Gynecology

Paget's Disease

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The condition is relatively rare. [1] Usually occurs in younger patients, more often in men than women. The syndrome also became known as “thrombosis of the effort” in the 1960s [2], when the connection of this syndrome with active physical activity was discovered, [3] although the condition may occur spontaneously. It also occurs when there are neck ribs. May develop a complication in the form of chest exit syndrome.

Symptoms may include sudden pain, warmth, redness, blueness and swelling in the shoulder area. Such manifestations indicate the need for early treatment, as they can lead to pulmonary emboli.

Traditional treatment of thrombosis is similar to the treatment of deep vein thrombosis of the lower extremities and includes the use of anticoagulants - heparin (usually low molecular weight) with the transition to warfarin. In recent years, new oral anticoagulants (rivaroxaban [4], dabigatran, edoxaban) have been increasingly used. In modern conditions, catheter-driven thrombolysis is preferred in young patients. The method consists in administering the drug (urokinase) through a special tube (catheter) directly into the thrombus. Thrombolysis, in contrast to conservative treatment, allows you to completely clear the lumen of the veins from thrombotic masses and restore the function of the limb.

The syndrome is named after James Paget [5], who first suggested venous thrombosis, causing pain and swelling of the upper extremities, [6] and Leopold von Schroetter, who later linked clinical syndrome with subclavian and axillary venous thrombosis. [7]

Paget's disease of the bones

In 1877, J. Pedzhet published an article about 5 patients whom he observed. Each of them had a specific lesion of the skeleton, mainly the skull and long tubular bones of the lower extremities, which was accompanied by a thickening of the bone tissue, its deformation and softening. He called the pathologist this disease a deforming osteitis, since it suggested an inflammatory etiology of bone damage, which was refuted only in the 20s of the last century, and the disease itself was named Paget's disease.

According to modern concepts, Pedzhet's bone disease is a chronic, acquired disease of the bone tissue caused by impaired remodeling (resorption and formation) of bones.

The most commonly diagnosed pathology in people aged 55-60 years. The presence of the disease at a young age is very rare.

The essence of pathology

The mechanism of the disease is as follows. Under the influence of causal factors, the function of osteoclasts of bone tissue is disrupted, the task of which is to destroy the skeleton so that other cells (osteoblasts) can build a “new” bone tissue.

It is important to know! The human skeleton is not at all a static formation. Every day, bone tissue is destroyed and replaced with new and more durable material. This is achieved thanks to the coordinated work of osteoblasts and osteoclasts.

With excessive activation of osteoclasts and increased bone tissue resorption, the body reacts with a compensatory increase in the formation of new matter. But, unfortunately, it is inadequate: the collagen fibers in such a bone are arranged in a disordered manner, which is accompanied by the formation of coarse-fibrous and loose bone tissue, not compact. Such a mosaic structure of the skeleton is accompanied by its local deformations, softening and increased fragility.

A characteristic feature of the disease is the locality of the pathological process. As a rule, any one bone is affected (most often it is the lumbar vertebrae, the flat bones of the skull, the pelvis and the long tubular bones of the lower extremities), but several skeletal sections may be affected at the same time. The joints in this pathology, as a rule, do not suffer.

Causes and risk factors

Unfortunately, to date, the exact cause of deforming osteitis has not been found, but 2 theories are being considered: slow viral infection and genetic predisposition.

Studies in recent years have allowed the discovery of "traces" of paramyxoviruses (measles, PC-virus, dog plague virus) in the pedjetov foci of bone remodeling.

The presence of deforming osteitis in close relatives speaks in favor of the genetic theory of the disease. Changes in the bones of relatives of the diseased are found in 40% of cases. Genetic studies, unfortunately, have not yielded results. Researchers suggest that the pathology is heterogeneous, or there is one genetic locus responsible for the pathology of this type of bone tissue, which has yet to be discovered.

Classification

In practice, experts distinguish 4 variants of Paget's disease:

  1. Pedzhet's disease in children.
  2. Active pathology options in young people.
  3. The typical form of the disease in older people. In this case, it is customary to distinguish the monosormal form (the defeat of only one bone) and the polyossal (pathological lesions in several bones). Also among these forms, slowly flowing and active variants are distinguished.
  4. Ozlokachestvlenie pedzhetovskogo lesions. In some cases, there is the development of malignant tumors on the background of pedzhetovskogo bone damage. The most common osteogenic sarcoma, fibrosarcoma and giant cell tumor.

Symptoms and diagnosis

The symptoms of the disease are very diverse and depend on the stage of the pathological process, the number, location and size of lesions, the activity of the disease and the presence of complications.

It is customary to distinguish 3 stages of deforming osteitis:

  • Initial (stage of bone loss).
  • Intermediate (stage of consolidation of affected areas).
  • Stabilization (coarse-fiber reorganization of bone tissue).

