For health professionals. This section uses clinical terms and is general education for doctors, nurses and students, not advice for an individual patient. Patients and families: please read our patient guides instead, Cancer Myth Busters · Understanding your cancer report · Living through cancer treatment, or speak with your own doctor.
102 clinical-vignette questions in three levels: 34 basics, 34 applied and innovative, and 34 challenging (58 Soft tissue, 30 Bone, 14 Metastatic; 7 built on schematic imaging, endoscopy or histology drawings). Practise question by question with instant marking, or take a timed mock test with a result summary by topic, level and section. You can also print the page as a booklet.
LevelScore 0 / 0 answered
Topic
Timed mock test. Random questions, about 80 seconds each (30 questions in 40 minutes). Answers stay hidden until you submit or time runs out; then you get a score by topic, level and section.
Level 1 · Basics
Core facts for NEET-PG and USMLE Step 1–2: histology, risk factors, presentation, staging and first-line principles.
Q1Soft tissueLevel 1At a glance
Which statement about the relative frequency of sarcomas is correct?
ASoft tissue sarcomas outnumber bone sarcomas by roughly four to one
BOsteosarcoma is the commonest sarcoma in older adults
CSoft tissue and bone sarcomas are about equally common
DSarcomas account for about 10% of adult cancers
EBone sarcomas outnumber soft tissue sarcomas by roughly four to one
Show answer
Answer: A. There are more than 100 subtypes, and soft tissue sarcoma is about four times commoner than bone sarcoma. Sarcomas are only about 1% of adult cancers but roughly 1 in 7 to 1 in 10 childhood and adolescent cancers.
Which three types are the commonest soft tissue sarcomas in adults?
ARhabdomyosarcoma, synovial sarcoma and Ewing sarcoma
BAngiosarcoma, Kaposi sarcoma and MPNST
CAlveolar soft part, clear cell and epithelioid sarcoma
DDesmoid tumour, GIST and dermatofibrosarcoma protuberans
EUndifferentiated pleomorphic sarcoma, liposarcoma and leiomyosarcoma
Show answer
Answer: E. UPS, liposarcoma and leiomyosarcoma lead in adults. Rhabdomyosarcoma and Ewing sarcoma are mainly tumours of children and young adults, and the others are uncommon. GIST and desmoid are separate diseases.
A 15-year-old boy has had knee pain for 3 months that now wakes him at night, with a firm swelling above the knee. Which tumour is most likely?
AConventional chondrosarcoma
BOsteochondroma
COsteosarcoma of the distal femur
DSynovial sarcoma of the knee joint
EChronic osteomyelitis
Show answer
Answer: C. Osteosarcoma peaks in teenagers and arises in the metaphysis around the knee. Conventional chondrosarcoma is a tumour of adults over 40. Night pain in a teenager is not growing pains.
This schematic radiograph is from a 15-year-old with night pain above the knee. Which finding points to osteosarcoma?
AMultiple well-defined lucent lesions in the epiphysis
BA uniformly sclerotic bone with no soft tissue mass
CA calcified cartilage cap on a bony stalk
DA sunburst periosteal reaction with a Codman triangle in the metaphysis
EOnion-skin layering of the diaphysis
Show answer
Answer: D. Bone-forming osteosarcoma gives a mixed lytic and sclerotic metaphyseal lesion with sunburst spicules and a Codman triangle. Onion-skin layering with a permeative diaphyseal pattern suggests Ewing sarcoma, and a cartilage cap suggests an osteochondroma or chondrosarcoma.
Where is Ewing sarcoma most characteristically found?
AIn the epiphysis of the proximal tibia in older adults
BIn the metaphysis of the distal femur in teenagers only
CIn the diaphysis of a long bone, the pelvis or the ribs, in children and young adults
DIn the phalanges of the hand in middle age
EIn the skull vault in adults over 60
Show answer
Answer: C. Ewing sarcoma arises in long-bone diaphyses, the pelvis and the ribs, and can also arise in soft tissue. It affects children and young adults.
Which statement about conventional chondrosarcoma is correct?
