Tumori kosti u djece: učestalost, dijagnoza, kirurško liječenje i komplikacije

Bone tumor treatment changed considerably during past decades. Due to improvements in basic research in the areas of tumor biology, tumor detection and imaging, surgical procedures and development of modern surgical instruments, discovery and use of new technical equipment, and especially in the use of chemotherapy and radiotherapy, significant progress has been achieved. About 100 new patients with primary bone tumor (both benign and malignant) are discovered in Croatia and treated at the Department of Orthopedic Surgery, Clinical Hospital Center Zagreb and Zagreb University School of Medicine per year, and almost a half of them (45%) are younger than 20 years. The most common benign bone tumors are osteochondroma, simple (juvenile) bone cyst, and osteoid osteoma, and the most common malignant bone tumors are osteosarcoma and Ewing’s sarcoma. Bone tumors are localized mostly in the long bones (femur, tibia, humerus), and the knee joint region is the most common site of tumors. Patient history and examination with laboratory and imaging methods remain the essence in tumor diagnosis. A conventional X-ray examination must be the first and the most important part in tumor imaging, followed by bone scan, CT, MRI, ultrasound, and histological or cytological analysis. The biopsy, as the most accurate method in tumor diagnosis must be performed as the last diagnostic procedure. Cytological analysis is useful in tumors with soft tissue. An individual approach to patient is essential in order to choose the most suitable surgical treatment for bone tumor. Depending on the tumor type, location and other patient-related risk factors, one of the various forms of surgical therapy should be chosen. Limb salvage surgery means “en-bloc” tumor resection with the affected part of the bone through a healthy tissue and defect reconstruction, with preservation of the affected limb; this procedure is performed nowadays in about 85% of patients with malignant bone tumors. Reconstruction can be biologic (with bone auto- or homotransplant) or with foreign material (i.e. endoprosthesis or bone cement). Soft tissue reconstruction is a great challenge in this part of a treatment, and complications are not rare. Amputation still has and will have in the future an important place in treating patients with malignant bone tumor. Decision on additional chemo- and/or radiotherapy should be made between pediatric, oncology and orthopedic surgeons, according to international guidelines. Future devolvement in treating patients with bone tumors points into supporting and/or establishing reparative procedures. Bone and surrounding tissue regeneration is always better than defect reconstruction.Liječenje tumora kosti posljednjih se desetljeća znatno promijenilo. Zahvaljujući napretku temeljne znanosti u području biologije tumora, boljoj mogućnosti otkrivanja i snimanja tumora, kirurškim metodama i suvremenim kirurškim instrumentima, otkriću i primjeni nove tehničke opreme, a posebice kemoterapiji i radioterapiji postignuto je mnogo. U Hrvatskoj se godišnje otkrije oko 100 novih bolesnika s primarnim tumorom kosti (dobroćudnih i zloćudnih) i toliko ih se liječi u Klinici za ortopediju Kliničkog bolničkog centra Zagreb i Medicinskog fakulteta Sevučilišta u Zagrebu, a gotovo polovica tih bolesnika (45%) mla|a je od 20 godina. Najčešći dobroćudni tumori kosti su osteohondrom, jednostavna (juvenilna) koštana cista i osteoidni osteom, dok su najčešći zloćudni tumori kostiju osteosarkom i Ewingov sarkom. Tumori kostiju uglavnom se nalaze u dugim kostima (femur, tibija, humerus), a najčešće sijelo tumora je područje koljenoga zgloba. Povijest bolesti i pregled upotpunjen laboratorijskim i radiološkim nalazima i dalje su temelj za postavljanje dijagnoze tumora. Konvencionalni rtg pregled mora biti prvi i najvažniji dio snimanja tumora, nakon čega slijede scintigrafija, CT, MRI, ultrazvuk te histološka ili citološka analiza. Biopsija je najtočnija metoda u dijagnozi tumora i zadnji dijagnostički postupak koji se mora obaviti. Citološka analiza je korisna kad je riječ o tumorima mekog tkiva. Individualni pristup bolesniku nužan je za izbor najprikladnijeg oblika kirurškog liječenja tumora kosti. Izbor - kirurške terapije ovise o vrsti tumora, sijelu i ostalim čimbenicima rizika svakog pojedinog bolesnika. Kirurgija spašavanja ekstremiteta znači resekciju tumora ’en bloc’ sa zahvaćenim dijelom kosti kroz zdravo tkivo i rekonstrukcijom defekta, uz sačuvanje zahvaćenog ekstremiteta; taj se postupak danas primjenjuje u oko 85% bolesnika sa zloćudnim tumorima kosti. Rekonstrukcija se može izvesti biološki (auto- ili homotransplantatom) ili stranim materijalom (endoprotezom ili koštanim cementom). Rekonstrukcija mekog tkiva velik je izazov u tome dijelu liječenja i komplikacije nisu rijetka pojava. Amputacija još uvijek ima i ubuduće će imati važno mjesto u liječenju bolesnika sa zloćudnim tumorima kosti. Odluku o naknadnoj primjeni kemo- i/ili radioterapije zajedno trebaju donijeti pedijatar, onkolog i ortopedski kirurg u skladu s me|unarodnim smjernicama. Daljnja zadaća u liječenju bolesnika s tumorima kosti jest podupirati i/ili odrediti reparativne postupke. Regeneracija kosti i okolnoga tkiva uvijek je bolja solucija od rekonstrukcije defekta

