Pathology - Tumours Of Infancy & Childhood Flashcards
What are some examples of Hamartomas?
Hemangiomas, lymphangiomas, rhabdomyomas of the heart, adenomas of the liver, and developmental cysts in the kidney, pancreas, or lungs could be considered hamartomas.
Define a Hamartoma
An excessive but focal overgrowth of cells and tissues native to the organ in which it occurs. They do NOT reproduce the normal architecture of the surrounding tissue.
Name the 4 more common benign tumours of infancy and childhood:
Hemangiomas, lymphangiomas, fibrous lesions, and teratomas.
What underlying genetic disease may hemangiomas represent?
von Hippel-Lindau disease. A rare, autosomal dominant disease due to mutation of vHL tumour suppressor gene (3p.25.3). Increases risk of a number of tumours.
Where do teratomas usually occur?
40% of childhood teratomas occur as sacrococcygeal teratomas. They are 4 times more common in females. Other sites include the testes or ovaries, and various midline locations, such as the mediastinum, retroperitoneum, and head and neck.
10% of teratomas are associated with what kind of other congenital anomalies?
Midline defects, such as defects of the hindgut and cloacal region, as well as meningocoele and spina bifida. These are not related to local effects of the teratoma.
What are the most frequent childhood cancers? Contrast this with adult cancer frequency.
Childhood:
Haematopoetic, nervous tissue (inc. sympathetic NS, adrenal medulla, retina), soft tissues, bone, and kidney.
Adult:
Epithelial; Lung, Breast, Prostate, Colon, Skin!
Which neoplasms display striking incidence in children under the age of 10?
(10 to name)
Leukaemia (ALL) Neuroblastoma Wilm's tumour Hepatoblastoma Retinoblastoma Rhabdomyosarcoma Teratoma Ewing sarcoma Posterior fossa neoplasms (medullablastoma & epyndymoma)
What is the differential of a “small round blue cell tumour”?
Neuroblastoma Wilms tumour Lymphoma Rhabdomyosarcoma Ewing sarcoma/primitive NEcto tumour
What are the characteristics of the neuroblastic tumour family?
(3 to name)
Spontaneous or therapy induced differentiation into mature elements.
Spontaneous tumour regression.
A wide range of clinical behaviour and prognosis.
What is the most lethal childhood malignancy?
Leukaemia accounts for more deaths than all other tumours combined in children under 15.
Where do neuroblastomas most commonly arise?
Where else can they occur?
40% arise in the adrenal medulla.
The remainder can occur anywhere along the sympathetic chain (brain to pelvis).
Histological features of neuroblastomas include?
Name 3
(Small, blue, round cell tumour)
Karyorrhexis, Homer-Wright pseudorossetes, and mitotic activity.
What are two important aspects of Schwann cells appearing in neuroblastomas?
- Indicates maturation into ganglioneuroma or ganglioneuroblastoma.
- Favourable prognosis.
Clinical features of Neuroblastomas?
Age, PC, metastatic spread
Usually children <2 years or age.
Large abdominal mass, fever, wt loss. Metastatic disease is widespread via blood and lymph.
PC may be due to compressive symptoms of metastatic disease.
What does the “blue berry muffin baby” have?
Metastatic Neuroblastomata: cutaneous deposits.
90% of neuroblastomas produce?
Catecholamines BUT hypertension is less commonly seen that with phaeo’s.
Hyperploidy and near-triploidy... Non-amplification of N-myc... Low telomerase expression... CD44 expression... Absence of 17q gain...
Are all what?
Favourable features for neuroblastoma disease.
Diploidy, near di/tetraploidy... N-myc expression... Telomerase expression... 17q gain... CD44 absence...
Are all what?
Unfavourable features of neuroblastoma disease.
What are the 3 groups of congenital malformations associated with (10%) of Wilms tumours?
- WAGR Syndrome:
Aniridia, genital anomalies, retardation - Denys-Drash syndrome:
Gonadal dysgenesis, early-onset nephropathy, 90% get Wilms. - Beckwith-Weidemann syndrome:
Big organs, big tongue, big head.
Clinical features of Wilms tumours?
Age, PC, metastatic
Children usually between 2-5
Present with abdominal mass, haematuria, obstruction, or hypertension.
Pulmonary metastases are often present at presentation.
Prognosis of Wilms with nephrectomy and chemo?
Very good.
90% 2 year survival.
Worse with diffuse anaplasia, hence histology is important.
Unfavourable histological feature of a Wilms tumour?
Diffuse anaplasia.
Prognosis of neuroblastomas?
Dependant on stage, if 1-2 then 90% 5 year survival.
Even with unfavourable features, survival can be 50%.
