Teratomas, Dermoids, and Soft Tissue Tumors

Question 1

Regarding germ cell tumour all of the following are correct except:

A. Germ cells develop from pluripotent cells.

B. Dysgerminoma is a hormone secretion tumour.

C. Immature teratoma is a premature condition.

D. Yolk sac endodermal sinus tumour develops from intraembryonic differentiation.

E. Trophoblastic choriocarcinoma is a hormone-secreting tumour.

Answer 1

D

Yolk sac endodermal sinus tumor develops from extraembryonic differentiation.

Syed/MCQ

Question 2

Which of the following statements regarding type of sacrococcygeal teratoma is false?

A. Type I is predominantly external with minimum presacral component.

B. Type II is external with significant intrapelvic component.

C. Type III is external with predominant pelvic mass with extension into the abdomen.

D. Type IV is entirely presacral with external presentation.

E. All of the above are false.

Answer 2

E

All statements A, B, C, and D related to types of sacrococcygeal teratoma are true.

Syed/MCQ

Question 3

Alpha-fetoprotein is high in all of the following conditions except:

A. Embryonal carcinoma.

B. Endodermal sinus tumour

C. Seminoma.

D. Hepatic malignancy.

E. Hypothyroidism.

Answer 3

C

Alpha-fetoprotein does not rise in seminoma.

Syed/MCQ

Question 4

In all of the following conditions, Beta HCG is high except:

A. Choriocarcinoma.

B. Hydatidiform mole.

C. Germ cell tumour without trophoblastic component.

D. Hepatoma.

E. Hepatoblastoma.

Answer 4

C

Beta-HCG is secreted in germ cell tumors containing trophoblastic component (eg choriocarcinoma).

Syed/MCQ

Question 5

The commonest site of germ cell tumour is:

A. Sacrococcygeal.

B. Mediastinal.

C. Abdominal.

D. Ovarian.

E. Testicular.

Answer 5

Sacrococcygeal area is the commonest site of germ cell tumour.

According to site, germ cell is divided as follows. Extragonadal

(a) Sacrococcigeal 41 percent
(b) Mediastinal 6 percent
(c) Abdominal 5 percent
(d) Intracranial 6 percent
(e) Head and neck 4 percent
(f) Vagina 1 percent

Gonadal

(a) Ovarian 29 percent
(b) Testicular 7 percent

Syed/MCQ

Question 6

Factors associated with worst prognosis in malignant germ cell tumour include all except:

A. An extragonadal location.

B. Age less than 11 years.

C. Extent of disease.

D. Inability to perform complete resection.

E. Germinoma or mixed germ cell tumour.

Answer 6

B

Age greater than eleven years old results in bad prognosis.

Syed/MCQ

Question 7

Regarding teratoma in children, all of the following are true except:

A. Contains all the three germ layer.

B. Tumour size does not relate to the risk of malignancy.

C. Children less than two years of age risk of malignancy is 10–20 percent.

D. After 2 years of age, risk of malignancy is almost 80 percent.

E. Radiation is commonly used in this disease.

Answer 7

E

Radiation is rarely used in this condition.

Syed/MCQ

Question 8

Which of the following is not germ cell tumour.

A. Leydig cell. 
B. Yolk sac. 
C. Mixed germ cell tumour. 
D. Seminoma. 
E. Teratoma.

Answer 8

A

Leydig cell tumor is gonadal stromal cell tumor.

Syed/MCQ

Question 9

Which of the following is commonest testicular tumour?

A. Rhabdomyosarcoma. 
B. Yolk sac tumour. 
C. Teratoma. 
D. Mixed germ cell tumour. 
E. Seminoma.

Answer 9

C

Teratoma is commonest;
testicular tumor accounts for 54%.

Syed/MCQ

Question 10

Regarding rhabdomyosarcoma, which of the following is false?

A. It arises from embryonic mesenchyme.

B. It has the potential to differentiate into smooth muscle.

C. It accounts 15 percent of solid tumours.

D. It accounts 50 percent of soft tissue sarcoma.

E. It is sensitive to chemotherapy and radiotherapy.

Answer 10

B.

(It has the potential to differentiate into skeletal, not smooth, muscle.)

Syed/MCQ

Question 11

The commonest site of rhabdomyosarcoma is:

A. Head and neck. 
B. Genitourinary. 
C. Prostate. 
D. Limbs. 
E. Chest.

Answer 11

A

Head and neck is the commonest site and accounts for 35 percent of cases. Others include genitourinary without prostate and urinary bladder 16 percent, prostate and bladder 10 percent, limbs 19 percent and others 20 percent.

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Question 12

Commonest histological type of rhabdomyosarcoma is:

A. Alveolar. 
B. Botroid. 
C. Embryonal. 
D. Pleomorphic. 
E. Undifferentiated

Answer 12

C

Commonest histological type of rhabdomyosarcoma is embryonal, which accounts for 54 percent.

Others include botroid 4.5 percent, alveolar 18.5 percent, pleomorphic 0.5 percent, and undifferentiated 6.5 percent.

Syed/MCQ

Question 13

Which one of rhabdomyosarcoma has the best prognosis?

A. Alveolar. 
B. Pleomorphic.
C.  Embryonal. 
D. Spindle cell. 
E. Botroid.

Answer 13

E

Botroid has the best prognosis.

Syed/MCQ

Question 14

Which of the following comes in category IIC, according to clinical group classification of rhabdomyosarcoma?

A. Microscopic residual tumour at primary site and pathological negative lymph node.

B. Non-residual tumour at primary site and pathological positive lymph node.

C. Gross residual.

D. Distant metastasis.

E. None of the above.

Answer 14

E None of the above.

In the clinical group classification of rhabdomyosarcoma, statement A is IIA, statement B is IIB, statement C is III, statement D is IV.

Category IIC is explained as microscopic residual tumour at primary site and pathological positive lymph node.

Syed/MCQ

Question 15

All of the following are considered unfavourable site for rhabdomyosarcoma except:

A. Parameningeal head and neck.

B. Bladder.

C. Prostate.

D. Limb.

E. Orbit.

Answer 15

E

Orbit is considered a favourable site. Other favourable sites of rhabdomyosarcoma are superficial head and neck, testes, vagina and uterus.

Syed/MCQ

Question 16

Regarding sarcoma of bone in children, which of the following statements is not true?

A. Sarcoma of bone comprises 5 percent of all malignant paediatric tumours.

B. For osteosarcoma of long bones, long-term survival is less than 30 per cent.

C. Evan’s sarcoma is small, blue round cell tumour.

D. Ewing’s sarcoma local control rate is 90 percent.

E. Ewing’s sarcoma is chemo-sensitive.

Answer 16

B

For the osteosarcoma of the long bones long-term survival is about 68 percent.

Syed/MCQ

Question 17

Regarding ovarian cyst and tumour, all of the following are correct except:

A. Preconscious puberty may occurs in luteal cyst.

B. Elevated oestrogen is cause of preconscious puberty.

C. High beta HCG is cause of preconscious puberty.

D. Peutz-Jegher syndrome is associated with corpus luteal cyst.

E. Gross residual is considered as stage III germ cell tumour.

Answer 17

D

Peutz-Jegher syndrome is associated with granulosa cell tumor, ovarian cystadenoma and sex cord stromal tumor.

Corpus luteal cysts are usually small and asymptomatic.

Syed/MCQ

Question 18

Regarding neoplastic ovarian cyst, which of the following statements is false?

A. Among epithelial tumours, 10 per cent are malignant.

B. Sertoli-Leydig cell tumour may produce alpha-fetoprotein.

C. Fibroma are most common among sex cord stromal tumour.

D. Mucinous are epithelial variety of tumour.

E. Sclerosing stromal tumour associated with Chediak-Higashi syndrome.

Answer 18

C

Granulosa cell tumor is the most common variety of sex cord stromal tumor.

Syed/MCQ

Question 19

Regarding germ cell tumour of ovary, all of the following statements are true except:

A. Mature teratoma are more common than immature teratoma.

B. Dysgerminoma is most common among malignant ovarian tumour.

C. Choriocarcinoma shows high alpha-fetoprotein.

D. Gonadoblastoma develops from dysgenetic gonads.

E. Contralateral ovary should be examined at the time of surgery.

Answer 19

C

Choriocarcinoma shows normal alpha-fetoprotein and high beta HCG.

