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Photomicrograph showing the typical starry-sky appearance of a case of Burkitt lymphoma (H and E, ×400)
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Primary non-Hodgkin's lymphoma (NHL) of the gastrointestinal (GI) tract is the most common extranodal lymphoma in pediatric age group. Yet, the overall incidence is very low. The rarity of the disease as well as variable clinical presentation prevents early detection when the possibility of cure exists.
We studied six cases of primary GI NHL in ped...
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Burkitt's lymphoma is an undifferentiated non-Hodgkin's B-cell lymphoma. Three clinical subtypes are recognized: African (endemic), American and other areas (sporadic) and human immunodeficiency virus (HIV) associated. Sporadic Burkitt's lymphoma is a rare malignancy among Asian population. This report describes a case of sporadic Burkitt's lymphom...
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Non-Hodgkin's lymphoma (NHL) constitutes a diverse group of lymphoid neoplasm that differs in manner of presentation, response to therapy and prognosis. They are less predictable and have a greater prediction to disseminate to extranodal sites. Their incidence has been increased over the last four decades and, head and neck is the second most commo...
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Citations
... Furthermore, as our systematic review and meta-analysis included studies with > 10 participants, we may have not captured rarer relevant and important clinical presentations. These include gastrointestinal presentations such as intussusception [70] and bowel obstruction [71], upper respiratory tract symptoms such as asymmetrical tonsillar enlargement [72], sleep apnoea [73], wheeze and stridor [74], as well as rheumatic symptoms such as arthritis [75]. There were several limitations to this study. ...
Background
The diagnosis of B-cell lymphoma, one of the commonest cancers seen in childhood and adolescence, is challenging. There is a crucial need to identify and delineate the prevalence of associated symptoms in order to improve early diagnosis.
Aims
To identify clinical presentations associated with childhood and adolescent B-cell lymphomas and estimate symptom prevalence.
Methods
A systematic review of observational studies and meta-analysis of proportions was carried out. Medline and EMBASE were systematically searched, with no language restrictions, from inception to 1st August 2022. Observational studies with at least 10 participants, exploring clinical presentations of any childhood and adolescent lymphoma, were selected. Proportions from each study were inputted to determine the weighted average (pooled) proportion, through random-effects meta-analysis.
Results
Studies reported on symptoms, signs and presentation sites at diagnosis of 12,207 children and adolescents up to the age of 20. Hodgkin’s lymphoma most frequently presented with adenopathy in the head-and-neck region (79% [95% CI 58%-91%]), whilst non-Hodgkin’s lymphoma presented abdominally (55% [95% CI 43%-68%]). Symptoms associated with lymphoma included cervical lymphadenopathy (48% [95% CI 20%-77%]), peripheral lymphadenopathy (51% [95% CI 37%-66%]), B-symptoms (40% [95% CI 34%-44%]), fever (43% [95% CI 34%-54%]), abdominal mass (46% [95% CI 29%-64%]), weight loss (53% [95% CI 39%-66%]), head-and-neck mass (21% [95% CI 6%-47%]), organomegaly (29% [95% CI 23%-37%]), night sweats (19% [95% CI 10%-32%]), abdominal pain (28% [95% CI 15%-47%]), bone pain (17% [95% CI 10%-28%]) and abnormal neurology (11% [95% CI 3%-28%]).
Conclusion
This systematic review and meta-analysis of proportions provides insight into the heterogeneous clinical presentations of B-cell lymphoma in childhood and adolescence and provides estimates of symptom prevalence. This information is likely to increase public and clinical awareness of lymphoma presentations and aid earlier diagnosis. This review further highlights the lack of studies exploring childhood and adolescent lymphoma presentations in primary care, where patients are likely to present at the earliest stages of their disease.
... The ideal treatment approach in GI lymphoma is [5] debatable as per literature . Radical tumor resection followed by chemotherapy in early disease(St. ...
... The evolving literature on PGL has identified that early diagnosis is key to preventing complications and improving prognosis [24]. For instance, a specific diagnosis of the histopathological type before chemotherapy can enable patients to receive appropriate chemotherapy regimens and improve the possibility for a cure [25]. ...
Background
Whether surgery can improve the prognosis of patients with primary pediatric gastrointestinal lymphoma (PPGL) who experienced bowel perforation remains controversial. This study aimed to evaluate the prognosis of such patients.
Methods
Nine patients pathologically diagnosed with PPGL who experienced perforation at our center between January 2010 and December 2020 were enrolled and divided into two groups: those with perforation during ( n = 4) and before ( n = 5) chemotherapy. Their medical records were reviewed, and long-term follow-up was conducted by telephone in February 2021.
Results
All patients with perforation during chemotherapy were diagnosed with PPGL in the outpatient department. The mean time from outpatient visit to chemotherapy was 17.3 ± 6.1 days. Two patients experienced perforation during the first chemotherapy regimen and received conservative treatment, while the others developed perforation after multiple chemotherapy regimens and underwent surgery. All of the patients received regular chemotherapy and survived for a mean follow-up time of 3.8 ± 1.9 years. No patient with perforation before chemotherapy had a definite diagnosis in the outpatient department. Among these patients, 4 experienced perforation and underwent surgery, of whom 3 developed perforation-related complications and died; the other recurred after chemotherapy. Only the patient who received conservative treatment was diagnosed with PPGL before chemotherapy, received regular chemotherapy, and survived without a recurrence for 1.0 year.
Conclusion
Prompt diagnosis and chemotherapy improve the prognosis of PPGL. Surgery does not affect the prognosis of patients with perforation during chemotherapy but may accelerate disease progression in patients with perforation before chemotherapy.
... The rarity of the disease as well as variable clinical presentation prevents early detection when the possibility of cure exists. [5] First described by Dennis Burkitt in 1958, Burkitt's lymphoma is a highly aggressive NHL often presenting in extra-nodal sites or as an acute leukemia. [6] Burkitt's lymphoma usually is encountered within young people and is the most frequent type of the juvenile NHL. ...
Small bowel lymphoma is a comparatively rare disease. Usually neither typical nor explicit symptoms are determined during its course
and the first manifestation can be noticed when a complication occurs. Burkitt’s lymphoma is a high‑grade, aggressive and rapidly
growing B‑cell neoplasm, which has low long‑term survival rates. Sporadic Burkitt’s lymphoma accounts for 1-2% of lymphomas
in adults and for 40% of lymphomas in children in the worldwide population. The abdomen is the most frequent site of onset in
nonendemic (sporadic) Burkitt’s lymphoma. Symptoms are often misleading and make diagnosis difficult. We present a case of a
5‑year‑old male child who presented with symptoms of acute intestinal obstruction. Emergency therapeutic and diagnostic laparotomy
was performed and biopsy from thickened ileal wall was taken. Histopathologically, it was diagnosed as non‑Hodgkin’s lymphoma
possibly Burkitt’s lymphoma and confirmed on immunohistochemistry.
... Although there are data available on adult GI lymphomas, there is sparse literature available on pediatric PGILs. [7,8] Original Article IntRoductIon P rimary gastrointestinal lymphomas (PGIL) are infrequent in children and account for <5% of all pediatric malignancies. Lymphomas are one of the most common tumors affecting pediatric population with PGILs being the most common extranodal lymphoma. ...
... The first six cases have been published earlier as a part of a smaller series. [7] On macroscopic examination, majority of the lesions were mass forming (17/20; 85%), and remaining three cases were diffuse infiltrating lesions. Following histological analysis and IHC staining, the most common (9/20) subtype was diffuse large B-cell lymphoma (DLBCL). ...
... Lymphomas constitute the third-common cause of pediatric malignancies. [7] Pediatric PGILs represent a heterogeneous group of diseases with regard to its histological subtypes. Their response to therapy and outcomes are also different. ...
Background:
Primary gastrointestinal lymphomas (PGIL) are uncommon in children and account for <5% of all pediatric malignancies. The objective of our study was to analyze the prognostic factors of pediatric PGILs with reference to its histological subtypes, stage, and outcomes using immunohistochemistry.
Materials and methods:
Twenty cases were studied over 11 years. Clinicopathological profiles, histological details, and immunohistochemical (IHC) profiles were analyzed.
Results:
The mean age at the presentation was 6 years. Using IHC stains (CD CD10, CD19, CD20, CD3, terminal deoxynucleotidyl transferase, BCL 2 and 6, PAX 5, and MUM1), diffuse large B-cell lymphoma (DLBCL) was most prevalent (45%), followed by Burkitt lymphoma (35%) and lymphoblastic lymphoma (20%). DLBCLs (9) were classified using the Han's algorithm. Six were activated B-cell and 3 were germinal center B-cell subtypes. The cases of lymphoblastic lymphoma and those in Stage I disease had the best prognosis.
Conclusion:
Pediatric PGILs have variable presentation, outcomes, and diverse treatment modalities depending on the histological subtypes. A panel of IHC stains can be a useful adjunct for the categorization and prognosis of pediatric PGILs.
... The male to female ratio of childhood GI NHL is reported to be from 7:1 to 1.8:1 (14). Primary lymphoma of GI tract accounts for 30-40% of extranodal lymphomas and comprises 10-15% of all non-hodgkins lymphomas (15) . ...
Extranodal Lymphomas are most commonly located in gastrointestinal tract. The most common cause of small intestinal obstruction in children is intussusception. However GI lymphomas are a rare cause of intussusception. So we are reporting a case of extranodal lymphoma in a female child presenting as intussusception of small intestine. a 9 years old female child presented with features of subacute intestinal obstruction. On examination a lump was felt in the abdomen. On further evaluation, a computed tomography (CT) revealed a possibility of lymphoma. The bowel segment was excised and sent for histopathological examination. On gross examination a growth was seen along with satellite lesion. 10 lymph nodes were also identified. Representative sections were taken. Microscopic examination showed the presence of medium size to large size lymphoid cells. After immunohistochemical staining a diagnosis of Non Hodgkins Lymphoma (Extranodal Marginal Zone) was made. 1 lymph node was positive for tumour invasion while other 9 were showing reactive hyperplasia. Since both lymphomas and intussesception are common in pediatric age group, lymphoma should always be kept as a differential diagnosis while dealing with a case of intussusception in pediatric age group.
