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Tumors and tumor-like formations of soft tissues

Soft tissue neoplasms are traditionally referred to as mesenchymal tumors that occur in extraskeletal and non-epithelial tissues, excluding the parenchyma of the internal organs, the lining of the brain and the lymphoreticular system. They are classified according to the tissue type (muscle, fat, connective tissue, blood vessels, nerves), which they reproduce. Some of these tumors do not have a tissue analogue, however, they have constant clinical and pathological manifestations that justify their classification status. It is difficult to estimate the true frequency of soft-tissue neoplasms, since so many, if not most, benign mesenchymal tumors are not subjected to surgical removal. These benign neoplasms predominate over their malignant counterparts in a ratio of approximately 100: 1. Currently in the United States about 5,700 soft-tissue sarcomas are recognized annually, which is 0.8% of all malignant tumors. These sarcomas account for up to 2% of all deaths from malignant neoplasms.

The etiology of most soft tissue tumors is unknown. However, the relationship between radiation therapy, rare cases of chemical and thermal burns, as well as injuries, on the one hand, and the subsequent development of sarcoma, on the other, has been traced. In some cases, environmental factors play a certain etiological role, in particular, effects on the body of phenoxyherbicides and chlorophenol. The increase in the incidence of Kaposi's sarcoma in AIDS and in patients with depressed immunity also suggests that viruses and an impaired immunocompetence are likely to be the cause. Almost all soft tissue tumors are found in the form of sporadic neoplasms, but a small number of them are associated with genetic syndromes. Among the latter should be noted neurofibromatosis type I, or Recklinghausen's disease (FD von Recklinghausen; malignant schwannoma), Gardner syndrome (EJGardner; fibromatosis), and Osler-Weber-Rendu syndrome (W.Osler, FPWeber, HJL Rendu; body strain. In some patients with soft tissue tumors, abnormalities in certain tumor suppressor genes, such as p53 and the retinoblastoma gene, have been identified. However, the molecular development mechanisms of most of these tumors remain unknown.

Tumors of soft tissue may have different localization. Approximately 40% of tumors occur in the lower limbs (especially in the hip area), 20% in the upper limbs, 10% in the head and neck, 30% in the trunk and retroperitoneal space. If we talk only about soft tissue sarcomas, then the information on the age and sex distribution is as follows. Men are more often affected by women (1.4: 1), and approximately 40% of the neoplasms develop on average and old age. About 15% of soft tissue sarcomas occur in children. In children, these tumors account for 25% of the total number of the most common malignant tumors. Often (with primary multiple neoplasia) they accompany brain tumors, hematopoiesis systems, and Wilms tumors. Some types of sarcomas appear in certain age groups, for example, rhabdomyosarcoma develops mainly in childhood, synovial sarcoma in adolescents, liposarcoma, and malignant fibrous histiocytoma in mature and elderly people.

As with other neoplasms, the size of soft tissue tumors significantly affects the prognosis (the larger, the worse). Important prognostic signs are the shape of tumor cells (spindle-shaped, round, polygonal) and their structure (rod-shaped, lymphocyte-like, epithelioid cell). Unfortunately, often these symptoms may not be sufficient to distinguish one form of sarcoma from another (especially with poorly differentiated and aggressive tumors). In this case, the main differential diagnostic criteria are based on the data of immunohistochemical, ultrastructural, cytogenetic and molecular genetic analysis.

Regardless of tissue type, the degree of histological differentiation of soft-tissue sarcoma also has a very important prognostic value. Graduation by degree (I – III) in most cases is based on the level of differentiation of the tumor cells themselves, the number of mitosis figures, the density of the cells (the so-called cellularity), the size of the necrosis zones. There is no single point of view on the importance of each of these signs, however, the magnitude of necrosis is considered especially significant. The next extremely important prognostic criterion is the stage of growth (spread) of soft tissue tumors. On the TNM system designate:

• stage T1 — tumors with a diameter of 5 cm or less;

• stage T2 - tumors of more than 5 cm, but without damage to the bones, main blood vessels and nerves;

• stage T3 — sarcomas that spread to the bones, vessels, and nerves;

• N2 - metastases in regional lymph nodes;

• M1 - distant hematogenous metastases.

