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Classically acute radiation syndrome is divided into three main presentations: hematopoietic, gastrointestinal, and neurological/vascular. These syndromes may or may not be preceded by a prodrome. The speed of onset of symptoms is related to radiation exposure, with greater doses resulting in a shorter delay in symptom onset. These presentations presume whole-body exposure and many of them are markers which are not valid if the entire body has not been exposed. Each syndrome requires that the tissue showing the syndrome itself be exposed. The hematopoietic syndrome requires exposure of the areas of bone marrow actively forming blood elements (i.e., the pelvis and sternum in adults). The neurovascular symptoms require exposure of the brain. The gastrointestinal syndrome is not seen if the stomach and intestines are not exposed to radiation. Some areas affected are:
1. Hematopoietic. This syndrome is marked by a drop in the number of blood cells, called aplastic anemia. This may result in infections due to a low amount of white blood cells, bleeding due to a lack of platelets, and anemia due to few red blood cells in the circulation. These changes can be detected by blood tests after receiving a whole-body acute dose as low as 0.25 Gy, though they might never be felt by the patient if the dose is below 1 Gy. Conventional trauma and burns resulting from a bomb blast are complicated by the poor wound healing caused by hematopoietic syndrome, increasing mortality.
2. Gastrointestinal. This syndrome often follows absorbed doses of 6–30 Gy (600–3000 rad). The signs and symptoms of this form of radiation injury include nausea, vomiting, loss of appetite, and abdominal pain. Vomiting in this time-frame is a marker for whole body exposures that are in the fatal range above 4 Gy. Without exotic treatment such as bone marrow transplant, death with this dose is common. The death is generally more due to infection than gastrointestinal dysfunction.
3. Neurovascular. This syndrome typically occurs at absorbed doses greater than 30 Gy (3000 rad), though it may occur at 10 Gy (1000 rad). It presents with neurological symptoms such as dizziness, headache, or decreased level of consciousness, occurring within minutes to a few hours, and with an absence of vomiting. It is invariably fatal.
The prodrome (early symptoms) of ARS typically includes nausea and vomiting, headaches, fatigue, fever, and a short period of skin reddening. These symptoms may occur at radiation doses as low as 0.35 Gy (35 rad). These symptoms are common to many illnesses, and may not, by themselves, indicate acute radiation sickness.
Cutaneous radiation syndrome (CRS) refers to the skin symptoms of radiation exposure. Within a few hours after irradiation, a transient and inconsistent redness (associated with itching) can occur. Then, a latent phase may occur and last from a few days up to several weeks, when intense reddening, blistering, and ulceration of the irradiated site are visible. In most cases, healing occurs by regenerative means; however, very large skin doses can cause permanent hair loss, damaged sebaceous and sweat glands, atrophy, fibrosis (mostly Keloids), decreased or increased skin pigmentation, and ulceration or necrosis of the exposed tissue. Notably, as seen at Chernobyl, when skin is irradiated with high energy beta particles, moist desquamation (peeling of skin) and similar early effects can heal, only to be followed by the collapse of the dermal vascular system after two months, resulting in the loss of the full thickness of the exposed skin. This effect had been demonstrated previously with pig skin using high energy beta sources at the Churchill Hospital Research Institute, in Oxford.
The sarcoma first appears as a bruise mark, a purplish discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
Up to 10% of invasive cancers are related to radiation exposure, including both ionizing radiation and non-ionizing radiation. Additionally, the vast majority of non-invasive cancers are non-melanoma skin cancers caused by non-ionizing ultraviolet radiation. Ultraviolet's position on the electromagnetic spectrum is on the boundary between ionizing and non-ionizing radiation. Non-ionizing radio frequency radiation from mobile phones, electric power transmission, and other similar sources have been described as a possible carcinogen by the World Health Organization's International Agency for Research on Cancer, but the link remains unproven.
