Pediatric soft tissue tumor surgery removes lumps that grow in the fat, muscle and nerves of children, from benign lipoblastomas to rhabdomyosarcoma and other sarcomas. Every deep or growing lump is imaged with MRI and biopsied before removal, so that the first operation is the right one. At Biruni Hospital in Istanbul, our pediatric surgeons plan surgery, sentinel node biopsy and reconstruction at the tumor board and protect function in a growing child.
About This Department
Pediatric Surgery
Most lumps under a child's skin are harmless. The few that are sarcomas need a planned operation from the very first cut.
Pediatric soft tissue tumor surgery treats lumps that grow in the fat, muscle, nerves and connective tissue of babies, children and teenagers, from benign lipoblastomas and fibrous tumors to rhabdomyosarcoma and other soft tissue sarcomas. Planning comes first. In an Indian cancer center, nearly half of children referred with a non-rhabdomyosarcoma sarcoma had already had the lump removed elsewhere without planning, and tumor remained in 45.7 percent of those who underwent re-excision (Qureshi and colleagues, Journal of Pediatric Surgery, 2017). Results after a planned operation are good. In a Children's Oncology Group study of 529 young patients, five-year survival reached 96.2 percent in low risk non-rhabdomyosarcoma sarcomas, and most were cured by surgery alone (Spunt and colleagues, Lancet Oncology, 2020). This page explains benign lumps, warning signs, imaging and biopsy, rhabdomyosarcoma, other sarcomas, desmoid tumors, lymph nodes, reconstruction, follow-up, and how families from abroad arrange soft tissue tumor surgery for a child at Biruni Hospital in Istanbul.
96
Percent five-year survival in low risk non-rhabdomyosarcoma sarcomas treated by risk group
46
Percent of children with tumor left behind after an unplanned excision elsewhere
17
Percent of limb rhabdomyosarcomas whose stage changed after sentinel node biopsy
Free
Review of your child's MRI and pathology by our pediatric tumor board
Limbs, trunk, head and neck, the bladder and prostate, the genital area and deep in the abdomen or pelvis.
How common
Soft tissue sarcomas make up around seven percent of childhood cancers, and rhabdomyosarcoma accounts for half of them. Benign soft tissue lumps are far more common.
Vascular birthmarks and lymphatic malformations are soft tissue lumps too, and many need no surgery. We treat them through a separate vascular anomalies pathway.
Benign lumps
Common benign lumps
Does a benign lump need removal?
Not always. Small, typical lumps confirmed on ultrasound can be watched. We remove benign lumps that grow, cause pain, press on nerves or vessels, limit movement or bother the child, and lipoblastomas are removed completely to prevent regrowth.
Benign soft tissue lumps
Lump
Typical age
Treatment
Lipoblastoma
Under three years
Complete excision
Dermoid cyst
Infants, near the eyebrow
Excision
Fibrous hamartoma of infancy
First two years
Excision
Neurofibroma
Any age, more with NF1
Excision if growing or painful
A benign diagnosis rests on imaging and, for anything deep or unusual, on a biopsy. We treat every deep, growing lump as a possible sarcoma until proven otherwise.
Warning signs
When to see a specialist
Most lumps that parents notice are lymph nodes, cysts or benign fat tumors. A few features point to a sarcoma and call for a specialist review within two weeks.
Signs that need specialist assessment
Sign
Why it matters
Next step
Larger than five centimeters
Higher chance of sarcoma
MRI
Deep to the muscle covering
Benign lumps sit closer to the skin
MRI and biopsy
Growing over weeks
Active tumor
Review within days
Lump in the bladder area, vagina or behind the eye
Typical rhabdomyosarcoma sites
Same week
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Painless does not mean harmless
Many sarcomas are painless. Size, depth and growth matter more than pain.
Blood from the vagina in a young girl, urinary blockage or a bulging eye need assessment the same week.
Ultrasound is the first test, and MRI follows for any lump with a warning sign.
Biopsy before removal
A sarcoma should never be shelled out.
When a lump is removed without imaging and a biopsy first, the surgeon cuts through the tumor or close to it, spreads cells into the surrounding tissue and leaves no clear margin, and a second, wider operation, sometimes with radiotherapy, then becomes necessary to clean the area, removing more tissue than a planned operation would have taken at the start. In the Indian series, 40 of 84 children with non-rhabdomyosarcoma sarcomas arrived after an unplanned excision, and 45.7 percent of those re-operated still had tumor in the scar (Qureshi and colleagues, 2017). Re-excision brought outcomes back in line with planned surgery.
MRI first. Biopsy second. Operation third.
Imaging and biopsy
Tests before soft tissue tumor surgery
Test
Purpose
Note
MRI with contrast
Size, depth, nerves and vessels
Key test for planning
Core needle biopsy
Diagnosis and molecular tests
Through the line of the future incision
Chest CT
Lung metastases
For sarcomas
PET-CT
Nodes and distant spread
Rhabdomyosarcoma and high grade sarcomas
The biopsy track is placed where the later operation will remove it, so no tumor cells are left behind in untouched tissue. The surgeon who will operate plans the biopsy with the radiologist.