AIt mostly affects children under 10 and responds well to chemotherapy
BIt mainly affects adults over 40 and is chemoresistant, so surgery is the only effective treatment
CIt is treated with the same chemotherapy as osteosarcoma
DIt is radiosensitive and is managed with radiotherapy alone
EIt never occurs in the pelvis
Show answer
Answer: B. Conventional chondrosarcoma occurs in adults (pelvis, femur, shoulder girdle) and does not respond to chemotherapy. Surgery is the treatment; dedifferentiated and mesenchymal variants are handled differently.
A 45-year-old has a painless, slowly enlarging 7 cm lump deep in the thigh. What is the most appropriate next step?
AObservation for 3 months
BFNAC alone, with reassurance if it is benign
CUltrasound only, and discharge if there is no pain
DMRI of the thigh with contrast before any biopsy or excision
EExcision biopsy under local anaesthesia
Show answer
Answer: D. A lump that is large (over 5 cm), deep or growing needs MRI before a knife. Pain does not separate benign from malignant soft tissue masses; size, depth and growth do.
Which feature most raises the suspicion that a soft tissue lump is a sarcoma?
AA history of a single minor injury
BPain only at night
CA soft consistency
DTenderness on palpation
ESize over 5 cm, depth below the fascia, or growth
Show answer
Answer: E. Size, depth and growth are the clues. Pain is uncommon until the tumour is large, and an injury often only draws attention to a tumour that was already present.
Which biopsy approach is preferred for a suspected limb sarcoma?
AFrozen section during a planned excision elsewhere
BExcision of the whole lump as the biopsy
CA transverse incisional biopsy
DImage-guided core needle biopsy after MRI, along a track that will be removed with the tumour
EFNAC alone
Show answer
Answer: D. Core biopsy with several cores, planned with the surgeon, is the default. FNAC alone is inaccurate for subtype and grade, and a transverse cut contaminates more tissue and enlarges the resection.
Which investigation should every adult with a new soft tissue sarcoma have to look for metastases?
ACT of the chest
BMRI of the brain
CSerum LDH
DChest radiograph only
EBone scan
Show answer
Answer: A. The lung holds about 80% of metastases, so CT chest is standard. A chest radiograph is acceptable only for small low-grade tumours, and brain MRI is kept for particular subtypes.
Answer: E. SS18::SSX is the diagnostic fusion of synovial sarcoma. EWSR1::FLI1 is Ewing sarcoma, FUS::DDIT3 myxoid liposarcoma, PAX3::FOXO1 alveolar rhabdomyosarcoma and COL1A1::PDGFB dermatofibrosarcoma protuberans.
MDM2 amplification on FISH helps to distinguish which pair?
AGIST from desmoid tumour
BSynovial sarcoma from Ewing sarcoma
COsteosarcoma from osteomyelitis
DLeiomyosarcoma from leiomyoma
EWell-differentiated liposarcoma from lipoma
Show answer
Answer: E. MDM2 amplification separates atypical lipomatous tumour or well-differentiated liposarcoma from lipoma, and dedifferentiated liposarcoma from UPS. Any retroperitoneal “UPS” should be tested.
A 28-year-old woman has an osteosarcoma. Her mother had breast cancer at 36 and her brother had an adrenocortical tumour in childhood. Which germline gene should be tested?
AVHL
BTP53
CAPC
DMLH1
ENF2
Show answer
Answer: B. This pattern suggests Li-Fraumeni syndrome (germline TP53): sarcoma at a young age with family cancers. Radiotherapy is avoided where possible in carriers.
What is the usual latency of a radiation-associated sarcoma?
AAbout 1–2 years
BUnder 6 months
CUsually 7–16 years, shorter (4–8 years) for angiosarcoma after breast radiotherapy
DAlways over 40 years
EThere is no latency; they appear during radiotherapy
Show answer
Answer: C. The absolute risk is low (well under 1%) but persists for decades. The criteria are a sarcoma in a previously irradiated field, a different histology from the first cancer, and a latency of at least 3 years (classically over 5).
A “lipoma” is excised in a minor theatre and histology shows a high-grade sarcoma with involved margins. What should be done next?
AMRI of the site and re-excision of the scar and tumour bed, after expert pathology review
BNo further local treatment, because the lump has been removed
CReassure and review in 3 months
DPostoperative chemotherapy alone
EWait for a local recurrence
Show answer
Answer: A. Residual tumour is found in about half of unplanned excisions (Noria 1996). Re-image with MRI, review the pathology and re-excise the scar and bed; radiotherapy can secure control where re-excision would cost function.
