Tumors Lf3

Front 1

most common tumors of infancy?

Back 1

hemangiomas

Front 2

what are port wine stains?

Back 2

In
 children
 most
 hemangiomas
 are
 located
 in
 the
 skin,
 pa rticularly
 on
 the
 face
 and
 scalp,
 
where
 they
 produce
 flat
 to
 elevated,
 irregular,
 red -­‐blue
 masses;
 the
 flat,
 larger
 lesions
 are
 
referred
 to
 as
  port
 wine stains .
 

Front 3

clinical significance of hemangiomas?

Back 3

Usually
 of
 no
 clinical
 significance,
 but
 they
 may
 be
 a
 manifestation
 of
 a
 hereditary
 disorder
 
associated
 with
 disease
 within
 internal
 organs,
 such
 as
 the
 von
 Hippel -­‐Lindau
 and
 Sturge -­‐
Weber
 syndromes.

Front 4

what is von hippel lindau sy. (VHL)?

Back 4

Von Hippel–Lindau (VHL) disease is a rare, autosomal dominant genetic condition that predisposes individuals to benign and malignant tumours. The most common tumours found in VHL are central nervous system and retinal hemangioblastomas, clear cell renal carcinomas, pheochromocytomas, pancreatic neuroendocrine tumours, pancreatic cysts, endolymphatic sac tumors and epididymal papillary cystadenomas.[1][2] VHL results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome 3p25.3.[3]

Front 5

hemangiomatosis?

Back 5

some hemangiomas can involve large portions of the body (called angiomatosis)

Front 6

what is a pregnancy tumor?

Back 6

Pregnancy tumor (granuloma gravidarum) is a pyogenic granuloma that occurs in the gingiva of 1% of pregnant women. These lesions can spontaneously regress (especially after pregnancy) or undergo fibrosis; in some cases surgical excision is required. Recurrence is rare.

Front 7

clinical significance of lymphangiomas?

Back 7

Though histologically benign, they tend to increase in size after birth and may encroach on mediastinal structures or nerve trunks in axilla.

Front 8

are sacrococcygeal teratomas malignant?

Back 8

Approximately 75% of these tumors are histologically mature with a benign course, and about 12% are unmistakably malignant and lethal

Front 9

small round blue cell tumors?

Back 9

Because of their primitive histologic appearance, many childhood tumors have been collectively referred to as small, round, blue cell tumors. These are characterized by sheets of cells with small, round nuclei.

e.g. neuroblastoma, lymphoma, rhabdomyosarcoma, Ewing sarcoma (peripheral neuroectodermal tumor), and some cases of Wilms' tumor.

Front 10

describe neuroblastoma

Back 10

The term "neuroblastic tumor" includes tumors of the sympathetic ganglia and adrenal medulla that are derived from primordial neural crest cells populating these sites; neuroblastoma is the most important member of this family.

Front 11

what is heterotopia? examples?

Back 11

Heterotopia or choristoma refers to microscopically normal cells or tissues that are present in abnormal locations.

Examples include a pancreatic tissue "rest" found in the wall of the stomach or small intestine, or a small mass of adrenal cells found in the kidney, lungs, ovaries, or elsewhere. Heterotopic rests are usually of little significance, but they can be confused clinically with neoplasms.

Front 12

what is choristomia? examples?

Back 12

Heterotopia or choristoma refers to microscopically normal cells or tissues that are present in abnormal locations.

Examples include a pancreatic tissue "rest" found in the wall of the stomach or small intestine, or a small mass of adrenal cells found in the kidney, lungs, ovaries, or elsewhere. Heterotopic rests are usually of little significance, but they can be confused clinically with neoplasms.

Front 13

define hamartoma. cellular properties? tissue architecture? is it a neoplastic phenomenon?

Back 13

Hamartoma refers to an excessive but focal overgrowth of cells and tissues native to the organ in which it occurs. Although the cellular elements are mature and identical to those found in the remainder of the organ, they do not reproduce the normal architecture of the surrounding tissue. Hamartomas can be thought of as the linkage between malformations and neoplasms. The line of demarcation between a hamartoma and a benign neoplasm is frequently tenuous and is variously interpreted. Hemangiomas, lymphangiomas, rhabdomyomas of the heart, and adenomas of the liver are considered by some to be hamartomas and by others to be true neoplasms.

Front 14

three hallmarks of neoplasia in infancy/childhood?

