Malt lymphoma is a lowgrade marginal zone bcell lymphoma that is most commonly found in the stomach. International journal of radiation oncologybiologyphysics. Extranodal nonhodgkins lymphoma enhl approximately account for one third of non hodgkins lymphomas and arise primarily from sites other than lymph nodes. Extranodal lymphomas enls comprise about a third of all nonhodgkin lymphomas nhl. The most common extranodal sites of anaplastic large cell lymphoma are skin, subcutaneous tissue, bone, lung, and gastrointestinal organs. Prior to treatment baseline muga scan for doxorubicin baseline pfts for bleomycin progressive disease evaluation ppd progression of single site defines progression. Immune or lymphoid cells in the stomach, lung, in structures around the eyes, in the thyroid gland, salivary glands, and small. Sep 23, 2019 extranodal lymphoma can also arise in the lymphoid tissue of the spleen, bone marrow, thymus, tonsils, and adenoidssmall patches of tonsillike tissue hidden up where the nasal passages meet the throat.
Lymphomas that potentially fall into the hgbl categories can morphologically resemble blymphoblastic leukemia lymphoma blbl, bl, and dlbcl as well as lymphomas that are intermediate between dlbcl and bl dlbclbl. Extranodal natural killer tcell lymphoma, nasal type. Multiple lymphomas may rarely occur simultaneously and cause diagnostic problems in hematology practice. Radiation therapy rt is frequently used as either primary therapy particularly for indolent enl, consolidation after systemic therapy, salvage treatment, or palliation. Typically, it presents with nasal obstruction, sinusitis, ulcer, and epistaxis. The national comprehensive cancer network recommends cancer patient participation in clinical trials as the gold standard for treatment. The aim of this study was to retrospectively investigate the site of occurrence of lymphomas in the head and neck area and to analyze the typical symptoms of patients who presented at an oral and maxillofacial surgical department. Extranodal lymphoma can arise in any organ, and can multifocally affect 2 or more organs. The term extranodal disease refers to lymphomatous infiltration of anatomic sites other than the lymph nodes. Lymphoma is the 2nd most common neoplasm of the head and neck, and up to 30% of the time it can present as an extranodal mass. Jun 07, 2018 extranodal natural killertcell lymphoma, nasal type enkl is a wellcharacterized subtype of mature t and natural killer nk cell lymphoma in the world health organization classification. Extranodal nktcell lymphoma, nasal type, is a predominantly extranodal lymphoma of nkcell or tcell lineage, characterized by vascular damage and destruction, prominent necrosis, cytotoxic phenotype, and association with ebv. Malt lymphoma maltoma is a form of lymphoma involving the mucosaassociated lymphoid tissue malt, frequently of the stomach, but virtually any mucosal site can be afflicted.
Often the primary extranodal presentation requires sitespecific strategies either for diagnosis or therapy. Here, we present an uncommon and rarely reported disease pattern. In 60 out of 71 cases of gi lymphoma and 38 of 49 cases of head and neck lymphoma upper ci tract endoscopy was performed at presentation. The two major types of lymphoma are hodgkin lymphoma hl and nonhodgkin lymphoma nhl. This lymphoma develops at extranodal sites outside the lymph nodes, also known as glands most often. Follicular lymphoma is a cancer that develops in the white blood cells, lymphatic system and bone marrow. Winner of the standing ovation award for best powerpoint templates from presentations magazine. Synchronous presentation of two extranodal lymphomas. Spectrum of ct findings in thoracic extranodal nonhodgkin lymphoma1 nonhodgkin lymphoma nhl frequently manifests in extranodal structures in the chest, often in the form of secondary involvement but occasionally as primary disease. Clinical characteristics of 26 patients with primary extranodal. Illustrate the characteristic imaging features of extranodal lymphoma of the head and neck enl hn with a variety of case examples based on anatomic pattern 2.