What is the standard preoperative radiotherapy dose for a soft tissue sarcoma of the limb?
A50 Gy in 25 fractions
B66 Gy in 33 fractions
C20 Gy in 5 fractions
D30 Gy in 10 fractions
E75 Gy in 30 fractions
Show answer
Answer: A. Preoperative RT is 50 Gy in 25 fractions with surgery 4–6 weeks later. Postoperative doses are 60 Gy for a negative margin, 66 Gy for a positive margin and up to 75 Gy for gross residual disease. Shorter schedules remain investigational.
Compared with postoperative radiotherapy, preoperative radiotherapy for limb sarcoma causes:
AWorse local control
BWorse overall survival
CMore late fibrosis and joint stiffness
DNo wound complications
EMore wound complications but less permanent late fibrosis
Show answer
Answer: E. In NCIC SR2 local control and survival were equal, wound complications were 35% versus 17%, and late fibrosis was lower with preoperative RT, which is why it is preferred for most patients.
Which statement about retroperitoneal sarcoma is correct?
AIt is usually under 3 cm at diagnosis
BIt is cured by biopsy and radiotherapy alone
CIt nearly always presents with early jaundice
DIt commonly spreads to lymph nodes
EIt is often silent until large, about 15 cm at diagnosis
Show answer
Answer: E. Retroperitoneal sarcoma (10–15% of soft tissue sarcomas) produces fullness, early satiety or leg symptoms late. Nodal spread is uncommon, and complete resection at the first operation is the only cure.
Which regimen is the backbone of Ewing sarcoma chemotherapy?
AMAP
BVDC/IE (vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide)
CDoxorubicin plus trabectedin
DGemcitabine plus docetaxel
EWeekly paclitaxel
Show answer
Answer: B. Adding ifosfamide and etoposide to VDC improved 5-year event-free survival in localised disease (INT-0091). Adults receive the same protocol at full intensity.
Which drug requires an echocardiogram before treatment and a record of the lifetime cumulative dose?
ADoxorubicin
BEribulin
CTrabectedin
DMethotrexate
EPazopanib
Show answer
Answer: A. Doxorubicin causes cumulative cardiomyopathy, so echo or MUGA beforehand and tracking of the lifetime dose are needed, with precautions against extravasation.
Which measure prevents ifosfamide haemorrhagic cystitis?
ADexrazoxane
BMesna with hydration
CLeucovorin
DAllopurinol
EAmifostine
Show answer
Answer: B. Ifosfamide also causes encephalopathy and a proximal tubulopathy (Fanconi syndrome), so kidney function, phosphate and bicarbonate are monitored. Leucovorin is the rescue for methotrexate.
In advanced soft tissue sarcoma, pazopanib is not used for which group?
AMPNST
BSynovial sarcoma
CLeiomyosarcoma
DAdipocytic sarcomas (liposarcoma)
EUndifferentiated pleomorphic sarcoma
Show answer
Answer: D. Pazopanib (PALETTE: PFS 4.6 versus 1.6 months, no overall survival gain) is for non-adipocytic sarcomas. Eribulin is the later-line drug with a survival benefit in liposarcoma.
When do most relapses of soft tissue sarcoma occur?
ARelapse is rare after complete resection
BIn the first 2–3 years, so surveillance is front-loaded
COnly in the first month
DEvenly across 10 years
EAfter 15 years
Show answer
Answer: B. Examination and chest CT are most frequent in the first 2–3 years, to catch lung metastases and local relapse while they can be resected. Retroperitoneal liposarcoma is the exception and recurs late.
Before cisplatin, ifosfamide or other alkylating chemotherapy in a 15-year-old boy, which step is often forgotten?
AThyroid screening
BA live vaccine
CSperm banking
DDental extraction
EBone marrow harvest
Show answer
Answer: C. Fertility preservation (sperm banking, or ovarian protection) must be arranged before starting treatment, and it is often forgotten in the rush to begin.
Which investigation should come first in a teenager with persistent bone pain?
ASerum calcium only
BBone biopsy at the local hospital
CA plain X-ray of the whole bone in two planes
DA bone density scan
EA trial of antituberculous treatment
Show answer
Answer: C. The plain X-ray is still the first and most useful bone test. MRI of the whole bone follows, and the patient should be referred before any biopsy.