Exposure to ionizing radiation is known to increase the future incidence of cancer, particularly leukemia. The mechanism by which this occurs is well understood, but quantitative models predicting the level of risk remain controversial. The most widely accepted model posits that the incidence of cancers due to ionizing radiation increases linearly with effective radiation dose at a rate of 5.5% per sievert. If the linear model is correct, then natural background radiation is the most hazardous source of radiation to general public health, followed by medical imaging as a close second.
According to the prevalent model, any radiation exposure can increase the risk of cancer. Typical contributors to such risk include natural background radiation, medical procedures, occupational exposures, nuclear accidents, and many others. Some major contributors are discussed below.
Basal-cell skin cancer (BCC) usually presents as a raised, smooth, pearly bump on the sun-exposed skin of the head, neck or shoulders. Sometimes small blood vessels (called telangiectasia) can be seen within the tumor. Crusting and bleeding in the center of the tumor frequently develops. It is often mistaken for a sore that does not heal. This form of skin cancer is the least deadly and with proper treatment can be completely eliminated, often without scarring.
There are a variety of different skin cancer symptoms. These include changes in the skin that do not heal, ulcering in the skin, discolored skin, and changes in existing moles, such as jagged edges to the mole and enlargement of the mole.
Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma.
Individuals with a basal-cell carcinoma typically present with a shiny, pearly skin nodule. However, superficial basal-cell cancer can present as a red patch similar to eczema. Infiltrative or morpheaform basal-cell cancers can present as a skin thickening or scar tissue – making diagnosis difficult without using tactile sensation and a skin biopsy. It is often difficult to visually distinguish basal-cell cancer from acne scar, actinic elastosis, and recent cryodestruction inflammation.
Temporal lobe necrosis is a late-stage and serious complication usually occurring in persons who have undergone radiation treatment for nasopharyngeal carcinoma (NPC). It is rather rare and occurs in 4-30% of patients who receive radiation treatment for NPC. Many patients who experience temporal lobe necrosis are asymptomatic. This demonstrates a need for consistent imaging follow up, such as MRI and/or PET/CT, to help with the potential management of it. Those who are symptomatic usually suffer from "vague" symptoms including headaches, dizziness, intracranial pressure, personality changes, seizures, and short-term memory loss. The rarity of this disease has led to difficulty in finding optimal treatments, however, most treatments include one or some of the following: steroids, hyperbaric oxygen, surgery, and decadron.
It presents as a slow growing mass that especially affects tendons and aponeuroses and it is deeply situated. Patients often perceive it as a lump or hard mass. It causes either pain or tenderness but only until it becomes large enough. This kind of tumor is commonly found in the extremities especially around the knee, feet and ankle. Patients diagnosed with clear cell sarcoma are usually between the ages of 20 and 40.
The lungs are a radiosensitive organ, and radiation pneumonitis can occur leading to pulmonary insufficiency and death (100% after exposure to 50 gray of radiation), in a few months. Radiation pneumonitis is characterized by:
- Loss of epithelial cells
- Edema
- Inflammation
- Occlusions airways, air sacs and blood vessels
- Fibrosis
Basal-cell carcinoma (BCC), also known as basal-cell cancer, is the most common type of skin cancer. It often appears as a painless raised area of skin, that may be shiny with small blood vessels running over it or it may present as a raised area with ulceration. Basal-cell cancer grows slowly and can damage the tissue around it but is unlikely to spread to distant areas or result in death.
Risk factors include exposure to ultraviolet light, having lighter skin, radiation therapy, long term exposure to arsenic, and poor immune system function. UV light exposure during childhood is particularly harmful. Tanning beds are becoming another common source of ultraviolet radiation. Diagnosis is often based on skin examination and confirmed by tissue biopsy.
It is not clear if sunscreen affects the risk of basal-cell cancer. Treatment is typically by surgical removal. This can be by simple excision if the cancer is small, otherwise Mohs surgery is generally recommended. Other options may include application of cold, topical chemotherapy, laser surgery, or the use of imiquimod. In the rare cases in which distant spread has occurred, chemotherapy or targeted therapy may be used.
