IPSO Surgical Practice Guidelines

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IPSO Surgical Practice Guidelines PDF
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Authors

Simone de Campos Vieira Abib¹, Chan Hon Chui², Sharon Cox³, Abdelhafeez H Abdelhafeez⁴, Israel Fernandez-Pineda⁵, Ahmed Elgendy⁶, Jonathan Karpelowsky⁷, Pablo Lobos⁸, Marc Wijnen⁹, Jörg Fuchs¹⁰, Andrea Hayes¹¹ and Justin T Gerstle¹²

Pediatric Oncology Institute, GRAACC, Federal University of São Paulo, Rua Pedro de Toledo, 572 – Vila Clementino, São Paulo, SP 04021-001, Brazil
Surgery Centre for Children, Mount Elizabeth Medical Centre, 3 Mount Elizabeth, 228510, Singapore
Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital, University of Cape Town, Cape Town, South Africa
Department of Surgery, St Jude Research Hospital 262 Danny Thomas Place. MS133, Memphis, TN 38105, USA
Department of Pediatric Surgery, Virgen del Rocio Children’s Hospital, Av Manuel Siurot S/NN, Sevilla 41013, Spain
Surgical Oncology Unit, Faculty of Medicine, Tanta University, Elgiesh Street, 31111, Tanta, Gharbeya, Egypt
Department of Paediatric Surgery, Children’s Hospital at Westmead, Westmead NSW 2145, Australia
Pediatric Surgery Division, Hospital Italiano de Buenos Aires, Andrés Lamas 812, Buenos Aires 1406, Argentina
Department of Surgery, Princess Maxima Center for Pediatric Oncology, Huispostnummer KE 01.129.2, Postbus 85090, Utretcht 3508AB, The Netherlands
Department of Pediatric Surgery and Pediatric Urology, University of Tuebingen, Hoppe-Seyler-Str. 3, Tübingen 72076, Germany
Department of Surgery, Howard University Hospital, 1851 9th Street NW, 4th Floor, Washington, DC 20059, USA
Department of Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA

Abstract

Most children with tumors will require one or more surgical interventions as part of the care and treatment, including making a diagnosis, obtaining adequate venous access, performing a surgical resection for solid tumors (with staging and reconstruction), performing procedures for cancer prevention and its late effects, and managing complications of treatment; all with the goal of improving survival and quality of life. It is important for surgeons to adhere to sound pediatric surgical oncology principles, as they are closely associated with improved local control and survival. Unfortunately, there is a significant disparity in survival rates in low and middle income countries, when compared to those from high income countries.

The International Society of Paediatric Surgical Oncology (IPSO) is the leading organization that deals with pediatric surgical oncology worldwide. This organization allows experts in the field from around the globe to gather and address the surgical needs of children with cancer. IPSO has been invited to contribute surgical guidance as part of the World Health Organization Initiative for Childhood Cancer. One of our goals is to provide surgical guidance for different scenarios, including those experienced in High- (HICs) and Low- and Middle- Income Countries (LMICs). With this in mind, the following guidelines have been developed by authors from both HICs and LMICs. These have been further validated by experts with the aim of providing evidence-based information for surgeons who care for children with cancer.

We hope that this initiative will benefit children worldwide in the best way possible.

Simone Abib, IPSO President
Justin T Gerstle, IPSO Education Committee Chair
Chan Hon Chui, IPSO Secretary

Keywords: paediatric oncology surgery, paediatric cancer, surgery, children

Disclaimer

The document, IPSO Surgical Practice Guidelines, and the information it contains are for authorised use by surgeons. IPSO cannot accept any liability and responsibility for any claims, loss or damage arising from the use of this document and its contents.