Which statement about lymph node metastasis in sarcoma is correct?
ANodes are involved in most soft tissue sarcomas
BNodal spread is the commonest route of metastasis
CNodal disease never occurs in sarcoma
DSentinel node biopsy is routine in all sarcomas
ENodal involvement is uncommon (under 5%) except in clear cell, epithelioid, angiosarcoma and rhabdomyosarcoma
Show answer
Answer: E. Lymph nodes are rarely involved, so routine node dissection or sentinel biopsy is not used. In the subtypes named, examine the regional nodal basin; nodal spread then makes a limb or trunk sarcoma stage IV.
A 52-year-old man had an 8 cm “lipoma” of the thigh excised under local anaesthesia, with a drain brought out 10 cm away. Histology: high-grade pleomorphic sarcoma with involved margins. Which plan is most appropriate?
AAdjuvant chemotherapy alone
BMRI of the thigh and CT chest, expert pathology review, preoperative RT that includes the scar and drain track, then re-excision
CHip disarticulation
DObservation with MRI at 6 months
ERe-excise only the scar, without radiotherapy
Show answer
Answer: B. The scar and drain site are contaminated and must be treated; an unplanned excision enlarges both the radiotherapy field and the definitive operation. For stage III UPS, pembrolizumab with RT can be discussed (SU2C-SARC032).
In SU2C-SARC032 (stage III limb UPS or pleomorphic or dedifferentiated liposarcoma), adding pembrolizumab to preoperative RT and surgery:
AHad no effect on disease-free survival
BImproved 2-year disease-free survival (67 vs 52%) with more grade 3 or worse toxicity (56 vs 31%); overall survival is not yet known
CWas tested together with doxorubicin–ifosfamide chemotherapy
DImproved overall survival by 20% with no extra toxicity
EReplaced the need for surgery
Show answer
Answer: B. It is the first immunotherapy result in localised sarcoma. Offer it after shared decision-making where access allows; survival data are immature and it was not tested with chemotherapy.
Which patient with a limb soft tissue sarcoma can reasonably be treated with wide excision alone?
AA deep 9 cm grade 3 tumour
BAny tumour next to a nerve
CA tumour with a positive margin that cannot be re-excised
DAn 8 cm myxofibrosarcoma with infiltrative margins
EA superficial 4 cm grade 1 tumour with clear margins
Show answer
Answer: E. Surgery alone is reasonable for superficial, ≤5 cm, low-grade tumours with clear margins (Yang 1998). Radiotherapy adds local control, but not survival, for most tumours that are over 5 cm or deep, and grade 2–3.
After preoperative RT, a thigh sarcoma is resected with a 2 mm margin where it abuts an intact fascial plane, and the sciatic nerve is preserved. How is this margin regarded?
AUnacceptable unless a 5 cm margin is achieved
BOften adequate: fascia, periosteum and vessel adventitia are good barriers, and planned close margins beside a preserved nerve or vessel are acceptable after preoperative RT
CAlways an R2 resection
DAn indication for amputation
EImpossible to judge without a PET scan
Show answer
Answer: B. An adequate margin is not a single number. About 1 cm of fat or muscle, or an intact fascial plane, is usually enough with radiotherapy.
What target volume was used for preoperative RT in RTOG 0630?
AThe tumour only, with no margin
BThe tumour plus 10 cm in every direction
CThe regional lymph node basin
DThe tumour plus about 1.5 cm radially and 3–4 cm along the muscle, including T2 oedema where feasible
EThe whole limb
Show answer
Answer: D. Image-guided preoperative RT to these reduced volumes gave few marginal failures and late grade 2 or worse toxicity of about 10%, showing that smaller margins are safe.
A 34-year-old man has this deep posterior thigh mass (schematic T2 MRI). Core biopsy: myxoid liposarcoma with a FUS::DDIT3 fusion. Which staging is specific to this subtype?
APET-CT alone
BBone marrow aspirate only
CCT chest only
DCT chest, CT abdomen-pelvis and whole-spine MRI; PET may miss disease
EMRI of the brain
Show answer
Answer: D. Myxoid liposarcoma spreads to soft tissue, the retroperitoneum and bone marrow without lung disease, so the spine and abdomen are imaged. It is exceptionally radiosensitive and trabectedin is active.
