nuchal fibroma is a poorly delineated proliferation of dense fibrous tissue. FIGURE 6.1.6    Nuchal fibroma is a poorly delineated proliferation of dense fibrous tissue. Breast spindle cell tumors (BSCTs), although uncommon, constitute a heterogeneous group of benign and malignant lesions, often necessitating different therapeutic approaches. Extensive or diffuse infiltration should not be present. Histologically, this lesion is composed of a moderately cellular proliferation of spindled cells arranged in interlacing fascicles. Histologically, they are comprised of a poorly delineated dermal proliferation of spindled cells (Figure 6.1.1). Individual bundles of plexiform neurofibroma may have a myxoid central portion, but usually have a thin fibrous capsule at the periphery. Send thanks to the doctor. The defining feature of inclusion body fibromatosis is the presence of small intracytoplasmic red inclusions.    They are often excised for cosmetic purposes. Nerve. The individual tumor cells have very indistinct cytoplasmic boundaries and elongated, sometimes wavy, nuclei. Calcifying aponeurotic fibroma is a rare tumor with a predilection for children and adolescents. Rare stripped nuclei are often identified in the background. COVID-19 is an emerging, rapidly evolving situation. Histochemical stains for elastin can be helpful in highlighting the degenerated fibers (Figure 6.2.6). 6.7      Nodular Fasciitis Fibrous hamartoma of infancy is a lesion that occurs in the first 2 years of life. This site needs JavaScript to work properly. FIGURE 6.3.3    Fibromatosis is variable in overall cellularity. The third and final subtype of neurofibroma is the plexiform subtype. They comprise a mixture of normal fat and hypocellular fibrous tissue (Figure 6.2.2).    Tags: Atlas of Soft Tissue and Bone Pathology National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error. The fat component may be interspersed throughout or, in more mature lesions, confined to the periphery. WordPress theme by UFO themes FIGURE 6.3.4    A more cellular example of fibromatosis.  |  This example is extensively hyalinized and sclerotic to the point that it almost resembles a keloid. Nuchal fibroma is a rare, benign lesion with a strong predilection … As with apsirates of schwannoma, aspirates from neurofibroma may show significant nuclear atypia. The key feature for diagnosis is the presence of large, fragmented elastic fibers. Examination of histologic sections reveals multiple nodules of neural tissue, corresponding to different tissue planes or sections of the tumor bundles (Figure 6.4.5). As mentioned in the schwannoma section (Section 6.5), “hybrid” types of lesions, with features of both schwannoma and neurofibroma, are not uncommon. Mesenchymal Tumors General Comments Gastrointestinal (GI) spindle cell tumors caused diagnostic confusion for decades. } Unlike schwannomas, cystic degeneration is not a common feature. Histologically, each tumor displayed islands of spindle or stellate cells embedded in a collagenous matrix. In more cellular lesions, mitotic figures may be prominent. Keywords: The cytoplasmic processes are indistinct, and cell nuclei are small and spindled or ovoid. The tumor is diverse in appearance and several different cell lines produce tumors of similar appearance. NF1 is caused by a mutation in the NF1 gene located on chromosome band 17q11.2 that encodes for a tumor suppressor protein known as neurofibromin. Variations on the classic histologic appearance include focal myxoid or cystic degenerative change as well as osseous or cartilaginous metaplasia. FIGURE 6.1.14    Juvenile hyaline fibrosis is characterized by an extensive sclerotic hyalinized matrix, which entraps small plump fibroblasts. PEComas: a family of tumors made up of abnormal cells called perivascular epithelial cells. Occasionally, it occurs on the wrist, fingers, or more proximal soft tissues of the extremity. // If there's another sharing window open, close it. Spindle cell carcinoma, also termed carcinosarcoma, pseudosarcoma, polypoid carcinoma, sarcomatoid carcinoma, and spindle cell variant of squamous cell carcinoma, is a rare type of malignant tumor that often grows as an exophytic polypoid lesion (see also Chapter 20). Spindle cells should make think: 1. FIGURE 6.4.3    Diffuse type of neurofibroma that infiltrates between the adnexal structures of the skin. Treatment of fibromatosis is largely surgical. windowOpen.close(); 2.2. var windowOpen; They tend to infiltrate around the adnexal structures of the skin and can cause extensive thickening of the dermis. FIGURE 6.1.4    A low-power view of calcifying aponeurotic fibroma showing its fibromatosis-like infiltrating appearance.    These are summarized in Table 6.3.1. 