Site specific - Children Teenagers and Young Adults (CTYA)

ICD-10 codes

Note:

• please refer to Appendix A and B for site specific registerable ICD codes for CTYA patients, where the patient is under 25 at the time of diagnosis

Retired data items

The following data items have been retired from v10:

• CT7000 - Treated According to CCLG Guidelines
• CT7010 - CCLG Guideline Name
• CT7090 - Urine VMA / Creatinine Ratio
• CT6160 - Specialty Sub Code (Chemotherapy Consultant)

Overview

The following age groupings are used for COSD:

• paediatric = under 16 years at time of diagnosis
• teenage = 16 - 18 years (under 19) at time of diagnosis
• young adult = 19 - 24 at time of diagnosis

The following guidelines are intended to support the decision on which data sets should be submitted.

Where the patient is discussed by an age specific (paediatric or TYA) MDT at a designated paediatric or TYA Principal Treatment Centre (PTC), the responsibility for completing the CTYA data set rests with the PTC. For patients (of any age) who are also discussed at a site specific MDT, or where the disease is not specified in the CTYA data set, (for example the diagnosis of a colorectal carcinoma), the appropriate site specific data set should also be completed by the relevant MDT.

National guidance offers patients (aged 19 to 24 years) the option of referral to a TYA PTC, although the guidance also indicates that all such patients should be discussed at a TYA MDT even if they are not referred to the PTC for treatment. If, despite this, the patient is only discussed by a site specific MDT, that team should complete the appropriate site specific data set and the relevant additional (non-disease-specific) items in the CTYA data set.

Where a disease is covered by both the CTYA and a site specific data set (such as some haematological diseases), only one set of disease specific items needs to be completed (either CTYA or site specific according to the speciality of the treating team). The non-disease-specific items in the CTYA data set should however be completed as per the preceding paragraphs.

Disease specific data items

A table has been created, which shows which data items are applicable to each specific diagnosis. It is important to note that some of these have now moved to other sections within COSD to help improve ascertainment, however the disease specific groupings have not changed.

May be up to one occurrence per CORE – Referrals and First Stage of Patient Pathway (0..1)

Specialty (Referrer to Specialist)

The specialty of the person referring to the patients Principal Treatment Centre or age specific Specialist TYA MDT.

Cancer Symptoms First Noted Date

Record the time when the symptoms were first noted related to this diagnosis as agreed between the consultant and the patient. This will normally be recorded by the consultant first seeing the patient in secondary care.

Depending on the length of time this should normally include at least the month and year. The day should also be included if known. If symptoms have been present for a long time then it may only be possible to record the year. In these various circumstances the Format/Length will be:

• date: (including year, month and day): CCYY-MM-DD
• year and month: YYYY-MM
• year only: YYYY

Notes:

• this has been moved from CORE to CTYA and is ‘ONLY’ required for CTYA cases
• this data item has a new data item number in v10, previously CR2000

May be up to one occurrence per CORE – Diagnosis (0..1)

Consultant Specialty (at Diagnosis)

The specialty of the consultant responsible for the patient at the time of diagnosis.

Consultant Age Specialty (at Diagnosis)

The age group specialty of the consultant responsible for the patient at the time of diagnosis. This will be defined by the MDT.

May be up to one occurrence per CORE – Diagnosis (0..1)

Banked Tissue at Diagnosis

To carry Banked Tissue details for CTYA patients

After discussions with Subject Matter Experts, this data item has moved from ‘CORE – Diagnosis – Banked Tissue’ and is ‘ONLY’ required for CTYA cases from v10.

Notes:

• this data item has a new data item number in v10, previously CR7700
• ‘9 – Not Known (Not Recorded)’ has been retired in v10

May be up to one occurrence per CORE - Diagnosis (0..1)

Life Threatening Symptoms at Presentation

Record if there were any life-threatening symptoms at presentation.

May be multiple occurrences per record (0..*)

Note:

• the following two data items have moved from CORE to CTYA and are ‘ONLY’ required for CTYA cases

Patient Trial Status (Cancer)

This is now a mandatory data item from v10. An indication of whether a patient who is eligible for a cancer clinical trial is taking part in it. These attributes have been updated so that they better reflect the CTYA clinical trial process.

Notes:

• this data item has a new data item number in v10, previously CR1290
• attributes 03 and 09 have been retired from v10
• Attributes 04 and 05 are new from v10

Clinical Trial Decision Date (Patient)

Record the patient's decision date for each clinical trial, provided it is related to the recorded diagnosis.