A 58-year-old woman has abdominal fullness. This schematic CT shows a large fatty left retroperitoneal mass with thick septa and a solid enhancing nodule. What does the nodule suggest, and what is the best next step?
AA benign lipoma; observe
BA germ cell tumour; orchidectomy
CLymphoma; excision biopsy at laparotomy
DRenal angiomyolipoma; embolise
EDedifferentiation within a well-differentiated liposarcoma; percutaneous posterior core biopsy of the nodule and discussion at a sarcoma MDT
Show answer
Answer: E. Fat with a solid non-fatty nodule indicates dedifferentiation. Percutaneous posterior core biopsy of a retroperitoneal mass is safe and does not raise local recurrence; open or laparoscopic biopsy is avoided.
How should the result of STRASS be applied to retroperitoneal sarcoma?
APreoperative RT is standard for every retroperitoneal sarcoma
BPreoperative RT did not improve abdominal recurrence-free survival overall; it is an option in selected well-differentiated and low-grade dedifferentiated liposarcoma after MDT discussion
CRadiotherapy should never be used
DChemotherapy replaces surgery
EPostoperative RT is preferred
Show answer
Answer: B. Postoperative RT is avoided because bowel falls into the tumour bed. Surgery by an experienced team remains the key treatment.
At the first operation for a resectable retroperitoneal liposarcoma, which approach gives the best local control?
ACompartmental en bloc resection including adherent organs, by an experienced team
BEnucleation along the capsule
CLaparoscopic morcellation
DDebulking with planned reoperation
EOpen biopsy followed by chemotherapy
Show answer
Answer: A. Extended resection improves local control in liposarcoma (Bonvalot 2009). Never enucleate, morcellate or “debulk” at the first operation, and refer before laparotomy.
A 47-year-old woman had a hysterectomy for a rapidly growing “fibroid” that proved to be leiomyosarcoma, and now has a cough. Which staging is most appropriate?
ABone scan only
BMRI of the brain
CChest X-ray only
DNo imaging, because the uterus has been removed
ECT of the chest and abdomen including the liver
Show answer
Answer: E. Leiomyosarcoma metastasises to lung and liver, so both are scanned. Power morcellation of a fibroid with suspicious features should be avoided because it can spread an unsuspected sarcoma.
A 64-year-old woman treated with breast-conserving surgery and RT 7 years ago has had this violaceous, bruise-like patch for 2 months and was given antibiotics for “cellulitis”. What is the next step?
ATopical steroids
BA longer course of antibiotics
CObservation for 6 months
DMultiple punch biopsies from the darkest areas
EMammography alone and reassurance
Show answer
Answer: D. A bruise that will not fade on an irradiated breast is angiosarcoma until a punch biopsy says otherwise; reassess any “bruise” persisting beyond 2 weeks. MYC amplification supports radiation-associated angiosarcoma.
A 15-year-old’s X-ray shows a sunburst lesion of the distal femur. Which sequence is correct?
AImmediate biopsy at the nearest hospital, then referral
BMRI of the whole femur, CT chest and PET-CT or bone scan, then core biopsy at the centre that will operate
CChemotherapy before any tissue diagnosis
DAmputation without biopsy
ECurettage as for a suspected infection
Show answer
Answer: B. A poorly placed biopsy track can turn limb salvage into amputation, so plan the biopsy with the treating team. The whole bone is imaged for skip lesions, and baseline audiometry, echo and kidney function precede chemotherapy.
A 24-year-old man has 4 months of thigh pain, fever and a raised ESR, and antituberculous treatment was started for a “lytic lesion” without a biopsy. His radiograph (schematic) shows a permeative, moth-eaten pattern with onion-skin layering and a soft tissue mass. What should be done?
ASix weeks of antibiotics without a biopsy
BCT-guided core biopsy sent for histology, molecular testing and culture, and stop the unproven antituberculous treatment
CCurettage and cementing
DBone scan only
EContinue antituberculous drugs for 6 months
Show answer
Answer: B. Fever, raised ESR and a bone lesion can be Ewing sarcoma. Send tissue for histology and culture together, and never curette a suspected sarcoma as if it were infection.
A 52-year-old has a high-grade osteosarcoma. Which chemotherapy is usual?
