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Cervical lymphadenopathy can be thought of as "local" where only the cervical lymph nodes are affected, or "general" where all the lymph nodes of the body are affected.
Cervical lymphadenopathy refers to lymphadenopathy of the cervical lymph nodes (the glands in the neck). The term "lymphadenopathy" strictly speaking refers to disease of the lymph nodes, though it is often used to describe the enlargement of the lymph nodes. Similarly, the term "lymphadenitis" refers to inflammation of a lymph node, but often it is used as a synonym of lymphadenopathy.
Cervical lymphadenopathy is a sign or a symptom, not a diagnosis. The causes are varied, and may be inflammatory, degenerative, or neoplastic. In adults, healthy lymph nodes can be palpable (able to be felt), in the axilla, neck and groin. In children up to the age of 12 cervical nodes up to 1 cm in size may be palpable and this may not signify any disease. If nodes heal by resolution or scarring after being inflamed, they may remain palpable thereafter. In children, most palpable cervical lymphadenopathy is reactive or infective. In individuals over the age of 50, metastatic enlargement from cancers (most commonly squamous cell carcinomas) of the aerodigestive tract should be considered.
Lymphadenopathy of the axillary lymph nodes can be defined as solid nodes measuring more than 15 mm without fatty hilum. Axillary lymph nodes may be normal up to 30 mm if consisting largely of fat.
Lymphadenopathy can occur in one or more groups of lymph nodes. Among 358 cases of Rosai–Dorfman disease that Rosai collected in a disease registry for which the location of lymphadenopathy was specified, 87.3% had cervical lymphadenopathy. Axillary, inguinal, and mediastinal lymphadenopathy are also found in Rosai–Dorfman disease.
Accumulation of histiocytes may occur outside of lymph nodes. The most common sites of extranodal disease in Rosai's registry were skin, nasal cavity/paranasal sinuses, soft tissue, eyelid/orbit, bone, salivary glands, and central nervous system.
The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.
Lymphadenopathy or adenopathy is disease of the lymph nodes, in which they are abnormal in size, number, or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In clinical practice, the distinction between lymphadenopathy and lymphadenitis is rarely made and the words are usually treated as synonymous. Inflammation of the lymphatic vessels is known as lymphangitis. Infectious lymphadenitides affecting lymph nodes in the neck are often called scrofula.
The term comes from the word lymph and a combination of the Greek words , "adenas" ("gland") and , "patheia" ("act of suffering" or "disease").
Lymphadenopathy is a common and nonspecific sign. Common causes include infections (from minor ones such as the common cold to dangerous ones such as HIV/AIDS), autoimmune diseases, and cancers. Lymphadenopathy is also frequently idiopathic and self-limiting.
Lymph nodes or 'glands' or "nodes" or "lymphoid tissue" are nodular bodies located throughout the body but clustering in certain areas such as the armpit, back of the neck and the groin. They are part of the lymphatic system.
The lymphatic system is part of the body's immune surveillance system. Blood contains fluid and blood cells. The fluid, which may contain suspended foreign material such as bacteria and viruses, seeps through blood vessel walls into the tissues, where it bathes the body cells and exchanges substances with them. Some of this fluid is then taken up by lymphatic vessels and passed back to the heart, where it is again mixed with the blood. On its way the fluid passes through the lymph nodes. If nodes detect something foreign passing through them such as a bacterium or a cancer cell they will swell up. This is called "lymphadenopathy" or "swollen glands". Usually this is localised (for example an infected spot on the scalp will cause lymph nodes in the neck on that same side to swell up), but when it is in two or more regions, it is called "generalized lymphadenopathy".
Usually this is in response to a body-wide infectious disease such as influenza and will go away once the person has recovered, but sometimes it can persist long-term, even when there is no obvious cause of disease. This is then called "persistent generalized lymphadenopathy" (PGL).
Bilateral hilar lymphadenopathy is a bilateral enlargement of the lymph nodes of pulmonary hila. It is a radiographic term that describes the enlargement of mediastinal lymph nodes and is most commonly identified by a chest x-ray.
Lymphatic disease is a class of disorders which directly affect the components of the lymphatic system.
Examples include Castleman's disease and lymphedema.