Note:

• this data item has a new data item number in v10, previously CR6700

It is important that all CTYA stageable cancers at staged for every case. All site specific staging fields are mandatory and a child of ‘CORE – Site Specific Staging’ Section, and together mandates the collection of:

• the date the sample was taken which provided a positive site specific stage outcome or the MDT where the stage was agreed
• the organisation who carried out the stage
  • this is a ‘required’ data item from v10, but important to collect if known
• the stage itself

Note additional CTYA staging is required in the following areas of COSD:

• for CTYA sarcomas, carcinomas, melanomas and extracranial germ cell tumours the TNM staging system MUST be provided per submission (see relevant site-specific section)
• for CTYA Hodgkin and non-Hodgkin lymphomas the Ann Arbor and/or Murphy (St Jude) stage MUST be provided per submission (see Haematological section)
• For CTYA ALL and AML, to comply with the Toronto Cancer Staging benchmarking project, Children’s Oncology Group (COG) Staging System Stage and/or Central Nervous System Involvement are required (see Haematological section)
• for CTYA medulloblastomas, other embryonal CNS tumours, ependymomas and intracranial germ cell tumours the Chang staging system MUST be provided per submission (see CNS section)
• for CTYA leukaemia’s and other CTYA CNS tumours are unstageable

The following data items are specific to paediatric renal tumours, including adult Wilms tumour, neuroblastomas, paediatric liver tumours (including adult hepatoblastoma), and retinoblastomas. If applicable, one of the following Site Specific Staging Sections MUST be provided per record.

May be one occurrence per Core - Site Specific Staging (0..1)

CTYA - Site specific staging choice - Choice 1 - Renal Tumours (1..1)

End of CTYA - Site specific staging choice - Choice 1

CTYA - Site specific staging choice - Choice 2 – Neuroblastoma (1..1)

End of CTYA - Site specific staging choice - Choice 2

CTYA - Site specific staging choice - Choice 3 – Hepatoblastoma (1..1)

Start of repeating item - 'Pretext Annotation Factors'

End of repeating item - 'Pretext Annotation Factors'

End of CTYA - Site specific staging choice - Choice 3

CTYA - Site specific staging choice - Choice 4 – Retinoblastoma (1..1)

End of CTYA - Site specific staging choice - Choice 4

End of CTYA - Site specific staging choice

It is possible that some legacy data may not have all the required mandatory fields. The recommendation is for Trusts to update their data to meet the new requirements and improve/enrich their data submissions, or not upload the legacy data items in the new record (if that data is not available).

Wilms Tumour Stage

Stage is determined by the results of the imaging studies and both the surgical and pathologic findings at nephrectomy. It is essential to record the stage for this group of patients and this information should be available to the MDT following treatment.

Stage 1 – the tumour is only affecting the kidney. The tumour has not spread, and it was completely removed during surgery.

Stage 2 – the tumour has spread beyond the kidney to the nearby structures. There are no cancer cells in distant organs, such as the lungs. It was completely removed during surgery.

Stage 3 – the tumour has either:

• not been completely removed during surgery
• spread to the lymph nodes in the tummy area (abdomen)
• burst, before or during, the surgery

Stage 4 – the tumour has spread to a distant part of the body. This is most commonly the lungs, but might be the liver, bone, brain or lymph nodes in an area outside the tummy (abdominal) or pelvic area.

Stage 5 – there are tumours in both kidneys. This is called bilateral Wilms’ tumour. Doctors stage each of the tumours separately.

International Neuroblastoma Risk Group (INRG) Staging System

The International Neuroblastoma Risk Group Staging System (INRGSS) was designed for the International Neuroblastoma Risk Group (INRG) pre-treatment classification system. Unlike the INSS, the INRGSS uses only the results of imaging tests taken before surgery. It does not include surgical results or spread to lymph nodes to determine the stage. Knowledge regarding the presence or absence of image defined risk factors (IDRF) are required for this staging system. Find out more from the INRGSS in an article from the Journal of Clinical Oncology.  

Note:

• the descriptions have been updated on the advice of CTYA clinical leads

Stage L1 - tumours are localised tumours that do not involve vital structures as defined by the list of IDRFs (Table 1). The tumour must be confined within one body compartment, neck, chest, abdomen, or pelvis. The isolated finding of intraspinal tumour extension that does not fulfil the criteria for an IDRF (Table 1) is consistent with stage L1.

Stage L2 - tumours are locoregional tumours with one or more IDRFs. The tumour may be ipsilaterally continuous within body compartments (such as, a left-sided abdominal tumour with left-sided chest involvement should be considered stage L2). However, a clearly left-sided abdominal tumour with right-sided chest (or vice versa) involvement is defined as metastatic disease.