Basal cell cancer accounts for at least 32% of all cancers globally. Of skin cancers other than melanoma, about 80% are basal-cell cancers. In the United States about 35% of white males and 25% of white females are affected by BCC at some point in their life.
The early lesions are usually asymptomatic. The patients presenting with an advanced stage of the disease comprises around 66-77% of the cases.
The most important signs include a lump in the neck when palpated and weight loss.
People may also present with fatigue as a symptom.
The primary tumor does not have readily discernible signs or symptoms as they grow within the tonsillar capsule. It is difficult to notice anything suspicious on examination of the tonsil other than slight enlargement or the development of firmness around the area.
The carcinoma may occur in one or more sites deep within the tonsillar crypts. It may be accompanied by the enlargement of the tonsil. The affected tonsil grows into the oropharyngeal space making it noticeable by the patient in the form of a neck mass mostly in the jugulodiagastric region.
As the tonsils consist of a rich network of lymphatics, the carcinoma may metastasise to the neck lymph nodes which many are cystic.
Extension of tumor to skull or mediastinum can occur.
The additional symptoms include a painful throat, dysphagia, otalgia (due to cranial nerve involvement), foreign body sensation, bleeding, fixation of tongue (infiltration of deep muscles) and trismus (if the pterygoid muscle is involved in the parapharyngeal space).
On the other hand, the tumor may also present as a deep red or white fungating wound growing outwards, breaking the skin surface with a central ulceration. This wound-like ulcer fails to heal (non-healing) leading to bleeding and throat pain and other associated symptoms.
During biopsy, the lesion may show three signs: Gritty texture, Firmness and cystification owing to keratinization, fribrosis and necrosis respectively.
Cervical lymphydenopathy may be present.
Chronic radiation syndrome is a constellation of health effects that occur after months or years of chronic exposure to high amounts of ionizing radiation. Chronic radiation syndrome develops with a speed and severity proportional to the radiation dose received, i.e., it is a deterministic effect of radiation exposure, unlike radiation-induced cancer. It is distinct from acute radiation syndrome in that it occurs at dose rates low enough to permit natural repair mechanisms to compete with the radiation damage during the exposure period. Dose rates high enough to cause the acute form (> ~0.1 Gy/h) are fatal long before onset of the chronic form. The lower threshold for chronic radiation syndrome is between 0.7 and 1.5 Gy, at dose rates above 0.1 Gy/yr. This condition is primarily known from the Kyshtym disaster, where 66 cases were diagnosed, and has received little mention in Western literature. A future ICRP publication, currently in draft, may recognize the condition but with higher thresholds.
In 2013, Alexander V. Akleyev described the chronology of the clinical course or CRS while presenting at ConRad in Munich, Germany. In his presentation, he defined the latent period as being 1-5 years, and the formation coinciding with the period of maximum radiation dose. The recovery period was described as being 3-12 months after exposure ceased. He concluded that "CRS represents a systemic response of the body as a whole to the chronic total body exposure in man." In 2014, Akleyev's book "Comprehensive analysis of chronic radiation syndrome, covering epidemiology, pathogenesis, pathoanatomy, diagnosis and treatment" was published by Springer.
Radiation-induced lung injury is a general term for damage to the lungs which occurs as a result of exposure to ionizing radiation. In general terms, such damage is divided into early inflammatory damage ("radiation pneumonitis") and later complications of chronic scarring ("radiation fibrosis"). Pulmonary radiation injury most commonly occurs as a result of radiation therapy administered to treat cancer.
UPS occurs most commonly in the extremities and retroperitoneum, but has been reported in other sites. Metastasis occurs most frequently in the lungs (90%), bones (8%), and liver (1%).
In the extremities, it presents itself as a painless enlarging soft tissue mass.
When the tumor is large and there is presence of necrosis and local recurrence, the prognosis is poor. Presence of metastasis occurs in more than 50% cases and the common places of its occurrence are the bone, lymph node and lungs. Five-year survival rates, which are reported to be between 50-65%, can be misleading because the disease is prone to late metastasis or recurrence. Ten and twenty-year survival rates are 33% and 10%, respectively.