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Molecular tests
Fusion genes such as PAX3-FOXO1 in rhabdomyosarcoma, SS18 in synovial sarcoma and NTRK in infantile fibrosarcoma decide risk and sometimes allow targeted drugs.
Results reach the tumor board within one to two weeks, and the treatment plan follows.
Rhabdomyosarcoma
The most common sarcoma of childhood
Maintenance chemotherapy in high risk rhabdomyosarcoma, 371 patients
Outcome
With maintenance
Without
Five-year disease-free survival
77.6 percent
69.8 percent
Five-year overall survival
86.5 percent
73.7 percent
Treatment
Six months of vinorelbine and oral cyclophosphamide
Stop after standard therapy
Rhabdomyosarcoma arises from cells that form muscle. Treatment combines chemotherapy for every child with surgery, radiotherapy or both for local control. In the European RMS 2005 trial, six months of low dose maintenance chemotherapy after standard treatment improved five-year survival in high risk patients (Bisogno and colleagues, Lancet Oncology, 2019).
Embryonal rhabdomyosarcoma, the more common type, has a better outlook than the alveolar type. Site, size, age and lymph nodes also shape the risk group.
Local control of rhabdomyosarcoma
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The principle
Remove the tumor completely when this is possible without loss of function or appearance. When it is not, chemotherapy shrinks the tumor first, and radiotherapy or delayed surgery follows.
A small tumor of the limb or trunk may be removed at diagnosis. Tumors of the bladder, prostate, vagina or head and neck are treated first with chemotherapy, and organ sparing surgery or radiotherapy follows, so that bladder function, fertility and facial appearance are protected.
Will our child need radiotherapy?
Many children do, as part of local control, especially for alveolar tumors, head and neck sites and tumors that could not be removed with clear margins. Surgery after chemotherapy can lower the radiotherapy dose. The tumor board decides on the combination for each child.
Local control by site
Site
First step
Local treatment
Limb or trunk, small
Surgery or biopsy
Wide excision, radiotherapy if margins involved
Bladder and prostate
Chemotherapy
Partial cystectomy, radiotherapy or brachytherapy
Vagina and uterus
Chemotherapy
Limited surgery, often no radiotherapy
Head and neck
Chemotherapy
Radiotherapy, selected surgery
Lymph nodes and the sentinel node
Lymph node spread changes the risk group and the treatment. Nodes are checked in every child with rhabdomyosarcoma of the limbs and in boys over ten with tumors near the testis.
Sentinel node procedure in limb rhabdomyosarcoma, 55 patients
Finding
Result
Meaning
Sentinel node found
All patients
Feasible in children
Stage changed
17.0 percent
Different treatment
False negative rate
10.5 percent
Combined with scans
A sentinel node is the first lymph node that drains the tumor. Finding and removing it gives more accurate staging than scans alone.
How the sentinel node is found
Step
How
When
Tracer injection
Radioactive tracer and blue or fluorescent dye
Day of surgery
Mapping
Scan and handheld probe
Before the cut
Removal
Small separate incision
Same operation
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Evidence
A systematic review and cohort study recommended the sentinel node procedure for children with limb rhabdomyosarcoma, alongside clinical and scan assessment (Jeremiasse and colleagues, Annals of Surgical Oncology, 2021).
Our nuclear medicine unit performs tracer mapping, and the surgeon removes the sentinel node through a small incision under the same anesthetic as the biopsy or tumor removal.
Other soft tissue sarcomas
Risk groups in 529 young patients with non-rhabdomyosarcoma sarcomas
Risk group
Five-year event-free survival
Five-year overall survival
Low, resected
88.9 percent
96.2 percent
Intermediate, large high grade or unresected
65.0 percent
79.2 percent
High, metastatic
21.2 percent
35.5 percent
Synovial sarcoma, malignant peripheral nerve sheath tumor, infantile fibrosarcoma and other rare sarcomas depend more on surgery than rhabdomyosarcoma does. Low risk tumors removed with clear margins were cured by surgery alone in most children in the American study, sparing them chemotherapy and radiotherapy (Spunt and colleagues, 2020). Large high grade tumors receive chemotherapy and radiotherapy before surgery.
What is different about infantile fibrosarcoma?
This tumor of babies grows fast but rarely spreads. Most carry an NTRK gene fusion, and targeted drugs such as larotrectinib can shrink it dramatically, which allows smaller, function sparing operations or avoids surgery in some infants.
Desmoid tumors
The European conservative strategy for desmoid tumors, 173 young patients
First approach
Share of patients
Five-year progression-free survival
Wait and see
35 percent
26.7 percent
Immediate surgery
31 percent
41.2 percent
Immediate chemotherapy
34 percent
42.8 percent
Desmoid tumors grow locally and never spread, but they can return after surgery. Some stop growing or shrink by themselves. The European pediatric group recommends watching first, surgery only when it does not harm function, and low toxicity chemotherapy for growth, and all but one patient were alive at analysis (Orbach and colleagues, Lancet Child and Adolescent Health, 2017).