APazopanib
BVDC/IE
CNo chemotherapy, because adults do not benefit
DMAP exactly as for a teenager
EDoxorubicin–cisplatin; the benefit of high-dose methotrexate is unproven at this age
Show answer
Answer: E. MAP is standard up to age 40. Where methotrexate cannot be monitored safely, carboplatin–ifosfamide–doxorubicin is an alternative, with more second leukaemias.
A teenager’s osteosarcoma shows 60% necrosis after preoperative MAP. What should the postoperative chemotherapy be?
AAdd ifosfamide–etoposide, which improved survival
BSwitch to VDC/IE
CContinue MAP to complete about 29 weeks; changing or intensifying treatment did not help in EURAMOS-1
DStop chemotherapy
EAdd zoledronate to improve survival
Show answer
Answer: C. Adding ifosfamide–etoposide added toxicity only. Zoledronate (OS2006) and interferon maintenance for good responders (EURAMOS-1) were also negative.
Which is the correct overall sequence for localised Ewing sarcoma in a 17-year-old?
AVDC/IE only after surgery
BVDC/IE every 14 days for 12 weeks, local control (surgery or RT), then chemotherapy to a total of 14–17 cycles
CSurgery first, then 6 cycles of MAP
DInterferon maintenance after 4 cycles
ERadiotherapy alone
Show answer
Answer: B. If radiotherapy is the local treatment, chemotherapy continues during it with doxorubicin omitted. From age 18, cycles may be given every 14 or 21 days.
Answer: C. Interval compression with growth-factor support is the benefit. Higher doses per cycle (Granowetter 2009) and adding vincristine–topotecan–cyclophosphamide (AEWS1031) did not help.
A Ewing sarcoma of the iliac wing with a large mass is judged unresectable without mutilation. Which local treatment is appropriate?
ARadiotherapy after 3 years
BDefinitive RT (about 55 Gy) with chemotherapy continuing, omitting doxorubicin during RT
CHemipelvectomy regardless of function
DNo local treatment; chemotherapy alone
EIntralesional curettage
Show answer
Answer: B. Definitive RT is used when the tumour is unresectable or resection would be mutilating (spine, much of the pelvis, skull base). Postoperative RT is given for positive margins, gross residual disease or high-risk chest wall tumours.
A fit 47-year-old woman has metastatic uterine leiomyosarcoma (lung and liver). Which first-line regimen improved both PFS and overall survival in a randomised trial?
AGemcitabine–docetaxel
BDoxorubicin plus trabectedin, followed by trabectedin maintenance (LMS-04)
CPazopanib alone
DImatinib
EObservation
Show answer
Answer: B. PFS was 12 versus 6 months and overall survival 33 versus 24 months, but grade 3 or worse toxicity was 97%, so growth-factor support and LFT and CK monitoring are needed. Doxorubicin plus dacarbazine is an alternative.
A young adult with an advanced sarcoma has an NTRK fusion found by RNA sequencing. Which treatment gives high, durable responses?
APazopanib
BMifamurtide
CImatinib
DLarotrectinib or entrectinib
EAtezolizumab
Show answer
Answer: D. TRK inhibitors give high, durable responses in any tumour type. Screen with pan-TRK IHC and confirm with RNA NGS; NTRK fusions are rare in adult sarcoma but common in infantile fibrosarcoma.
Three years after treatment of a grade 3 synovial sarcoma near the knee, a 29-year-old woman has two new right-lung nodules on surveillance CT (schematic), with nothing elsewhere on PET-CT. What is the best approach?
AAdjuvant MAP after resection
BObserve until symptoms develop
CWhole-lung radiotherapy
DRepeat CT at 6–8 weeks to test the tempo, then VATS resection if no new lesions, with no routine adjuvant chemotherapy
EStart palliative chemotherapy at once
Show answer
Answer: D. Selection explains much of the benefit: long disease-free interval, few nodules, controlled primary and no extrathoracic disease. Five-year survival after resection is about 30–40% in series.
Which medicines should be avoided during high-dose methotrexate for osteosarcoma, and what is used for severely delayed clearance?