The most common symptom of bone tumors is pain, which will gradually increase over time. A person may go weeks, months, and sometimes years before seeking help; the pain increases with the growth of the tumor. Additional symptoms may include fatigue, fever, weight loss, anemia, and/or unexplained bone fractures. Many patients will not experience any symptoms, except for a painless mass. Some bone tumors may weaken the structure of the bone, causing pathologic fractures.
The most common cancers in children are (childhood) leukemia (32%), brain tumors (18%), and lymphomas (11%). In 2005, 4.1 of every 100,000 young people under 20 years of age in the U.S. were diagnosed with leukemia, and 0.8 per 100,000 died from it. The number of new cases was highest among the 1–4 age group, but the number of deaths was highest among the 10–14 age group.
In 2005, 2.9 of every 100,000 people 0–19 years of age were found to have cancer of the brain or central nervous system, and 0.7 per 100,000 died from it. These cancers were found most often in children between 1 and 4 years of age, but the most deaths occurred among those aged 5–9. The main subtypes of brain and central nervous system tumors in children are: astrocytoma, brain stem glioma, craniopharyngioma, desmoplastic infantile ganglioglioma, ependymoma, high-grade glioma, medulloblastoma and atypical teratoid rhabdoid tumor.
Other, less common childhood cancer types are:
- Neuroblastoma (6%, nervous system)
- Wilms tumor (5%, kidney)
- Non-Hodgkin lymphoma (4%, blood)
- Childhood rhabdomyosarcoma (3%, many sites)
- Retinoblastoma (3%, eye)
- Osteosarcoma (3%, bone cancer)
- Ewing sarcoma (1%, many sites)
- Germ cell tumors (5%, many sites)
- Pleuropulmonary blastoma (lung or pleural cavity)
- Hepatoblastoma and hepatocellular carcinoma (liver cancer)
Radiation dermatitis (also known as radiodermatitis) is a skin disease associated with prolonged exposure to ionizing radiation. Radiation dermatitis occurs to some degree in most patients receiving radiation therapy, with or without chemotherapy.
There are three specific types of radiodermatitis: acute radiodermatitis, chronic radiodermatitis, and eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy. Radiation therapy can also cause radiation cancer.
With interventional fluoroscopy, because of the high skin doses that can be generated in the course of the intervention, some procedures have resulted in early (less than two months after exposure) and/or late (two months or more after exposure) skin reactions, including necrosis in some cases.
Radiation dermatitis, in the form of intense erythema and vesiculation of the skin, may be observed in radiation ports.
As many as 95% of patients treated with radiation therapy for cancer will experience a skin reaction. Some reactions are immediate, while others may be later (e.g., months after treatment).
Undifferentiated pleomorphic sarcoma (UPS), also undifferentiated pleomorphic sarcoma (PUS) and previously malignant fibrous histiocytoma (abbreviated MFH), is a type of cancer and soft tissue sarcoma.
It is considered a diagnosis of exclusion for sarcomas that cannot be more precisely categorized. Other sarcomas are cancers that form in bone and soft tissues, including muscle, fat, blood vessels, lymph vessels, and fibrous tissue (such as tendons and ligaments).
Acute radiodermatitis occurs when an "erythema dose" of ionizing radiation is given to the skin, after which visible erythema appears up to 24 hours after. Radiation dermatitis generally manifests within a few weeks after the start of radiotherapy. Acute radiodermatitis, while presenting as red patches, may sometimes also present with desquamation or blistering. Erythema may occur at a dose of 2 Gy radiation or greater.
Common symptoms include seizure, headaches, nausea and vomiting, memory loss, changes to personality, mood or concentration; and localized neurological problems.
The kind of symptoms produced depends more on the location of the tumor than on its pathological properties. The tumor can start producing symptoms quickly, but occasionally is an asymptomatic condition until it reaches an enormous size.
There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.
First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.
Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses.