DIAGNOSIS AND REFERRAL OF HODGKIN LYMPHOMA

 

DIAGNOSIS AND REFERRAL OF HODGKIN LYMPHOMA

 

SIOP Childhood Cancer Early Diagnosis & Appropriate Referral (CEDAR) Project

 

Dr. Maya Prasad and Dr. Reineke Schoot (Members, SIOP Education and Training Committee)

On behalf of the SIOP Education and Training Committee and IPA SAG on NCD

 

The SIOP CEDAR Project is improving the knowledge and understanding of healthcare providers who are involved in the initial care, diagnostic workup, and referral of pediatric patients suspected of having cancer.

The fifth CEDAR webinar, “Diagnosis and referral of Hodgkin Lymphoma” is taking place on 20th January 2023. SIOP and IPA encourage Pediatricians, Family Practitioners, Community Nurses, and other healthcare professionals who are involved in the care of children to register for this webinar.

 A video of this webinar is available here!


What is Hodgkin Lymphoma?

Hodgkin Lymphoma (HL) constitutes around 5% of childhood cancers. The two main types of childhood Hodgkin lymphoma are classic and nodular lymphocyte-predominant. While HL presents with a bimodal age distribution (one peak in adolescents and young adults; other in older adults >50 years) in upper-income countries (UICs), a third peak in younger children is noted in lower-income and lower-middle-income countries (L&LMICs). The most common histologic subtype in L&LMICs is mixed cellularity probably related to early exposure to Epstein-Barr virus. Over 95% of children treated for Hodgkin Lymphoma in UMICs and under 70% in LLMICs go on to become long-term survivors, with poorer outcomes being attributable to delayed diagnosis, advanced presentations, treatment toxicities, and abandonment of treatment.

 

Misdiagnosis or late diagnosis of Hodgkin Lymphoma

The commonest presentation of childhood HL is painless adenopathy, predominantly in the cervical or supraclavicular location. The lymph nodes are typically firm, rubbery, non-tender, matted, and have contiguous spread. Children may also present with pallor, fatigue, anorexia, enlargement of other lymphoid organs, mediastinal masses (more frequent in adolescents) or “B symptoms” (fever, weight loss, night sweats). Childhood HL may also present as an emergency with +/- superior vena cava syndrome and/or airway obstruction due to large mediastinal masses and pleural or pericardial effusion.

Since the presentations of HL overlap significantly with non-malignant childhood conditions, including HIV and tuberculosis (TB), children often receive inappropriate, empirical treatment for these conditions, leading to delayed diagnosis and advanced presentation of HL. Analysis of a recent childhood HL trial from India found that 29% of children had received empirical antitubercular treatment (ATT) prior to establishing a definitive diagnosis of HL, with a significant proportion of these children presenting with advanced-stage disease and B symptoms. Cervical lymphadenopathy in children warrants investigation if there is either/ and progressive increase in size, generalized lymphadenopathy, nodes at unusual sites (mediastinal, epitrochlear, supraclavicular ), firm, rubbery or matted nodes, associated organomegaly, fever, anemia, thrombocytopenia and are non-responsive to antibiotics / ATT.

Diagnosis of HL ideally requires an excision biopsy from the largest and firmest node. Enlarged axillary lymph nodes are more likely to be pathological than cervical or inguinal nodes. Hodgkin lymphoma in children may also present concurrently with HIV and TB, further confounding diagnosis and complicating treatment.

 

Treatment of Hodgkin Lymphoma

The current management of childhood HL is based on risk stratification and adapted to the response to initial treatment. Prognostic factors used to determine include stage, presence of B symptoms, bulky disease, extranodal involvement, and/or erythrocyte sedimentation rate.

Current trials in childhood HL focus on decreasing the toxicities of treatment while maintaining excellent outcomes. Multiagent chemotherapy is used in all children, with or without low-dose involved-field or involved-site radiation therapy. Recent trials have found improved outcomes in high-risk HL with the addition of targeted therapy. Immunotherapy and high-dose chemotherapy with stem cell transplant are used in relapsed HL. Children treated for HL need long-term follow-up for late medical effects of treatment as well as surveillance for late recurrences which may occur rarely.