Syed/MCQ

Question 20

What is the mean age of children with hepatoblastoma?

A. 3.5 years. 
B. 5.5 years. 
C. 7.5 years. 
D. 9.5 years. 
E. 11.5 years.

Answer 20

A.

The mean age children develop hepatoblastoma is 3.5 years. The hepatoblastoma is an embryonal tumor that typically develops at 1-3 years of age.

Syed/MCQ

Question 21

Regarding diagnostic studies in hepatic tumours, all of the following are correct except:

A. MRI is more accurate in diagnosis than CT scan.

B. CT scan of the chest is indicated to see metastatic disease.

C. Arteriography is used in therapy for chemoembolization and intra-arterial infusion of cytotoxic drugs.

D. Alpha-fetoprotein has diagnostic value.

E. Thrombocytosis develops because of release of cytokines from tumour.

Answer 21

E

Thrombocytopenia develops from release of cytokines from tumor.

Syed/MCQ

Question 22

Regarding histological subtypes of hepatoblastoma, which of the following statements is correct?

A. Small cell is more common than foetal.

B. Macrotrabacular is more common than embryonal.

C. Teratoid is more common than non-teratoid.

D. Foetal is more common than teratoid.

E. Epithelial and mixed epithelial are not subtypes of hepatoblastoma.

Answer 22

D

Histological subtypes of hepatoblastoma are as follows:

1. Epithelial
   (a) Fetal 31%
   (b) Embryonal 19%
   (c) Macrotrabacular 05%
   (d) Small cell 03%
2. Mixed epithelial/mesenchymal
   (a) Teratoid 10%
   (b) Nonteratoid 34%

Syed/MCQ

Question 23

Regarding pathology of hepatoblastoma, which of the following statements is false?

A. Usually it is bulky and solitary mass.

B. It is surrounded by pseudocapsule.

C. Bilober disease is common.

D. Aneuploid lesion has better prognosis than diploid tumour.

E. Increased mitotic activity shows poor prognosis.

Answer 23

D

Diploid tumor has somewhat better prognosis than aneuploidy tumor.

Syed/MCQ

Question 24

With regard to hepatic tumour in children, which of the following is not true?

A. Primary tumour of liver is uncommon.

B. Forty percent of primary liver tumours are malignant.

C. Hepatoblastoma is the most common primary liver tumour in children.

D. Hepatoblastoma affects boys as frequently as girls.

E. Right lobe is more commonly involved.

Answer 24

B

Approximately 75% of primary liver tumors are malignant.

Syed/MCQ

Question 25

Regarding Wilms tumour, all of the following are false except:

A. Tumour produces alpha-fetoprotein, which causes hypertension.

B. WT1 is an oncogene, which causes tumour development.

C. Beta HCG is present in urine and has prognostic value.

D. Stage I disease is limited to the capsule.

E. Bilateral renal involvement is stage IV disease.

Answer 25

E

Bilateral renal involvement is stage IV disease. Tumour produces renin, which causes hypertension. BFGF is produced in urine and has prognostic value. WT1 and WT2 are tumour suppressor genes and must be inactivated to cause the cancer.

Stage I disease is limited to kidney and renal capsule; sinus and vessels are not involved.

Involvement of capsule, sinus or vessels is considered stage II disease.

Syed/MCQ

Question 26

What percentage of Wilms tumour are bilateral?

A. 1–5 percent. 
B. 5–10 percent. 
C. 10–15 percent. 
D. 15–20 percent. 
E. 20–25 percent.

Answer 26

B

5-10%

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Question 27

What is survival rate for bilateral Wilms tumour?

A. 25 percent. 
B. 50 percent. 
C. 75 percent. 
D. 100 percent. 
E. 0 percent.

Answer 27

C

75%

Syed/MCQ

Question 28

Features of cervical lymphadenitis due to lymphoma include all except:

A. Discharging sinus. 
B. Longer duration. 
C. No pain. 
D. Weight loss. 
E. Other enlarge lymph nodes in the body.

Answer 28

A

Discharge from lymph node is a feature of tuberculous lymphadenitis.

Syed/MCQ

Question 29

Regarding undifferentiated lymphoma, which one is not correct?

A. It is variety of non-Hodgkin lymphoma.

B. It develops from B cells.

C. Burkitt’s lymphoma is its one variety.

D. The majority of these present as thymic or mediastinal masses.

E. Chemotherapy is primary modality of treatment.

Answer 29

D

90% of these present as abdominal tumors.

Syed/MCQ

Question 30

Regarding lymphoblastic lymphoma which one of the following statements is false?

A. It is a variety of non-Hodgkin lymphoma.

B. It originates from T cells.

C. Non-Burkitt lymphoma is one of the varieties.

D. The majority of these present as thymic or mediastinal masses.

E. Chemotherapy is the primary modality of treatment.

Answer 30

C

Both Burkitt’s and non-Burkitt’s lymphoma are varieties of undifferentiated lymphoma.

Syed/MCQ

Question 31

Which of the following develops from posterior mediastinum?

A. Thymoma. 
B. Lymphoma. 
C. Neuroblastoma. 
D. Teratoma. 
E. Dermoid cyst.

Answer 31

C

Neuroblastoma develops from posterior mediastinum.

Other mediastinal masses developing from posterior mediastinum include bronchogenic and enteric duplication cyst.

Thymoma, teratoma, dermoid cyst and germ cell tumors develop in anterior mediastinum.

Syed/MCQ

Question 32

Which one is the commonest mediastinal mass?
A. Neurogenic tumour, such as neuroblastoma and others.
B. Lymphoma.
C. Germ cell tumour.
D. Mesenchymal tumour.
E. Thymic lesion.

Answer 32

B

Lymphoma is most common.

The incidence is like lymphoma 41%, neurogenic tumour 33%, germ cell tumor 7%, mesenchymal tumour 7%, cystic lesion (such as pericardial, bronchogenic and enteric) 7%, and thymic lesion 2.5%.

Syed/MCQ

Question 33

Regarding diagnosis and management of mediastinal masses, which of the following statements is not true?

A. CT is superior with ability to define to define calcification within the mass.

B. Oesophagogram is indicated in foregut duplication.

C. Urinary catecholamine for anterior mediastinal masses.

D. Pre-operative alpha fetoprotein and beta HCG particularly for anterior mediastinal masses.

E. Common approach for anterior mediastinal masses is median sternotomy.

Answer 33

C.

Urinary catecholamine measurement is indicated in posterior mediastinal masses.

Syed/MCQ

Question 34

With regard to lymphoma, what is false?

A. It is a variety of small blue cell tumour.

B. C-myc proto-oncogene translocation between chromosomes number 14 and 18 is associated with Burkitt’s lymphoma.

C. Almost all intestinal lymphoma are non-Hodgkin’s type.

D. Lymphoblastic lymphoma occurs in predominantly in anterior mediastinum.

E. It is associated with increases serum alpha-fetoprotein.

Answer 34

E

None of the small, round cell tumors are associated with increased serum alpha-fetoprotein level.

Syed/MCQ

Question 35

Small round cell tumours include all except:

A. Lymphoma.

B. Primary neuroectodermal tumour.

C. Rhabdomyosarcoma.

D. Hepatoblastoma.

E. Ewing’s sarcoma.

Answer 35

D

Hepatoblastoma is not a small blue cell tumour.

In addition to A, B, C and E, other blue cell tumors include neuroblastoma.

Syed/MCQ

Question 36

Which one is the most common site among following for extra-adrenal pheochromocytoma? L

A. Lymph node. 
B. Liver. 
C. Lung. 
D. Bone. 
E. Organ of Zuckerkandl.

Answer 36

E

Organ of Zuckerkandl is chromatin body, derived from the neural crest, located at the bifurcation of the aorta or at the origin of the inferior mesenteric artery.

Syed/MCQ

Question 37

In comparison of adult pheochromocytoma, paediatric pheochromocytoma has all of the following features except:

A. More malignant.

B. More bilaterally.

C. More sustained hypertension.

D. More familial pattern.

E. More extra-adrenal site.

Answer 37

A

Pediatric pheochromocytoma is less malignant 3% compared to adult (10%).

The incidence of bilaterality in children is 20-50%, while adults 10%.

Sustained hypertension in children 90%, while adult 50%.

Familial pattern in children 10%, while adults 3%.

Extra-adrenal site in children 30%, while adults 10%.