As a rule, at the initial stage, any symptoms of Paget's disease are absent, which leads to a late diagnosis of the pathology, and in some cases the illness remains unrecognized at a late stage, since the majority of elderly patients experience symptoms of deforming osteitis as an aging and fading process.

In about 25% of cases, the pathology has an asymptomatic course and is detected quite accidentally by X-ray examination for another reason or when searching for causes of an increase in the level of alkaline phosphatase in the blood. In other cases, the disease is accompanied by certain signs.

The first symptoms of Paget's disease that lead a patient to a doctor are pain syndrome and deformity of the affected part of the skeleton. Pain usually occurs during physical exertion at the site of the bone lesion. In some cases, the pain syndrome is constant, it is characterized by an aching character.

Deformation of the skeleton, as a rule, is typical. Characterized by a thickening of the affected area of ​​the limb. For the legs is very typical saber-varus deformity. In case of damage to the bones of the skull, its arch becomes hilly, it gradually increases. If the spine is affected, then pathological kyphosis, kyphoscoliosis is often formed.

Very often, patients develop neurological disorders, especially in lesions of the bones of the skull and spine. Also characterized by pathological fractures of the lower extremities and compression fractures of the affected vertebrae.

The diagnosis is made on the basis of the patient's complaints, typical clinical signs and X-ray data. An additional criterion is the increase in the enzyme alkaline phosphatase in the blood. To clarify the localization and number of pedzhetovskikh foci conduct scintigraphy of the skeleton.

Over the past 20 years, tremendous changes have occurred in the treatment of osteitis deformans. In addition to symptomatic therapy, now there are drugs that can influence the course of the disease and slow its progression.

Pathology therapy is not always needed. Indications for its appointment are:

  • severe pain,
  • polyosal form
  • monosupple form in case of an unfavorable prognosis, for example, the location of the bone rearrangement near large joints (hip, knee), skull damage, the risk of neurological disorders, high activity of the process,
  • in case of a high risk of developing a pathological fracture.

Unfortunately, if there are complications of the disease (deformity, fractures, pinching of nerves, spinal cord, skull structures), this therapy will not be effective. If the size of the lesions is small, there is no risk of developing deformity, fractures, neurological pathology, the activity of the process is low, then doctors use waiting tactics.

Antiresorption therapy (prevents the destruction of bone tissue) includes the use of bisphosphonates (etidronate, clodronate, tiludronat, pamidronate, alendronate, residronat), calcitonin preparations and plicamycin.

Symptomatic treatment includes taking analgesics and nonsteroidal anti-inflammatory drugs.

From non-pharmacological therapies, special orthoses and corsets are used.

In severe cases, surgical treatment of deformities, fractures, neurological complications, and the development of malignant tumors is resorted to.

Pedzheta cancer nipple

Paget's cancer is an eczema-like form of a malignant lesion of the mammary gland in the region of the nipple-areolar complex. It occurs in 5% of breast cancers in patients aged 50-60 years. For the first time the disease was described by J. Pedget in 1874.

This form of malignant lesions of the breast is found not only in women but also in men. In the latter case, it has a more aggressive course. There are extramammary forms of Paget's cancer. They can be localized in those parts of the body where apocrine sweat glands are present (vulva, perineum, penis, scrotum).

Unfortunately, the etiology of this type of malignant breast tumor, like most of the others, remains unknown.

Treatment methods and prognosis

In the case of diagnosis of this type of tumor, the patient is shown surgery. Depending on the oncoprocess stage, a simple mastectomy or an extended one is performed.

Mandatory prescribed complex treatment, which includes, in addition to the removal of the breast, chemotherapy, radiation, hormone therapy.

The prognosis depends on the timeliness of pathology detection. By itself, the tumor is considered very aggressive, and even after performing a radical operation there is a very high risk of recurrence. The average life expectancy in patients with this pathology is only 3 years.

Paget-Shretter Syndrome

This pathological condition is also called thrombosis effort. It consists in the development of acute thrombosis of the deep venous vessels of the shoulder, as a rule, in the subclavian and axillary veins.

This disease is quite rare. Usually develops in people of young age, more often in men. The right hand suffers 2 times more often than the left.

Most researchers associate the development of the subclavian vein thrombosis with trauma or physical strain of the arm.

The main and earliest symptom of the syndrome is swelling of the upper extremity, and in some cases of all the upper half of the chest. Patients complain of a feeling of fullness in the hand, weakness, fatigue. The skin of the affected limb acquires a bluish tint. All patients develop pain syndrome. The pain, as a rule, has a dull arching character. Most of all, the pain is intense at the time of thrombosis, with time it decreases slightly, but returns again with active or passive movements. Also, the patients have a well-marked venous network of the affected arm and upper torso.

The acute stage of the syndrome lasts up to 3 weeks. Further, all the symptoms subside, but may reappear, for example, after physical labor with the involvement of the upper limbs.