 

SIOP CEDAR Project

The International Society of Pediatric Oncology (SIOP) has launched “Childhood Cancer Early Diagnosis and Appropriate Referral” (CEDAR) project in collaboration with the International Pediatric Association (IPA). In the first phase of the CEDAR project, SIOP and IPA are organizing a series of monthly webinars (started in September 2022) to highlight the importance of early diagnosis, with a focus on the six WHO index cancers (acute lymphoblastic leukemia, Hodgkin lymphoma, Burkitt’s lymphoma, Wilms tumor, retinoblastoma, and low-grade glioma). Each CEDAR webinar is accredited by the European Accreditation Council for Continuing Medical Education (EACCME) with 1 credit point. These webinars are free to attend and open to everyone, and we are offering simultaneous Spanish translation.

Recordings of the first two webinars are available on the following links:

1.      Outcomes of childhood cancers: Gaps and Disparities to be addressed

https://www.youtube.com/watch?v=3d2U0sajKr4

 

2.      Diagnosis and Referral of Acute Leukemia

https://youtu.be/VBp8nbRlRDQ

 

3.      Diagnosis and Referral of Retinoblastoma

https://www.youtube.com/watch?v=nFQMMh-2GlE

 

 

CEDAR Webinar – Diagnosis and referral of Hodgkin Lymphoma

The fifth CEDAR webinar is on 20th January 2023 at 2-3 PM UTC/GMT on “Diagnosis and referral of Hodgkin Lymphoma”. This webinar will be co-moderated by SIOP Education and Training Committee members Dr. Maya Prasad (India) and Dr. Reineke Schoot (UK) and a member of IPA SAG on NCD Dr. Basim Al-Zoubi (Jordan). The agenda of this educational session is as follows:

1.      Case presentation: Dr. Ganda Ilmana (Indonesia)

2.      When to suspect Hodgkin Lymphoma? : Dr. Mauricio Castellanos (Guatemala)

3.      Approach for diagnosis, initial management, and referral: Dr. Jennifer Geel (South Africa)

4.      Panel Discussion with Expert Panelists

a.      Dr. Ramandeep Arora (India)

b.      Dr. Francesco Ceppi (Switzerland)

c.      Dr. Szymon Skoczen (Poland)

 


Link for registration: https://bit.ly/3uDmNpW

 




FUTURE CEDAR WEBINARS

Please save the dates and register for our future CEDAR webinars:

Date

Subject

Link to register

Webinar 6: 3rd February 2023

Diagnosis and Referral of Burkitt’s Lymphoma

https://bit.ly/3Fd1n82

Webinar 7: 10th March 2023

Diagnosis and Referral of Wilms Tumor

https://bit.ly/3UGzlHK

For queries or further information, please visit siop-online.org/cedar or email info@siop-online.org

 


 

Maya Prasad (MD) is Professor of Paediatric Oncology at Tata Memorial Centre, Mumbai, India. She has been serving as co-chair of the SIOP Nutrition Network and an active member of the SIOP Education and Training Committee. She has special interests in Paediatric Solid Tumors, Supportive Care in Paediatric Oncology, and Late Effects in Survivors of Childhood Cancer.

 

Dr. Reineke Schoot (MD) is paediatric oncologist at Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands. She is an active member of SIOP Europe (SIOPE) and the SIOP Education and Training Committee. She has a special interest in soft tissue sarcoma and early phase clinical trials.

 

SIOP

The International Society of Paediatric Oncology (SIOP) is the only global multidisciplinary society entirely devoted to pediatric and adolescent cancer. SIOP, as a non-state actor in official relations with WHO, is committed to contribute substantially to WHO Global Initiative for Childhood Cancer. We have over 2,600 members worldwide including doctors, nurses, other healthcare professionals, scientists, and researchers.

Visit siop-online.org for more information.

 

IPA

The International Pediatric Association (IPA) exists to create a world where all children, regardless of age, location, or family situation can live healthy lives. IPA advocates globally, nationally, and locally for high-quality, evidence-based, and child-centered pediatric care. We are a respected partner of WHO and UNICEF and provide global leadership on emerging child health issues through our work with leading global health players. Visit ipa-world.org for more information.

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