Contents Overview only

IPSO SURGICAL PRACTICE GUIDELINES			1
	Introduction		1
	Disclaimer		2
	Contributing Authors		9
	1. Role of Surgery in Paediatric Cancer Diagnosis		9
	2. Management of Lymph Node Enlargement in Children		9
	3. Venous Access for the Paediatric Cancer Patient		9
	4. Neuroblastoma		10
	5. Wilms Tumour		11
	6. Rhabdomyosarcoma and Non-Rhabdomyosarcoma Soft Tissue Sarcoma		11
	7. Osteosarcoma and Ewing Sarcoma		11
	8. Liver Tumours		11
	9. Germ Cell Tumours		12
	10. Thoracic Tumours		13
	11. Pulmonary Metastasis		13
	12. Surgical Strategies in Pelvic Tumours		13
	13. Rare Tumours		14
	14. Surgery for Lymphoma		15
	15. MIS in Paediatric Oncology		15
	16. Surgical Emergencies in Paediatric Surgical Oncology		16
	17. Fertility Preservation		17
	18. Paediatric Surgical Oncology and Palliative Care		18
ROLE OF SURGERY IN PAEDIATRIC CANCER DIAGNOSIS			19
	Introduction		19
	Abdominal Mass		19
	Scrotal Mass		22
	Thoracic Mass		23
	Neck Mass		23
	Extremity Mass		28
	Tips and Pitfalls in the Diagnosis of Paediatric Cancer		28
MANAGEMENT OF LYMPH NODE ENLARGEMENT IN CHILDREN			33
	Preoperative: Evaluation, Images, Special Needs and Biopsy Need		33
	Surgical Goals		34
	Pitfalls		34
	Surgery		35
	Complications		35
	Conclusions		35
VENOUS ACCESS FOR THE PAEDIATRIC CANCER PATIENT			36
	Devices Available		36
	Surgical Goals		36
	Preoperative Evaluation, Images and Special Needs		36
	Surgery		37
	Postoperative Period		39
	Complications		39
	Tips and Pitfalls		40
NEUROBLASTOMA			42
	Epidemiology		42
	Preoperative Evaluation		42
	Indications for Surgery		44
	Perioperative Management		45
	Surgical Goals		45
	Postoperative Considerations		45
	Complications		47
	Tips and Pitfalls		47
WILMS TUMOUR			55
	Evaluation		55
	Indications and Principles of Biopsy		55
	Perioperative Management		56
	Surgery		57
	Tips, Pitfalls and Complications		59
	Postoperative Considerations		59
	Prognosis, Prognostics and Follow-up		59
RHABDOMYOSARCOMA AND NON-RHABDOMYOSARCOMA SOFT-TISSUE SARCOMA			62
	Introduction		62
	Rhabdomyosarcoma		63
	Treatment of RMS		63
	Guidelines for Surgery for RMS		65
	Surgical Guidelines for Various Sites		66
	Complications of Surgery		67
NON-RHABDOMYOSARCOMA SOFT-TISSUE SARCOMA			68
	Surgical Principles		68
	Surgical Considerations		69
OSTEOSARCOMA AND EWING SARCOMA			71
	Epidemiology		71
	Preoperative Evaluation, Images, Special Needs, Biopsy and Indications for Surgery		71
	Surgical Goals		71
	Perioperative Management		72
	Surgical Approach		72
	Complications		74
	Postoperative Considerations		75
	Tips		76
	Pitfalls		76
LIVER TUMOURS: HEPATOBLASTOMA			80
	Evaluation		80
	Indications and Principles of Biopsy versus Resection at Diagnosis		81
	Role and Timing of Multimodality Therapy		82
	Surgical Management		82
	Key Steps of the Surgical Procedure: Hepatectomy		84
	Types of Liver Resections		86
	Post-operative Management		87
	Pitfalls, and Potential Surgical Complications		87
	Other Surgical Considerations		88
	Outcome		89
LIVER TUMOURS: PAEDIATRIC HEPATOCELLULAR CARCINOMA			92
	Evaluation		92
	Treatments		94
	Surgical Procedures in Paediatric HCC		94
	Chemotherapy		95
	Ablative Therapies		97
	Outcome		98
EXTRACRANIAL GERM CELL TUMOUR			101
	Introduction		101
	Sacrococcygeal Germ Cell Tumour		101
	Mediastinal Germ Cell Tumour		102
	Abdominal and Retroperitoneal Germ Cell Tumour		103
	Head and Neck Germ Cell Tumour		104
	Genitourinary Germ Cell Tumour		105
	Gonadal Germ Cell Tumour		105
	Testicular Tumours		108
THORACIC TUMOURS			114
	Background		114
	Tumours of the Chest Wall		114
	Mediastinal Tumours		117
	Primary Pulmonary Tumours		121
		Pleuropulmonary Blastoma (PPB)	121
		Pulmonary Carcinoid Tumours	123
		Inflammatory Myofibroblastic Tumour	124
SURGICAL APPROACH TO PULMONARY METASTASIS IN CHILDREN			129
	Introduction		129
	Surgical Goals		129
	Work-Up		129
	Surgical Technique		129
	Tumour Specific Management		130
	Complications		132
	Conclusion		133
SURGICAL STRATEGIES IN PELVIC TUMOURS			138
	Evaluation		138
	Workup		139
	Indications and Principles of Biopsy		140
	Perioperative Management		140
	Surgery		141
	Key Steps		143
	Tips, Pitfalls and Complications		144
	Postoperative Considerations		145
	Prognosis and Follow-up		145
RARE TUMOURS			148
	Introduction		148
	Pancreatic Tumours		150
		Background	150
		Solid-Cystic Papillary Tumour (SCPT) of The Pancreas	150
		Pancreatoblastoma (PBL)	152
		Neuroendocrine Tumours (NET)	154
		Pseudopapillary Tumours	155
		Pancreatic Carcinoma	155