Syed/MCQ

Question 38

Pheochromocytoma shows following clinical feature except:

A. Headache. 
B. Polyuria. 
C. Polydipsia. 
D. Hypotension. 
E. Hyperglycaemia.

Answer 38

D

Most characteristic clinical feature of pheochromocytoma is hypertension.

Syed/MCQ

Question 39

With regards to pheochromocytoma in children, all are true except:

A. It is commonly bilateral.

B. The tumour rarely involves blood vessels.

C. The hypertension is typically sustained rather than episodic.

D. Thirty per cent are malignant.

E. Thirty per cent have extra-adrenal disease.

Answer 39

D

About 3% of childhood tumors are malignant.

Syed/MCQ

Question 40

Scan for diagnosis of pheochromocytoma is

A. DTPS. 
B. DMSA. 
C. MAG 3. 
D. HIDA. 
E. MIBG.

Answer 40

E

MIBG is a radioisotope scan for localization of pheochromocytoma.

Syed/MCQ

Question 41

Regarding features of neuroblastoma, all of the following are false except:

A. Proptosis because of bleeding in eyeball.

B. Hypertension because of cortisol secretion.

C. Diarrhoea because of VIP (vasoactive intestinal polypeptide) secretion.

D. Bleeding because of thrombocytosis.

E. Horner’s syndrome, when it arises from the adrenal gland.

Answer 41

C

Diarrhea because of VIP secretion, hypertension because of production of catecholamine, proptosis because of ocular metastasis, and bleeding because of thrombocytopenia. Horner’s syndrome is when the tumour arises from upper mediastinum involving stellate ganglion.

Syed/MCQ

Question 42

All of the following are bad prognostic factors of neuroblastoma except:

A. Age more than one year.

B. Stage IV-S.

C. When adrenal gland is the site of development.

D. Elevated serum ferritin level.

E. Stoma poor Shimada histology.

Answer 42

B

Stage I, II, and IV-S have a good prognosis, while stage III and IV have a poor prognosis. Age less than one year has a good prognosis, while more than one year has a bad prognosis. Adrenal gland and celiac axis are sites of a bad prognosis. Elevated level of serum ferritin indicates a bad prognosis.

On histology stroma rich has a good prognosis, while stroma-poor has a bad prognosis.

Syed/MCQ

Question 43

Which of the following mediastinal mass shows features of Horner’s syndrome.

A. Lymphoma. 
B. Teratoma. 
C. Thymic hyperplasia. 
D. Neuroblastoma. 
E. Neurogenic duplication cyst.

Answer 43

D

Neuroblastoma shows features of Horner’s syndrome.

Syed/MCQ

Question 44

Which of the following mediastinal lesions in relation to others presents with paraplegia?

A. Bronchogenic cyst. 
B. Neurogenic duplication cyst. 
C. Neuroblastoma. 
D. Neratoma. 
E. Thymic hyperplasia.

Answer 44

B

One of the presentations of neurogenic duplication cyst is paraplegia.

Syed/MCQ

Question 45

Neuroblastoma which is localised primary tumour with dissemination limited to skin, liver, and/or bone marrow in infants younger than 1 year of age is labelled as:

A. Stage I. 
B. Stage II.
C. Stage III. 
D. Stage IV. 
E. Stage IV-S.

Answer 45

E

Stage IV-S

Syed/MCQ

Question 46

Common effects of neuroblastoma on kidneys are:

A. Dilatation. 
B. Displacement. 
C. Distortion. 
D. All of the above. 
E. None of the above.

Answer 46

B

Neuroblastoma commonly causes downward displacement of kidney.

Syed/MCQ

Question 47

Sunburst appearance is a radiological feature of which of the following tumors?

A. Pheochromocytoma
B. Ewing sarcoma
C. Teratoma
D. Osteosarcoma
E. Neuroblastoma

Answer 47

D

X-rays in osteosarcoma may demonstrate perpendicular or radiating striations, called “sunburst”.

Syed/MCQ

Question 48

Regarding testicular tumors, which of the following is false?

A. Testicular tumors account for 1% of tumors in children.

B. Teratoma is mostly benign.

C. Most common malignant tumor is germ cell tumor.

D. Sertoli cell tumor causes gynecomastia.

E. Seminoma is the most common tumor.

Answer 48

E

Most common malignant tumor is germ cell tumor.

Syed/MCQ

Question 49

Microscopic residual tumor is a feature of which stage of testicular tumor?

A. Stage I
B. Stage II
C. Stage III
D. Stage IV
E. None of the above.

Answer 49

B

Stage II

Syed/MCQ

Question 50

Regarding testicular tumor marker, which of the ff statements is not true?

A. Alpha-fetoprotein and beta-HCG both are glycoprotein.

B. Alpha-fetoprotein half-life is 5 days.

C. Beta HCG half-life is 24 hours.

D. Alpha-fetoprotein level comes to normal after 2 days of surgery.

E. B-HCG level comes to normal after 5-7 days of surgery.

Answer 50

D

AFP level comes to normal after 25-30 days of surgery.

Syed/MCQ

Question 51

A tumor that develops from the dysgenetic gonad is:

A. Yolk sac tumor
B. Teratoma
C. Gonadoblastoma
D. Leiomyoma
E. Seminoma

Answer 51

C

Gonadoblastoma

Syed/MCQ

Question 52

Which of the following is not germ cell tumor?

A. Leydig cell
B. Yolk sac
C. Mixed germ cell tumor
D. Seminoma
E. Teratoma

Answer 52

A

Leydig cell tumor is gonadal stromal cell tumor.

Syed/MCQ

Question 53

Regarding syndactyly, which of the following is true?

A. It is a transverse arrest of development.

B. It is a longitudinal deficiency.

C. It is a failure of differentiation.

D. It is commonly seen between the index and middle finger.

E. Syndromic features of syndactyly include Down syndrome.

Answer 53

C

Syndactyly is a failure of differentiation. It is common between middle and ring finger.

Syndromic features of syndactyly include Apert syndrome, Crouzon syndrome, trisomy 13, Pfeiffer syndrome, Golz syndrome and Poland syndrome.

An example of transverse arrest in the development is Amalia.

Examples of longitudinal deficiencies include radial club hand and ulnar ray deficiencies.

Syed/MCQ

Question 54

Regarding deformities of digits, which of the following is false?

A. Clinodactyly means medial or lateral curve of the digit.

B. In polydactyly, central digit duplication is the most common.

C. Mirror hand possess seven or eight digits.

D. Macrodactyly is associated with neurofibromatosis.

E. Brachydactyly means shortened digits.

Answer 54

B

In polydactyly, most common is little finger duplication and second most common is thumb duplication.

Central digit duplication is uncommon.

Syed/MCQ

Question 55

The cause of conjoint twin is:

A. Zygote divided in the first to seventh day of gestation.

B. Two separate ova are fertilised.

C. Inner cell mass incompletely divides.

D. All of the above.

E. None of the above.

Answer 55

C

Conjoint twins result when inner cell mass incompletely divides after first seven days when monozygotic twining occurs.

Conjoint twin with fused organs therefore usually have incomplete development, as manifested as in conjoint heart, liver, GIT and genitourinary tract.

Complete division of zygote in first seven days of gestation yields monozygotic identical twins. They are identical in sex, karyotype and that they share amnion and yolk sac.

Dizygotic twins result from fertilization of two separate ova and each foetus has its own amnion, yolk sac and umbilical cord. These twins may be of same or opposite sex and have different karyotype.

Syed/MCQ

Question 56

The commonest type of conjoint twin is

A. Thoracopagus.

B. Omphalopagus.

C. Pyopagus.

D. Ischiopagus.

E. Craniopagus

Answer 56

A

Thoracopagus is commonest variety of conjoint twin, constitutes 74 per cent. Other incidence: omphalopagus 1 percent, pyopagus 17 percent, ischiopagus 6 percent, and craniopagus 2 percent.

Syed/MCQ

Question 57

Regarding organs potentially involved in Ischiopagus, which of the following groups is most perfect .

A. Heart, liver and intestine.

B. Liver, biliary tract and intestine.

C. Spine, rectum and genitourinary tract.

D. Pelvis, intestine, genitourinary tract and liver.

E. Brain.

Answer 57

D

Organs potentially involved in Ischiopagus includes pelvis, intestine, genitourinary tract and liver.

