The Lugano staging system is a lymphoma staging classification system resulting from recommend changes to the Cotswolds-modified Ann Arbor classification following meetings in 2011.
The goal was to simplify and standardize the response assessment enabling better understanding and communication ...
The Lugano classification is a lymphoma staging system resulting from recommended changes in 2011 to the Cotswolds modified Ann Arbor staging. This article outlines the lesion measurement guidelines for the system:
lymph nodes: the longest diameter in axial plane should be >1....
The Lugano classification is a lymphoma staging system resulting from recommended changes in 2011 to the Ann Arbor staging with Cotswolds modifications. This article outlines the classification's response to treatment based on CT.
Also included in the classification are staging and reponse to ...
The Lugano classification is a lymphoma staging system resulting from recommended changes in 2011 to the Cotswolds modified Ann Arbor staging. This article outlines the classification's response to treatment based on PET/CT.
Also included in the classification are staging and reponse to treatm...
Lymph node enlargement (rarely lymphadenomegaly) is often used synonymously with lymphadenopathy, which is not strictly correct.
Lymphadenopathy (or adenopathy) is, if anything, a broader term, referring to any pathology of lymph nodes, not necessarily resulting in increased size; ...
Lymphoma is a malignancy arising from lymphocytes or lymphoblasts. Lymphoma can be restricted to the lymphatic system or can arise as extranodal disease. This, along with variable aggressiveness results in a diverse imaging appearance.
Lymphoma accounts for ~4% of all cancers 4. T...
Lymphoma of the spinal cord is an uncommon manifestation of lymphoma. Although lymphoma more commonly involves the vertebral body (vertebral body tumors) or epidural compartment, intramedullary lymphoma may rarely occur.
Apparent intramedullary spinal cord lymphoma may often, in fact, represe...
Lymphoma of the uterine cervix is generally uncommon and when it does occur tends to present as cervical involvement with added background multi-organ disease rather than isolated primary cervical lymphoma 1. It is often considered part of the spectrum of uterine lymphoma.
In the ...
The Lymphoma Response to Immunomodulatory Therapy Criteria (LYRIC) represents an adaptation of the Lugano classification for the evaluation of lymphoma after immune-based treatment.
During immunomodulatory agents (e.g. checkpoint inhibitors) therapy, imaging findings suggestive of progressive d...
There are a number of lymphoma staging systems for both Hodgkin lymphoma and non-Hodgkin lymphoma including the Ann Arbor classification, Cotswolds-modified Ann Arbor classification, the Lugano classification and the most current LYRIC classification
Evolution of lymphoma staging and treatment ...
Lymphomatoid granulomatosis (LG), also known as angiocentric lymphoma or angiocentric immunoproliferative lesion, is a rare type of non-Hodgkin's lymphoma.
There is a recognised association with antecedent infection with the Epstein Barr virus (EBV).
It can affect a variety of org...
Lymphomatoid granulomatosis of the central nervous system is uncommon, but represents the second most common site of involvement in patients with systemic lymphomatoid granulomatosis, after the lungs, which are most commonly involved. It is considered one of the immunodeficiency-associated CNS l...
Pulmonary manifestations of lymphomatoid granulomatosis are important since the lung is one of the most frequent sites of involvement in lymphomatoid granulomatosis. It falls under the group of pulmonary angiitis and granulomatosis.
Some consider the condition to be midway between ov...
Mucosa-associated lymphoid tissue (MALT) lymphoma, also called extranodal marginal zone B-cell lymphoma, is a type of low-grade extranodal lymphoma.
MALT lymphoma represents ~7.5% of non-Hodgkin lymphomas. The average age of presentation is 60 years with a slight female predomina...
Mannose-binding lectin (MBL) deficiency is a condition that affects the immune system.
It may be prevalent in 10-30% of the general population.
Affected individuals have low levels of an immune protein named mannose-binding lectin in their blood. They are prone to recu...
Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma (NHL) and accounts for ~5% of all NHL. It is a malignant neoplasm of virgin B cells that closely resemble normal mantle zone B cells surrounding germinal centers.
They occur in older adults (mean age ~60 years). There is...
Marginal zone lymphomas are a group of low grade Non Hodgkin's lymphoma (NHL) that arise from the marginal zone of B cell germinal follicles in lymph nodes. There are three types of marginal zone lymphomas depending on the site of origin. These include: Mucosa-accosiated lymphoid tissue (MALT) s...
Mediastinal lymph node enlargement can occur from a wide range of pathologies. It may occur on its own or in association with other lung pathology.