The diagnosis is made on the basis of a typical clinical picture and phlebography (X-ray examination of the deep veins of the shoulder with the introduction of a contrast agent into the lumen of the vessel). In the case of Paget-Schretter syndrome, there is a lack of contrast in the subclavian or axillary vein along with the expansion of the collateral venous network of blood vessels.

All patients in the acute stage of the syndrome are hospitalized. The injured arm is given an exalted position and is prescribed a comprehensive drug treatment, which includes anticoagulants, antiplatelet agents, fibrinolytic agents. In severe cases, surgical treatment is indicated - thrombectomy with subsequent subclavian vein plasty.

The prognosis for patients with this syndrome is favorable. Severe complications such as limb gangrene or pulmonary thromboembolism occur in isolated cases.

Eczematoid breast cancer

This rare disease of the mammary gland is found in both women and men.

For the first time, the clinical picture of the development of the nipple oncological pathology was described almost 200 years ago by the famous British pathologist D. Pedget. In addition to the description, Pedzhet, in his writings, emphasized that the defeat of the skeletal-alveolar region during the year leads to breast cancer. Podet disease can develop not only in the nipple area, but also in those areas where the apocrine glandular ducts (penis, perineum) are located.

Medical science has not yet established the exact reason for the development of this pathology. But there are two theoretical directions:

  1. Epidemotropic. A certain number of atypical cells migrate along the ducts of the mammary gland to the nipple. Then they implanted and expanded. Practice shows that almost always Paget's disease goes side by side with intraductal breast cancer.
  2. Transforming. It is argued that a tumor appears when healthy epithelial cells of the areola or breast nipple are simply regenerated, and this process is not associated with anything else.

Malignant breast pathology manifests itself in three forms:

  1. Psoriatic - pale pink papules with dry scales.
  2. Eczematoid in acute form - fine-grained rash with sores and wet nipple of the chest.
  3. Eczematoid in chronic form - dry crusts, under which there are moist areas.

Symptoms of breast disease manifests nonspecific. In the initial stage, the process goes unnoticed. Then the symptoms of precursors appear: on the skin of the breast, in the area of ​​the juice, slightly reddens and peels off. The nipple and the nipple itself is covered with irritation.

After lubrication with corticosteroid ointments, irritation and peeling disappear.

After a while, the symptoms increase. There is a strong itching with burning sensation begins to appear. The skin of the breast burns, tingles, and serous and hemorrhagic contents begin to stand out from the nipple. The shape of the nipple changes: it can be retracted inwards or compacted and convex.

Further, the malignant process progresses, and infiltrates appear, covered with sores, crusts or erosions.

Under dry crusts, a moist, wetting area is found on the nipple of the breast.

The formation of cones and seals - the doctor discovers these symptoms during examination and palpation. The affected area of ​​the breast with a clear contour, which is slightly elevated above the surface. Additional symptoms: an increase in axillary lymph nodes, discharge of bloody contents from the nipple of the breast.

Diagnosis of the disease pedzhet and medical tactics

The diagnosis of Paget's disease begins with a medical examination of the mammary gland and mammography. As a result of mammographic examination, the size and location of the tumor is determined. The study of the breast is carried out in full in all projections, since Paget’s disease can be combined with other tumor nodules of other structures. In the primary survey plan with negative data on mammography, breast ultrasound, magnetic resonance imaging is assigned. To clarify the diagnosis, a biopsy is taken, scraping from the affected area.

Further treatment of Pedet disease depends on the stage of the oncological process.

The most common method by which Paget’s disease is treated is surgery. During surgery, the surgeon removes the breast along with axillary lymph nodes (radical mastectomy). After reconstructive mammoplasty is performed. After surgery, a course of chemo and hormone therapy is prescribed, which helps prevent relapses with metastasis.

The prognosis for recovery directly depends on the stage in which Paget's disease is and what its biological aggressiveness is, age data, lymph node damage, degree of malignancy. The average life expectancy is about three years. With metastasis and infiltrates, it is reduced to one year.

Pedzhet's disease is difficult to detect in the initial stages. Therefore, it is important to regularly attend a breast specialist and be examined. This will give a chance to improve the forecast.

Thrombosis effort

Симптомы другого заболевания, который еще называют тромбозом усилия или травматическим веноспазмом, описаны в трудах Педжета и австралийского врача Шреттера.

Paget's disease — Schretter syndrome often develops in well-developed physically young men.

The first manifestations of the Péjdet-Schrötter syndrome coincide with an excessive muscular load on the upper limbs. The tension is strong, habitual and stereotyped, which comes from the muscle, injures the subclavian vein, breaks the vascular wall. This leads to the development of thickening, spasms or thrombosis. Syndrome Pedeta-Shretter mainly affects weightlifters, armwrestlers and athletes.