Syed/MCQ

Question 58

Which is not part of the staging procedure for a patient with an ovarian teratoma?

A palpation of lymph nodes and biopsy of enlarged nodes

B examination and biopsy of suspicious lesion in the omentum

C peritoneal washings

D routine wedge biopsy/bivalving of the contralateral ovary

E preoperative tumour markers

Answer 58

D

The paradigm for staging patients with teratomas includes an examination of the entire peritoneal cavity.

The omentum should be examined and suspicious nodules biopsied. Peritoneal washings or ascitic fluid should be taken for cytologic analysis. All suspicious lymph nodes should be biopsied; however, there is no indication for a full retroperitoneal lymph node dissection.

Routine wedge biopsy of the contralateral ovary is not indicated; biopsy should be performed only if suspicious lesions are present.

SPSE 1

Question 59

What is the most common germ cell tumour in the neonatal age group?

A mediastinal teratoma
B ovarian neoplasm
C cervical teratoma
D testicular neoplasm
E sacrococcygeal teratoma (SCT)

Answer 59

E

SCT is the most common germ cell tumour in the fetal/neonatal age group (50%).

mediastinal teratomas and gonadal neoplasms are seen in children in their second decade of life.

The rate of germ cell tumours in the 0–4 age group is 7 per million children.

Gonadal tumours are rare in children; they are much more common in girls aged 10–15 years.

SPSE 1

Question 60

Which of the following is not generally included in the preoperative workup of an infant with SCT?

A blood screen for tumour markers

B echocardiogram prior to chemotherapy

C MRI of abdomen and pelvis

D chest CT scan

E spinal radiographs

Answer 60

B

SCTs require extensive preoperative workup that should include an AFP tumour marker. This is generally used as a baseline and to predict recurrence in future follow-up.

Spine films should be performed to rule any sacral anomalies. Currarino’s triad includes a presacral tumour, anorectal malformation and a scimitar type sacral anomaly.

Examination of the chest should be performed to ensure there is no metastatic spread to the lungs.

An echocardiogram is not required as 95% of SCTs are benign in the neonatal period.

SPSE 1

Question 61

What is the risk of recurrence of a SCT?

A 10%

B 25%

C 80% if the coccyx is not removed

D 50%

E 70%

Answer 61

B

The risk of recurrence of SCT is generally thought to be around 25%.

Risk factors for recurrence include age greater than one year, immature teratoma on histology, and an elevated AFP.

The risk of recurrence is 40% if the coccyx is not removed.

Patients should be monitored with serial rectal examinations and serum markers to detect any sign of recurrence.

Patients with local recurrence should have resection and be considered for chemotherapy.

SPSE 1

Question 62

Which of the following is false regarding the presentation of cervical teratomas?

A They present in utero with antenatal polyhydramnios.

B They may have respiratory distress due to airway compression and hypoplastic lungs.

C They may require an EXIT procedure for management of the airway.

D They are usually malignant.

E They contain elements from all three germ cell layers.

Answer 62

D

Cervical teratomas comprise 10% of all teratomas and are most often diagnosed on prenatal ultrasound. They can present with polyhydramnios secondary to obstruction.

many patients also have hypoplastic lungs.

The teratomas contain all three germ cell layers and are almost always benign (80%).

They are found at the level of the strap muscles and extend posteriorly towards the trachea.

SPSE 1

Question 63

What is the standard therapy for a completely resected mature teratoma in a child under age 15?

A radiation therapy to primary site

B neo-adjuvant chemotherapy if extragonadal

C observation

D radiation to primary site and six cycles of chemotherapy with cisplatin, etoposide and bleomycin (PEB)

E six cycles of chemotherapy with PEB

Answer 63

C

As long as no elements of carcinoma are found, patients with mature germ cell teratomas (regardless of location), may simply be observed at regular interval without the need for any adjuvant therapy.

Immature teratomas contain tissue from all three germ cell layers, but predominantly neuroepithelial elements. These are graded from I–III based on the amount of immature tissue.

If malignant teratomas are noted the PEB regimen is standard of care.

SPSE 1

Question 64

Which of the following is true regarding SCTs?

A Type I SCT are predominantly external and have the highest incidence of malignancy.

B Type IV is completely external.

C Type III has a small external component but the majority of the mass is internal.

D Type II is the least common type of SCT.

E Type IV tumours usually present with congestive heart failure.

Answer 64

C

The Altman classification of SCTs is based on location.

Type I is a predominantly external.

Type II has both an intra-abdominal and an external component.

Type III is predominantly internal with a small external component.

Type IV is exclusively internal with no external component.

Tumours with an intra-abdominal component are more likely to be malignant, primarily because of the late stage at diagnosis.

In neonates the tumour is benign in about 95% of patients, children greater than 1 year of age have a higher rate of malignancy (80%).

Congestive heart failure can be secondary to shunting or haemorrhage into the tumour and is most common with the large external SCT.

SPSE 1

Question 65

Which of the following statements is not true regarding ovarian germ cell tumours?

A All patients except those with stage I, grade I immature teratoma and stage IA dysgerminoma require postoperative chemotherapy.

B Adjuvant platinum-based chemotherapy for ovarian germ cell tumours is thought to be superior to vincristine, dactinomycin and cyclophosphamide.

C Recurrence rate of immature teratomas is inversely related to the grade of neural elements.

D Second-look laparotomy is not beneficial in patients with initially completely resected tumours who receive cisplatin-based adjuvant treatment.

E Radiation therapy for recurrent germ cell tumours is not effective.

Answer 65

C

The recurrence rate of immature teratomas is directly related to the grade of neural elements.

Both size and histology are the major factors determining prognosis for patients with immature teratomas.

Prognosis is poor for patients with large tumours when more than one-third of the tumour was composed of endodermal sinus elements, choriocarcinoma, or grade III immature teratoma.

Tumours greater than 10 cm in size also have a poor prognosis.

Studies show treatment with PEB appears to be superior to vacuum-assisted closure (VAC) as there is a 25% recurrence rate in patients treated with 6 months of VAC.

In the modern era patients treated with PEB-based chemotherapy have been found not to benefit from second-look surgical procedures because of the effectiveness of the platinum-based chemotherapy.

SPSE 1

Question 66

What percentage of patients with SCTs have associated abnormalities?

A 5%
B 10%
C 20%
D 40%
E 50%

Answer 66

C

Twenty per cent of children with SCTs have some sort of associated abnormality.

These can include cardiac, genitourinary, musculoskeletal or neurologic abnormalities.

SPSE 1

Question 67

Diagnostic criteria for Neurofibromatosis Type 1?

Answer 67

A neurofibroma is a benign neoplasm of Schwann cells, usually of peripheral nerves with several clinical forms.

Multiple neurofibromas with café-au-lait skin lesions suggests neurofibromatosis type 1 (NF-1) or von Recklinghausen disease.

This autosomal dominant disorder arises from mutations or deletions of chromosome 17.

Genetic diagnosis should be sought when 2 of 7 clinical criteria are present:

Six or more café-au-lait spots

Two or more neurofibromas or one plexiform neurofibroma

Osseous lesions such as sphenoid dysplasia or tibial pseudarthrosis

First-degree relative with NF-1

Lisch nodules (iris hamartomas)

Axillary or inguinal freckles

Optic glioma

H&A

Question 68

What are prognostic findings in the prenatal assessment of sacrococcygeal teratoma?

Answer 68

The assessment and follow-up of SCT starts with the unborn patient. Multiple prognostic variables have been described, including size, volume, diameter in comparison to biparietal diameter, vascularity, and percentage of solid component.

All of these have been found to correlate with outcomes, but the most reproducible and useful parameters for prognostication have been the TFR and growth rate.

A TFR ratio above 0.12 on a fetal ultrasound performed before 24 weeks of gestation is highly predictive of fetal complications including polyhydramnios, hydrops fetalis, and intrauterine fetal demise.

A high TFR has also been found to correlate with need for fetal interventions and/or early delivery, two factors which are clearly associated with worse neonatal outcomes.

Growth rate is highest with solid lesions, and shows similar correlations.

A growth rate of more than 60 cm3 / week correlates with adverse outcomes, while a rate of more than 160 cm3 /week correlates with mortality.

An increase in tumor volume by less than 150 mL/week, or growth slower than the fetus, has been shown to be a good prognostic sign. Nevertheless, close follow-up of the fetus and patient should continue, with an ultrasound performed every 2 weeks, and more frequently if the trajectory changes.

