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Sarcomas-Recognition and referral

Rare cancers – heterogeneous group of malignant neoplasms arising from mesenchymal cells -can happen in any anatomical site but happens more frequently in connective tissues They are complex and rare hence difficult to study and manage Several databases have been developed which are long – term institutional , multi-institutional and national which collect epidemiological and clinical data on sarcomas WHO has recognised over 70 histotopes of sarcoma with two main categories – Soft tissue sarcoma ( STS ) and Bone sarcoma ( BS ) In Europe SST are the predominant type and constitute about 84 % of all the sarcomas diagnosed Amongst the bone sarcomas Osteosarcoma is the most frequent bone sarcoma overall.

Seen more frequently in young adults and adolescents compared to other cancers They make up < than 1 % of all cancers There is a male preponderance Most cases the aetiology is unknown , certain genetic associations are known for e.g risk of malignant nerve sheath tumour ( MPNST ) in individuals with familial neurofibromatosis due to mutation in the NF1 gene Soft tissue sarcoma -rare malignancy- derived embryologically from the mesenchyme often form in muscles , joints , fat , nerves , seep skin tissues and blood vessels commonly seen in lower extremities and trunk ( lower limbs account for up to 36.6 % of cases ) STS can present at any age although most common in middle ages and older adults , they are relatively more common in children and young adults accounting for 7 % to 10 % of paediatric malignancies dozens of subtypes of STS are known arising from cartilage , muscle , blood vessels , nerves and fat classification is based on the mature tissue that they resemble with WHO classification containing nearly 100 histologic types About 11,000 new cases seen in US / each year ( Eyal et al 2017 ) can present at any age but the median age of diagnosis is 56-65 yrs peaking in the 8th decade male preponderance soft tissue lumps are common but STS account for only 1 % of adult cancers a family doctor is likely to see 1 case of STS for every 24 yrs of practice distinguishing between benign and malignant lumps can be difficult a suspicious lump is any lump that is (1 ) deep to the fascia (2 ) > 5 cm (3 ) increasing in size 
(4) painful the more of these clinical features present , greater the risk of malignancy with increasing size being the best individual indicator can happen anywhere but more commonly found in lower extremities with thigh being the most common site of disease expectedly the initial presenting symptoms of a STS may vary based on the site of the tumour , subtype[e and grade

Since the publication of NICE guidance on sarcoma in 2006 -specialised centralised service centers have been established NICE has produced updated guideline for primary care in 2015 for early diagnosis of STS

Consider an urgent direct access US scan within 2 weeks-n adults with an un-explained lump that is increasing in size

Consider a USC referral to be seen within 2 weeks if US scan findings are suggestive of STS or if the US findings are uncertain and clinical concerns persist.

multidisciplinary team decision making triple assessment ie history , imaging and biopsy Plain radiographs can reveal bone remodelling , bone invasion and soft tissue calcification or ossification US – readily accessible and can confirm the presence and evaluate the size , depth of the soft tissue mass and help with core-biopsy sampling If malignancy is suspected – MRI ( Gold-standard ) and CT and PET Pathological assessment is necessary before definitive treatment Surgical resection with or without adjuvant radiation with aim of limb salvage

multidisciplinary team decision making ( MDT’s made of radiologists , surgeons , medical and clinical oncologists .pathologists , specialist nurses ) the goal is to provide the patient with a functional extremity without local tumour relapse surgical resection with or without adjuvant radiation with aim of limb salvage

Bone sarcoma -Bone sarcomas constitute about 14 % of of sarcomas diagnosed in Europe Osteosarcomas and chondrosarcomas are the most commonly diagnosed malignant bone tumours WHO has determined 6 categories of Primary bone cancers , chondrogenic , osteogenic , notochondral , vascular and other malignant mesenchymal and miscellaneous ( including Ewing’s sarcoma ) Osteosarcoma is the most common malignancy in the paediatric population No clear defined cause but certain factors increase the likelihood of primary bone cancers as
○ previous treatment involving radiation , chemotherapy or stem cell transplantation
○ certain inherited conditions as children who had hereditary retinoblastoma , members with Li- Fraumeni syndrome
○ people aged over 40 with Paget’s disease are at increased risk of developing osteosarcoma NCRAS ( National Cancer Registration and Analysis Service ) of UK reports that
○ primary bone tumours are exceptionally rare 
○ they affect males more than females
○ they represent 4 % of all malignancies in children aged up to 14 yrs.