Tumori kosti u djece: učestalost, dijagnoza, kirurško liječenje i komplikacije

Bone tumor treatment changed considerably during past decades. Due to improvements in basic research in the areas of tumor biology, tumor detection and imaging, surgical procedures and development of modern surgical instruments, discovery and use of new technical equipment, and especially in the use of chemotherapy and radiotherapy, significant progress has been achieved. About 100 new patients with primary bone tumor (both benign and malignant) are discovered in Croatia and treated at the Department of Orthopedic Surgery, Clinical Hospital Center Zagreb and Zagreb University School of Medicine per year, and almost a half of them (45%) are younger than 20 years. The most common benign bone tumors are osteochondroma, simple (juvenile) bone cyst, and osteoid osteoma, and the most common malignant bone tumors are osteosarcoma and Ewing’s sarcoma. Bone tumors are localized mostly in the long bones (femur, tibia, humerus), and the knee joint region is the most common site of tumors. Patient history and examination with laboratory and imaging methods remain the essence in tumor diagnosis. A conventional X-ray examination must be the first and the most important part in tumor imaging, followed by bone scan, CT, MRI, ultrasound, and histological or cytological analysis. The biopsy, as the most accurate method in tumor diagnosis must be performed as the last diagnostic procedure. Cytological analysis is useful in tumors with soft tissue. An individual approach to patient is essential in order to choose the most suitable surgical treatment for bone tumor. Depending on the tumor type, location and other patient-related risk factors, one of the various forms of surgical therapy should be chosen. Limb salvage surgery means “en-bloc” tumor resection with the affected part of the bone through a healthy tissue and defect reconstruction, with preservation of the affected limb; this procedure is performed nowadays in about 85% of patients with malignant bone tumors. Reconstruction can be biologic (with bone auto- or homotransplant) or with foreign material (i.e. endoprosthesis or bone cement). Soft tissue reconstruction is a great challenge in this part of a treatment, and complications are not rare. Amputation still has and will have in the future an important place in treating patients with malignant bone tumor. Decision on additional chemo- and/or radiotherapy should be made between pediatric, oncology and orthopedic surgeons, according to international guidelines. Future devolvement in treating patients with bone tumors points into supporting and/or establishing reparative procedures. Bone and surrounding tissue regeneration is always better than defect reconstruction.Liječenje tumora kosti posljednjih se desetljeća znatno promijenilo. Zahvaljujući napretku temeljne znanosti u području biologije tumora, boljoj mogućnosti otkrivanja i snimanja tumora, kirurškim metodama i suvremenim kirurškim instrumentima, otkriću i primjeni nove tehničke opreme, a posebice kemoterapiji i radioterapiji postignuto je mnogo. U Hrvatskoj se godišnje otkrije oko 100 novih bolesnika s primarnim tumorom kosti (dobroćudnih i zloćudnih) i toliko ih se liječi u Klinici za ortopediju Kliničkog bolničkog centra Zagreb i Medicinskog fakulteta Sevučilišta u Zagrebu, a gotovo polovica tih bolesnika (45%) mla|a je od 20 godina. Najčešći dobroćudni tumori kosti su osteohondrom, jednostavna (juvenilna) koštana cista i osteoidni osteom, dok su najčešći zloćudni tumori kostiju osteosarkom i Ewingov sarkom. Tumori kostiju uglavnom se nalaze u dugim kostima (femur, tibija, humerus), a najčešće sijelo tumora je područje koljenoga zgloba. Povijest bolesti i pregled upotpunjen laboratorijskim i radiološkim nalazima i dalje su temelj za postavljanje dijagnoze tumora. Konvencionalni rtg pregled mora biti prvi i najvažniji dio snimanja tumora, nakon čega slijede scintigrafija, CT, MRI, ultrazvuk te histološka ili citološka analiza. Biopsija je najtočnija metoda u dijagnozi tumora i zadnji dijagnostički postupak koji se mora obaviti. Citološka analiza je korisna kad je riječ o tumorima mekog tkiva. Individualni pristup bolesniku nužan je za izbor najprikladnijeg oblika kirurškog liječenja tumora kosti. Izbor - kirurške terapije ovise o vrsti tumora, sijelu i ostalim čimbenicima rizika svakog pojedinog bolesnika. Kirurgija spašavanja ekstremiteta znači resekciju tumora ’en bloc’ sa zahvaćenim dijelom kosti kroz zdravo tkivo i rekonstrukcijom defekta, uz sačuvanje zahvaćenog ekstremiteta; taj se postupak danas primjenjuje u oko 85% bolesnika sa zloćudnim tumorima kosti. Rekonstrukcija se može izvesti biološki (auto- ili homotransplantatom) ili stranim materijalom (endoprotezom ili koštanim cementom). Rekonstrukcija mekog tkiva velik je izazov u tome dijelu liječenja i komplikacije nisu rijetka pojava. Amputacija još uvijek ima i ubuduće će imati važno mjesto u liječenju bolesnika sa zloćudnim tumorima kosti. Odluku o naknadnoj primjeni kemo- i/ili radioterapije zajedno trebaju donijeti pedijatar, onkolog i ortopedski kirurg u skladu s me|unarodnim smjernicama. Daljnja zadaća u liječenju bolesnika s tumorima kosti jest podupirati i/ili odrediti reparativne postupke. Regeneracija kosti i okolnoga tkiva uvijek je bolja solucija od rekonstrukcije defekta