Metastases develop in 80% of patients with deep-seated high-grade sarcomas (poorly differentiated) that are more than 20 cm in diameter. It is also known that the total 10-year survival rate for patients with soft tissue tumors is about 40%.

Before turning to individual tumors and tumor-like lesions of soft tissues, we note that many of them are described in other chapters. For example, information about the most common benign soft tissue neoplasm of a person, such as leiomyoma (fibroids), is given in Chapters 7 and 21; Chapter 7 describes such common tumors as lipoma and liposarcoma; Neoplasms of vascular origin, including Kaposi's sarcoma, are discussed in Chapter 11; tumors of the peripheral nerves are discussed in Chapters 8 and 26. To avoid repetition, these tumors are not considered here.

Tumors and tumor-like formations of connective (fibrous) tissue. Reactive pseudosarcomatous proliferates. They are changes of a non-tumor nature that develop in response to certain types of local physical or ischemic damage. These changes are proliferates from metabolically active fibroblasts or related mesenchymal cells. They appear unexpectedly and grow rapidly, imitating sarcomas. Such an imitation also finds microscopic evidence in the form of increased cellularity and mitotic activity of the tissue, as well as relatively simplified histological differentiation. Consider 4 variants of reactive pseudosarcomatous proliferates.

1. Nodular fasciitis. It is also called infiltrative, or pseudosarcomatous, fasciitis. Such fasciitis is considered the most common form of reactive pseudosarcomatous proliferates. Nodular fasciitis occurs, as a rule, on the palmar surface of the forearm in adults, followed by the frequency of the spread of the chest and back. Patients usually develop single, rapidly growing, and sometimes painful tumors over a period of several weeks. In 10-15% of patients noted prior injury. Nodular fiscitis occurs in the deep layers of the dermis, subcutaneous fat or muscle. The lesion reaches several centimeters in diameter, has a nodular shape and poorly defined borders. Under the microscope, nodular fasciitis is multicellular; it contains bundles of immature fibroblasts, which have either a random orientation or are assembled into non-uniform and short bundles similar to the strands of fibroblasts in an in vitro tissue culture. Cells vary in size and shape (stellate and spindle-shaped). In their nuclei nucleoli and numerous figures of mitosis are clearly visible. That part of the lesion, which can be conditionally attributed to the stroma, often looks mucous and contains small groups of red blood cells outside the vessels.

The list of diseases that should be considered in the differential histological diagnosis is very significant. First of all, it is important to exclude fibromatosis and spindle cell sarcoma, since nodular fasciitis is a reactive process that rarely recurs after surgical removal. Of the other pseudosarcomas related to nodular fasciitis, proliferative fasciitis and proliferative myositis should be mentioned. They develop in the proximal extremities. In the first case, under a microscope, large, rounded proliferating fibroblasts are visible, having noticeable nucleoli and resembling ganglion cells.

2. Ossifying miosis. It differs from other fibroblastic proliferates in that bone appears as a result of metaplasia in tissue (more often in skeletal muscle). The disease usually occurs in young people after injury, often sports. The lesion appears under the skin and in the muscles of the proximal limbs. Clinical symptoms are determined by the stage of development of the process. In the early stage, the affected area is swollen and sore. Over the next few weeks, it becomes more delimited and dense. In the final stage, this area becomes painless, ossified and clearly limited. The maximum diameter of the center of ossifying myositis reaches 3-6 cm. In most cases, there are clear boundaries of the lesion, in the center of which soft shiny fabric is defined on the incision and dense, crumbly fabric at the edges. Microscopic data also correspond to the stage of damage: in the early stages, the focus of ossifying myositis is more cellular and contains bundles of elongated fibroblast-like cells distributed in poorly shaped bundles. After 3 weeks from the onset of the disease, morphological zones are determined in the outbreak. The center maintains a population of fibroblasts. However, the center merges with the adjacent intermediate zone containing osteoblasts, which create a vaguely delineated coarse-fibered trabeculae. Most of the peripheral zone of the outbreak has well-formed and mineralized beams, similar to analogues in the cancellous bone. Skeletal muscle fibers are often involved in this zone of ossification, and regenerating giant muscle cells are also found here. As a result, the entire focus is ossified and the intergranular spaces are filled with bone marrow.

