Germ cell tumours present a dichotomy in their incidence peaks, the first strikingly early between 0-4 years, labelled Type I GCT. The second peak, observed during adolescence and young adulthood, characterizes Type II GCT. Notably, between the ages of 15-30 years, germ cell tumours become the predominant malignancy, accounting for 15% of all cases within this age group.
Symptoms
The clinical presentation of germ cell tumours in children is a mosaic of symptoms, mirroring the heterogeneity that defines these neoplasms. The manifestations vary based on the tumour’s location, with common symptoms encompassing:
l Abdominal Distension: Tumours in the abdominal region lead to noticeable swelling, prompting medical attention.
l Pain and Discomfort: Children may experience localized discomfort, signalling the presence of a tumour in areas like the abdomen, pelvis, or gonads.
l Changes in Puberty Development: Type II GCTs, emerging with the onset of puberty, may disrupt normal pubertal development, manifesting as irregularities in menstrual cycles for girls or alterations in secondary sexual characteristics for boys.
l Respiratory Distress: In cases where tumours are situated in the thoracic region, respiratory symptoms like coughing, shortness of breath, or chest pain may manifest.
Diagnosis
The diagnosis of germ cell tumours in children is a multifaceted process that involves a combination of clinical evaluation, imaging studies, and pathological assessment. Early detection is crucial for accurate prognosis, but the heterogeneity of GCTs makes this task particularly challenging. Clinical evaluation begins with a thorough medical history and physical examination. In infants and young children, this might involve careful monitoring of growth patterns, while in adolescents, attention is directed towards the beginning of puberty and complications in reproductive system. Furthermore, imaging studies play a pivotal role in unravelling the mysteries of GCTs. Ultrasound, CT scans, and MRI scans offer a detailed look at the tumour’s location, size, and characteristics. In Type I GCTs, abdominal and pelvic imaging may be crucial, while Type II GCTs often involve imaging of the reproductive organs.
Besides, pathological assessment, through biopsy or surgical resection, is crucial for confirming the diagnosis and classifying GCTs into subtypes, guiding treatment decisions.
Treatment
The diverse nature of germ cell tumours requires a collaborative approach between pediatric oncologists and surgical disciplines. Treatment, tailored to tumour characteristics, includes surgery, chemotherapy, and, when necessary, radiotherapy.
Surgery is often a cornerstone of GCT treatment, aiming for complete resection while preserving organ function. The complexity of surgical interventions highlights the need for specialized expertise, with pediatric surgeons, urologists, gynaecologists, thoracic surgeons, and neurosurgeons contributing their skills to achieve optimal outcomes. Chemotherapy plays a crucial role in managing GCTs, especially in cases where complete surgical resection is challenging.
In certain scenarios, radiotherapy becomes part of the treatment plan, targeting residual tumour cells or preventing recurrence. The judicious use of radiotherapy requires careful consideration of the patient’s age, overall health, and the potential long-term impact on normal tissue.
The collaborative efforts of the medical team extend beyond the treatment phase, encompassing long-term follow-up and survivorship care. Monitoring for late effects of treatment and addressing the unique challenges faced by survivors of pediatric GCTs underline the holistic approach to managing these rare neoplasms.
(The author, Dr. Vinay Bhatia, is the Head, Molecular Biology National Reference Lab, at Oncquest Laboratories Limited, Gurugram.)