The most frequent primarysites are the stomach 50 60% and small bowel 20 30%. Pdf onset of oral extranodal large bcell nonhodgkins. Bcell lymphoma of the oral cavity are a painless swelling, a nonhealing ulcer, fever, sweats and weight loss. Differentiation of extranodal nonhodgkins lymphoma from. Advances in the treatment of extranodal nktcell lymphoma. An 89yearold japanese man visited our hospital with a threemonth history. This disease was called hodgkins disease for about 170 years. Extranodal nkt cell lymphoma, nasal type, is more common in asia, central america and south america than it is in north america. Extranodal involvement in lymphoma a pictorial essay and. The lymphoid system consists of circulating t and b lymphocytes and the lymphoid organs, including lymph nodes, thymus, spleen, tonsils, and adenoids. In lymphoma, malignant lymphocytes cancer cells grow uncontrollably.
Anaplastic large cell lymphoma is a rare type of nonhodgkin lymphoma. The patient was a 48yearold hivnegative male who presented with five months history of recurrent genera lized tonicclonic seizures, weakness and numbness of. Extranodal lymphomas associated with hepatitis c virus infection. This balance ensures that the body functions properly. Review differential diagnosis educational objectives introduction contact lymphoma is the 2nd most common neoplasm of the head and neck, and up to 30% of the time it can present as an extranodal. Marginal zone bcell lymphomas account for approximately 8% of nonhodgkin lymphoma nhl and rank as the third most frequent histologic subtype, after diffuse large bcell lymphoma and follicular lymphoma 1. Lymphomas encompass a group of lymphoproliferative malignant diseases that originate from t and bcells in the lymphatic system. Primary gastric lymphomas primary gastric lymphoma account for 7% to 20% of all lymphoma, and the gastrointestinal tract is the most common extranodal site of lymphoma. Occurrence and prognosis of extranodal lymphomas freeman. Worlds best powerpoint templates crystalgraphics offers more powerpoint templates than anyone else in the world, with over 4 million to choose from.
A previous pioneer study from north india has shown the incidence of extranodal lymphoma to be 44%. The information in this document does not replace a medical consultation. Extranodal nk tcell lymphoma enktl, nasal type is a rare and aggressive malignancy most commonly seen in south east asia. Biological characterization of nodal versus extranodal presentation of diffuse large bcell lymphoma using immunohistochemistry min kyoung kim,1 sung hwa bae,2 young kyung bae,3 yoonseup kum,4 hun mo ryoo,2 hee soon cho,5 kyung hee lee,1 sung ae koh,1 ha young lee,1 so yeon yun,2 jun hyeok choi,2 myung soo hyun1 abstract. It has been reported that approximately 20% of all small bowel malignancies are primary malignant. Biological characterization of nodal versus extranodal. It is a cancer originating from b cells in the marginal zone of the malt, and is also called extranodal marginal zone b cell lymphoma.
The aim of this study is to evaluate the role of petct in identification of different patterns of extranodal involvement in hodgkins disease hd and nonhodgkins lymphoma nhl and to enlist the common sites of extranodal involvement in each histological type and compare our results with the existing literature. Primary extranodal bcell lymphoma chinese clinical oncology. Mar 30, 2018 extranodal fl, although rare, may occur in any area such as the skin, gastrointestinal tract, breast, and testis. It is for personal use only and cannot be modified. Nonhodgkin lymphoma nhl arises primarily in sites other than lymph nodes, including extranodal tissues. Pmnhls are defined as involvement of mediastinal lymph nodes, thymus, andor mediastinal organs heart, lung, pleura, pericardium by nhl without evidence of systemic disease at presentation. Both b and tnkcell lymphomas can occur in extranodal sites, in which bcell lymphoms are more common. Infiltration of malignant lymphomatous cells in the organs other than lymph node is termed as extranodal lymphoma.
A destructive mass most often forms inside the nose or in the sinuses air. Extranodal marginal zone bcell lymphoma maltlymphoma. Cancer therapy selection, dosing, administration, and the management of related adverse events can be a complex process that should. Apr 24, 2017 extranodal nasal nkt cell lymphoma nktcl is a rare type of cancer. Extranodal nkt cell lymphoma, nasal type, is a rare, fastgrowing aggressive type of nonhodgkin lymphoma nhl. Primary mediastinal nodal and extranodal nonhodgkin. At most of extranodal sites, dlbclnos is the most frequent lymphoma subtype. This issue of radiologic clinics of north america focuses on extranodal lymphoma from head to toe, and is edited by dr.