The first and most indicative symptom of Pieddet-Shretter's disease is swelling of the arm. In one day, it spreads from the subclavian region to the finger tips. The edema is so intense that there is no trace when pressed. The next symptom of Pieddet-Schretter disease is intense cyanosis of the skin on the limb. The sick person complains of a strong arching pain. It migrates and covers different parts of the hand, forearm, shoulder, and clavicle area.

After a painful attack, the pain intensifies only with a change in the position of the hand or during physical exercise. On the skin surface, subcutaneous venous collaterals are visible. The veins on the forearm and in the place of the elbow bend are dilated and tense. After 3 weeks, the acute period of Paget-Shretter syndrome subsides, and the deterioration of well-being happens during physical exertion.

The diagnosis of the Paget-Schrötter syndrome is established by objective clinical manifestations and during the radiopaque study (phlebography).

Sick lie down compulsory hospitalizitsii. The affected limb is fixed and given an exalted position. Therapeutic measures of Paget-Schrötter syndrome are held by antithrombotic drugs, antiplatelet agents and fibrinolytic agents.

In order to improve the venous outflow, prescribe a course of physiotherapy. In severe cases of Paget-Schretter disease, surgery is indicated. The surgeon performs a thrombectomy with subsequent venous plasty. The prognosis is favorable.

Bone disease

The bone disease of Padet is a deforming pathology of the bones. Paget first described this disease and in his work proved that the cause of its development is chronic inflammation of a viral nature. The disease is familial in nature and more often in middle-aged men. It affects the bone tissue of the legs, skull, upper torso. The bones weaken, begin to bend and gradually collapse. Ill troubled aching pain. The skull is deformed and hearing is reduced in the victim due to compression of the auditory nerves. Lower limbs acquire X-shaped or wheel-shaped form.

The diagnosis is made on the basis of a physical examination and is confirmed radiographically. On the radiograph visible foci of osteoporosis, alternating with foci of seals. The skull box thickens 2 times higher than normal. Vertebrae deformed and flattened. The course of treatment of the disease is prescribed by an orthopedic traumatologist. As a rule, the doctor prescribes the administration of thyrocalcitonin for a month with a repeated course in six months. Nonsteroidal anti-inflammatory drugs, a course of vitamin D and calcium supplements are prescribed for pain. With the diagnosis of Pedzhet's bone disease, the patient is under regular medical check-up with regular visits to the doctor and examination. This helps reduce the risk of dangerous complications and relapses.

Causes of Paget's Disease

Paget's disease of the bone, usually occurs in people over 40 years of age and progresses with age. The exact reasons have not yet been established, however, the following options are put forward in medicine:

  • violation of phosphorus-calcium metabolism,
  • viral diseases,
  • heredity.

A combination of several reasons. In this case, dystrophic processes occur, as a result, the nutrition of the bone tissue is disturbed and its renewal is suspended. The bones become softer and lose their ability to bear the usual load.

Doctors distinguish two forms of this disease:

  1. Monoossal - defeat only one bone.
  2. Polyoesal - defeat several bones.

Bone change processes

Separate bones are exposed to Paget's disease, not a single case of generalized lesion has been encountered. Changes in the bones occur constantly, and it does not depend on the loads. The disease has three phases, which depend on whether the process prevails - resorption (osteolysis) or bone reconstruction (osteogenesis):

  1. Osteolytic. Lots of bone dissolve, hollow pits form,
  2. Active It combines two processes - hollow pits are formed and at the same time the bone grows. It acquires an uneven cellular form,
  3. Inactive. Bone tissue is replaced by connective (osteosclerosis).

The changes are serious - the tubular bones are bent, the surfaces become rough. More bones become thicker, but their weight, on the contrary, decreases. With the defeat of the skull, it thickens to three to five centimeters, head deformation occurs. If the spinal column is affected, then the vertebrae grow and become flattened; as a result, the curvature of the spine is formed.

Symptoms of Paget's Disease

The very first symptoms include:

  • Bone lesion in the area near the joints, the joint is involved in the disease,
  • Constant pain, which only gets stronger after resting,
  • Stiffness, impaired mobility,
  • Frequent fractures even from a weak effect on the bone,
  • Curvature of the feet,
  • Stoop, back pain,
  • You can feel the thickening or irregularities,
  • Head shape changes
  • Neuritis, compression of the nerves between the cervical vertebrae,
  • Partial or complete loss of hearing and / or vision in case of problems with the bones of the skull.

Diagnosis of Paget's Disease

When Paget's disease is suspected, the patient must undergo a laboratory blood test, which should determine the level of calcium, magnesium, alkaline phosphatase, phosphorus. So you can determine the degree of dystrophic process that occurs in the affected area.