Back 14

1) Relatively frequent demonstration of a close relationship between abnormal development (teratogenesis) and tumor induction (oncogenesis)
2) Prevalence of constitutional genetic abnormalities or syndromes that predispose to cancer
3) Tendency of fetal and neonatal malignancies to spontaneously regress or undergo "differentiation" into mature elementsImproved survival or cure of many childhood tumors, so that more attention is now being paid to minimizing the adverse delayed effects of chemotherapy and radiotherapy in survivors, including the development of second malignancies

Front 15

which kind of rosettes can we find in neuroblastoma histology preparations?

Back 15

Typically, rosettes (Homer-Wright pseudo-rosettes) can be found in which the tumor cells are concentrically arranged about a central space filled with neuropil.

Front 16

what is the difference between a neuroblastoma and a ganglioneuroma?

which is considered a more serious condition?

Back 16

In fact, the presence of a "schwannian stroma" composed of organized fascicles of neuritic processes, mature Schwann cells, and fibroblasts is a histologic prerequisite for the designation of ganglioneuroblastoma and ganglioneuroma; ganglion cells in and of themselves do not fulfill the criteria for maturation. (in that case, with only ganglion cells and not schwann cells, i think this is called something inbetween - ganglioneuroblastoma)


evidence of schwannian stroma and gangliocytic differentiation is indicative of a "favorable" histology.!

Front 17

in which neoplastic disorder can we find an amplification of the MYCN oncogene?

Back 17

neuroblastoma. MYCN amplification is present in about 25% to 30% of primary tumors, most in advanced-stage disease; the greater the number of copies, the worse the prognosis. MYCN amplification is currently the most important genetic abnormality used in risk stratification of neuroblastic tumors.

Front 18

what is meant by "blueberry muffin baby"?

Back 18

In neonates, disseminated neuroblastomas may present with multiple cutaneous metastases with deep blue discoloration to the skin (earning the rather unfortunate moniker of "blueberry muffin baby")

Front 19

about 90% of neuroblastoma secrete ........ which is important for diagnostics

Back 19

About 90% of neuroblastomas, regardless of location, produce catecholamines (similar to the catecholamines associated with pheochromocytomas), which are an important diagnostic feature (i.e., elevated blood levels of catecholamines and elevated urine levels of catecholamine metabolites such as vanillylmandelic acid [VMA] and homovanillic acid [HVA]). Despite the elaboration of catecholamines, hypertension is much less frequent with these neoplasms than with pheochromocytomas (Chapter 20).

Front 20

neuroblastoma arises from which cell type?

Back 20

Neuroblastomas and related tumors arise from neural crest-derived cells in the sympathetic ganglia and adrenal medulla

Front 21

Hereditary tumor genetically characterized by deletion of the short arm of chromosome13 (13q14p) is called?

Back 21

retinoblastoma

Front 22

which type of rosettes are specific for retinoblastoma histo-slides?

Back 22

Differentiated structures are found within many retinoblastomas, the most characteristic of these being the rosettes described by Flexner and Wintersteiner (Flexner-Wintersteiner rosettes; Fig. 7-35B). These structures consist of clusters of cuboidal or short columnar cells arranged around a central lumen (contrast with the pseudo-rosettes of neuroblastoma, which lack a central lumen).

Front 23

cat's eye reflex is seen in which neoplastic disorder?

Back 23

retinoblastoma

Front 24

781. The retinoblastoma gene regulates:
a) DNA transcription
b) signal transmission in cells
c) cell cycle progression
d) apoptosis
e) repair DNA

Back 24

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Front 25

which congenital disorders are associated with Wilm's tumor?

Back 25

Patients with the WAGR syndrome, characterized by aniridia, genital abnormalities, and mental retardation, have a 33% chance of developing Wilms' tumor. Another group of patients, those with the so-called Denys-Drash syndrome (DDS) also has an extremely high risk (∼90%) of developing Wilms' tumor

Front 26

709. Tumor associated with congenital deletion on chromosome 11 is?

Back 26

Wilm's tumor

Front 27

755. Which of the following tumor types has an autosomal dominant mode of inheritance?
a) Squamous cell carcinoma in lungs
b) Colloid colon cancer
c) Wilms tumor
d) Pleomorphic adenoma
e) Teratoma

Back 27

Wilm's tumor


... are there any other tumors with AD inheritance i should know of?

Front 28

what is a germ cell tumor?

770. Which of the following cancers belong among germ cell tumors?
a) Embryonal rhadbomyosarcoma
b) Wilms tumor (nephroblastoma)
c) Teratoma
d) Medulloblastoma
e) Hamartoma

Back 28

i have no idea what it is,

but teratoma is the right answer i think.