Diseases and disorder
Hodgkin's Disease/Hodgkin's Lymphoma
This is a type of cancer of the lymphatic system. It can start almost anywhere in the body. It is believed to be caused by HIV, Epstein-Barr Syndrome, age and family history. Symptoms include weight loss, fever, swollen lymph nodes, night sweats, itchy skin, fatigue, chest pain, coughing or trouble swallowing.
Non-Hodgkin's Lymphoma
Lymphoma is a usually malignant cancer. It is caused by the body producing too many abnormal white blood cells. It is not the same as Hodgkin's Disease. Symptoms usually include painless, enlarged lymph node or nodes in the neck, weakness, fever, weight loss, and anemia.
Lymphadenitis is an infection of the lymph nodes usually caused by a virus, bacteria or fungi. Symptoms include redness or swelling around the lymph node.
Lymphangitis
Lymphangitis is an inflammation of the lymph vessels. Symptoms usually include swelling, redness, warmth, pain or red streaking around the affected area.
Lymphedema
Lymphedema is the chronic pooling of lymph fluid in the tissue. It usually starts in the feet or lower legs. It's also a side-effect of some surgical procedures.
Lymphocytosis
Lymphocytosis is a high lymphocyte count. It can be caused by an infection, blood cancer, lymphoma, or autoimmune disorders that are accompanied by chronic swelling.
Secondary lymphedema is a condition characterized by swelling of the soft tissues in which an excessive amount of lymph has accumulated, and is caused by certain malignant diseases such as Hodgkin's disease and Kaposi sarcoma.
Secondary lymphedema also can be caused by several non-malignant diseases, such as lipedema, and can result from the removal of lymph nodes during various cancer surgeries, especially for breast and prostate cancers.
A urogenital pelvic malignancy is a regional lymph node involvement in urogenital malignancies (category N in the TNM classification system) is a significant radiologic finding, with important implications for treatment and prognosis. Male urogenital pelvic cancers commonly spread to iliopelvic or retroperitoneal lymph nodes by following pathways of normal lymphatic drainage from the pelvic organs. The most likely pathway of nodal spread (superficial inguinal, pelvic, or paraaortic) depends on the tumour location in the prostate, penis, testes, or bladder and whether surgery or other therapy has disrupted normal lymphatic drainage from the tumour site; knowledge of both factors is needed for accurate disease staging. At present, lymph node status is most often assessed with standard anatomic imaging techniques such as multidetector computed tomography or magnetic resonance imaging (MRI). However, the detection of nodal disease with these techniques is reliant on lymph node size and morphological characteristics, criteria that provide limited diagnostic specificity. Functional imaging techniques, such as diffusion-weighted MRI performed with or without a lymphotropic contrast agent and positron emission tomography, may allow a more accurate nodal assessment based on molecular or physiologic activity .
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.
General signs and symptoms include depression, fever, weight loss, loss of appetite, loss of hair or fur and vomiting. Lymphoma is the most common cancerous cause of hypercalcemia (high blood calcium levels) in dogs. It can lead to the above signs and symptoms plus increased water drinking, increased urination, and cardiac arrhythmias. Hypercalcemia in these cases is caused by secretion of parathyroid hormone-related protein.
Multicentric lymphoma presents as painless enlargement of the peripheral lymph nodes. This is seen in areas such as under the jaw, the armpits, the groin, and behind the knees. Enlargement of the liver and spleen causes the abdomen to distend. Mediastinal lymphoma can cause fluid to collect around the lungs, leading to coughing and difficulty breathing. Hypercalcemia is most commonly associated with this type.
Gastrointestinal lymphoma causes vomiting, diarrhea, and melena (digested blood in the stool). Low serum albumin levels and hypercalcemia can also occur.
Lymphoma of the skin is an uncommon occurrence. The epitheliotropic form typically appears as itchy inflammation of the skin progressing to nodules and plaques.
The non-epitheliotropic form can have a wide variety of appearances, from a single lump to large areas of bruised, ulcerated, hairless skin. The epitheliotropic form must be differentiated from similar appearing conditions such as pemphigus vulgaris, bullous pemphigoid, and lupus erythematosus.
Signs for lymphoma in other sites depend on the location. Central nervous system involvement can cause seizures or paralysis. Eye involvement, seen in 20 to 25 percent of cases, can lead to glaucoma, uveitis, bleeding within the eye, retinal detachment, and blindness. Lymphoma in the bone marrow causes anemia, low platelet count, and low white blood cell count.