An x-ray will also be assigned, which will show the changes that have occurred - the appearance of cellularity, the area of ​​absorption. X-rays must be repeated periodically so that the process can be monitored and monitored. Additionally, bone scintigraphy can be prescribed.

Treatment of Paget's Disease

Orthopedists are engaged in the treatment of this disease. The main treatments are drugs such as bisphosphonates. They can significantly slow down the process of rebuilding bone tissue, but the reception of these funds should be quite long.

Paget's disease is not amenable to complete treatment, but the progression of the disease can be slowed down by years. To do this, you need to undergo a course of treatment with a duration of about six months and, if necessary, it must be repeated. But the drugs have side effects, so the dose and duration of the course can be set only by a doctor.

For severe pain, nonsteroidal drugs are prescribed, for example, ibuprofen, paracetamol or Diclofenac. Also required is vitamin D and calcium.

Possible complications

The main complications that may arise in the process of Paget's disease include:

  • pathological fractures that cause movement disorders,
  • hearing impairment
  • development of sarcoma.

In order to prevent or at least make the process of formation of a complication slower, timely treatment and constant monitoring by an orthopedic surgeon are necessary.

Causes of Paget's Cancer

The exact causes of Paget’s cancer are currently unknown, but there are a number of theories about this:

  1. The epidermotropic theory says that when diagnosed with breast cancer, Pedzhet's cells can migrate through the ducts, get into the nipples, and there begin to implant and grow. This theory was based on the fact that Paget's cancer in almost 95% goes along with other forms of breast cancer. And the most frequent combination is with intraductal carcinoma.
  2. Transforming theory. Here the idea is being advanced that Paget's cancer develops due to the sharp transformation of epithelial cells into malignant forms.
  3. Family predisposition. More often, this theory is advanced when Paget's cancer is found in a man. Among the factors that can provoke cancer are nipple injuries and carcinogenic effects.

Symptoms of Paget's Cancer

Early symptoms have no particular specificity, so the patient does not show concern and is not in a hurry to pay a visit to the mammologist. The disease begins to manifest itself with a slight redness, mild irritation of the skin of the nipple-areolar complex, peeling. Symptoms may periodically disappear, but eventually reappear.

In the next stage of the disease, symptoms such as burning, itching and tingling can be monitored. Sensitivity is increased, there is a probability of formation of sero-hemorrhagic discharge, and the nipple becomes denser or compressed. The progression of symptoms is accompanied by hyperemia, erosions, the appearance of crusts and expressions. When removing the crust, wet moist areas are exposed.

Eczematous changes can also spread to the skin around the nipple and areola. The affected area rises slightly above the clear skin and has clear outlines. On palpation in most cases enlarged lymph nodes and tumor or nodular formations are palpable. In the later stages abundant bleeding often appears.

Paget's diagnosis of cancer

Diagnostics begin consultation with the mammologist, general examination. Then the patient must undergo an ultrasound scan and a breast biopsy, as well as MRI and mammogram.

During the examination, a number of changes are detected: changes in the shape and condition of the nipple, areas around, and palpation, tumors and inflamed lymph nodes are palpated. Investigating the smear, detect the Paget cells.

The quality of mammography increases if tumor nodes are present. In cases where there are none, the sensitivity of X-ray diagnostics is no more than 50%. When analyzing mammography, the doctor looks at the shape of the nipple, the thickness of the skin, the presence of microcalcifications, the location of the tumor and its size.

Usually, the changes that could not be detected with the help of mammography can be recognized after an ultrasound scan. It also helps the echography, which determines the nature of angiogenesis and shows the status of the ducts. MRI helps to identify changes that have not yet been manifested clinically. Biopsy makes it possible to establish a histological diagnosis before the operation.

Paget's cancer treatment

With an invasive form, as well as with intraductal carcinoma, radical mastectomy should be performed. Thus, the mammary glands, the pectoral muscles and the lymph nodes are surgically removed.

In the early stages, radical resection is sufficient, which includes the removal of the nipple and only part of the mammary gland where the tumor is located. After that do reconstructive mammoplasty.

Complementing the surgery, the patient must undergo gamma, chemo and hormone therapy. Systemic treatment is considered the best prevention of metastasis, as well as the occurrence of relapses.

Due to the fact that recognize the Paget's cancer is not always possible in time, treatment may be delayed for a long time. If we take into account that the growth of the tumor is quite aggressive, then the forecast is very difficult, because, judging by the reviews of doctors, even after a successful operation, there is a very high risk of recurrence.

The average life expectancy of people with Paget's cancer is approximately three years, but in the presence of metastases, the period is reduced to one year.

The pathogenesis of the disease and its clinical forms

In Paget's cancer, the large milk and sweat glands and their ducts are always the object of a cancer, so the disease can develop not only in the chest, but also in the armpits, on the genitals, around the anus of men and women. The disease is detected mainly in both sexes over 50 years of age. The average age of patients with this diagnosis is 68 years for men and 61 years for women.

Among the representatives of the strong half of humanity, Paget's cancer is distinguished by a high flow rate, aggressiveness and serious consequences. This is because in men, the pectoral muscles are small, so the cancer cells quickly infect the muscle tissue, then penetrate the lymph nodes, where they quickly metastasize and spread throughout the body.

Forms of Pedzhet's cancer differ in their manifestations. The table reflects the main clinical forms of the disease and their manifestations.

In 50% of cases, there are lesions in the nipple and its areola. In 40% of the nodal neoplasm is detected, which can be probed. In 10% of Paget's disease is detected by chance, after the study of discharge from the nipple.

The exact cause of the development of Paget's disease has not yet been established. There are only two assumptions that explain the appearance of this pathological process.

From the point of view of epidermotropy, it is believed that during the development of breast cancer, tumor cells, called Paget's cells, are localized in the deep layers of the gland, enter the milk ducts and spread through them, reaching the nipple. Once in the nipple tissue, they begin to actively grow. Proof of the epidermotropic theory is the fact that in 95% of cases, when detecting Paget's cancer, other types of breast tumors are simultaneously found in patients. Tumor cells, which are located in the nipple tissues, are formed by the epithelium of the milky ducts and have a glandular nature.

According to the theory of transformation, the cause of the development of Paget's cancer is the rapid degeneration of epithelial cells of the nipple tissue and the areola circle into malignant. Proof of this theory are the cases of detection of Paget's cancer without the development of malignant neoplasms from another location.

The main risk factors for the occurrence of Paget's disease are:

  • the presence of a large number of carcinogens in the environment,
  • injury to the chest and in particular the nipple and areola,
  • sunbathing topless in solariums and under open sunshine,
  • poor breast skin hygiene,
  • genetic predisposition to this disease, especially in the male line.

Symptomatology

The early symptoms of Paget's cancer do not cause anxiety in the patient, they are not specific and not alarming. Therefore, almost no one visits a doctor.

The onset of the disease is accompanied by a slight redness, irritation and peeling of the skin of the nipple and areola. Many people associate this with an allergic reaction to synthetic clothing, dry skin, and do not see anything unusual. Symptoms disappear rather quickly, which further aggravates the situation. The patient calms down without seeing any signs of the disease, does not consult a doctor, and in the meantime the disease continues to progress in his body. Sometimes the external manifestations are eliminated with the help of moisturizing ointments or therapeutic agents containing corticosteroids.

As the disease progresses, more serious symptoms begin to appear that are no longer impossible to notice. The nipple becomes very sensitive, it is impossible to touch it due to painful sensations. A person is regularly worried about tingling, itching, burning in the chest, in the nipple area. On linen you can see traces that leave a serous-hemorrhagic discharge from the nipple. The nipple itself changes its shape, it flattens and retracts. In the future, ulcers, erosive changes, dry crusts appear on the areola and nipple. If you remove these crusts, remain wet areas.

Paget's disease mainly affects one breast, but there are cases when cancer cells are formed in two mammary glands at once. Further eczematous manifestations spread to the skin of the breast. The affected area rises slightly above healthy tissue.

In half of the cases, when probing the breast, nodal neoplasms in the mammary gland tissues and enlargement of the adjacent lymph nodes can be felt. When the disease passes to the late stage, a bloody fluid begins to be released from the nipple due to the destruction of the nipple-areolar complex.

Mammography

If Paget's cancer is suspected, a mammography is performed in 3 projections, which makes it possible to exclude the presence of other types of malignant tumors. Mammography is the most informative way to detect tumor tumors in the breast. The procedure is most sensitive if there is a nodular tumor. If the tumor site is not observed, then the diagnostic sensitivity does not exceed 50%.

Breast ultrasound

This is a mandatory procedure, which is carried out in cases of suspected cancer of Paget. Ultrasound often reveals pathological processes that are not reflected in mammography.

Magnetic resonance imaging is able to identify the pathological process in the early stages, even if there are no visible clinical signs, as well as to differentiate the detected tumors. МРТ довольно точно выявляет место расположения опухоли, показывает распространенность опухоли (глубину ее проникновения, размеры пораженного участка). Позволяет определить утолщение тканей соска и ареолы, обнаружить опухоль в протоках, когда заболевание протекает бессимптомно.

Сцинтиграфия

Позволяет выявить самые незначительные опухоли. This is very important for early diagnosis. The method is based on the introduction into the body of radioactive isotopes with the subsequent study of the results of their distribution in the tissues. Breast scintigraphy is a highly informative procedure for detecting Paget's cancer, since the radioactive isotope 99mTc is actively accumulated in the Paget's dividing intraductal cells.

This laboratory test is designed to identify tumor cells in the material taken. For analysis, take nipple tissue and areola. The doctor takes a small sample of tissue, which is then examined under a microscope to detect Paget cells.