GIANT CELL TUMOR OF BONE: RESULTS AND TREATMENT COMPLICATIONS

Gigantocelularni tumor kosti (GCT) je najčešće benigni, lokalno agresivan tumor sklon recidiviranju. Liječenje GCT je u pravilu kirurško, a kirurški pristup određuje lokalizacija i lokalna agresivnost tumora. U Klinici za ortopediju KBC-a Zagreb je od 1995. do 2009. godine zbog GCT liječeno 39 bolesnika. Četiri bolesnika su izgubljena iz praćenja. U bolesnika s nižim stupnjem agresivnosti (n=12, 34 %) učinjena je marginalna resekcija, dok je u bolesnika s agresivnim GCT učinjena resekcija tumora u bloku i rekonstrukcija endoprotezom ili koštanim presatkom (n=22, 63 %). U jednog je bolesnika liječenje provedeno zračenjem. Komplikacije su se pojavile u trećine bolesnika. Najčešće su komplikacije bile lokalni recidiv tumora (n=6, 50 % komplikacija) i infekcija (n=2, 17% komplikacija). Amputacija je učinjena u dva pacijenta u kojih je u podlozi GCT otkriven osteosarkom. Ne računajući primarnu biopsiju, u 35 bolesnika učinjeno je ukupno 84 kirurških zahvata. Liječenje GCT je složeno, uz veliku mogućnosti komplikacija. Preporuča se multidisciplinarni pristup liječenju u specijaliziranim ortopedskim centrima s timom iskusnih ortopeda onkologa.Giant cell tumor of bone (GCT) is mostly benign, locally aggressive tumor with a high recurrence rate. GCT is treated primarily surgically, and the approach is determined according to localization and local tumor behavior. The aim of this study was to analyze results and complications of surgical treatment of GCT at a tertiary orthopedic clinical center in Croatia. We analyzed all patients treated at University Department of Orthopedics, Zagreb University Hospital Center, during a 15-year period. From 1995 to 2009, 39 patients were surgically treated for GCT. Four patients were lost from follow up. In patients with low-grade GCT (n=12, 34%), we performed marginal-intralesional resection, whereas in patients with locally aggressive GCT we performed en bloc resection and reconstruction with tumor endoprosthesis or bone allograft (n=22, 63%). In one patient, the only treatment was tumor irradiation. Complications were evident in one-third of our patients. The most common complications were tumor recurrence (n=6, 50% of all complications) and deep infection (n=2, 17% of all complications). We performed amputation in two patients in whom osteosarcoma was revealed under GCT radiologic and histologic appearance. We performed 84 operations in 35 patients, not counting primary biopsy. In conclusion, treatment of GCT is complex, with a high incidence of tumor recurrence. Diagnosis and treatment are best provided through a multidisciplinary approach in highly specialized centers for orthopedic oncology