Second most common primary anterior mediastinal mass in adults. Most are seen in the anterior compartment and rest are seen in middle compartment. Hodgkin's usually present in 40-50's with nodular sclerosing type (7), and non-Hodgkin's in all age groups. Can also be primary mediastinal B-cell lymphoma with exceptionally good prognosis. Common symptoms include fever, weight loss, night sweats, and compressive symptoms such as pain, dyspnea, wheezing, Superior vena cava syndrome, pleural effusions (10,11). Diagnosis usually by CT showing lobulated mass. Confirmation done by tissue biopsy of accompanying nodes if any, mediastinoscopy, mediastinotomy, or thoracotomy. FNA biopsy is usually not adequate. (12,13,14) Treatment of mediastinal Hodgkin's involves chemotherapy and/or radiation. 5 year survival is now around 75%. (15) Large-cell type may have somewhat better prognosis. Surgery is generally not performed because of invasive nature of tumor.
Of all cancers involving the same class of blood cell, 2% of cases are mediastinal large B cell lymphomas.
The cancer is classified into low and high grade types. Classification is also based on location. The four location types are multicentric, mediastinal, gastrointestinal, and extranodal (involving the kidney, central nervous system, skin, heart, or eye). Multicentric lymphoma, the most common type (by greater than 80 percent), is found in the lymph nodes, with or without involvement in the liver, spleen, or bone marrow. Mediastinal lymphoma occurs in the lymph nodes in the thorax and possibly the thymus. Gastrointestinal lymphoma occurs as either a solitary tumor or diffuse invasion of the stomach or intestines, with or without involvement in the surrounding lymph nodes, liver or spleen. Classification is further based on involvement of B-lymphocytes or T-lymphocytes. Approximately 70 percent are B-cell lymphoma. Cutaneous lymphoma can be classified as epitheliotropic (closely conforming to the epidermis) or non-epitheliotropic. The epitheliotropic form is typically of T-cell origin and is also called mycosis fungoides. The non-epitheliotropic form is typically of B-cell origin.
MCD clinical features range from waxing and waning mild enlargement of the lymph nodes with B symptoms to more severe cases involving intense inflammation, widespread enlargement of lymph nodes enlargement of the liver and spleen, vascular leak syndrome with anasarca, fluid collections in the space around the lungs, and fluid collection in the abdomen's peritoneal space, organ failure, and even death. The most common 'B Symptoms' of MCD are high fevers, night sweats, weight loss, and loss of appetite. Acute episodes can display significant clinical overlap with acute viral illnesses, autoimmune diseases, hematologic malignancies, and even sepsis. Laboratory findings commonly include low red blood cell count, low or high platelet counts, low albumin, high gamma globulin levels, elevated C-reactive protein levels, elevated erythrocyte sedimentation rate, IL-6, vascular endothelial growth factor (VEGF), and fibrinogen; positive anti-nuclear antibody, anti-erythrocyte autoantibodies, and anti-platelet antibodies; and protein spilling into the urine, and polyclonal marrow plasmacytosis. Castleman disease is seen in POEMS syndrome and is implicated in 10% of cases of paraneoplastic pemphigus.
Castleman disease, also known as giant lymph node hyperplasia, lymphoid hamartoma, or angiofollicular lymph node hyperplasia, is a group of uncommon lymphoproliferative disorders that share common lymph node histological features. The disease is named after Benjamin Castleman.
Castleman's disease has two main forms: It may be localized to a single lymph node (unicentric) or occur systemically (multicentric).
The unicentric form can usually be cured by surgically removing the lymph node, with a 10-year survival of 95%.
Multicentric Castleman disease (MCD) involves hyperactivation of the immune system, excessive release of proinflammatory chemicals (cytokines), proliferation of immune cells (B cells and T cells), and multiple organ system dysfunction. Castleman disease must be distinguished from other disorders that can demonstrate "Castleman-like" lymph node features, including reactive lymph node hyperplasia, autoimmune disorders, and malignancies. Multicentric Castleman's disease is associated with lymphoma and Kaposi's sarcoma.