Front 29

823. Supraclavicular Lymph node biopsy of a 60 year old man revealed metastasis. What is probably the tumor
a) leiomyosarcoma
b) Kidney Wilms tumor
c) humerus osteosarcoma
d) pancreatic cancer
e) embryonic carcinoma

Back 29

how can we answer this???

Front 30

what is mask of pregnancy?

Back 30

Melasma (also known as Chloasma faciei,[1]:854 or the mask of pregnancy[2] when present in pregnant women) is a tan or dark skin discoloration. Although it can affect anyone, melasma is particularly common in women, especially pregnant women and those who are taking oral or patch contraceptives or hormone replacement therapy (HRT) medications. It is also prevalent in men and women of Native American descent (on the forearms) and in men and women of German, Russian, and Jewish descent (on the face).[citation needed]

Melasma is thought to be the stimulation of melanocytes or pigment-producing cells by the female sex hormones estrogen and progesterone to produce more melanin pigments when the skin is exposed to sun.

Front 31

what is the difference between Lentigines and ephelis?

Back 31

A lentigo (plural: lentigines) is a small pigmented spot on the skin with a clearly-defined edge, surrounded by normal-appearing skin. It is a harmless (benign) hyperplasia of melanocytes which is linear in its spread.

Lentigines are distinguished from freckles (ephelis) based on the proliferation of melanocytes. Freckles have a relatively normal number of melanocytes but an increased amount of melanin.

Front 32

can a hairy mole be malignant?

Back 32

no, malignant cells would destroy the hair appendages!

Front 33

713. Which of the following process is an example of dysplasia?
a. Actinic keratosis
b. Chronic cystitis
c. Chronic bronchitis
d. Ulcerative colitis
e. Pigmented nevus

Back 33

???????

Front 34

difference between a mixed tumor and teratoma?

Back 34

The multifaceted mixed tumors should not be confused with a teratoma, which contains recognizable mature or immature cells or tissues representative of more than one germ-cell layer and sometimes all three.

Front 35

what is a mixed tumor (pleomorphic neoplasm???)

Back 35

WIKI: A Mixed tumor is a tumor that derives from multiple tissue types.[1]

For example, a "Chondroid syringoma" is a cutaneous condition characterized histologically by nests of cuboidal or polygonal epithelial cells in the dermis.[2]:667

and it's of two types : 1-derived from a single germ cell layer that differentiates into more than one cell type 2-derived from more than one germ cell layer(totipotent cells)


ROBBINS:

neoplasms are of monoclonal origin, as is discussed later. In some instances, however, the tumor cells may undergo divergent differentiation, creating so-called mixed tumors. The best example is mixed tumor of salivary gland. These tumors have obvious epithelial components dispersed throughout a fibromyxoid stroma, sometimes harboring islands of cartilage or bone (Fig. 6-2). All of these diverse elements are thought to derive from epithelial cells, myoepithelial cells, or both in the salivary glands, and the preferred designation of these neoplasms is pleomorphic adenoma. Fibroadenoma of the female breast is another common mixed tumor. This benign tumor contains a mixture of proliferated ductal elements (adenoma) embedded in a loose fibrous tissue (fibroma). Although studies suggest that only the fibrous component is neoplastic, the term fibroadenoma remains in common usage

Front 36

give some examples of tumors with the benign suffix -oma, but which are actually malignant.

Back 36

lymphoma, mesothelioma, melanoma, and seminoma

Front 37

describe "choristoma".

Back 37

Another misnomer is the term choristoma. This congenital anomaly is better described as a heterotopic rest of cells. The term choristoma, connoting a neoplasm, imparts to the heterotopic rest a gravity far beyond its usual trivial significance.

Front 38

describe hamartoma

Back 38

Hamartoma is a malformation that presents as a mass of disorganized tissue indigenous to the particular site. One may see a mass of mature but disorganized hepatic cells, blood vessels, and possibly bile ducts within the liver, or there may be a hamartomatous nodule in the lung containing islands of cartilage, bronchi, and blood vessels.

Front 39

difference between hamartoma and choristoma?

Back 39

long story short; hamartoma represents disorganized tissue native to its location (e.g. one may see a mass of mature but disorganized hepatic cells, blood vessels, and possibly bile ducts within the liver, or there may be a hamartomatous nodule in the lung containing islands of cartilage, bronchi, and blood vessels.)

choristoma is basically the same phenomenon, but the disorganized tissue is located in an abnormal site, e.g. pancreatic rests in the stomach submucosa.

Front 40

what are xanthomas? etiology?

