Pediatric Soft Tissue Tumor Surgery
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
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.
What soft tissue tumors are
| Group | Examples | Behavior |
|---|---|---|
| Benign | Lipoma, lipoblastoma, fibrous hamartoma, nerve sheath tumors | Do not spread |
| Intermediate | Desmoid tumor, infantile fibrosarcoma, dermatofibrosarcoma | Grow locally, rarely spread |
| Malignant | Rhabdomyosarcoma, synovial sarcoma, malignant nerve sheath tumor | Can spread to nodes and lungs |
Where they grow
How 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?
| 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 |
Warning signs
When to see a specialist
| 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 |
Blood from the vagina in a young girl, urinary blockage or a bulging eye need assessment the same week.
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
| 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 |
Rhabdomyosarcoma
The most common sarcoma of childhood
| 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 |
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
Will our child need radiotherapy?
| 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.
| 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.
| 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 |
Other soft tissue 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 |
What is different about infantile fibrosarcoma?
Desmoid tumors
| 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 |
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.
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
Microsurgery, used for free flaps and nerve repair, has its own page on this site.
Recovery and follow-up
| 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
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
Children needing chemotherapy can receive it in Istanbul or at home on the same protocol, with surgery and radiotherapy planned here.
Support in Istanbul
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
| 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.
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 Assistant Professor Ömer DOĞRU, Pediatric Hematology and Oncology.
Medically reviewed by
Assistant Professor Ömer DOĞRU
Pediatric Hematology and Oncology
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