The dynamics of radiological data corresponds to the stages of morphological progression. At first, only a soft-tissue seal is determined, but after about 3 weeks, spotty flocculent darkening forms at its edges. Over time, the area of ​​X-ray darkening expands and slowly absorbs the center of the focus. Ossifying myositis is usually cured by surgical excision and is rarely malignant. It should be differentiated from extraskeletal osteosarcoma. The latter occurs in elderly patients, its actively proliferating cells have morphological signs of malignancy. This tumor has no zonal structure, as in the case of ossifying myositis.

3. Palmar, plantar and penile fibromatosis. They constitute a small group of superficial fibromatosis, characterized by the presence of nodular or indistinctly limited bundles of mature fibroblasts, surrounded by numerous and developed collagen fibers. Ultrastructural studies indicate that many of these cells are myofibroblasts and are likely to shrink.

With palmar fibromatosis (Dupuytren's contracture; G. Dupuytren), an inordinate knotted unilateral or bilateral thickening of the palmar fascia arises.
After a few years, the involvement of the overlying skin causes its folding and retraction. At the same time, slowly progressing flexion contracture (mainly GU and V fingers) is developing, with which the possibility of extension of the corresponding interphalangeal joints is limited.

Similar changes are observed in plantar fibromatosis, but flexion contractures and bilateral lesions are rare. After a certain time, as a rule, after several years, the palmar and plantar forms of fibromatosis stabilize and do not progress further. Cases of their gradual and spontaneous regression are described. After surgical removal of these lesions, relapses are possible, especially in the palmar form.

In penile fibromatosis (Peyronie's disease; F. de la Peyronie), a palpable seal appears on the back side of the penis. It can cause abnormal curvature of the penis and constriction of the urethra.

4. Desmoid (aggressive fibromatosis). From a biological point of view, desmoid occupies an intermediate position between rapidly growing fibrous formations and fibrosarcomas of low malignancy (well-differentiated). On the one hand, during desmoid, large masses of infiltratively growing fibrous tissue are often formed that can recur after incomplete removal, but on the other hand, small sections can be formed here that are constructed of ordinary highly differentiated fibroblasts that are not capable of metastasis.

Desmoid occurs at any age, often at the 2nd – 4th decades of life, and may be accompanied by local pain. It manifests itself in the following forms, extra-abdominal, abdominal, intra-abdominal. All these forms have similar macro- and microscopic features. Extraabdominal desmoid occurs with equal frequency in men and women. It occurs mainly in the skeletal muscles of the shoulder girdle, chest, back and thigh. Abdominal desmoid usually develops in the muscle-neurotic structures of the anterior abdominal wall in women during or after pregnancy. Intraabdominal desmoid usually grows in the mesentery or pelvic wall. It is often found in individuals with Gardner syndrome (see Chapter 16). In all these forms, the desmoid is characterized as a unicentric, grayish-white dense, unclearly limited formation, varying in diameter from 1 to 15 cm. It has a rubbery texture and sometimes infiltrates surrounding tissues. Under the microscope, it is seen that the desmoid tissue is represented by several swollen fibroblasts with minimal differences in the size of the cells themselves and their nuclei. These fibroblasts are located among the developed collagen fibers (Fig. 24.16). Figures of mitosis are rare. In the case of involvement in the process of skeletal muscle can be found regenerating muscle cells, taking the form of giant multinuclear elements.

Fibroma. Despite the widest distribution of connective tissue in the human body, the localization of fibroids is strikingly few, and fibrosarcomas are among the rarest soft tissue tumors. Many lesions, previously called fibromas, were later renamed fibromatosis. Most often, fibromas occur in the ovaries or are formed as neurofibromas along the nerve trunks. Clearly limited nodules, also related to fibromas, are formed in the oral cavity near the teeth, but these nodules can be of a reactive, rather than tumor, nature. As a rule, fibromas are small dense encapsulated tumors, pearl-gray in the cut. Regardless of the location, these neoplasms are always constructed from typical spindle-shaped fibroblasts, which are located in weakly or, conversely, strongly developed collagen. Figures of mitosis are rare, therefore it is difficult to distinguish such fibroids from fibrosarcomas.