Extranodal mzl of mucosa associated lymphoid tissue malt type including gastric and nongastric malt lymphomas 2. Hodgkin described several cases of people with symptoms of a cancer involving the lymph nodes. In the united states in 20, about 9,290 persons were expected to be diagnosed with hodgkin lymphoma. Various factors have been attributed to this changing trend of lymphoma namely hiv, increasing use of immunosuppressive therapy and indolent viral infection. Extranodal disease is prognostically important in any lymphoma. Approximately onethird of nonhodgkin lymphomas nhls arise primarily from sites other than. Extranodal natural killer tcell lymphoma, nasaltype. Nhl is the name for many different types of cancer that. Several marginal zone lymphoma subtypes are recognized in the world health organization classification of lymphoid neoplasms.
Anaplastic lymphoma kinasenegative anaplastic large cell. Many arise or present in nonlymphoid sites such as the skin and gastrointestinal tract. A few people have more widespread lymphoma stage 3 or 4 when they are diagnosed. The imaging modalities and findings that best allow differentiation of lymphoma from other differential diagnoses will also be discussed. The term extranodal is used because this form of lymphoma is found outside of the traditional lymph node groupings. Ko, soo mee bang, jae hoon lee, dae ho lee, jooryung huh, sung yong oh, hyukchan kwon, hyo. Consortium for improving survival of lymphoma study. Follicular lymphoma is a welldefined subtype of nonhodgkin lymphoma nhl with cells of the lymphoid tissues in the lymphatic system multiplying uncontrollably to eventually cause tumours to grow. Extranodal marginal zone lymphoma of the parotid gland article pdf available in journal of maxillofacial and oral surgery 15suppl 2 march 2016 with 24 reads how we measure reads. Extranodal marginal zone lymphoma of the central nervous system cns emzbl is a rare disease.
Greater manchester cancer pathway lymphoma guidelines. Hematology a lymphoproliferation that arises in nonlymphoid tissues, which are often histologically diffuse and more aggressive than nodal lymphomas sites stomach, tonsils, skin, small intestine, salivary glands, breast. Extranodal involvement is seen not only in widespread nodal disease but also. However, several issues remain controversial such as the definition itself of primary extranodal lymphoma, and the most appropriate staging system to characterize the disease extent. Oral lymphoma often is a component of a disseminated disease process that may involve regional nodes as well. Peripheral tcell lymphoma ptcl is a form of lymphoma accounting for around 10% of lymphoma cases and develop from maturestage tcells and natural killer nk cells. It mainly affects men around 50 years of age, and usually arises in the nose, paranasal sinuses paranasal sinuses are cavities spaces or small tunnels. Lymphoma occur most commonly 80% in the nose and upper aerodigestive tract, less commonly 20% in nonnasal. What is extranodal nkt cell lymphoma and who gets it.
Primary extranodal nonhodgkins lymphoma mimicking a painful. Both the lymphoma subtype and the organ involved may influence the clinical behaviour and management of the lymphoma. Nonhodgkin lymphomas are malignant neoplastic proliferations of the immune system that can manifest as nodal or extranodal lymphomas. Primary pulmonary lymphoma is one of the rare ones, comprising only 4% of all extranodal nhls 11. Extranodal nkt cell lymphoma, nasal type enktclnt also termed angiocentric lymphoma, nasaltype nk lymphoma, nktcell lymphoma, polymorphicmalignant midline reticulosis, and lethal midline granuloma is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, andor pharynx at these sites, the disease often takes the form of massive, necrotic, and. Around 30% of all nonhodgkin lymphoma nhl cases arise from extranodal sites. Clinical characteristics of primary extranodal versus. Fs4 mantle cell lymphoma facts i page 1 revised november 2014 introduction lymphoma is the general name for many related subtypes of cancer that arise from a type of white blood cell called a lymphocyte. Mucous membranes line many organs, and they contain lymphoid tissue, which is part of the lymphatic system. However, specific sites of extranodal lymphoma involvement. Technically, extranodal marginal zone bcell lymphoma of mucosaassociated lymphoid tissue malt lymphoma.