Although mediastinal lymphadenopathy is used interchangeably - by some - with "mediastinal lymph node enlargement", they are not synon...
Monoclonal gammopathy of undetermined significance (MGUS) is the most common plasma cell disorder and refers to the presence of an abnormal monoclonal antibody in the blood but the absence of the overt bone marrow and clinical signs of multiple myeloma.
MGUS is the most common pla...
MR liver iron quantification is a non-invasive means of measuring liver iron concentration, a key indicator in the management of patients with hemochromatosis (primary or secondary).
Apart from being non-invasive, sampling occurs in a large cross-section of the liver, as opposed to ...
Multiple myeloma is the most common primary malignant bone neoplasm in adults. It arises from red marrow due to monoclonal proliferation of plasma cells and manifests in a wide range of radiographic abnormalities. Multiple myeloma remains incurable.
Four main patterns are recognise...
Extraosseous myeloma refers to any manifestation of multiple myeloma where there is plasma cell proliferation outside the skeletal system. This can potentially affect any organ system and the reported disease spectrum includes:
lymph nodes (considered to be most comm...
Muscle lymphoma is a rare manifestation of lymphoma.
Muscle lymphoma is rare, representing <2% of all lymphomas. The average age of presentation is 70 years 1.
Focal swelling and/or pain along with B-type symptoms 2. Any muscle can be involved but most comm...
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal haematologic stem cell disorders. It has sometimes been referred to as a preleukaemia or a preleukaemic condition.
Its overall incidence is thought to be around 3.3 per 100,000. The incidence in patients older tha...
Myelofibrosis is a haematologic disorder where there is the replacement of bone marrow with collagenous connective tissue and progressive fibrosis. It is also classified as a myeloproliferative disorder. It is characterized by:
Myeloproliferative disorders (MPDs) are a diverse group of conditions that are characterized by the overproduction of red cells, white cells and/or platelets in bone marrow. There are numerous conditions considered in this group but the most common are:
Necrobiotic xanthogranulomas are a rare form of cutaneous non-Langerhans cell histiocytosis.
The condition occurs mostly in older adults 2.
Patients demonstrate cutaneous papules that develop into large, firm plaques, papules and nodules, which are often ye...
Neurolymphomatosis is a rare condition characterized by the direct invasion of the cranial and peripheral nerves and roots by lymphoma, in the great majority B-cell non-Hodgkin lymphoma.
It should be differentiated from non-tumor conditions associated with lymphoma that also affect the peripher...
Nodular lymphoid hyperplasia (NLH) is a type of rare, benign, lymphoproliferative disease. It is most commonly reported affecting the gastrointestinal and respiratory systems.
The presence of gut/mucosa associated lymphoid tissue (GALT/MALT) can be seen in children and young adults as a normal ...
Non-Hodgkin lymphoma (NHL) is a catch-all term for lymphomas that are not of the Hodgkin subtype. It is a heterogeneous group of malignancies in terms of histology, clinical presentation, and prognosis.
See 2008 WHO classification for further information on subtypes.
There are several non lymphomatous lymphoid disorders that can affect the lung. This implies that they consist of lymphoid tissue but may not have progressed as far as an overt lymphoma. They include:
lymphocytic interstitial pneumonia
This article lists examples of normal imaging of the gastrointestinal tract and surrounding structures, divided by modality.
example 1: abdominal film
example 2: erect and supine
example 3, example 4: pediatric
example 5: young adult male
example 1, example...
This article lists examples of normal imaging of the liver and biliary tree and surrounding structures, divided by region and modality.
liver silhouette: example
example 1 with shear wave elastography
liver Doppler ultrasound: example ne...
In the pre-multidetector CT era, mesenteric lymph nodes were only really appreciated when enlarged. Following the advent of routine volume acquisition CT (and especially coronal reformats) they are commonly seen in normal individuals, which has raised the obvious question of what counts as mesen...
Pancreatic lymphoma is most commonly a B-cell sub-type of non-Hodgkin lymphoma.
Pancreatic lymphoma is typically seen in middle-aged patients with a mean age of around 55 years old and is more common in immunocompromised patients.
Symptoms are often non-spe...
Paraneoplastic syndromes occur secondary to the indirect effects of a malignancy and occur remotely to the primary malignancy. Symptoms are mediated by cytokines, hormones or immune cross-reactivity. These syndromes can cause a diverse range of symptoms and can affect multiple systems.
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder whereby some of the red blood cells produced are defective and are susceptible to premature destruction by the immune system, leading to haemoglobinuria.
The term comes from a mistaken 19th c...