Fig. 24.16.

Desmoid soft tissue of the anterior abdominal wall


And - a general view of the lesion; B - a zone in which fibroblasts are oriented in one direction.

Fig. 24.16. Continued.

AT -

fibroblasts that are among the developed collagen pods

(negatives of A.Ye.Kolosova).

Fibrosarcoma. This is a malignant neoplasm that is found throughout the body. Most often it is found in the retroperitoneal space, the tissues of the thigh and the area near the knee joint, as well as in the leaf sections of the limbs. Many tumors, previously regarded as fibrosarcomas, are referred to as aggressive fibromatosis (desmoid) or malignant fibrous histiocytoma (see below). A typical fibrosarcoma does not have a capsule, has infiltrative growth, a soft consistency, and on the cut it resembles the meat of red fish. In this tissue, you can sometimes find areas of hemorrhage and necrosis. Highly differentiated fibrosarcoma sometimes has a false capsule, which is a pressed back and compressed local stroma. Histological examination showed different degrees of differentiation of tumor tissue. Slow-growing fibrosarcomas are similar to fibromas, which contain fusiform cells (Fig. 24.17) and sometimes grow in the form of a herringbone. Rapidly progressive fibrosarcomas are distinguished by pronounced cellularity, disordered architectonics of cellular structures, noticeable polymorphism and proliferative activity of tumor elements, as well as areas of necrosis. Re-

Fig. 24.17.

Highly differentiated fibrosarcoma

(drug IAP).

Cydivision is noted in more than 50% of patients, and in about 25% of them, at the time of diagnosis, metastases are detected. With modern treatment methods, 60–80% of patients survive for 5 years.

Fibrogistiocytic tumors. In their tissue, they contain both fibroblastic and histiocytic elements. Initially, they were regarded as histiocyte tumors capable of expressing the basic properties of fibroblasts. However, according to the latest research, the main phenotype of tumor cells is closest to fibroblasts. Обе разновидности фиброгистиоцитарных опухолей, о которых речь идет ниже, часто встречаются в дерме (см. главу 25).

1. Доброкачественная фиброзная гистиоцитома. Это относительно часто встречающееся новообразование, которое обычно возникает в дерме и под кожей. Оно не вызывает боли, растет медленно и обнаруживается, как правило, в среднем возрасте. Внешне это маленькая (до I см) плотная, подвижная, нечетко ограниченная опухоль. Примерно в 30 % случаев она множественная. Большинство доброкачественных фиброзных гистиоцитом отличается развитой целлюлярностью и состоит из массы веретеновидных клеток, расположенных в виде завихрений. Их часто называют дерматофибромами. Иногда эти опухоли содержат многочисленные кровеносные сосуды и отложения гемосидерина, дающие повод к названию «склерозирующая гемангиома». В ряде случаев веретеновидные опухолевые элементы могут чередоваться с гистиоцитами, обладающими пенистой цитоплазмой. Такие варианты называют гистиоцитомами. В краях таких новообразований видны признаки инфильтрирующего роста, однако инвазия в вышележащий и нередко вторично гиперплазированный эпидермис не происходит.

2. Злокачественная фиброзная гистиоцитома. Она вызывает многочисленные споры; встречается у взрослых лиц. Существует мнение о том, что все случаи злокачественной фиброзной гистиоцитомы представляют собой неправильные интерпретации других типов сарком. Злокачественная фиброзная гистиоцитома обычно развивается в скелетной мускулатуре проксимальных отделов конечностей и забрюшинном пространстве. Внешне это серовато-белый, лишенный капсулы узел, который может иметь обманчиво четкие границы и весьма крупные размеры (диаметр 5—20 см). Различают полиморфные, миксоидные, воспалительные, гигантоклеточные и ангиоматоидные гистологические варианты злокачественной фиброзной гистиоцитомы. Примерно у 60 % больных выявляют полиморфный вариант, построенный из малигнизированных веретеновидных клеток, которые создают фигуры завихрений. Среди этих клеток встречаются крупные округлые и полиморфные элементы. В большинстве случаев злокачественная фиброзная гистиоцитома отличается агрессивным клиническим поведением. Если резекция опухоли выполнена нерадикально, то уровень метастазирования может достигать 30—50 %. Правда, сюда не относятся кожные злокачественные фиброзные гистиоцитомы, которые метастазируют редко. Ангиоматоидный вариант тоже обладает более спокойным течением и в отличие от остальных вариантов злокачественной фиброзной гистиоцитомы встречается у подростков и молодых людей.