GIANT CELL TUMOR OF BONE: RESULTS AND TREATMENT COMPLICATIONS

Gigantocelularni tumor kosti (GCT) je najčešće benigni, lokalno agresivan tumor sklon recidiviranju. Liječenje GCT je u pravilu kirurško, a kirurški pristup određuje lokalizacija i lokalna agresivnost tumora. U Klinici za ortopediju KBC-a Zagreb je od 1995. do 2009. godine zbog GCT liječeno 39 bolesnika. Četiri bolesnika su izgubljena iz praćenja. U bolesnika s nižim stupnjem agresivnosti (n=12, 34 %) učinjena je marginalna resekcija, dok je u bolesnika s agresivnim GCT učinjena resekcija tumora u bloku i rekonstrukcija endoprotezom ili koštanim presatkom (n=22, 63 %). U jednog je bolesnika liječenje provedeno zračenjem. Komplikacije su se pojavile u trećine bolesnika. Najčešće su komplikacije bile lokalni recidiv tumora (n=6, 50 % komplikacija) i infekcija (n=2, 17% komplikacija). Amputacija je učinjena u dva pacijenta u kojih je u podlozi GCT otkriven osteosarkom. Ne računajući primarnu biopsiju, u 35 bolesnika učinjeno je ukupno 84 kirurških zahvata. Liječenje GCT je složeno, uz veliku mogućnosti komplikacija. Preporuča se multidisciplinarni pristup liječenju u specijaliziranim ortopedskim centrima s timom iskusnih ortopeda onkologa.Giant cell tumor of bone (GCT) is mostly benign, locally aggressive tumor with a high recurrence rate. GCT is treated primarily surgically, and the approach is determined according to localization and local tumor behavior. The aim of this study was to analyze results and complications of surgical treatment of GCT at a tertiary orthopedic clinical center in Croatia. We analyzed all patients treated at University Department of Orthopedics, Zagreb University Hospital Center, during a 15-year period. From 1995 to 2009, 39 patients were surgically treated for GCT. Four patients were lost from follow up. In patients with low-grade GCT (n=12, 34%), we performed marginal-intralesional resection, whereas in patients with locally aggressive GCT we performed en bloc resection and reconstruction with tumor endoprosthesis or bone allograft (n=22, 63%). In one patient, the only treatment was tumor irradiation. Complications were evident in one-third of our patients. The most common complications were tumor recurrence (n=6, 50% of all complications) and deep infection (n=2, 17% of all complications). We performed amputation in two patients in whom osteosarcoma was revealed under GCT radiologic and histologic appearance. We performed 84 operations in 35 patients, not counting primary biopsy. In conclusion, treatment of GCT is complex, with a high incidence of tumor recurrence. Diagnosis and treatment are best provided through a multidisciplinary approach in highly specialized centers for orthopedic oncology