When planning an interventional procedure a patient’s coagulation status must be assessed and optimised to best balance the risk of bleeding and thrombosis. The following must be considered;
bleeding risks associated with the procedure
medications the patient is taking that alter coagulation o...
Perisplenitis is acute inflammation of the splenic capsule and its peritoneal covering.
It is seen uncommonly but there is no data on its actual incidence.
It is seen usually in young and middle-aged patients, with acute left hypochondrial or lower chest pa...
Pernicious anemia (PA) is the commonest cause of vitamin B12 deficiency (or hypocobalaminaemia) in the world, due to an autoantibody against intrinsic factor, the cofactor required for absorption of this vitamin.
Pernicious anemia is the commonest cause of hypovitaminosis B12 glob...
PET-CT is a combination of cross-sectional anatomic information provided by CT and the metabolic information provided by positron emission tomography (PET).
PET is most commonly performed with 2-[F-18]fluoro-2-deoxy-D-glucose (FDG). Fluorine-18 (F-18) is an unstable radioisotope and has a half-...
Pituitary lymphoma is very rare, although lymphomatous (or leukemic) infiltration of the perisellar dura is not infrequently encountered as part of more widespread CNS disease. This article concerns itself with involvement of the pituitary itself rather than the region more broadly. For a genera...
Plasmacytomas are discrete, solitary tumors of neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary). It is a rare tumor that is associated with latent systemic disease in the majority of affected patients. In contradistinction to multiple myeloma, there is minimal or...
Pleural involvement with lymphoma can occur in two situations:
primary pleural lymphoma
primary effusion lymphoma
secondary involvement of the pleura with lymphoma
Pneumocystis pneumonia (PCP), also referred to as Pneumocystis jirovecii pneumonia (PJP), is an atypical pulmonary infection and the most common opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS).
Classically, PCP was the acronym for Pneumocystis car...
POEMS syndrome is the acronymic name for a rare multisystem paraneoplastic disorder comprising of a minimum of three of the following features in the setting of a plasma cell dyscrasia:
hepatomegaly, splenomegaly or lymphadenopathy
Polycythemia vera (older term: polycythemia rubra vera) is a myeloproliferative disorder that results in an excess of red blood cells in the bloodstream.
The estimated prevalence is around 2-3 per 10,000 people. It typically presents in older individuals. There may be a slightly g...
Polyglandular autoimmune syndromes (PAS) are a rare set of diseases characterized by the presence of ≥2 autoimmune endocrine disease.
Three types of PAS have been described.
PAS type I
a.k.a. APECED (autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy) or MEDAC (mu...
Primary bone (skeletal/osseous) lymphoma (PBL) is a less common manifestation of lymphoma than secondary involvement from disseminated lymphoma. It is rare, accounting for <5% of bone tumors and <1% of non-Hodgkin lymphoma.
PBL is defined as the presence of lymphoma isolated to one...
Primary effusion lymphoma is a rare form of non-Hodgkin lymphoma (diffuse large cell B cell lymphoma) characterized by malignant fluid accumulation in the absence of lymphadenopathy.
Typical sites of accumulation include
Primary hepatic lymphoma (PHL) is rare accounting for roughly 100 described cases. If it is being considered as a diagnosis, distal lymphadenopathy, splenomegaly, bone marrow disease, and leukemia should not be present for at least 6 months after the liver tumor is detected (see: secondary hepat...
Primary immunodeficiency states are heterogenous group of disorders that occur when there is an impairment of humoral or cell-mediated immunity in the absence of any recognised precipitating cause such as drug therapy or infective agent such as human immunodeficiency virus (HIV).
Primary mediastinal large B-cell lymphoma is a distinct entity, recognised in the WHO classification of lymphoma.
Primary mediastinal large B-cell lymphoma accounts approximately 5% of large B-cell lymphoma, which is usually disseminated or found in the abdomen. There appears to b...
Primary ovarian lymphoma (POL) refers to involvement of the ovary with lymphoma but without the involvement of any other site. It is an extremely rare yet well-recognised condition.
POL accounts for ~1.5% of ovarian tumors 5.
The rarity of this condition is probably co...
Primary pleural lymphoma is extremely rare, especially in immunocompetent patients.
Primary pleural lymphoma accounts for <0.5% of all non-Hodgkin lymphoma 2 and ~2.5% of primary chest wall tumors 4.
Primary pleural lymphoma may be Hodgkin or non-Hodgkin lymphoma with...
Primary pulmonary lymphomas refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is much rarer than secondary pulmonary lymphoma and is most frequently represented by lymphoma of B-cell lineage - often marginal zone B-cell lymphoma of m...