	Pleuropulmonary Blastoma		159
		Evaluation	159
		Perioperative Management	160
		Advanced Stages and Relapsed Disease	161
		Postoperative Considerations	161
		Prognosis, Prognostics and Follow-up	161
	Phaeochromocytoma and Paraganglioma		163
		Introduction	163
		Diagnostic Investigations	164
		Assessment of Patients on Admission	166
		Surgical Technique	166
		Post-operative Management	168
		Postoperative Outcome & Follow-up	168
	Non-Germ Cell Gonadal Tumours		172
		Evaluation	172
		Perioperative Management	173
		Prognosis, Prognostics and Follow-up	176
	Colorectal Carcinoma		178
		Incidence	178
		Clinical Presentation	178
		Diagnosis	178
		Management	178
		Prognosis	179
	Adrenocortical Tumours		180
		Background	180
		Epidemiology	180
		Clinical Presentation	180
		Workup	181
		Imaging	181
		Additional Assessments	182
		Diagnosis	182
		Surgery	182
		Metastases	183
		Preoperative Considerations	183
		Role and Timing of Multimodality Therapy	183
		Staging	183
		Postoperative Considerations	183
		Treatment for Malignant ACT	184
		Follow-up	184
	Gastrointestinal Stromal Tumours (GIST)		186
		Epidemiology, Biology and Clinical Aspects	186
		Treatment	186
	Melanoma		189
		Evaluation	189
		Pre-Operative Management	190
		Surgical Guidelines	190
		Postoperative Management	190
	Salivary Gland Tumours		192
		Evaluation	192
		Indications and Principles of Biopsy	193
		Perioperative Management	193
		Surgery	193
		Postoperative Considerations	193
		Prognosis and Follow-up	194
	Neuroendocrine Tumours of the GI Tract		196
		Evaluation	196
		Perioperative Management	197
		Surgery	198
		Postoperative Considerations	199
		Prognosis, Prognostics and Follow-up	199
SURGERY FOR LYMPHOMA			202
	Introduction		202
	Surgical Goals		202
	Preoperative: Evaluation, Images, Special Needs, Biopsy Need?		202
	Special Considerations for Lymph Node Sampling		204
	Special Considerations for Abdominal Burkitt Lymphoma		204
	Post Operation		205
	Complications		206
	Tips		206
	Pitfalls		206
MINIMALLY INVASIVE SURGERY IN PAEDIATRIC SURGICAL ONCOLOGY			208
	1. Introduction		208
	2. Incidence		208
	3. Principles of Surgical Resection		208
	4. Principles of MIS		209
	5. MIS Approach to Renal Tumours in Children		209
	6. MIS Approach to Adrenal Tumours in Children		211
	7. MIS Approach to Gonadal Tumours in Children		211
	8. MIS Approach to Pancreatic Tumours in Children		212
	9. MIS Approach to Hepatic Tumours in Children		213
	10. Minimally Invasive Fertility Preserving Procedures in Children		213
	11. MIS Approach to Thoracic Tumours in Children		214
	12. Upcoming Technologies		214
	Surgical Emergencies and Complications in Paediatric Oncology		218
	Classification of Commonly Encountered Surgical Emergencies:		218
		Emergencies Related to Chemotherapy: Part 1	218
		Emergencies Related to Tumour Bulk or Mass Effect: Part 2	218
		Emergency Access Procedures: Part 3	218
		Surgical Biopsies in the Management of BMT Patients: Part 4	218
	Emergencies Related to Chemotherapy		219
	Introduction		219
		Neutropenic Colitis (Typhlitis)	219
		Pancreatitis	224
		Cholelithiasis and Cholecystitis	225
		Gastrointestinal Haemorrhage	225
		Anorectal Complications	226
		Haemorrhagic Cystitis	227
		Invasive Fungal Infections (Invasive Aspergillosis)	228
	Extremities		229
	Emergencies Related to Tumour Bulk		232
		Bowel Obstruction	232
		Bowel Gangrene and Perforation	233
		Intussusception	235
		Ovarian Tumour Torsion	237
		Rupture of Renal and Liver Tumours as Emergencies	239
		Haemoperitoneum	240
		Urinary Tract Obstruction	240
		Spinal Cord Compression	242
		Ventilatory Compromise	244
		Take Home Messages	244
	Emergency Access Procedures:		248
		Tube or Pigtail Thoracostomy	248
		Central Venous Line Complications	249
		Insertion of Dialysis Catheter	250
		Vascular Access	252
		Tracheostomy Insertion in Certain Emergency Situations	252
	Take Home Messages		253
	Surgical Biopsies in the Management of BMT Patients		256
FERTILITY PRESERVATION IN CHILDREN			258
	Epidemiology		258
	Induced Infertility Mechanisms		258
	Preoperative Workup		258
FERTILITY PRESERVATION IN GIRLS			259
	Ovarian and Genital Tract Sparing Surgery		259
	Ovarian Transposition (Oophoropexy)		260
	Uterine Transposition		260
	Ovarian Tissue Cryopreservation		261
FERTILITY PRESERVATION IN BOYS			261
	Testicular Sparing Surgery		261
	Testicular Shielding		262
	Testicular Transposition		262
	Semen/Testicular Cryopreservation		262
CONCLUSION			263
PAEDIATRIC SURGICAL ONCOLOGY AND PALLIATIVE CARE			267