RECURSOS RECURSOS HUMANOS

22.9.1 Parameningeal site

Complete surgical resection is difficult and generally not possible. Radiotherapy is always necessary in patients over 3 years and should be given at week 13 regardless of response to initial chemotherapy.

An initial resection will not be accepted if permanent severe uncorrectable functional dysfunction or mutilation results. In all cases where resectability is uncertain a resection should not be attempted and only a biopsy taken. Neck dissections should not be performed initially.

Only after radiotherapy a secondary resection is acceptable. Secondary resections in PM site should only be performed in centres with experience in this field.

22.9.2 Orbit

Biopsy is usually the only surgical procedure required for orbital tumours.

Secondary resections are not recommended. Enucleation or exenteration are very rarely indicated 5_. Depending on the age of the child microsurgical reconstruction with a free flap or forearm flap in combination with an appropriate prosthetic device are recommended after exenteration of the orbit.

22.9.3 Head and Neck

Complete surgical excision is difficult but major resections with reconstruction may be appropriate in some circumstances, after neoadjuvant chemotherapy. Such operations should only be realised in centres with an interdisciplinary surgical team and with experience in microsurgical free flap reconstruction.

A combination of surgery and brachytherapy (“Amore” technique) is practised in some Centres 6.

22.9.4 Bladder/Prostate

Cystoscopy should be done at diagnosis and during follow up.

Initial resection (rather than biopsy alone) should only be done in the case of very small tumours arising in the dome of the bladder, far from the trigone.

Secondary operations:

Conservative surgery of bladder /prostate tumours could be done where feasible (partial cystectomy and/or partial prostatectomy) in conjunction with brachytherapy particularly in very young boys 7,8 or external beam radiotherapy.

Partial prostatectomy, without radiotherapy, carries a high risk of local relapse9.

Where conservative treatment is not feasible, the treatment will include total cystectomy and/or total prostatectomy with or without post-operative radiotherapy.

22.9.5 Vagina

Partial vaginectomy may be feasible after chemotherapy but brachytherapy is often preferable after ovarian transposition 10.

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22.9.6 Paratesticular

These should be excised via an inguinal incision, first ligating the cord at the internal inguinal ring. Orchidectomy is essential. In rare cases, if the tumour is very large and delivery into the groin would be difficult or traumatic, it is better to make a scrotal incision (keeping the tunica vaginalis intact) and deliver the testis and cord via this.

Retroperitoneal lymphadenectomy or nodal sampling at diagnosis is not recommended unless there is uncertainty on imaging 11,12.

If the initial operation before referral was scrotal then primary re-operation should be done to excise the cord at the internal ring. Complementary hemiscrotectomy is not necessary 13,14 if the patient is upstaged, being treated according to the Subgroup B strategy (i.e. if in the Low Risk group the child will be upstaged). When there is a doubt about scrotal contamination, hemiscrotectomy should be performed.

22.9.7 Extremities

Particular attention is recommended initially in evaluating the regional lymph nodes. Upper and lower limb tumours must have surgical evaluation of axillary or inguinal nodes, respectively, even if nodes are clinically/radiologically normal. New techniques of sentinel node mapping (with blue dye and/or radioactive tracer) are recommended whenever feasible 4.

At secondary operation, formal compartmental resection (en bloc resection of the tumour and the entire compartment of origin, where tumour was entirely anatomically confined) may be appropriate for some tumours but less “anatomical” wide resections (en bloc resection through normal tissue, beyond the reactive zone, with the removal of the tumour with its pseudocapsule and a margin of normal tissue) is usually sufficient, providing an adequate margin of normal tissue. A wide cutaneous incision will be made along traditional lines (along the major axis of the tumour- bearing anatomical compartment), and must include en bloc the scar and the holes-track of previous biopsies or surgery. Once the skin-fat flaps have been prepared the tumour will be isolated within the tumour-bearing structure, with prompt recognition and careful dissection of the main vascular structures and motor nerves (femoral, sciatic, sciatic-popliteal, external/internal, median, ulnar and radial). These structures must not show tumour infiltration. Should doubt arise about a possible oedema or suspect thickening of the delimiting fascia (vascular external tunica, perineurium), it will be prudent to perform frozen section biopsy.

Care must be taken to avoid contamination of the surgical field, which can also occur if the tumour is allowed to emerge on the surface of resection. When minimal contamination has occurred, the patient will be classified as IRS group II, and complementary radiation therapy will have to be planned in any case. Once the malignancy has been isolated, it must be removed en bloc with the surrounding soft tissue, covered at every point by healthy tissue.

Compartmental operations will be performed only if made necessary by the site and dimensions of the tumour. If the lesion is near structures such as the vascular-nervous fascia or bone, it must be cautiously prepared by also removing the fascia covering these structures (vascular external tunica, perineurium or periostium). If these structures are also found to be infiltrated, they must be resected en bloc with the tumour, assessing the possibility of performing vascular, neurological or bone reconstruction as an alternative to mutilating procedures.

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Specific problems that can arise from the combination with the irradiation should be considered already at the operation planning. These are:

• disturbance of growth because of irradiation of growth plates • pathological fractures after marginal bone resection

• lymph oedema after regional lymph node dissection and nevertheless necessary irradiation, especially in the region of the shoulder and groin

• scarred contracture.

When considering radiotherapy, it should be remembered that amputation may be preferable in young children, bearing in mind the serious effects of radiation on growth and function.

22.9.8 Abdomen/Pelvis

If radiotherapy is anticipated for pelvic tumour the surgeon should consider exclusion of the ovaries from the radiotherapy field by transposition and could consider exclusion of small bowel from the pelvis by insertion of a tissue expander or absorbable mesh.