Protein C deficiency is a hypercoagulable state due to reduced activity or an absolute deficiency of protein C, an anticoagulant protein. Protein C deficiency increases the risk of venous thrombosis.
The prevalence of protein C deficiency in the general population is around 1 per ...
Protein S deficiency is a hyperocoagulable state associated with increased risk of venous thrombosis (up to 5% of patients with deep venous thrombosis may carry this deficiency).
Protein S deficiency may be expected in ≈1 of every 500 people 3.
Protein S, a vitamin K-d...
Pulmonary artery stump thrombosis describes thrombus formation occurring at the site of the pulmonary arterial stump, a complication following lobectomy or pneumonectomy. It is usually discovered as an incidental finding on routine follow-up chest CT scans.
The prevalence of pulmo...
Pulmonary leukostasis is a medical emergency that is most commonly seen as a complication of chronic myeloid leukemia (CML) in blast crisis, and acute myeloid leukemia when white blood cell (WBC) counts are over 100 x 109/L (100,000/microL). It needs to be considered in any patient with myelogen...
Pulmonary lymphoma refers to lung parenchymal involvement with lymphoma.
It can be broadly divided as primary or secondary:
primary pulmonary lymphoma: (rare) usually non-Hodgkin lymphoma which is limited to the lung with or without mediastinal lymph node involvement and with no evi...
The Rai staging system is one of the two staging systems currently adopted in assessment of chronic lymphocytic leukemia (CLL).
It comprises of stages 0 to IV and classifies chronic lymphocytic leukemia into low, intermediate and high-risk categories, which correspond with stages 0, I & II, and...
A number of sonographic features are helpful in distinguishing reactive versus malignant lymph nodes.
Grey scale features
Features that favour reactive/infective nodes over malignancy include:
surrounding soft tissue edema
Doppler examination is particularly ...
Reed-Sternberg cells are a classical finding diagnostic of Hodgkin lymphoma. They are giant, multinucleated cells with abundant pale cytoplasm. Reed-Sternberg cells are rare, making up <1% of lymphoid tissue, with the background consisting of lymphocytes, plasma cells, eosinophils and macrophages.
Renal lymphoma is usually a part component of multi-systemic lymphoma - primary renal lymphoma is unusual.
Typical imaging findings are multiple bilateral hypodense or infiltrative renal masses.
While renal lymphoma has an autopsy incidence of ~45% (range 30-60%) in lymphoma pati...
The reticuloendothelial system (RES) comprises a number of tissues:
liver Kupffer cells
Retrorenal spleen refers to variant position of the spleen, when its inferior portion lies posterior to the upper pole of the left kidney.
This normal variant occurs in ~20% of patients, with the finding more common in patients who lie supine 1.
Recognition of this variant is important to avoi...
Richter transformation is defined as development of high-grade non-Hodgkin lymphoma (NHL) in patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma.
It has been expanded to include other lymphoid malignancies that develop in CLL patients, including Hodgkin disease, proly...
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign idiopathic proliferative disease that involves phagocytic histiocytes.
The disease predominantly occurs in young adults with a mean age at presentation of 21 years. There may be...
Rubinstein-Taybi syndrome (RTS) is a very rare genetic multi-system disorder primarily characterized by mental retardation, broad and often angulated thumbs and halluces, and distinctive facial features.
The estimated incidence is 1 in 100,000-125,000 live births 5.
Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a recently recognised, rare, non-neoplastic vascular splenic lesion of uncertain etiology.
The term SANT first appeared in the literature in a 2004 paper by Martel et al. which examined a series of 25 cases 3. T...
Secondary hepatic involvement with lymphoma (secondary hepatic lymphoma) is common, much more so than primary hepatic lymphoma.
Hepatomegaly with deranged liver function tests is the most common presentation. Jaundice is common. Rarely, patients may present with acute li...
Secondary involvement of the bone with lymphoma, also referred as secondary bone lymphoma, is much more common than primary bone lymphoma, occurring in ~15% of disseminated lymphomas.
Secondary bone lymphoma is defined as lymphoma involving the bone with nodal disease occurring wit...
Secondary involvement of the pleura with lymphoma (secondary pleural lymphoma) is very common, occurring in ~20% of lymphomas. It may be a result of an extension of lymphoma into the visceral or parietal pleura or be a complicating pleural effusion and is a poor prognostic factor.
Secondary organising pneumonia (SOP) refers to organising pneumonia that can be attributed to a specific cause, in contrast to cryptogenic organising pneumonia (COP).