Most common primary anterior mediastinal tumor (20%) in adults but rarely seen in children. It can be classified as lymphocytic, epithelial, or spindle cell histologies, but the clinical significance of these classifications is controversial. Tonofibrils seen under electron microscopy can differentiate thymoma from other tumors such as carcinoid, Hodgkin's, and seminoma. Patients are usually asymptomatic but can present with myasthenia gravis-related symptoms, substernal pain, dyspnea, or cough. Invasive tumors can produce compression effects such as superior vena cava syndrome. (3,4) Thymomas are diagnosed with CT or MRI revealing a mass in anterior mediastinum. Therapy in stage I tumors consists of surgical resection with good prognosis. Stage II-III requires maximal resection possible followed by radiation. Stage IV disease requires addition of cisplatin-based chemotherapy in addition to those in stage II and III. For those with invasive thymoma, treatment is based on induction chemotherapy, surgical resection, and post-surgical radiation. 5-year survival for invasive thymoma is between 12-54% regardless of any myasthenia gravis symptoms (5,6).
Paget's disease of the breast can affect the nipple and areola. Symptoms typically only affect one breast. Symptoms may include:
- "Skin." The first symptom is usually an eczema-like rash. The skin of the nipple and areola may be red, itchy and inflamed. After a period of time, the skin may become flaky or scaly.
- "Discharge." A discharge, which may be straw-colored or bloody, may ooze from the area.
- "Sensation." Some women have a burning sensation. These symptoms usually occur in more advanced stages, when serious destruction of the skin often prompts the patient to consult. Lumps or masses in the breast occur in 50% of the patients. In more advanced stages, the disease may cause tingling, increased sensitivity and pain.
- Nipple changes. The nipple may turn inwards be inverted.
- Breast changes. There may or may not be a lump in the breast, and there may be redness, oozing and crusting, and a sore that does not heal.
The symptoms usually affect the nipple and then spread to the areola and then the breast. It is common for the symptoms to wax and wane.
Most women do not visit the doctor because they assume Paget's disease to be minor contact dermatitis or eczema. A lump or skin irritation that does not seem to heal for over a month indicates that attention by a specialist is needed.
Apocrine gland anal sac adenocarcinomas first appear as small lumps associated with one of the anal sacs (rarely bilateral), but they can grow to a large size. Smaller tumors are undetectable without a rectal examination, while larger tumors can cause pain and straining to defecate. Between 25 and 40 percent of dogs with these tumors will also develop hypercalcaemia through secretion of parathyroid hormone-related protein by the tumor. Symptoms of hypercalcaemia include increased drinking and urination, vomiting, loss of appetite, weight loss, and bradycardia (slow heart rate). Apocrine gland anal sac adenocarcinomas also have a tendency to metastasize to the regional lymph nodes, spleen, and eventually lungs and, less commonly, bones. The sublumbar (iliac) lymph nodes are the most common site of metastasis and can become larger than the original tumor.
The symptoms of laryngeal cancer depend on the size and location of the tumour. Symptoms may include the following:
- Hoarseness or other voice changes
- A lump in the neck
- A sore throat or feeling that something is stuck in the throat
- Persistent cough
- Stridor - a high-pitched wheezing sound indicative of a narrowed or obstructed airway
- Bad breath
- Earache (""referred"")
- Difficulty swallowing
Treatment effects can include post-operative changes in appearance, difficulty eating, or loss of voice that may require learning alternate methods of speaking.
Lymphohemangioma, or lymphohaemangioma, () is a disease characterized by swelling of the lymph nodes and blood vessels. It is variously described as a "mixture of clear fluid and blood-filled cysts", a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels. It is also sometimes described as a misnomer for combined lymphatic and capillary malformation.
Lymphohemangioma commonly appears in and around the eyes and mouth, but may also occur in other areas, including the pelvis, urethra, bladder, and in the limbs. Amputation of extremities may be necessary if the disease renders the extremity non-functional.
It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical Halstedian radical mastectomy, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment—and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as Stewart-Treves syndrome (which can be both lymphangiosarcoma and hemangiosarcoma following mastectomy). The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs. Schreiber "et al." proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.
Anal sac adenocarcinomas are often suspected due to location (palpable masse in anal sac) and behavior, but a biopsy and histopathology is necessary for a definitive diagnosis. Fine needle aspiration and cytology is a common first step. Cytopathology reveals clusters of cells with uniform round nuclei. These cells do not have many of the features usually associated with malignancy, such as a high nucleus to cytoplasm ratio or prominent nucleoli. Ultrasonography and radiography are performed to look for metastasis.
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.