... The diagnosis of inflammatory bowel disease requires correlation of clinical, biological, endoscopic, histological and imaging findings [48] as shown in the revised Porto criteria proposed by the European Society for Paediatric Gastroenterology Hepatology and Nutrition [50,51] and exclusion of potential mimickers (Table 3) [19,[52][53][54][55][56][57][58]. US Fig. 9 Chronic recurrent multifocal osteomyelitis in an 11year-old girl with a 3-week history of persistent left hip pain, daily fevers and weight loss. ...
Autoinflammatory diseases are a family of disorders characterized by aberrant stimulation of inflammatory pathways without involvement of antigen-directed autoimmunity. They can be further divided in monogenic and polygenic types. Those without an identified genetic mutation are known as polygenic and include systemic-onset juvenile idiopathic arthritis, idiopathic recurrent acute pericarditis, Behçet syndrome, chronic recurrent multifocal osteomyelitis and inflammatory bowel disease among others. Autoinflammatory diseases are characterized by recurrent flares or persistent systemic inflammation and fever, as well as lymphadenopathy and cutaneous, abdominal, thoracic and articular symptoms. Although these syndromes can mimic infections clinically, the inflammatory lesions in autoinflammatory disorders are aseptic. However, because of their infrequency, varied and nonspecific presentation, and the new genetic identification, diagnosis is usually delayed. In this article, which is Part 2 of a two-part series, the authors review the main polygenic autoinflammatory diseases that can be seen in childhood, with special emphasis wherever applicable on imaging features that may help establish the correct diagnosis. However, the major role of imaging is to delineate organ involvement and disease extent.
... Data on natural history and outcomes of PGINHL is limited and one year and 5-yr overall survival (OS) of 65.6% and 35.6%, respectively, has been reported, with no improvement in survival with surgical resection (5,6). Our current knowledge regarding pediatric PGINHL is limited to case reports and small case series (7)(8)(9)(10)(11). Therefore, to better understand the clinicodemographic features and outcomes of these tumors in the pediatric population, we utilized a population-based database to analyze a higher number of patients. ...
Background/Aim: The aim of this study was to present the clinical characteristics, natural history and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in the pediatric population.
Patients and Methods: Surveillance, Epidemiology, and End Results (SEER) database were queried for patients aged 0 to 19 years with PGINHL between 1973 and 2014.
Results: A total of 452 cases were identified [mean age 11.0 (±5.1)] years, whites 84.1%, males (76.5%). The majority of tumors were noted in the small bowel (SB) (47.6%), followed by large bowel (LB) (28.5%) and the stomach (10.0%). Overall, the most common histological subtype was Burkitt lymphoma (51.8%), followed by diffuse large B-cell lymphoma (DLBCL) (26.1%). Mean overall survival (OS) of the entire cohort was 33,33 years with a 5-yr, 10-yr and 30-yr survival rate of 86%, 86% and 79%, respectively. Large bowel tumors had the best long-term survival rates whereas; gastric tumors had the worst with 30-yr survival rate 84% and 74%, respectively. Overall, 328 (72.6%) patients received surgery. No significant survival difference was noted between patients who underwent surgery and those who did not.
Conclusion: This study presents the largest dataset of pediatric PGINHL and describes the clinical features and outcomes of these patients in addition to summarizing the literature.
... A higher prevalence of DLBCL is found in pediatric intestinal mature B cell lymphoma compared to other series [4,10]. That is against our finding in CCHE as the higher prevalence of Burkitt's lymphoma is found which account 95.4% of the cases in the overall group, and Burkitt's lymphoma was the pathology of all the subgroup patients. ...
... Dr. Hafiz: The pediatric population constitutes 32.4% of the total population of this subcontinent and the malignancies are emerging major childhood killer. 1 The primary tumor of the gastrointestinal tract is rare and it is less than 5% of all childhood malignancies. 2 The scarcity of the disease and early variable clinical presentation preclude early diagnosis when the likelihood of cure exists. Among the gastrointestinal malignancies, non-Hodgkin's lymphoma is the predominant variety. ...
This article has no abstract. The first 100 words appear below:
A 2-year-old male child, the second issue of non-consanguineous parents, from average socio-economic status hailing from Dinajpur, Bangladesh was attended at Pediatric Surgery outpatient department with the complaints of vague diffuse abdominal pain in the lower right side of abdomen and around the umbilical region for last 3 days. His mother also reported the feeling of a solid mass in the abdomen during dressing of her child. Then, gradually his problems were increasing in nature with several times of nausea, vomiting, and the passage of blood mixed stool three times before his admission.