SOP can be attributed to the following causes 1:
atypical pneumonias (e.g. Legi...
Secondary pulmonary lymphomas refer to pulmonary involvement with lymphoma when the condition is not limited to the lung and has mediastinal lymph node involvement or evidence of extrathoracic dissemination for at least three months after the initial diagnosis. This is a more common form of pulm...
Sézary syndrome (SS) is a type of cutaneous T-cell lymphoma. It is sometimes considered an advanced and leukemic form of cutaneous T-cell lymphoma (CTCL).
It is clinically characterized by an extensive erythematous rash covering most of the body as well as the presence of...
Sickle cell disease (SCD) is a hereditary (autosomal recessive) condition resulting in the formation of abnormal hemoglobin (a hemoglobinopathy), which manifests as multisystem ischemia and infarction, as well as hemolytic anemia.
There is no recognised gender predilection. The h...
Abdominal manifestations of sickle cell disease (SCD) are wide and can involve many organs.
For a general discussion, please refer to sickle cell disease.
may occur transiently with the sequestration syndrome, where rapid pooling of blood occurs in the spleen, resulting ...
Acute chest syndrome (ACS) in sickle cell disease is a leading thoracic complication - as well as leading cause of mortality - in those affected by sickle cell disease. The diagnosis is made on the combination of new pulmonary opacity on chest x-ray with at least one new clinical symptom or sign...
Sideroblastic anemia (also known as sideroachrestic anemia) is a rare cause of anemia characterized by the presence of ring sideroblasts rather than normal red blood cells in the bloodstream. Sideroblastic anemia is either congenital or acquired.
Symptoms and signs reflec...
Small bowel lymphoma is the most common small bowel malignancy, accounting accounts for ~25% of all primary small bowel malignancies and ~40% of all primary gastrointestinal lymphomas.
Small bowel lymphoma is most commonly secondary extranodal involvement in widespread systemic ly...
Small lymphocytic lymphoma (SLL) is a non-Hodgkin lymphoma affecting the B-lymphocytes of the immune system. It is considered the same disease process as chronic lymphocytic leukemia (CLL) although unlike CLL, the tumor cells are mostly confined to the lymph nodes rather than the blood stream. ...
Smouldering multiple myeloma (SMM) is a precursor to multiple myeloma.
Similar to MGUS patients, smoulder multiple myeloma patients are asymptomatic.
The criteria for smouldering multiple myeloma diagnosis according to the International Myeloma Worki...
Solitary plasmacytoma with minimal bone marrow involvement refers to a type of plasmacytoma with bone marrow cytology positive for monoclonal plasma cell infiltration.
In order to fulfill the International Myeloma Working Group criteria for the diagnosis of solitary plasmacytoma with ...
The spleen is an organ of the haematologic system and has a role in immune response, storage of red blood cells and hematopoiesis.
The spleen is a wedge-shaped organ lying mainly in the left upper quadrant (left hypochondrium and partly in the epigastrium) and is protected by the...
Splenic artery aneuryms are the commonest visceral arterial aneurysm formation as well as the 3rd commonest abdominal aneurysm (after the aorta and iliac vessels). Aneurysms are usually saccular in configuration and they can either be in the form of a true aneurysm (much more common) or as a pse...
Splenic epithelial cysts, also referred as splenic epidermoid cysts or primary splenic cysts, are unilocular fluid lesions with thin and smooth walls and no enhancement. They represent ~20% of cysts found in the spleen, and are usually an innocuous incidental imaging finding.
Note that most (~8...
Splenic (artery) embolization is an endovascular technique for treatment of splenic and splenic artery pathology as an alternative to splenic artery ligation or splenectomy. It often results in successfully treating the underlying pathology, while maintaining at least partial splenic function.
Splenic granulomatous disease refers to sequelae arising from granulomatous infection-inflammation of the spleen.
They are a result of:
In latent s...
Splenic hamartomas are very rare lesions commonly found incidentally on imaging. They are most often solitary but may be present as multiple nodules in patients with tuberous sclerosis or Wiskott-Aldrich syndrome.
The recently-described sclerosing angiomatoid nodular transformatio...
Splenic lymphoma, also termed as lymphomatous involvement of the spleen, represents the most common malignancy to involve the spleen. They are commonly secondary, rarely being primary (referred as primary splenic lymphoma).
This article focuses on the location-specific primary and secondary l...
Splenic metastases are relatively rare on imaging, although they are more commonly found on autopsy. Typically they are part of a widespread metastatic disease.
The rate of splenic metastases varies between 1-10% of autopsy studies, depending on whether microscopic or macroscopic ...