AAvoid NSAIDs, PPIs and co-trimoxazole; glucarpidase for delayed clearance
BAvoid allopurinol; give hydroxocobalamin
CAvoid statins; give dexrazoxane
DAvoid mesna; give amifostine
EAvoid antiemetics; give rasburicase
Show answer
Answer: A. Hydration, urine alkalinisation, drug levels and leucovorin rescue are routine. NSAIDs, PPIs and co-trimoxazole delay clearance, and glucarpidase is used for delayed clearance.
Which long-term effects should be anticipated after cisplatin and ifosfamide for a bone sarcoma?
APulmonary fibrosis and hypothyroidism only
BCardiomyopathy from cisplatin
CHair depigmentation
DPeripheral neuropathy only from ifosfamide
EHearing loss and renal tubular loss of phosphate and magnesium
Show answer
Answer: E. Audiometry and magnesium monitoring are needed with cisplatin, and kidney tubular damage follows ifosfamide and cisplatin. Anthracyclines cause cardiomyopathy and alkylators cause infertility and second cancers.
For how long should retroperitoneal liposarcoma be followed with imaging?
AFor 6 months
BOnly if symptoms develop
CFor 2 years
DBeyond 10 years, because liposarcoma recurs late and can dedifferentiate
EUntil the first scan is clear
Show answer
Answer: D. CT abdomen-pelvis and chest is done every 3–6 months for 2–3 years, then every 6–12 months. Leiomyosarcoma, by contrast, metastasises early.
Which imaging is preferred to follow the primary site after limb sarcoma resection with positive margins or at a hard-to-examine site?
ANo imaging
BPET-CT every 3 months
CUltrasound every 3 months only
DMRI about 6-monthly for 2 years, then yearly to 5 years
EChest radiograph yearly
Show answer
Answer: D. MRI is preferred to CT for the primary site (better sensitivity, no radiation). Chest CT surveillance for lung metastases continues alongside.
Which statement about late effects after treatment of a bone sarcoma in a young person is correct?
ASurvivors cannot play sport
BAn echocardiogram is needed only at diagnosis
CAnthracycline cardiomyopathy, cisplatin hearing loss, infertility and second cancers can appear years later and need survivorship follow-up
DLate effects do not occur after chemotherapy
EOnly radiotherapy causes late effects
Show answer
Answer: C. Many young survivors walk, run and play sport. Late effects also include kidney damage, radiotherapy fibrosis, fracture and growth disturbance.
Why is preoperative chemotherapy preferred in osteosarcoma?
AIt gives better survival than postoperative chemotherapy
BIt prevents all metastases
CIt allows radiotherapy to be omitted
DIt eases limb salvage and allows necrosis to be assessed; survival is similar to postoperative timing
EIt removes the need for surgery
Show answer
Answer: D. Preoperative and postoperative timing give similar survival, but preoperative treatment makes limb-sparing surgery easier and shows the histological response.
DNB, DM and USMLE Step 3 level: landmark trials, newer targeted drugs and difficult decisions.
Q69BoneLevel 3Systemic therapy
What did the Multi-Institutional Osteosarcoma Study (MIOS) establish?
AAdjuvant chemotherapy after surgery raised relapse-free survival from about 17% to about 65%
BInterferon maintenance prolonged survival
CLimb salvage was inferior to amputation
DRadiotherapy can replace surgery
EZoledronate improved survival
Show answer
Answer: A. Chemotherapy before and after surgery raised long-term survival of localised osteosarcoma from under 20% to about 65–70%, making chemotherapy standard.
How should the pooled evidence for adjuvant doxorubicin–ifosfamide (Pervaiz 2008, EORTC 62931) be applied?
AGive it to every patient with soft tissue sarcoma
BGive it only to patients over 70
CGive it to all low-grade tumours
DNever give it
EOffer chemotherapy only to fit patients with high-risk tumours (over 5 cm, deep, grade 2–3, ideally chemosensitive types) after shared decision-making
Show answer
Answer: E. The meta-analysis suggested an 11% absolute survival gain, but the largest trial was negative, partly because it included small, low-grade and non-limb tumours. Benefit is modest and uncertain.
ISG-STS 1001 compared three cycles of epirubicin plus ifosfamide with histotype-tailored regimens in high-risk soft tissue sarcoma. What was the conclusion?