Stage M - is defined as distant metastatic disease (such as, not contiguous with the primary tumour) except as defined for MS. Nonregional (distant) lymph node involvement is metastatic disease. However, an upper abdominal tumour with enlarged lower mediastinal nodes or a pelvic tumour with inguinal lymph node involvement is considered locoregional disease. Ascites and a pleural effusion, even with malignant cells, do not constitute metastatic disease unless they are remote from the body compartment of the primary tumour.

Stage MS - is metastatic disease in patients younger than 12 months (365 days) with metastases confined to skin, liver, and/or bone marrow and other sites, but not Bone, Lung or CNS. MIBG scintigraphy must be negative in bone and bone marrow, provided there is MIBG uptake in the primary tumour, bone scans are not required. The primary tumour can be L1 or L2 and there is no restriction regarding crossing or infiltration of the midline.

Pretext Staging System Stage

Pretext 1 - 4 refers to sectors of liver involved.

Pretext Annotation Factors

This is a multiple repeating data item. Record any additional ‘Pretext Annotation Factors’ used to support Pretext Staging.

International Staging System for Retinoblastoma

The international staging system stage for intraocular and extraocular retinoblastoma.

This section is a child of ‘Core – Treatment’, and will mandate:

• the date the treatment started
• the treatment modality
• the organisation that provided the treatment

Must be one occurrence per CORE - Treatment (1..2)

CTYA - Treatment - Principal Treatment Centre choice - Choice 1 .. 2

CTYA - Treatment - Principal Treatment Centre - Choice 1

Start of repeating item - Principal Treatment Centre - Children's PTC

End of repeating item - Principal Treatment Centre - Children's PTC

End of CTYA - Treatment - Principal Treatment Centre - Choice 1

Start of CTYA - Treatment - Principal Treatment Centre - Choice 2

Start of repeating item - Principal Treatment Centre - Teenage Young Adult (TYA) PTC

End of repeating item - Principal Treatment Centre - Teenage Young Adult (TYA) PTC

End of CTYA - Treatment - Principal Treatment Centre - Choice 2

End of CTYA - Treatment - Principal Treatment Centre choice 

Childhood or TYA Principal Treatment Centre

Record the patient’s nominated childhood or TYA principal treatment centre (PTC), where they have chosen to have treatment. More than one centre can be selected.

There is no longer a pre-defined list of centres as these change over time. Instead from v10, record only the Trust or Hospital code of the principal treatment centre as defined by the Organisational Data Service (ODS).

May be one occurrence per record (0..1)

Risk Classification (Pathological) after Immediate Nephrectomy

Classification and timing of surgery determine histological risk. This information should be available for the MDT discussion following treatment but will only apply to a small number of cases.

The following definitions are used:

• favourable histology
  • non-anaplastic Wilms tumour (all subtypes); cystic partially differentiated nephroblastoma; mesoblastic nephroma; diffuse nephroblastomatosis
• unfavourable histology
  • anaplastic Wilms tumour (focal and diffuse); malignant rhabdoid tumour of kidney; clear cell sarcoma of the kidney; renal cell carcinoma

Risk Classification (Pathological) after Preoperative Chemotherapy

Classification after preoperative chemotherapy determines histological risk. This information should be available for the MDT discussion following treatment but will only apply to a small number of cases.

The following definitions are used:

• low risk:
  • cystic partially differentiated nephroblastoma; completely necrotic nephroblastoma; mesoblastic nephroma; diffuse nephroblastomatosis
• intermediate risk:
  • nephroblastoma type - epithelial; stromal; mixed; regressive; focal anaplasia
• high risk:
  • nephroblastoma blastemal type; nephroblastoma with anaplasia; malignant rhabdoid tumour of the kidney; clear cell sarcoma of the kidney; renal cell carcinoma

All cases of Retinoblastoma are referred to the national specialist centres who are requested to record this section in addition to TNM staging.

For many years the Rees-Ellsworth intraocular classification system was used to stage patients according to their likelihood of successful treatment with external beam radiotherapy. As treatment approaches have evolved and chemotherapy has replaced radiotherapy as the mainstay of conservative management, a new intraocular classification has been introduced and has been received with widespread approval from the international community.

The staging of extra-ocular disease is less well established although recently a panel of international experts have proposed a system which is gaining acceptance in published literature.

May be multiple occurrences per record (0..*)

Retinoblastoma Assessment Laterality

The laterality for which the retinoblastoma details were recorded.

International Classification for Intraocular Retinoblastoma

The intraocular classification for retinoblastoma as approved by the international community.