Biopsy is associated with a certain risk, since invasive effects (mechanical penetration into the body) can accelerate tumor metastasis. If discharge from the nipple is observed, then they are taken for further cytological examination.

During the diagnosis, Pedzhet's cancer is differentiated from dermatitis, psoriasis of scabies, superficial basiloma, nipple eczema, melanoma, tuberculosis and syphilis of the mammary glands, and herpes infection.

Treatment of Paget's disease is carried out in a complex. The main way to eliminate the disease is surgical intervention. But before and after surgery on the chest radiation therapy, hormone therapy, chemotherapy are mandatory.

When a tumor is detected at an early stage, the mammary gland is resected, as a result of which a part of the mammary gland and the nipple-areola complex are removed, containing the tumor. After some time, patients undergo surgery for reconstructive mammoplasty to give the breast a natural shape and hide the defect.

If Pedzhet's disease is detected in a late stage, combined with intraductal carcinoma, then a radical mastectomy is prescribed, consisting in the removal of the mammary gland, cellulose tissue, pectoral muscles, and lymph nodes.

Radiation therapy

In the future, systemic treatment of Paget's disease is aimed at preventing the recurrence of the disease, its metastasis. Radiation therapy is carried out at all stages of the detection of Paget's cancer. If there are contraindications to surgery (especially in older people), then radiation is used as an alternative treatment, replacing surgery.

Radiation therapy is combined with chemotherapy, and the course is carried out both before and after surgery. A modern method of irradiation with Intra Beam (Intra Beam) is used, in which, after the removal of a tumor, an irradiator is inserted into the wound for 15–20 minutes and only after that it is sutured. Rays contribute to the destruction of the remaining tumor cells, which significantly reduces the likelihood of relapse.

Chemotherapy

The procedure is necessarily assigned if there is a lesion of the lymph nodes. Chemotherapy is carried out in 2 stages. The first course is assigned to surgery. It is called non-adjuvant and is aimed at stopping the growth of a tumor. The second course is aimed at eliminating possibly left after surgery by malignant cells.

Treatment with hormone drugs

Hormone therapy is carried out with the use of Tamoxifen, Nolvadex, Zitazonium only when detecting receptors sensitive to steroid hormones. Before using hormones, patients of reproductive age are recommended to block the work of the ovaries.

The prognosis of the disease will depend on a number of factors.

  • the clinical stage of Pedzhet's cancer,
  • the age of the patient, the number of affected lymph nodes, the presence of aggravating morphological signs, the combination of the disease with infiltrative lobular cancer, the multiplicity of tumor neoplasms.

Pedzhet's cancer is very aggressive, the probability of its reappearance is extremely high, even after the operation.

The prognosis for the life of a patient with such a disease is unfavorable, on average, people live for 3 years, and if infiltration and metastases are found, then the period is reduced to one year. At the stage of metastases, complete cure will not be given, symptomatic and palliative therapy is prescribed. If the lymph nodes are not affected, then the prognosis is favorable, almost 90% of patients live more than 10 years.

Prevention

Paget's disease, like many cancers, is almost impossible to prevent, and it is difficult to detect it in the initial stage due to the absence of specific symptoms.

To preventive measures include:

  • regular examination of the mammary glands. Only by visiting a specialist mammologist in a timely manner, passing through his examination, can the disease be detected in time,
  • careful breast care (wear cotton underwear, monitor skin hygiene, do not sunbathe with open breasts),
  • getting rid of bad habits.

Early diagnosis and treatment of Paget's disease in the early stages, significantly improves the chances of survival of the sick person and the prognosis for later life.

What is Paget's Disease?

Also known by medical names: deforming osteitis, osteus deforming, osteodystrophy deforming.

Bone is a living tissue that is constantly destroyed and restored, this process is called "bone remodeling."

In the bones affected by Paget's disease, the remodeling process is disrupted, which leads to disruption of the structure or architecture of the bone, the bone does not recover properly, forming "weak" bones that deform, over-stretch, bend and are prone to fractures.

Paget's disease can occur in any bones of the body. In one third of patients, only one bone is affected, but the average number of damaged bones is about three.

The bones, most often involved in the process of the pelvis, spine, skull and long tubular bones of the arms and legs.

Up to 3% of people of European descent over 40 are affected by Paget's disease, and the prevalence of the disease increases with age. The disease occurs twice as often in men than in women.

Causes of Paget's Disease

The causes of Paget's disease are still unknown.

The most common opinion is about the viral nature of the disease (slow viral infection). A sick person may, for many years, be a carrier of the virus without any manifestations of the disease, after which, as a result of exposure to provoking factors, symptoms appear. One of the most significant viral infections is measles.

The role of hereditary predisposition is also not excluded. Paget’s disease is considered to be somewhat familial in nature. Therefore, it is recommended that brothers, sisters and children of patients with Paget's disease take a biochemical blood test every two to three years to monitor the level of alkaline phosphatase in the blood, and, if necessary, do a radiography of the bones.

These reasons are not mutually exclusive.

Symptoms of the disease

The symptoms of Paget's disease depend on the damaged bones and the severity of the disease.

Symptoms of Paget’s disease include pain around the damaged bone and osteoarthritis if the disease occurs near the bone joint.

Pain associated with damaged bone in Paget's disease is usually described as continuous, dull, aching and, unlike osteoarthritis, often aggravated at rest and after rest.

Stiffness, limited mobility in the affected joints are also increased after rest.

Bones in different parts of the body become strongly thickened and fragile, which leads to the destruction of bones that support body weight. Fractures can occur even with minor injuries, the back becomes stooped and deformed, and the legs are bent.

The abnormal growth of the bones of the skull, which results in a characteristic appearance of the head and spinal column, can put pressure on the surrounding nerves, the damage of which can cause serious consequences, such as permanent loss of hearing or sight, headaches or damage to the nerves of the spinal cord, causing tingling and numbness in arms and legs, feeling of "crawling goosebumps" on the body. In very rare cases, a severe form of Paget's disease can cause cardiac arrest or malignancy (malignancy) of the process with the development of a bone tumor.

Diagnosis of the disease

X-ray and blood analysis for serum alkaline phosphatase, which is formed as a result of bone remodeling, is used to diagnose the disease.

The level of serum alkaline phosphatase is often much higher than normal. Regular blood tests are needed to monitor any changes.

Scintigraphy of the bones of the skeleton is important in determining the exact location of the destruction of the bones, it provides visualization of the entire skeleton.

What can you do?

If you notice in yourself or your relatives the changes described above, you should immediately consult a doctor.

Since the course of the disease is unpredictable, it can flow for a long time and without any serious consequences, and can lead to disability in a few months, it is necessary to start treatment as soon as possible.

Self-treatment is just as dangerous as not treating a disease. Therefore, without consulting a doctor, do not try to heal yourself.

What can a doctor do?

The doctor will carry out examinations necessary for accurate diagnosis. After determining the stage of the disease, he will prescribe the necessary treatment, including medication, special exercises to strengthen the skeletal system.

The prognosis in the absence of complications is favorable. Patients should be at the dispensary observation at the orthopedist.

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Complications

The main complications of Paget's disease include:

  • pathological fractures with movement disorders,
  • damage to hearing due to changes in the bones in the area of ​​the inner ear,
  • development of bone sarcoma at the site of the pathological process.

Therefore, timely treatment and observation by an orthopedist is necessary, which will prevent or substantially slow down the formation of complications.

Causes of Paget's Cancer

The etiology of Paget's cancer is unknown. Currently, mammology has several theories regarding the cause of the development of this disease.

According to the epidermotropic theory, atypical cells (Paget cells) in breast cancer with deeper localization migrate along the ducts to the nipple where they are implanted and expanded.

This theory is based on observations showing that Paget's cancer in 95% of cases is combined with other forms of breast cancer, more often with intraductal carcinoma. Pedzhet's tumor cells that infiltrate the epidermis in nipple cancer are derived from the epithelium of the milky ducts and are glandular cells.

According to the transforming theory, the development of Paget's cancer results from the spontaneous degeneration of the epithelial cells of the nipple and areola into malignant ones.

This hypothesis is based on those cases in which breast cancer of other localization is not detected. The family predisposition to the development of Pedzhet's cancer in men is described, as well as its association with scleroderma, pemphigus, and cervical cancer.

Among the provoking factors are considered nipple injuries, various carcinogenic effects.

According to the variant of the clinical course, Pedzhet's cancer is released;

  • in the form of acute eczematoid (with a fine-grained rash, weeping and ulceration of the nipple)
  • in the form of chronic eczematoid (with the formation of crusts, which, when removed, form a soak area)
  • in psoriatic form (with the presence of small pink papules covered with dry flaky scales)
  • ulcerated (with crater-like ulceration)
  • in the tumor form (with the presence of a tumor in the thickness of the gland).

With Paget's cancer in 50% of cases, changes affect only the nipple-areola complex. In 40% of patients, against the background of changes in the areola and nipple, a palpable tumor site in the periphery region is detected. In 10% of cases, Paget's cancer is an accidental finding during cytological examination of a nipple smear in case of discharge from the breast ducts.

Paget's Prognosis for Cancer

The initial recognition of Paget's cancer is not always timely, and therefore adequate treatment may be delayed for many months.

Given the aggressive growth of the tumor, the prognosis for Paget's cancer is difficult. Even after surgery there is a high probability of relapse. The average life expectancy for Paget's cancer is about 3 years, with an infiltrative component and metastasis - about 1 year.

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