In some studies, cardiac function has also strongly predicted survival, with 100% survival in fetuses with normal cardiac function in utero, dropping to 70% in those with evidence of high output cardiac failure, and reaching only 25% in those with evidence of hydrops fetalis.

Reported fetal interventions have included laser vessel ablation, radiofrequency ablation, alcohol sclerosis, cyst drainage, vesicoamniotic shunt placement, amnioreduction, and open fetal surgery with debulking or resection.

The experience has not been extensive, and variable rates of fetal and neonatal survival have been reported. The most feared complication, maternal preeclampsia and mirror syndrome, may threaten the life of the mother.

If such a situation results, the fetus should be delivered by emergent Cesarean.

If the fetus is within the range of viability (>24 weeks), antenatal steroids should be administered to the mother prior to delivery.

Prematurity has been associated with worse prognosis in cases of SCT. However, babies with tumors larger than 5 cm in greatest diameter should be delivered by Cesarean section to avoid the risks of dystocia and tumor hemorrhage or rupture during delivery.

Sherif

Question 69

What laboratory tests should you obtain for SCT postnatally?

Answer 69

Routine laboratory testing should include complete blood count and a biochemical profile. Anemia may represent hemorrhage within the tumor.

The most important test is serum alpha fetoprotein (AFP), which will be used in follow-up after resection.

Serum beta hCG should also be measured.

Sherif

Question 70

Is a high initial AFP level indicative of malignancy in SCT?

Answer 70

No. AFP is synthesized by the fetal yolk sac and is therefore present in the serum of newborns.

It also serves as a tumor marker for a number of pediatric solid tumors, including yolk sac tumor, a germ cell tumor which is the most common malignant component in a malignant SCT.

AFP levels are normally very high in newborns, and higher yet in premature babies.

They drop gradually, but normal adult levels (<6–10 ng/mL) may not be seen until after the first year of life.

Therefore a high AFP level at birth is not indicative of malignancy.

However, it serves as a tumor marker in SCT. Failure to appropriately decrease or a re-elevation after normalization may be signs of residual disease or recurrence, respectively.

Sherif

Question 71

Do SCTs require further imaging postnatally?

Answer 71

Purely exophytic cystic tumors or those with minor internal components (Altman 1) may not require imaging.

However, in a patient with Altman II tumor with large external and internal components, imaging will likely aid in surgical planning.

Sherif

Question 72

What are the major principles of resection of SCT?

Answer 72

Blood should be typed and cross-matched.

Complete resection should be the goal of the surgical procedure.

The tumor may be decompressed in a controlled manner if needed, but spillage of solid component should be avoided.

The tumor should be carefully separated from the anal sphincters, rectal wall, and gluteal muscles, avoiding entry into the rectal wall or sacrifice of muscle.

Delineating the rectal wall by placing a large Hegar or packing the rectum with Vaseline gauze helps the dissection of the tumor from the rectum.

The coccyx should be resected to minimize recurrence.

The major blood supply comes from the middle sacral artery immediately anterior to the coccyx. This should be carefully isolated and ligated during resection.

The anal sphincter muscles should be sutured to the sacral margin, normalizing the anal position.

A drain is left in place, given the large amount of dead space.

The skin is closed to avoid redundant flaps as much as possible.

Sherif

Question 73

What are the main features the pathologist should look for when characterizing an SCT tumor?

Answer 73

The pathologist confirms the diagnosis of SCT by identifying histological elements of the three germ cell layers (ectoderm, mesoderm, and endoderm), evaluates the surgical margins for completeness of resection, and specifically searches for areas of tissue immaturity and malignancy.

This requires extensive sampling, especially in solid parts of the tumor.

Virtually all types of mature tissues can be seen in SCT, but the most commonly encountered elements are skin with appendages, cartilage, muscle, respiratory type epithelium, respiratory and digestive mucosa, and glands as shown on Figure 60.4.

Neuroectodermal components are also present in a majority of cases, including retinal epithelium and choroid plexuses.

The identification of immature neuroepithelial elements, often in the form of small blue cells forming pseudorosettes, as shown on Figure 60.4, warrants a diagnosis of SCT with immature elements.

There was no malignant component seen in this patient.

Although immature elements within SCT are not indicative of malignancy, recent studies report higher rate of recurrences and lesser favorable outcomes.

Contrary to teratomas in other sites, grading of immature SCT has not been shown to be of clinical significance.

Sherif

Question 74

How should an SCT patient be followed after discharge?

Answer 74

Close follow-up is warranted to detect any recurrence early.

Most recurrences occur in the first 2–3 years after resection, but rare recurrences as late as 10 years after resection have been reported.

A rectal exam to detect a recurrent presacral mass, along with a serum AFP level, should be performed every 3 months for the first 2–3 years.

If the baby continues to show no evidence of recurrence, follow-up interval can be prolonged to twice yearly for 2–3 years, followed by annual follow-up until adulthood.

In addition to screening for recurrence, urologic and gastrointestinal function should be evaluated at every visit.

Sherif

Question 75

What are the most common associated anomalies in SCT patients?

Answer 75

Associated anomalies are reasonably prevalent in patients with SCT, occurring in up to one-third of patients.

The two most common associations, hydronephrosis and hip dysplasia, are tumor-related.

However, associated abnormalities of almost every organ system have been reported.

The incidence of associated anomalies seems to increase with prematurity and Altman type IV lesions.

A thorough evaluation of the neonate with SCT is therefore indicated.

Targeted imaging and other investigations should be ordered to further assess any suspicious associated anomalies.

Sherif

Question 76

Primordial germ cells migrate from the yolk sac along the mesentery to the gonads in what week of gestation?

A week 1
B week 3
C week 12
D week 5
E week 9

Answer 76

D

The blastocyst forms primordial cells in week 1 of gestation.

At week 3, cells form on the wall of the yolk sac.

Actual migration of totipotential primordial germ cells does not occur until weeks 5–6, when cells migrate from the yolk sac along the mesentery to the gonads and then become gonadal or extragonadal.

Abnormal migration may give rise to midline tumours, which mainly occur in the cervical, retroperitoneal, sacrococcygeal, mediastinum and pineal gland.

SPSE 1

Question 77

What is the most common germ cell tumour in the neonatal age group?

A mediastinal teratoma

B ovarian neoplasm

C cervical teratoma

D testicular neoplasm

E sacrococcygeal teratoma (SCT).

Answer 77

E

SCT is the most common germ cell tumour in the fetal/neonatal age group (50%).

mediastinal teratomas and gonadal neoplasms are seen in children in their second decade of life.

The rate of germ cell tumours in the 0–4 age group is 7 per million children.

Gonadal tumours are rare in children; they are much more common in girls aged 10–15 years.

SPSE 1

Question 78

What malignant germ cell tumours secrete alpha-fetoprotein (AFP)?

A choriocarcinoma

B yolk sac tumours

C germinoma

D testicular tumours

E embryonal carcinoma

Answer 78

B

AFP is produced by yolk sac (endodermal sinus) tumours and has a half-life of 4 days.

Embryonal carcinoma, seminoma, dysgerminoma and choriocarcinoma can secrete β-hCG.

In addition, lactate dehydrogenase (lDH) and AlP can also be elevated in patients with germinoma tumours.

SPSE 1

Question 79

Klinefelter’s syndrome is associated with what type of germ cell tumour?

A mediastinal teratoma

B testicular seminoma

C endodermal sinus tumour

D choriocarcinoma

E embryonal carcinoma

Answer 79

A

mediastinal teratomas are the most common site of extragonadal germ cell tumours in older children.

They are found in the anterior mediastinum and present with airway symptoms.

Germ cells make up 20% of all mediastinal tumours.

About 80% of mediastinal germ cell tumours are benign; these occur with equal frequency in males and females. However, there is a male predominance (9 : 1) for malignant mediastinal germ cell tumours.

Germ cell tumours are associated with Klinefelter’s syndrome (47,XXY). In patients with Klinefelter’s syndrome, the relative risk of having a malignant mediastinal germ cell tumour is 66.7.

At least 8% of male patients with primary mediastinal tumours have Klinefelter’s syndrome (50 times the expected frequency).

SPSE 1

Question 80

Which of the following is not generally included in the preoperative workup of an infant with SCT?