Osteoid osteoma of the foot : Presentation, treatment and outcome of a multicentre cohort

Funding Information: The current study did not receive any funding. Publisher Copyright: © 2021, The Author(s).Background: Osteoid osteomas of the foot are rare, with a varying and atypical clinical as well as radiological presentation impeding early diagnosis and treatment. The aim of the present multicentre study was to 1) analyze epidemiological, clinical and radiological findings of patients with foot osteoid osteomas and to 2) deduce a diagnostic algorithm based on the findings. Methods: A total of 37 patients (25 males, 67.6%, mean age 23.9 years, range 8–57 years) with osteoid osteomas of the foot were retrospectively included, treated between 2000 and 2014 at 6 participating tertiary tumor centres. Radiographic images were analyzed, as were patients’ minor and major complaints, pain relief and recurrence. Results: Most osteoid osteomas were located in the midfoot (n = 16) and hindfoot (n = 14). Painful lesions were present in all but one patient (97.3%). Symptom duration was similar for hindfoot and midfoot/forefoot (p = 0.331). Cortical lesions required fewer x‑rays for diagnosis than lesions at other sites (p = 0.026). A typical nidus could be detected in only 23/37 of x‑rays (62.2%), compared to 25/29 CT scans (86.2%) and 11/22 MRIs (50%). Aspirin test was positive in 18/20 patients (90%), 31 patients (83.8%) underwent open surgery. Pain relief was achieved in 34/36 patients (outcome unknown in one), whilst pain persisted in two patients with later confirmed recurrence. Conclusions: As previously reported, CT scans seem to be superior to MRIs towards detection of the typical nidus in foot osteoid osteomas. In patients with unclear pain of the foot and inconclusive x‑rays, osteoid osteoma should be considered as differential diagnosis.publishersversionPeer reviewe

Molecular profiling of soft-tissue sarcomas with FoundationOne® Heme identifies potential targets for sarcoma therapy: a single-centre experience

Background: Molecular diagnosis has become an established tool in the characterisation of adult soft-tissue sarcomas (STS). FoundationOne ® Heme analyses somatic gene alterations in sarcomas via DNA and RNA-hotspot sequencing of tumour-associated genes. Methods: We evaluated FoundationOne ® Heme testing in 81 localised STS including 35 translocation-associated and 46 complex-karyotyped cases from a single institution. Results: Although FoundationOne ® Heme achieved broad patient coverage and identified at least five genetic alterations in each sample, the sensitivity for fusion detection was rather low, at 42.4%. Nevertheless, potential targets for STS treatment were detected using the FoundationOne ® Heme assay: complex-karyotyped sarcomas frequently displayed copy-number alterations of common tumour-suppressor genes, particularly deletions in TP53 , NF1 , ATRX , and CDKN2A . A subset of myxofibrosarcomas (MFS) was amplified for HGF ( n  = 3) and MET ( n  = 1). PIK3CA was mutated in 7/15 cases of myxoid liposarcoma (MLS; 46.7%). Epigenetic regulators (e.g. MLL2 and MLL3 ) were frequently mutated. Conclusions: In summary, FoundationOne ® Heme detected a broad range of genetic alterations and potential therapeutic targets in STS (e.g. HGF/MET in a subset of MFS, or PIK3CA in MLS). The assay’s sensitivity for fusion detection was low in our sample and needs to be re-evaluated in a larger cohort