Extranodal nkt cell lymphoma, nasal type, is an aggressive form of nonhodgkins lymphoma comprising specific clinicopathological characteristics. Extranodal marginal zone bcell lymphomas are a discrete clinicopathological entity arising in mucosaassociated lymphoid tissue malt. The nonhodgkin lymphoma subtype of marginal zone lymphoma represents a group of lymphomas that have been historically classified together because they appear to arise from postgerminal center marginal zone b cells and share a similar immunophenotype. What is follicular lymphoma european society for medical.
Because staging and treatment are affected by the presence of extranodal disease at imaging, radi. Hodgkin lymphoma hodgkin lymphoma hl, one of the most curable forms of cancer, was named for thomas hodgkin, a british pathologist. The involvement of the skeletal muscle has been described rarely in extranodal anaplastic large cell lymphoma. The nonhodgkin lymphoma subtype of marginal zone lymphoma represents a group of lymphomas that have been historically classified together because they appear to arise from postgerminal center marginal zone b cells.
Genetic risk of extranodal natural killer tcell lymphoma. Ppt lymphoma powerpoint presentation free to view id. Pdf extranodal marginal zone lymphoma of the parotid gland. Extranodal nktcell lymphoma, nasal type canadian cancer. Extranodal lymphoma from head to toe, an issue of radiologic clinics of north america, 1e the clinics. Onset of oral extranodal large bcell nonhodgkins lymphoma in a patient with polycythemia vera. A painless lymph node enlargement or a submucosal lesion in the junction between hard and soft palate are highly suspicious.
The deauville scale and lugano criteria are used for assessment, staging and response of hodgkin lymphoma hl and nonhodgkin lymphoma nhl. Clinical characteristics of 26 patients with primary extranodal hodgkin lymphoma. Pgl is the most common lymphatic tumor of the gi tract. All nodes lead to lymphoma a multimodality refresher of. The modified international prognostic index can predict the outcome of localized primary intestinal lymphoma of both extranodal marginal zone bcell and diffuse large bcell histologies international extranodal lymphoma study group ielsg british journal of haematology, 2002, 118, 218228 pdf. The addition of chemotherapy for advanced stages does not appear to improve survival compared radiotherapy alone, which remains the treatment of choice especially for localized stages. July 2019 chapter 16c page 3 orp manual, volume i version 5. According to the international agency for research on cancer, in 2015, approximately 414,772 new cases of nonhodgkin lymphoma nhl were diagnosed. Traditionally, lymphomas have been subcategorized into two groups. Extranodal nasal nkt cell lymphoma nktcl is a rare type of cancer. Field and dose guidelines from the international lymphoma radiation oncology group. Mantle cell lymphoma, lymphoblastic lymphoma, burkitts lymphoma, and mucosaassociated lymphoid tissue malt lymphoma, however, are more likely to affect extranodal sites 2.
Dec 12, 2019 in this case, the globular inclusions are confined to the cytoplasm of plasma cells, a finding usually related to defective secretion of the immunoglobulin product and most often seen in lymphoplasmacytic neoplasms. Response to chemotherapy and survival in 110 cats article pdf available in journal of small animal practice 5011. Theyll give your presentations a professional, memorable appearance the kind of sophisticated look that todays audiences expect. Constipated plasma cells in extranodal marginal zone lymphoma. Primary mediastinal nonhodgkin lymphomas pmnhls represent 5% of all nhls and comprise lymphomas of bcell and tcell origin. Marginal zone lymphomas are a heterogeneous subtype of indolent bnonhodgkin lymphoma that includes three distinct diseases.