6.5      Schwannoma It occurs most commonly on the palmar surfaces of the hands as well as the plantar surfaces of the feet. Rare fragments of dense stroma and bland spindle cells may be present. }); STAT6 expression in spindle cell lesions of the breast: An immunohistochemical study of 48 cases. Individual cell nuclei are sparse and often very widely spaced. Deep fibromatosis, or desmoid tumor, represents a more difficult problem. Fibroma of tendon sheath is benign, but may recur if not completely excised. There is rarely a single-cell population in aspirates of fibromatosis. It is more common to see stripped or bare nuclear fragments in the background of a cytologic preparation of a neurofibroma. The superficial fibromatoses are fairly common, affecting an estimated 20% of the population at some point in life. It is poorly delineated and presents as a slow-growing painless soft tissue mass. Elastofibromas are completely benign. These may be difficult to see on routine hematoxylin and eosin, but are often highlighted by Masson’s trichrome stain or can be highlighted by immunohistochemical staining for SMA (Figure 6.1.13). of numerous nerve small nerve fascicles. The tissue between the bundles may be fibrous, fat, or additional neurofibromatous tissue in a more diffuse pattern. FIGURE 6.1.11    Fibroma of tendon sheath is usually sclerotic and relatively paucicellular. The cytologic features of neurofibroma are indistinguishable from those of schwannoma. INCLUSION BODY FIBROMATOSIS  |  FIGURE 6.2.3    At first glance, the degenerated fibers of elastofibroma can be easily overlooked. HHS Gardner fibroma tends to show immunohistochemical expression of CD34 and in some instances, nuclear localization of beta-catenin. The requirements for a clinical diagnosis of NF1 and NF2 are summarized in Table 6.4.1. Neurofibroma is the one of the major subtypes of benign peripheral nerve sheath tumors (the other being schwannoma). If they cause dysfunction, they can be removed surgically with a marginal or lesional excision without risk of recurrence. 17 These are bulky intraluminal masses that most often develop in the mid esophagus of middle-aged to elderly men (80%). GNs represent the … FIBROUS HAMARTOMA OF INFANCY Spindle cells are of mesenchymal origin, and form the body's connective tissue, fat, muscle, bone, cartilage and blood vessels. Fibromas represent a very diverse set of lesions that have a common histologic feature: the presence of bland fibrosis. jQuery( document.body ).on( 'click', 'a.share-facebook', function() { FIGURE 6.2.6    An elastin stain highlights the abnormal elastic fibers. They tend to be spindled or occasionally stellate in shape (Figure 6.1.17). FIGURE 6.3.2    Diffuse or aggressive fibromatosis often entraps fat and individual fibers of adjacent skeletal muscle. } The second form of neurofibroma is the diffuse subtype. Clipboard, Search History, and several other advanced features are temporarily unavailable. FIGURE 6.3.3    Fibromatosis is variable in overall cellularity. Neurofibromas can occur in any site, both deep soft tissues and superficial cutaneous lesions. The plexiform variant of neurofibroma should be examined very carefully for evidence of malignant transformation. Examination of histologic sections reveals multiple nodules of neural tissue, corresponding to different tissue planes or sections of the tumor bundles (Figure 6.4.5). Histologically, fibroma of the tendon sheath is bland and paucicellular (Figure 6.1.11). Smooth. FIGURE 6.2.2    Elastofibroma is poorly defined and often appears as fibrous tissue infiltrating normal fat. var windowOpen; FIGURE 6.2.1    This location between the scapula and thoracic wall is classic for elastofibroma. _stq.push([ 'view', {v:'ext',j:'1:6.0.1',blog:'125224839',post:'311177',tz:'0',srv:'basicmedicalkey.com'} ]); Inclusion body fibromatosis is benign, but has a tendency to recur. 6.14      Myofibroma and Myofibromatosis    Individual cells have an indistinct cytoplasm, which has a tendency to imperceptibly merge with the background fibrous tissue. However, following decades of ultrastructural and immunohistochemical studies, and more recently genetic investigations, it is now evident that most GI mesenchymal tumors arise from interstitial cells … It occurs most commonly in adults, with a median patient age of around 50 years. The fat component may be interspersed throughout or, in more mature lesions, confined to the periphery. It presents as a small, very firm, and usually painless nodule. LIPOFIBROMATOSIS Benign and Low-Grade Spindle Cell Tumors CALCIFYING APONEUROTIC FIBROMA. Cytogenetic study of a single case has demonstrated a translocation t(2;11)(q31;q12) that is also present in desmoplastic fibroblastoma, raising the possibility that these may be closely related entities. The individual tumor cells have very indistinct cytoplasmic boundaries and elongated, sometimes wavy, nuclei. Perivascular epithelioid cell tumor of the lung, also known as clear cell “sugar” tumor, is a rare benign tumor arising from perivascular epithelioid cells. 6.3      Fibromatosis Individuals with NF1 may have multiple neurofibromas that need excision for functional purposes as well as to exclude the possibility of malignant transformation.    