Our approach
We watch a desmoid tumor with MRI every three months at first, and we avoid disfiguring or disabling surgery, because the tumor does not threaten life.
Reconstruction and function
Plastic surgeons cover large defects with local or free tissue flaps.
Nerve and tendon transfers restore movement where needed.
Limb sparing surgery is the rule, and amputation is rare.
1
Planned together
The tumor surgeon and the plastic surgeon plan the resection and reconstruction at the same time, so a wide margin does not leave a poor result.
Children grow, so the reconstruction must grow with them, and in young children we prefer living tissue, such as flaps from their own body, to implants.
Rehabilitation
Physiotherapists and occupational therapists start work the day after surgery. Children with limb operations receive a home program, splints when needed and regular reviews until they regain full use.
Microsurgery, used for free flaps and nerve repair, has its own page on this site.
Recovery and follow-up
Follow-up after soft tissue sarcoma treatment
Test
How often
Purpose
MRI of the tumor site
Every three months in year one
Local return
Chest imaging
Every three to six months at first
Lung metastases
Clinic review
Every three months, then less often
Growth, function, late effects
Most children leave hospital within two to five days after soft tissue tumor surgery, and longer after reconstruction.
Long-term checks
1
Late effects
Growth of a treated limb, heart function after doxorubicin, kidney function after ifosfamide, fertility and second tumors in the radiotherapy field are checked through childhood.
Pathology results arrive within one to two weeks after surgery, and the tumor board meets again to decide on further treatment.
Life after a soft tissue tumor
Children return to school and sport.
Most children treated for a soft tissue tumor live full lives, and even after sarcoma treatment with surgery, chemotherapy and radiotherapy they play, study and grow, while a few adjust to a scar, a weaker muscle or a shorter limb with the help of physiotherapy, prosthetic advice and psychological support, and our long-term follow-up clinic hands over a written treatment summary when the young person moves to adult care.
Function matters as much as cure.
For families from abroad
Before you travel
Send MRI images with reports, biopsy or surgery pathology reports and slides, and any operation notes. If a lump has already been removed elsewhere, send that report and photographs of the scar. Planning the re-excision depends on them.
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Length of stay
Plan for one to two weeks for biopsy and staging, and two to three weeks after a tumor operation, longer after reconstruction. Fitness to fly follows a wound check.
Children needing chemotherapy can receive it in Istanbul or at home on the same protocol, with surgery and radiotherapy planned here.
2
After you return home
Scans and blood tests continue with your local team, and our tumor board reviews results by video and message.
Support in Istanbul
One coordinator from the international patients office handles arrangements from the first message through discharge and answers the same WhatsApp number after you return. The office works in English, Arabic, French, Russian, Serbian, Romanian and Spanish and books interpreters for other languages, and it arranges the visa invitation letter, airport transfers, daily transport and accommodation near the hospital. Halal, vegetarian and diabetic meals, a prayer room on site, and a female physician on request wherever the rota allows.
Second opinions
A pathology review of slides before travel confirms the diagnosis or changes it in some cases, and we arrange it on request.
Cost
What a quote for soft tissue tumor surgery covers
Item
Included
Priced separately
Surgery
Surgeon, anesthesia, operating room, pathology
Reconstruction by plastic surgery
Hospital
Ward stay as planned
Extra days
Aftercare
Medicines at discharge, video follow-up
Chemotherapy, radiotherapy
Removal of a benign lump costs far less than sarcoma treatment, which combines several treatments over months.
1
Written quote
Sent after the tumor board review of MRI and pathology, before you travel.
The quote lists surgery, sentinel node mapping, reconstruction, chemotherapy and radiotherapy separately, so that families can see what each part of the plan costs.
Compare quotes for the same plan, and check that tumor board review and follow-up are included.
References
Spunt SL, Million L, Chi YY, et al. A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332), a Children's Oncology Group prospective study. Lancet Oncol. 2020;21(1):145-161.
Bisogno G, De Salvo GL, Bergeron C, et al. Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005), a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol. 2019;20(11):1566-1575.
Qureshi SS, Prabhu A, Bhagat M, et al. Re-excision after unplanned resection of nonmetastatic nonrhabdomyosarcoma soft tissue sarcoma in children, comparison with planned excision. J Pediatr Surg. 2017;52(8):1340-1343.
Jeremiasse B, van der Steeg AFW, Fiocco M, et al. Value of the sentinel node procedure in pediatric extremity rhabdomyosarcoma, a systematic review and retrospective cohort study. Ann Surg Oncol. 2021;28(13):9048-9059.
Orbach D, Brennan B, Bisogno G, et al. The EpSSG NRSTS 2005 treatment protocol for desmoid-type fibromatosis in children, an international prospective case series. Lancet Child Adolesc Health. 2017;1(4):284-292.
Editor's note
Written by the Biruni Hospital medical editorial team. Reviewed by Assoc. Prof. Dr. Aysel TAKTAK, Pediatric Nephrology.