A blood screen for tumour markers

B echocardiogram prior to chemotherapy

C MRI of abdomen and pelvis

D chest CT scan

E spinal radiographs

Answer 80

B

SCTs require extensive preoperative workup that should include an AFP tumour marker.

This is generally used as a baseline and to predict recurrence in future follow-up.

Spine films should be performed to rule any sacral anomalies.

Currarino’s triad includes a presacral tumour, anorectal malformation and a scimitar type sacral anomaly.

Examination of the chest should be performed to ensure there is no metastatic spread to the lungs.

An echocardiogram is not required as 95% of SCTs are benign in the neonatal period.

SPSE 1

Question 81

What is the risk of recurrence of a SCT?

A 10%

B 25%

C 80% if the coccyx is not removed

D 50%

E 70%

Answer 81

B

The risk of recurrence of SCT is generally thought to be around 25%.

Risk factors for recurrence include age greater than one year, immature teratoma on histology, and an elevated AFP.

The risk of recurrence is 40% if the coccyx is not removed.

Patients should be monitored with serial rectal examinations and serum markers to detect any sign of recurrence.

Patients with local recurrence should have resection and be considered for chemotherapy.

SPSE 1

Question 82

What is the current indication for a retroperitoneal lymph node dissection for testicular germ cell tumours in children?

A enlarged lymph nodes seen on CT scan

B routine for all children under 10 years of age

C if a sentinel lymph node test is positive

D for all germinomas

E if a patient underwent a trans-scrotal resection for testicular mass

Answer 82

A

In adults retroperitoneal lymph node dissection (RPlND) may be used as a primary treatment modality for low-volume non-seminomatous germ cell tumours localised to the retroperitoneum as well as a salvage therapy for residual masses following chemotherapy.

In children a routine selective lymph node dissection is indicated for patients with enlarged nodes.

RPlND may also be used in boys with persistently elevated levels of tumour markers after chemotherapy.

In children diagnosed with paratesticular rhabdomyosarcoma over age 10, routine retroperitoneal lymph node dissection is indicated.

SPSE 1

Question 83

Which is not part of the staging procedure for a patient with an ovarian teratoma?

A palpation of lymph nodes and biopsy of enlarged nodes

B examination and biopsy of suspicious lesion in the omentum

C peritoneal washings

D routine wedge biopsy/bivalving of the contralateral ovary

E preoperative tumour markers

Answer 83

D

The paradigm for staging patients with teratomas includes an examination of the entire peritoneal cavity.

The omentum should be examined and suspicious nodules biopsied.

Peritoneal washings or ascitic fluid should be taken for cytologic analysis.

All suspicious lymph nodes should be biopsied; however, there is no indication for a full retroperitoneal lymph node dissection.

Routine wedge biopsy of the contralateral ovary is not indicated; biopsy should be performed only if suspicious lesions are present.

SPSE 1

Question 84

Which of the following is false regarding the presentation of cervical teratomas?

A They present in utero with antenatal polyhydramnios.

B They may have respiratory distress due to airway compression and hypoplastic lungs.

C They may require an EXIT procedure for management of the airway.

D They are usually malignant.

E They contain elements from all three germ cell layers.

Answer 84

D

Cervical teratomas comprise 10% of all teratomas and are most often diagnosed on prenatal ultrasound.

They can present with polyhydramnios secondary to obstruction.

many patients also have hypoplastic lungs.

The teratomas contain all three germ cell layers and are almost always benign (80%).

They are found at the level of the strap muscles and extend posteriorly towards the trachea.

SPSE 1

Question 85

What is the standard therapy for a completely resected mature teratoma in a child under age 15?

A radiation therapy to primary site

B neo-adjuvant chemotherapy if extragonadal

C observation

D radiation to primary site and six cycles of chemotherapy with cisplatin, etoposide and bleomycin (PEB)

E six cycles of chemotherapy with PEB

Answer 85

C

As long as no elements of carcinoma are found, patients with mature germ cell teratomas (regardless of location), may simply be observed at regular interval without the need for any adjuvant therapy.

Immature teratomas contain tissue from all three germ cell layers, but predominantly neuroepithelial elements.

These are graded from I–III based on the amount of immature tissue.

If malignant teratomas are noted the PEB regimen is standard of care.

SPSE 1

Question 86

Which of the following is true regarding SCTs?

A Type I SCT are predominantly external and have the highest incidence of malignancy.

B Type IV is completely external.

C Type III has a small external component but the majority of the mass is internal.

D Type II is the least common type of SCT.

E Type IV tumours usually present with congestive heart failure.

Answer 86

C

The Altman classification of SCTs is based on location.

Type I is a predominantly external.

Type II has both an intra-abdominal and an external component.

Type III is predominantly internal with a small external component.

Type IV is exclusively internal with no external component.

Tumours with an intra-abdominal component are more likely to be malignant, primarily because of the late stage at diagnosis.

In neonates the tumour is benign in about 95% of patients, children greater than 1 year of age have a higher rate of malignancy (80%).

Congestive heart failure can be secondary to shunting or haemorrhage into the tumour and is most common with the large external SCT.

SPSE 1

Question 87

Which of the following statements about paediatric testicular seminoma is false?

A If discovered at stage I patients are thought to have nearly a 100% survival rate.

B Cryptorchidism is not a risk factor for seminoma.

C There is an equal incidence in both black and white people.

D The treatment for stage I resected seminoma does not include chemotherapy.

E Patients diagnosed with testicular seminoma on one side are at increased risk for developing a contralateral seminoma on the other side.

Answer 87

C

Cryptorchidism is a risk factor for the development of testicular germ cell tumours.

Ten per cent of testis tumours are associated with cryptorchidism.

A quarter of the tumours associated with cryptorchidism are actually within the contralateral normally descended testis.

The risk of testicular cancer in a boy with cryptorchidism is directly related to the degree of maldescent.

The risk is 1 in 20 if the testis is high within the abdomen and decreases to 1 in 80 if the testis is within the inguinal canal.

After radical orchidectomy, patients with stage I seminoma are treated only with external beam radiotherapy to retroperitoneal and ipsilateral pelvic lymph nodes, typically 2500 cGy, which gives nearly 100% survival rate, chemotherapy is not required.

Although in adults the incidence of testicular cancer is much higher in white people, among paediatric patients the incidence is equal among both black and white people.

SPSE 1

Question 88

Which of the following statements is not true regarding ovarian germ cell tumours?

A All patients except those with stage I, grade I immature teratoma and stage IA dysgerminoma require postoperative chemotherapy.

B Adjuvant platinum-based chemotherapy for ovarian germ cell tumours is thought to be superior to vincristine, dactinomycin and cyclophosphamide.

C Recurrence rate of immature teratomas is inversely related to the grade of neural elements.

D Second-look laparotomy is not beneficial in patients with initially completely resected tumours who receive cisplatin-based adjuvant treatment.

E Radiation therapy for recurrent germ cell tumours is not effective.

Answer 88

C

The recurrence rate of immature teratomas is directly related to the grade of neural elements.

Both size and histology are the major factors determining prognosis for patients with immature teratomas.

Prognosis is poor for patients with large tumours when more than one-third of the tumour was composed of endodermal sinus elements, choriocarcinoma, or grade III immature teratoma.

Tumours greater than 10 cm in size also have a poor prognosis.

Studies show treatment with PEB appears to be superior to vacuum-assisted closure (VAC) as there is a 25% recurrence rate in patients treated with 6 months of VAC.

In the modern era patients treated with PEB-based chemotherapy have been found not to benefit from second-look surgical procedures because of the effectiveness of the platinum-based chemotherapy.

SPSE 1

Question 89

What percentage of patients with SCTs have associated abnormalities?

A 5%
B 10%
C 20%
D 40%
E 50%

Answer 89

C

Twenty per cent of children with SCTs have some sort of associated abnormality.

These can include cardiac, genitourinary, musculoskeletal or neurologic abnormalities.

SPSE 1

Question 90

Which of the following should not be done to workup a testicular mass?

A serum markers including AFP, β-hCG

B abdominal CT scan

C testicular ultrasound

D trans-scrotal biopsy

E chest CT scan

Answer 90

D

Trans-scrotal biopsy should not be performed; an inguinal approach to testicular lesions should be done. markers including AFP, β-hCG, AlP and lDH are often helpful in testicular cancer.