Синовиальная саркома. Эта опухоль названа так потому, что по своему строению она напоминает развивающуюся синовиальную оболочку. Синовиальная саркома составляет около 10 % от всех мягкотканных сарком и по показателям заболеваемости находится на 4-м месте среди всех сарком. Как правило, синовиальная саркома встречается на 3—5-м десятилетии жизни. Несмотря на то, что само название опухоли предполагает ее развитие из суставной выстилки, лишь менее 10 % синовиальных сарком имеют внутрисуставную локализацию. Большинство таких новообразований развивается около крупных суставов конечностей, причем 60—70 % из них поражает нижние конечности, особенно зону коленного сустава. Больные обычно жалуются на глубоко расположенную опухоль, существующую у них уже несколько лет. Изредка синовиальная саркома возникает в окологлоточной области или в брюшной стенке.

Fig. 24.18.

Синовиальная саркома

(синовиома) с бифазным характером дифференцировки: среди саркоматозной стромы видны многочисленные трубки эпителиального типа (препарат К.М.Пожарисского),

Характерным гистологическим признаком синовиальной саркомы является так называемое бифазное строение (бифазная дифференцировка). Речь идет о двух морфологически различных компонентах опухоли: об эпителиоподобиых структурах, формирующих комплексы, похожие на железы, и о структурах веретеновидноклеточной саркомы (рис. 24.18). Несмотря на имитацию, опухолевые клетки не имеют признаков синовиоцитов. Эпителиоподобные элементы, ограничивающие железистые структуры, обладают кубической, иногда даже цилиндрической формой. Иногда они вместо железистых структур формируют солидные тяжи. Веретеновидные клетки растут в виде густых пучков, включающих эпителиоподобные.

Некоторые синовиальные саркомы имеют монофазный характер гистологической дифференцировки и состоят только из веретеновидно-клеточных, реже только из эпителиоподобиых структур. Первый вариант легко спутать с фибросаркомой. Дифференциально-диагностическими гистологическими признаками синовиальной саркомы, позволяющими надежно отличать ее от других сарком, являются: наличие в опухолевой ткани отложений извести (непостоянный признак); иммуногистохимические маркеры — цитокератины и эпителиальный мембранный антиген, экспрессируемые опухолевыми клетками как при бифазной, так и монофазной дифференцировке; специфические хромосомные аберрации, выражающиеся в реципрокной транслокации между хромосомами X и 18, -t (X; 18) (pll.2; qll.2) (см. главу 8). При адекватном лечени уровень 5-летней выживаемости больных варьирует в пределах 25—62 %. Метастазы при синовиальной саркоме обнаруживают в региональных лимфатических узлах, легких и костях.

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Tumors and tumor-like formations of soft tissues

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  15. SESSION 13 First aid for injuries. Closed soft tissue damage. Traumatic brain injury. Chest damage. Transport immobilization for injuries.
    Objective: To teach students how to diagnose various traumatic conditions and how to provide first aid to an injured person. Test questions 1. Trauma. Definition Classification of injuries. 2. Closed soft tissue damage. Injury. First aid. 3. Stretching. Complaints. First aid. 4. The gap. Complaints. First aid. 5. Prolonged crush syndrome. Pathogenesis. Clinical picture.
  16. Hematopoietic tissue tumors. Hemoblastosis. Tumor diseases of lymph nodes.
    1. Leukemia is 1. proliferation of bone marrow cells 2. infiltration of internal organs with leukocytes 3. primary tumor lesions of the bone marrow 4. lesions of the bone marrow leading to leukocytosis 5. tumor and metastatic lesions of the bone marrow 2. Chronic leukemia 1. lymphoblastic 3. non-lymphoblastic 2. lymphocytic 4. undifferentiated 3. Bone marrow in chronic
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