Most display small nuclei with comma or hook shapes embedded in a fibrous network. Histochemical stains for elastin can be helpful in highlighting the degenerated fibers (Figure 6.2.6). Premium Wordpress Themes by UFO Themes There is often a cracking artifact between the very dense and coarse bundles of fibrous tissue. jQuery( document.body ).on( 'click', 'a.share-twitter', function() { Histologically, this lesion is composed of cellular fascicles of spindled cells admixed with mature-appearing fat (Figure 6.1.18). It is a very aggressive form of cancer, and patients who are diagnosed generally do not live more than five years. Histologically, these lesions are characterized by an abundance of thick, hyalinized material with rare intervening bland, small spindle cells (Figure 6.1.14). This classic presentation has led to speculation that hormonal and/or traumatic stimuli may facilitate the growth of the lesion. FIGURE 6.1.16    Desmoplastic fibroblastoma typically appears well demarcated, but microscopic extension into adjacent soft tissues is identified in a significant percentage of cases. }); Histologically, calcifying aponeurotic fibroma is characterized by a proliferation of fibrous tissue that tends to simulate fibromatosis (Figure 6.1.4). FIGURE 6.2.1    This location between the scapula and thoracic wall is classic for elastofibroma. The cytologic features of neurofibroma are indistinguishable from those of schwannoma. When desmoid is suspected or in the differential of a low-grade fibroblastic lesion, immunohistochemical detection of beta-catenin can be useful. windowOpen.close(); Most display small nuclei with comma or hook shapes embedded in a fibrous network. The clinical, histologic, and diagnostic features of the spindle cell tumors of children are summarized in Table 6.1.1. Fibromas represent a very diverse set of lesions that have a common histologic feature: the presence of bland fibrosis. Neurofibromas demonstrate immunohistochemical staining for S100 protein, but not as diffusely or strongly as in schwannomas. It displays evidence of myofibroblastic differentiation in that it displays positive immunohistochemical staining for SMA. 2018 Oct;214(10):1544-1549. doi: 10.1016/j.prp.2018.07.011. This type of neurofibroma also likes to travel along fascial planes and insinuates itself into subcutaneous fat in the same manner as fibromatosis or dermatofibrosarcoma protuberans (Figure 6.4.4). When cells from this type of cancer are viewed under a microscope, they appear spindle-shaped. Alternative therapies, including radiation, tamoxifen, and tyrosine kinase inhibitors can augment the standard surgical approach in patients who experience multiple recurrences. Both forms of this disease are associated with a mutation of the capillary morphogenesis protein 2 gene on chromosome 4. The name of these often ends in “sarcoma.” The distinction is important, as patients with Gardner fibroma often have a germline genetic defect, which predisposes them to other forms of malignancy.     •    Osseous lesions: kyphoscoliosis, sphenoid dysplasia, cortical bone thinning, and others FIGURE 6.1.2    Deep extent of a dermatofibroma. It is poorly delineated and presents as a slow-growing painless soft tissue mass. Some refer to this as a “shredded carrots” type of pattern. There is a high incidence of local recurrence. Calcifying aponeurotic fibroma is a rare tumor with a predilection for children and... NUCHAL FIBROMA.    Individual cells have an indistinct cytoplasm, which has a tendency to imperceptibly merge with the background fibrous tissue. Purely benign mesenchymal spindle cell neoplasms of the breast are currently labeled under various terms in the literature (benign spindle cell tumor, fibroma, spindle cell lipoma, myofibroblastoma, solitary fibrous tumor, myogenic stromal tumor).    When material is obtained, it tends to be hypocellular and comprised of very dense fragments of fibrous material with associated small spindled cells (Figure 6.3.6). They are centered in the soft tissues, but cause a dome-shaped mass, which produces tightness and redness of the overlying skin. Tumors composed of benign spindle cells, with variable lines of differentiation. The dense calcification indicates this is probably a more mature lesion. FIGURE 6.1.7    Dense fibrous tissue of Gardner fibroma. 6.12      Gastrointestinal Stromal Tumor It is more common to see stripped or bare nuclear fragments in the background of a cytologic preparation of a neurofibroma.  |  Patients may eventually seek medical attention because of deformity or limitation of range of movement.    FIGURE 6.3.6    Aspirates of fibromatosis are almost always very hypocellular. Neurofibromas frequently display a cellular pattern referred to as “shredded carrots.” This corresponds to the haphazard arrangement of Schwann cells with wavy cytoplasmic extensions (Figure 6.4.1). This entity is also referred to as digital fibrous tumor of childhood and infantile digital fibroma. FIGURE 6.2.6    An elastin stain highlights the abnormal elastic fibers. However, spindle cell tumors can appear on the skin of a dog or cat as well, and be rather frustrating. NF1 is caused by a mutation in the NF1 gene located on chromosome band 17q11.2 that encodes for a tumor suppressor protein known as neurofibromin. They are centered in the soft tissues, but cause a dome-shaped mass, which produces tightness and redness of the overlying skin. Spindle cell carcinoma is a type of cancer which usually originates in the connective tissues of the body. NUCHAL FIBROMA FIGURE 6.1.17    Desmoplastic fibroblastoma with a hypocellular myxocollagenous background. Some histologic variations of elastofibroma include focal myxoid and cystic degenerative change.     •    Six or more cafe au lait macules >5 mm in size* Histologic variations include the presence of small whorled arrangements that resemble tactile elements as well as pigmented dendritic cells. Careful search reveals thick, deeply eosinophilic fragmented elastic fibers (Figures 6.2.5 and 6.2.6). There may be entrapment of small nerve fibers or subcutaneous fat within a nuchal-type fibroma (Figure 6.1.6). 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