Osteosarcoma -highly malignant tumour that can develop in any bone reported as 3rd most common cancer in adolescence after lymphoma and brain tumours mostly seen in areas of high bone turnover as distal femur , proximal tibia and proximal humerus they can be primary ( no underlying bone pathology ) or secondary forms
 ( underlying bone pathology that has undergone malignant degeneration / conversion ) a bimodal peak is seen with 1st peak in 10-14 age group 
( pubertal growth spurt ) & the next observable peak in adults over 65 when it mostly represents 2ary cancer resulting from malignant degeneration of Paget’s disease , sites of bone infarction etc Presentation can be non-specific , often late , bone pain with activity and can often be overlooked as symptoms are attributed to injuries , arthritis or growing pains MRI is gold standard but a standard radiograph is generally the 1st line study when potential bone mass is observed during physical exam.

Chondrosarcoma -Malignant cartilage producing bone tumour , typically seen in adults 40-75 yrs of age Least common bone cancer Diverse morphological features and clinical behaviour – arise in pelvis or long bones Mostly sporadic but they can also develop from malignant transformation of osteochondromas & enchondromas Local swelling and pain are the most common presenting features A plain XR is usually the first line test.

Ewing sarcoma -High grade aggressive sarcoma belonging to the group of round cell tumours ( ie made of small round cells ) Papers quote that ES is the 2nd most common bone tumour in childhood and adolescence Median age of diagnosis is 15 yrs Presentation can be with local symptoms as tumour mass formation , induration , pain , swelling , venous dilatation and unexplained fever Laboratory changes suggestive of inflammation , pathological fractures.

NICE guidance on Bone Sarcomas -Consider suspected cancer pathway referral for adults if an XR suggests the possibility of bone sarcoma

Adults -Consider a very urgent ( appt within 48 hrs ) for specialist assessment for children and young people if an XR suggests the possibility of bone sarcoma Consider a very urgent direct access X-ray ( within 48 hrs ) to assess for bone sarcoma in children and young people with unexplained bone swelling or pain.

References

  1. Limaiem F, Davis DD, Sticco KL. Chondrosarcoma. [Updated 2020 Dec 2]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538132
  2. Ozaki, Toshifumi. “Diagnosis and treatment of Ewing sarcoma of the bone: a review article.” Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association vol. 20,2 (2015): 250-63. doi:10.1007/s00776-014-0687-z
  3. Bone Sarcomas: incidence and survival rates in England – NCIN Data Briefing Bone Sarcomas: incidence and survival rates in England (ncin.org.uk)
  4. Pullan JE, Budh DP. Primary Bone Cancer. [Updated 2021 Mar 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560830/
  5. Prater S, McKeon B. Osteosarcoma. [Updated 2021 Feb 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK549868/
  6. Misaghi, Amirhossein et al. “Osteosarcoma: a comprehensive review.” SICOT-J vol. 4 (2018): 12. doi:10.1051/sicotj/2017028
  7. Ferguson JL, Turner SP. Bone Cancer: Diagnosis and Treatment Principles. Am Fam Physician. 2018 Aug 15;98(4):205-213. PMID: 30215968. Bone Cancer: Diagnosis and Treatment Principles – PubMed (nih.gov)
  8. Vodanovich, Domagoj Ante, and Peter F M Choong. “Soft-tissue Sarcomas.” Indian journal of orthopaedics vol. 52,1 (2018): 35-44. doi:10.4103/ortho.IJOrtho_220_17
  9. Ramu, Eyal M et al. “Management of soft-tissue sarcomas; treatment strategies, staging, and outcomes.” SICOT-J vol. 3 (2017): 20. doi:10.1051/sicotj/2017010
  10. Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res. 2016 Nov 15;6:20. doi: 10.1186/s13569-016-0060-4. PMID: 27891213; PMCID: PMC5109663.
  11. Suspected cancer: recognition and referral NICE guideline [NG12]Published:  Last updated: 

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