AFive cycles were superior to three
BThe standard regimen was better overall; high-grade myxoid liposarcoma, where the tailored drug is trabectedin, was the exception
CThe trial was negative for toxicity only
DTailored regimens were superior in every histotype
EBoth arms had identical results in every histotype
Show answer
Answer: B. Three cycles were also as effective as five in the earlier trial (Gronchi 2016), so three cycles is the regimen used when chemotherapy is given.
A 22-year-old woman has alveolar soft part sarcoma (ASPSCR1::TFE3) of the thigh with lung metastases. Which statement is correct?
AImatinib is standard
BChemotherapy is effective and curative
CDoxorubicin is highly effective and should start at once
DIt is indolent: limited stable disease can be observed, atezolizumab or pembrolizumab is used if it progresses, and brain MRI is part of staging
EIt never metastasises to the brain
Show answer
Answer: D. Chemotherapy is ineffective. Pembrolizumab plus axitinib is used if the disease is bulky or symptomatic (Wilky 2019); pazopanib or sunitinib are later options.
This schematic H&E field from a pelvic mass in a 19-year-old shows sheets of uniform small round blue cells. Which is the most useful next step?
AGleason grading
BS100 immunohistochemistry alone
COestrogen receptor testing
DCD99 and NKX2.2 immunohistochemistry with FISH or RNA-based fusion testing, on fresh or frozen tissue
ESerum CEA
Show answer
Answer: D. Small round cell sarcomas need molecular confirmation. Ewing sarcoma carries EWSR1::FLI1 (about 85%) or EWSR1::ERG (about 10%); CIC::DUX4 and BCOR::CCNB3 sarcomas are separate, more aggressive entities.
Which are the eligibility requirements for afamitresgene autoleucel in synovial sarcoma, and what is its status in India?
AHLA-A*02 and MAGE-A4 positivity; not yet available in India, so relevant for trial referral
BNTRK fusion; available in India
CPD-L1 positivity; widely available
DMDM2 amplification; available
EBRAF V600E; available
Show answer
Answer: A. SPEARHEAD-1 studied engineered T cells in synovial sarcoma and myxoid/round cell liposarcoma. Cytokine release syndrome and cytopenias are managed in a cell-therapy centre.
What does the Sarculator nomogram provide, and what level of risk marks “high risk” for a perioperative chemotherapy discussion?
ALocal recurrence only; 5%
BA PD-L1 score; 10%
CAn individual risk estimate for extremity soft tissue sarcoma that adds age, subtype and site to size and grade; a 10-year metastasis risk of about 40% or more
DResponse to MAP; 90%
ESurgical margin width; 1 mm
Show answer
Answer: C. Sarculator (Callegaro 2016) is used for extremity sarcoma; a histology-specific nomogram exists for the retroperitoneum (Gronchi 2013).
Which statement about added agents in osteosarcoma is correct?
AZoledronate added nothing (OS2006) and interferon maintenance was negative (EURAMOS-1); mifamurtide is used where licensed but is not available in India
BMifamurtide is widely available in India
CIfosfamide–etoposide improved outcomes for poor responders
DZoledronate improved survival
EInterferon improved survival for good responders
Show answer
Answer: A. Mifamurtide with MAP was studied in INT-0133 and is used where licensed.
Which statement about relapsed bone sarcoma is correct?
ARadiotherapy alone is standard
BRelapse is never curable
COnly chemotherapy is used
DResect lung metastases where possible; relapse over a year later with a few resectable nodules can still be cured by surgery; options include irinotecan–temozolomide or topotecan–cyclophosphamide in Ewing sarcoma and multitarget TKIs in osteosarcoma
EImatinib is standard
Show answer
Answer: D. Trials are preferred. Osteosarcoma TKI evidence comes from REGOBONE and CABONE.
Which osteosarcoma subtypes usually need surgery alone?
ARadiation-associated osteosarcoma
BHigh-grade osteosarcoma of the jaw
CLow-grade central and parosteal osteosarcoma
DConventional high-grade osteosarcoma
EOsteosarcoma in Paget disease
Show answer
Answer: C. Periosteal osteosarcoma is intermediate and chemotherapy is debated. High-grade tumours, including those of the jaw, usually get cisplatin-based chemotherapy.
Prepared and reviewed by Dr. Haritha Chiramana for teaching. Facts compiled from current guidelines, primary trials and evidence summaries, written in our own words; drawings are original schematics, not patient images; no doses are included.