It can start in t cells, but it develops most often in natural killer nk cells. Extranodal marginal zone lymphoma of the central nervous. The unusual morphologic appearance is a clue for the diagnosis of extranodal marginal zone lymphoma. Lymphoma cells can travel to any part of the body and be found in lymph nodes, the bone marrow, the spleen, blood, bone, skin and almost any organ or tissue. Extranodal lymphoma definition of extranodal lymphoma by. Yamaguchi m, tobinai k, oguchi m, ishizuka n, kobayashi y, isobe y, et al. Extranodal definition of extranodal by medical dictionary. Lymphoma is a general name for a group of cancers that affect the lymphatic system. Bhs guidelines for the treatment of marginal zone lymphomas. Me dian follow up time was 38 months range 2167 months. To illustrate some rarer case examples encoutered within our practice of extranodal lymphoma background the lymphoproliferative disorders can be divided into two hodgkin disease and nonhodkins lymphoma nhl, the former being distinguished from other subtypes by the.
Extranodal nktcell lymphoma, nasal type, is an aggressive form of nonhodgkins lymphoma comprising specific clinicopathological characteristics. We conclude that extranodal nhl is common among patients of arabic descent. A prognostic model from a retrospective multicenter study jeeyun lee, cheolwon suh, yeon hee park, young h. Principles of the realwho classification morphology may be sufficient for diagnosis in many cases immunophenotype and genetics often helpful in differential diagnosis have played a major role in defining disease entities clinical features play an important part in disease definition nodal and extranodal neoplasms are not equivalent. Emzls are a form of mzl in which malignant marginal zone bcells initially infiltrate malt tissues of the stomach 5070% of all emzl or, less frequently, the esophagus, small intestine, large intestine, rectum, conjunctiva of the eye, nasal passages, pharynx, lung bronchi, vulva, vagina, skin, breast, thymus gland, meninges i. The wide range of presentations of enl, involving any organ in the body and the. The lymphoma subtype, the b cell or t cell type, and the primary organ or tissue of origin can all be important prognostic factors.
The definition of primary extranodal lymphoma, particularly in the presence of both nodal and extra nodal disease, remains a controversial issue. Spectrum of ct findings in thoracic extranodal nonhodgkin. Extranodal fl, although rare, may occur in any area such as the skin, gastrointestinal tract, breast, and testis. The disease affects men and women equally, and any age group can be affected. Diffuse large b cell lymphoma is the most common histologic subtype. Almost any organ can be affected by lymphoma, with the most common extranodal sites of involvement being the stomach, spleen, waldeyer ring, central nervous system, lung, bone, and skin. The lymphatic system contains lymphocytes white blood cells that fight infections and cancer. The diagnosis of lymphoma is usually suggested by widespread lymphadenopathy. Extranodal natural killer nktcell lymphoma is an aggressive malignancy of putative nkcell origin, with a minority deriving from the tcell lineage. Fs25 peripheral tcell lymphoma facts i page 1 revised july 2014 introduction peripheral tcell lymphomas ptcls are uncommon and aggressive types of nonhodgkin lymphoma nhl that develop in mature white blood cells called t cells and natural killer nk cells. No longer indicated for the routine staging of hl and most dlbcl. Jing ma, yafei wang, haifeng zhao, su liu, qian li, li lin.
Splenic mzl with or without villous lymphocytes mzl including malt represent 11% of all nonhodgkins lymphomas. Extranodal nkt cell lymphoma is a rare type of nonhodgkin lymphoma nhl that develops from natural killer nk cells or t lymphocytes t cells. Bcell lymphomas account for 92 percent of all nhl cases in the united states. Extranodal nasal nkt cell lymphoma genetic and rare. Pathologically, the malignancy occurs in two forms, extranodal nktcell lymphoma, nasal type. Fs5 cutaneous tcell lymphoma facts i page 2 uaneus e pa facts normally, there is a balance in the body by which new cells replace old ones, and each cell carries out its specific tasks. Differentiation of extranodal nonhodgkins lymphoma from squamous cell carcinoma of the maxillary sinus. Fdgpet scanning for detection and staging of extranodal marginal zone lymphomas of the malt type. Fdg petct of extranodal involvement in nonhodgkin lymphoma.
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