Chest CT has now replaced chest X-ray as a means of evaluating the chest for spread.

Abdominal CT scan is necessary to look at retroperitoneal nodes for staging purposes.

Testicular ultrasound also helps differentiate a solid testicular mass from other masses – which may include hydrocele, hernia and varicoceles.

SPSE 1

Question 91

Which of these statements is true regarding the reduction of serum alpha-fetoprotein (AFP) levels during the first 8 months of life?

A 150 000 ± 50 000 decreases to 25 ± 10 ng/mL.

B 33 000 ± 15 000 decreases to 75 ± 10 ng/mL.

C 48 000 ± 35 000 decreases to 8.5 ± 5.5 ng/mL

D 250 000 ± 50 000 decreases to 15 ± 5.5 ng/mL

E 500 000 ± 50 000 decreases to 25 ± 5.5 ng/mL

Answer 91

C

Serum AFP at birth is high (48 000 ± 35 000 ng/ml), and rapidly declines to normal adult values of (8.5 ± 5.5 ng/ml) by 8 months of age.

High levels of serum AFP are suggestive of a malignant mass; milder elevations are seen in hamartoma and teratoma.

SPSE 1

Question 92

Kasabach–Merritt’s syndrome is characterised by:

A infantile hepatic haemangioendothelioma, thrombocytopenia, congestive cardiac failure and hypothyroidism

B infantile hepatic haemangioendothelioma, congestive cardiac failure and thrombocytopenia

C infantile hepatic haemangioendothelioma, thrombocytopenia, congestive cardiac failure and hyperthyroidism

D infantile hepatic haemangioendothelioma, congestive cardiac failure and thrombocytosis

E infantile hepatic haemangioendothelioma, congestive cardiac failure, thrombocytopenia and leucopenia.

Answer 92

B

Kasabach–merritt’s syndrome presents with congestive cardiac failure, thrombocytopenia and hepatic vascular anomalies such as infantile hepatic haemangioendothelioma.

SPSE 1

Question 93

Stage I hepatoblastoma and HCC is defined as:

A complete surgical resection

B microscopic residual tumour

C macroscopic residual tumour

D distant metastases

E disseminated disease.

Answer 93

A Staging of hepatoblastoma and HCC is as follows:

stage I – complete resection;
stage II – microscopic residual tumour;
stage III – macroscopic residual tumour; and
stage IV – distant metastases.

SPSE 1

Question 94

The five-year survival rate for gastrointestinal stromal tumours (GISTs) that have been completely resected is:

A 40%–50%
B 50%–65%
C 35%–45%
D 60%–70%
E 80%–90%.

Answer 94

B

The 5-year survival rate of completely resected GISTs is in the range of 50%–65%.

SPSE 1

Question 95

Which of the following statements about Barrett’s oesophagus is true?

A Barrett’s is a premalignant lesion leading to adenocarcinoma.

B Barrett’s is a premalignant lesion in all cases of oesophageal burns.

C Barrett’s is a premalignant lesion that never occurs in the distal oesophageal segment.

D Barrett’s is a premalignant lesion not retained in the cervical oesophagus post gastric transposition.

E Barrett’s is a premalignant lesion leading to squamous cell carcinoma.

Answer 95

A

Barrett’s oesophagus is a premalignant lesion that predisposes to oesophageal adenocarcinoma.

It can occur in the retained cervical oesophagus after gastric transposition.

The distal segment of the oesophagus must be removed as chronic inflammation from severe chronic oesophagitis predisposes to Barrett’s and adenocarcinoma after oesophageal replacement surgery.

There are case reports of adenocarcinoma of the oesophagus after chemical burns of the oesophagus.

SPSE 1

Question 96

Which of the following describes the typical location(s) of an appendiceal carcinoid tumour?

A 75% tip of the appendix, 14% body of the appendix, 11% appendicocaecal junction

B 50% tip of the appendix, 25% body of the appendix, 25% appendicocaecal junction

C 100% tip of the appendix

D 50% tip of the appendix, 50% appendicocaecal junction

E 100% appendicocaecal junction

Answer 96

A

Paediatric appendicular carcinoid is discovered at operation for appendicitis or incidentally.

The carcinoid occurs at the tip of the appendix in 75% of the cases and therefore proximal obstruction of the appendiceal lumen is not seen.

SPSE 1

Question 97

Which of the following statements is true?

A Breast cancer in mothers is the major associated malignancy in children with soft tissue sarcomas.

B Breast cancer in mothers is the major associated malignancy in children with Wilms’s tumour.

C Breast cancer in mothers is the major associated malignancy in children with yolk sac tumour.

D Breast cancer in mothers is the major associated malignancy in children with hepatocellular cancer.

E Breast cancer in mothers is the major associated malignancy in children with neuroblastoma.

Answer 97

A

In mothers of children afflicted with soft tissue sarcoma, the risk of breast cancer is 3–13.5 times greater than that of the normal population.

SPSE 1

Question 98

Which statement is true regarding sacrococcygeal teratomas?

A They are isolated and not associated with other anomalies.

B They are associated with anorectal anomalies only.

C They are associated with genitourinary and anorectal anomalies.

D They are associated with spinal anomalies only.

E They are associated with rectal atresia only.

Answer 98

C

Teratomas are associated with a spectrum of congenital anomalies.

Sacrococcygeal teratomas are associated with genitourinary tract anomalies, rectal atresia and caudal spinal cord.

SPSE 1

Question 99

Which statement is true regarding teratomas?

A Nasopharyngeal teratoma is associated with cleft palate.

B Sacrococcygeal teratomas are always isolated.

C Cranial teratomas are always isolated.

D Testicular teratomas are rare.

E Cranial teratomas are associated with cleft palates.

Answer 99

A

Nasopharyngeal teratomas are associated with disfiguring cleft palates.

SPSE 1

Question 100

A 2-year-old girl presents with intractable constipation. On examination the child was not thriving and has a large solid posterior rectal mass. What is the most likely diagnosis?

A Altman stage IV tumour

B Altman stage III tumour

C lipoma

D myelomeningocele

E dermoid

Answer 100

A

Sacrococcygeal teratomas can be classified according to Altman staging. Altman stage IV teratomas are purely intra-abdominal and usually are associated with distant metastases by the time of diagnosis.

SPSE 1

Question 101

Which of these statements regarding fetus in fetu is true?

A Fetus in fetu is a mature teratoma.

B Fetus in fetu is an immature teratoma.

C Fetus in fetu is a dermoid tumour with yolk sac components.

D Fetus in fetu is a dermoid tumour with mature cells.

E Fetus in fetu is a rhabdoid.

Answer 101

A

Fetus in fetu is rare and the current World Health organization categorisation is mature teratoma.

SPSE 1

Question 102

Which statement regarding sacrococcygeal teratoma is true?

A 80% of sacrococcygeal teratomas are benign, 10% have familial occurrence and 10% have associated anomalies.

B 70% of sacrococcygeal teratomas are benign, 5% have familial occurrence and 15% have associated anomalies.

C 90% of sacrococcygeal teratomas are benign, 10% have familial occurrence and 10% have associated anomalies.

D 80% of sacrococcygeal teratomas have immature mitotic cells, 15% have familial occurrence and 15% have associated anomalies.

E 70% of sacrococcygeal teratomas have immature mitotic cells, 5% have familial occurrence and 15% have associated anomalies.

Answer 102

A

80% of sacrococcygeal teratomas are benign and 10% have familial predilection.

Between 10% and 20% have associated anomalies such as oesophageal fistula with tracheo-oesophageal fistula, anorectal malformation, genitourinary malformations and myelomeningocele.

SPSE 1

Question 103

The cardinal step in sacrococcygeal teratoma surgery is:

A ligation of the abdominal aorta prior to excision of tumour

B excision of the coccyx after ligating the common iliacs

C excision of the coccyx and ligation of median sacral artery

D excision of the coccyx and ligation of the superior lateral sacral artery

E excision of the coccyx and ligation of the inferior lateral sacral artery.

Answer 103

C

median sacral vessels are ligated after the excision of the coccyx to achieve control over majority of the tumour vascularity.

SPSE 1

Question 104

Which statement is true regarding the incidence of intracranial tumours?

A Intracranial teratomas account for 50% of all brain tumours in early infancy.

B Gliomas account for 50% of all brain tumours in early infancy.

