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VIVA-ASIA RARE TUMOR BOARD WEBINAR is a virtual quarterly meeting that is organized by VIVA Foundation for Children with Cancer to enable doctors from all over the world to discuss the diagnosis and treatment of rare pediatric tumors.

PROGRAMME RUNDOWN

SYNOPSIS

NUT Carcinoma by Dr Jiang, Xuan Zhu

We present the case of a 16-year-old female admitted with a 3-day history of fever and cough. Chest CT revealed a right lung mass accompanied by pleural effusion and lymphadenopathy. Whole-body PET-CT suggested malignant lesions with metastases to the right pleura and lymph nodes. IHC analysis of a cervical lymph node biopsy demonstrated diffuse strong positivity for NUT, confirming the diagnosis of metastatic NUT carcinoma.

The patient received chemotherapy with ifosfamide, doxorubicin, and vincristine (IAV), complicated by myelosuppression and febrile neutropenia. Three weeks later, a second-line regimen of ifosfamide, doxorubicin, and cisplatin (IAP) was initiated. During treatment, she developed superior vena cava (SVC) syndrome, managed with fluid restriction and diuretics. Radiotherapy was administered following chemotherapy, with concurrent carboplatin; however, the tumor showed no significant regression.

Her clinical course deteriorated with progressive chest pain, worsening cough, and large-volume pleural effusion leading to respiratory distress. Palliative care was initiated, including intravenous morphine for symptomatic relief of dyspnea. The patient succumbed to respiratory failure and hypoxemia 3 months after initial diagnosis.

NUT Carcinoma by Dr Tong, Sun Wing

1. Pathological findings in this case (cervical lymph node core biopsy).

2. NUTM1-rearranged carcinoma, history & pathology.

3. Illustrative pathological differential diagnoses.

4. Approach to workup.

5. Summary.

Mucoepidermoid Carcinoma by Dr Lu, Evelyn

1 case of lung mucoepidermoid carcinoma will be presented follow by a short literature review.  Common approach to the disease, presentation and outcome would be illustrated.

Primary lung carcinoma in children and adolescents: Experience of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT) by Dr Brecht, Ines

Primary lung cancer is an extremely rare malignant disease in children, with an incidence of less than 2 cases per million per year, and is characterized by considerable clinical and histological diversity. The largest joint analysis to date, conducted by the European Cooperative Study Group on Paediatric Rare Tumors (EXPeRT), examined 38 pediatric cases diagnosed between 2000 and 2021. Mucoepidermoid carcinoma (MEC) was the most common subtype, followed by adenocarcinoma, squamous cell carcinoma, adenosquamous carcinoma and small cell lung cancer. Children with MEC were usually diagnosed at earlier stages and responded well to surgical resection alone, resulting in long-term remission in most cases. In contrast, children with other histological subtypes were often diagnosed with advanced disease and had significantly worse outcomes despite aggressive multimodal treatments, underscoring the urgent need for new therapeutic strategies. 

A recent genome study by the German Working Group on Rare Paediatric Tumors (STEP) found that pediatric lung adenocarcinomas and adenosquamous carcinomas frequently harbor ALK::EML4 fusions, a finding that is less common in adult lung cancer. The results of the analysis highlight both the unique and shared genetic characteristics of lung carcinomas in children and adults and point to the potential for mutation-driven, personalized treatment approaches in children with these rare tumors.

SPEAKERS

Dr Brecht, Ines

Pediatric Oncology and Hematology
University Children's Hospital Tuebingen
Vice Chair EXPERT (European Pediatric Rare Tumour Group)
Co-Chair STEP (German Rare Tumour Group)
Germany

 

 

 

Dr Abele, Michael

Pediatric Oncology, University Children’s Hospital Tuebingen
Germany

 

 

 

 

 

 

Dr Jiang, Xuan Zhu

Resident
Hong Kong University Shenzhen Hospital
China

 

 

 

 

 

 

Dr Tong, Sun Wing

Consultant pathologist
Chair, Department of Pathology
The University of Hong Kong-Shenzhen Hospital (HKU-SZH)
China

 

 

 

 

 

 

Dr Lu, Evelyn

Resident specialist, Department of Paediatrics and Adolescent Medicine, Hong Kong Children’s Hospital
Paediatric Haematology and Oncology subspecialty trainee
Honorary tutor, The University of Hong Kong

 

 

 

 

 

 

 

CONVENORS

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Prof Chan, Godfrey

Honorary Professor, Department of Paediatrics and Adolescent Medicine
School of Clinical Medicine, The University of Hong Kong
Consultant Paediatric Haematologist Oncologist
Hong Kong Sanatorium and Hospital
Hong Kong SAR

 

Dr Liu, Anthony

Honorary Clinical Assistant Professor
Department of Paediatrics & Adolescent Medicine
LKS Faculty of Medicine, The University of Hong Kong
Hong Kong SAR

Staff Oncologist, Pediatric Brain Tumour Program
The Hospital for Sick Children, Toronto
Canada