C Astrocytomas account for 50% of all brain tumours in early infancy.

D Craniopharyngiomas account for 50% of all brain tumours in early infancy.

E Medulloblastomas account for 50% of all brain tumours in early infancy.

Answer 104

A

Intracranial teratomas present in a bimodal age pattern and account for 50% of brain tumours in early infancy. The pineal gland is the most common site of origin.

SPSE 1

Question 105

Tumours of the testis should be approached by:

A inguinal incision
B inguinoscrotal incision
C scrotal incision
D abdominoperitoneal incision
E laparoscopy.

Answer 105

B

An inguinoscrotal incision is used. The testicular vessels are ligated at the level of the internal ring.

The tumour is excised with an island of scrotal skin.

Ipsilateral hemiscrotectomy may have to be performed.

Frozen section is useful for diagnosis and extent of excision.

SPSE 1

Question 106

Which of the following statements is true regarding testicular tumours?

A 60% of prepubertal testicular tumours are germ cell with 20% metastases to the lungs and 6% to the retroperitoneal nodes.

B 70% of prepubertal testicular tumours are germ cell with 20% metastases to the lungs and 6% to the retroperitoneal nodes.

C 50% of prepubertal testicular tumours are germ cell with 10% metastases to the lungs and 6% to the retroperitoneal nodes.

D 40% of prepubertal testicular tumours are germ cell with 20% metastases to the lungs and 10% to the retroperitoneal nodes.

E 50% of prepubertal testicular tumours are germ cell with 20% metastases to the lungs and 6% to the retroperitoneal nodes.

Answer 106

A

60% of prepubertal testicular tumours are germ cells.

Haematogenous spread to the lungs occurs in 20% and retroperitoneal lymph node metastases occur in 4%–6% of children.

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Question 107

The classic triad of presentation in a Leydig cell tumour is:

A 90% unilateral testicular mass, 10% precocious puberty and elevated 17-ketosteroid

B 90% unilateral testicular mass, 20% precocious puberty and elevated 17-ketosteroid and AFP

C 90% unilateral testicular mass, 10% precocious puberty and elevated 17-ketosteroid, AFP and human chorionic gonadotropin (hCG)

D 90% unilateral testicular mass, 20% precocious puberty and elevated 17-ketosteroid, AFP and hCG

E 90% unilateral testicular mass, 5% precocious puberty and elevated 17-ketosteroid.

Answer 107

A

Leydig cell tumour is the most common gonadal stromal tumour of adults and children.

The classic triad of presentation is precocious puberty in 10%, unilateral testicular mass in 90% and elevated 17-ketosteroid.

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Question 108

Which of the following statement is true regarding paediatric phaeochromocytoma?

A Paediatric phaeochromocytoma shows a familial pattern in 10%, occurs in extra-adrenal sites in 30% and paroxysmal hypertension is seen in 70%–90%.

B Paediatric phaeochromocytoma shows a familial pattern in 15%, occurs in extra-adrenal sites in 15% and sustained hypertension is seen in 70%–90%.

C Paediatric phaeochromocytoma shows a familial pattern in 10%, occurs in extra-adrenal sites in 30% and paroxysmal hypertension is seen in 70%–90%.

D Paediatric phaeochromocytoma shows a familial pattern in 10%, occurs in extra-adrenal sites in 30% and sustained hypertension is seen in 70%–90%.

E Paediatric phaeochromocytoma shows a familial pattern in 25%, occurs in extra-adrenal sites in 25% and sustained hypertension is seen in 70%–90%.

Answer 108

D

Ten per cent of paediatric phaeochromocytomas occur in families and 30% occur at extra-adrenal sites.

Sustained hypertension is seen in 70%–90% of children.

Paroxysmal hypertension occurs in adults.

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Question 109

Which statement regarding hypercalcaemia is false?

A Hypercalcaemia in younger and older patients presents acutely and is associated with co-morbid conditions.

B Between 10% and 20% of cancer patients develop hypercalcaemia.

C Cancer patients who develop hypercalcaemia have a 50% mortality at 1 month after it appears.

D The longer the duration of hypercalcaemia, the poorer the development of tolerance.

E Two common causes are hyperparathyroidism and malignancy.

Answer 109

D

Parathyroid hormone regulates serum calcium levels.

Calcium is bound to albumin and the most common cause of hypercalcaemia is hyperparathyroidism and malignancy.

Between 10% and 20% of cancer patients develop hypercalcaemia and it is associated with poor prognosis with 50% mortality at 1 month post hypercalcaemia clinical presentation.

The longer the duration of hypercalcaemia, the better is the tolerance, and slower the development of co-morbidities.

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Question 110

Which statement is false regarding Hodgkin’s lymphoma?

A It arises from B-cells.

B It is associated with Epstein–Barr virus.

C Reed–Sternberg cells are not characteristic.

D Positron emission tomography is a useful adjuvant to CT scan.

E Autologous bone marrow transplant is considered following relapse.

Answer 110

C

Reed–Sternberg cells are characteristic and are surrounded by a dense inflammatory infiltrate.

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Question 111

Which of the following statements is true regarding non-Hodgk lymphoma?

A Diffuse large B-cell lymphoma is the most common non-Hodgkin’s lymphoma, followed by low-grade follicular lymphoma.

B The incidence of lymphoma is high in young children.

C Cutaneous lymphomas are usually B-cell lymphomas.

D Altered immunity is not a risk factor.

E Antibiotics are contraindicated in gastric lymphoma.

Answer 111

A

Diffuse large B-cell lymphoma is the most common non-Hodgkin’s lymphoma.

The main risk factor is altered immunity.

Aggressive B-cell lymphoma is an AIDS defining illness and also can be associated with organ transplant, autoimmune disease and congenital immunodeficiency disorders.

It is associated with Epstein–Barr virus, Hepatitis C and Helicobacter pylori.

Antibiotics are the first line of treatment for low-grade localised gastric lymphoma followed by radiation as the second line of treatment.

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Question 112

A child with mixed gonadal dysgenesis presents for evaluation and further management. What advice would you give regarding gonadal surgery?

A bilateral gonadectomy

B unilateral gonadectomy of non-palpable gonad

C gonadal biopsies and observation

D do nothing

E annual assessment with pelvic CT scan and postpuberty biopsy

Answer 112

B

unilateral gonadectomy of the non-palpable gonad is recommended.

Annual review with ultrasound scan, followed by pre- and postpubertal gonadal biopsy of the gonad in the scrotum is advisable.

This is for early detection of testicular carcinoma in situ.

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Question 113

Which statement is false regarding gonadoblastoma in a child with mixed gonadal dysgenesis (45,X/46,XY)?

A Small bilateral tumours occur in 33%, and 10% are malignant.

B Early gonadectomy of non-palpable gonad is recommended.

C Early bilateral gonadectomy is recommended.

D There is a risk of seminoma in the gonad.

E These patients have neonatal testosterone imprints.

Answer 113

C

Although in the past early bilateral gonadectomy was indicated, this management policy has changed following the development of neonatal testosterone implants in the brain.

Because of the risk of seminoma, the non-palpable gonad is excised.

The palpable gonad is followed up annually with pre- and postpubertal biopsies to detect testicular carcinoma in situ.

If carcinoma in situ is detected, gonadectomy is advisable.

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Question 114

Which statement is false regarding the treatment of adrenocortical tumours?

A Surgical resection is the main stay in the treatment of these tumours.

B Debulking of unresectable tumours is beneficial.

C Chemotherapy is successful.

D Medical therapy with mitotane may benefit unresectable tumours.

E Chemotherapy is not successful.

Answer 114

C

Complete surgical resection of adrenocortical tumour is the treatment of choice.

It ensures good prognosis and long-term survival.

Chemotherapy has not been successful in these patients.

Debulking of unresectable tumour may be of benefit.

Common sites of metastases are lung, liver, lymph nodes, contralateral adrenal gland, bones, kidneys and brain. mitotane acts as an adrenolytic agent by altering mitochondrial function.

It is a useful adjuvant medical therapy in unresectable tumours.

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Question 115

Which is the most common malignant brain tumour of childhood?

A cerebellar astrocytoma
B ependymoma
C medulloblastoma
D brainstem glioma
E craniopharyngioma

Answer 115

C

medulloblastoma is the most common malignant brain tumour of childhood. Cerebellar astrocytomas are benign and curable by complete surgical excision.

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