Back 40

A xanthoma (pl. xanthomas or xanthomata) (condition: xanthomatosis), from Greek xanthos, ξανθος, "yellow", is a deposition of yellowish cholesterol-rich material in tendons or other body parts in various disease states.[1] They are cutaneous manifestations of lipidosis in which there is an accumulation of lipids in large foam cells within the skin.[1]

They are associated with hyperlipidemias, both primary and secondary types.

Front 41

what is Muir-Torre syndrome?

Back 41

Muir–Torre syndrome (MTS) is an inherited cancer syndrome[1]:663 that is thought to be a subtype of HNPCC. Individuals are prone to develop cancers of the colon, breast and genitourinary tract, and skin lesions, such as keratoacanthomas and sebaceous tumours. The genes affected are MLH1 & MSH2, and involved in DNA mismatch repair.

Front 42

histologic/morphologic features of actinic (sun-induced) keratosis?

Back 42

A
 useful
 acronym
 for
 remembering
 the
 histologic
 features
 is
 SPAIN
 (a
 sun-­‐
soaked
 country
 perfect
 for
 acquiring
 such
 lesions):
 
• Solar
 elastosis
 (dermal
 sun
 damage)
 
• Parakeratosis
 
 
• Atypia
 (keratinocytic)
 
• Inflammation
 (lymphocytes
 in
 the
 superficial
 dermis)
 
 
• Not
 full
 thickness
 (atypia)
 

Front 43

lesser trelat's sign?

Back 43

The Leser-Trélat sign is the explosive onset of multiple seborrheic keratoses[1] (many pigmented skin lesions),[2]:638[3] often with an inflammatory base. This can be an ominous sign of internal malignancy as part of a paraneoplastic syndrome.

Front 44

fibroblast growth factor (FGF) receptor 3 mutations is associated with which disorder?

Back 44

seborrheic keratosis

Front 45

how does UV light contribute to carcinogenesis?

Back 45

1) direct cause of mutations
2) impairs the antigen presentation on the langerhans cells - thus supressing immunosurveillance

Front 46

acantholytic and dyskeratinotic properties, varying degrees of anaplasia, sometimes lobules with zones of keratinization.... which tumor?

Back 46

squamous cell carcinoma in situ

Front 47

causes/predisposing conditions, of squamous cell carcinoma?

Back 47

In addition to sunlight, predisposing factors include industrial carcinogens (tars and oils), chronic ulcers, old burn scars, ingestion of arsenicals, and ionizing radiation.

Bowen's disease (BD) (also known as "squamous cell carcinoma in situ"[1]:655) is a neoplastic skin disease, it can be considered as an early stage or intraepidermal form of squamous cell carcinoma.

Front 48

what is Bowen's disease?

Back 48

Bowen's disease (BD) (also known as "squamous cell carcinoma in situ"[1]:655) is a neoplastic skin disease, it can be considered as an early stage or intraepidermal form of squamous cell carcinoma.

Front 49

what is keratoachantoma? etiology?

Back 49

Keratoacanthoma (KA) is a common low-grade (unlikely to metastasize or invade) skin tumour that is believed to originate from the neck of the hair follicle.[1] Many pathologists consider it to be a form of squamous cell carcinoma (SCC).

The tumors usually occur in older individuals (mean age 64 years old). Like squamous cell cancer, data suggests ultraviolet light from the sun causes the development of KA.[9] Just like its close relative, the squamous cell cancer, sporadic cases have been found co-infected with the human papilloma virus (HPV).

Front 50

which are more aggressive? squamous cell carcinomas of skin or mucous membranes?

Back 50

Invasive squamous cell carcinomas of the skin are often discovered while small and resectable; less than 5% have metastases to regional nodes at diagnosis. Mucosal squamous cell carcinomas (oral, pulmonary, esophageal, etc.) are generally a much more aggressive.

Front 51

PTCH dysregulation is commonly found in which tumor?

Back 51

basal cell carcinoma -- PTCH dysregulation is also often accompanied with developmental abnormalities, since the PTCH pathway is important in embryogenesis

Front 52

what are the two common growth patterns of basal cell carcinoma?

Back 52

Two common patterns are seen: either multifocal growths originating from the epidermis (superficial type), or nodular lesions growing downward into the dermis as cords and islands of variably basophilic cells with hyperchromatic nuclei, embedded in a fibrotic to mucinous matrix

Front 53

clincal features, and treatment of basal cell carcinoma?

Back 53

Clinically, these tumors present as pearly papules, often containing prominent, dilated subepidermal blood vessels (telangiectasia) (Fig. 22-18C). Some tumors contain melanin pigment and thus appear similar to melanocytic nevi or melanomas. Advanced lesions may ulcerate, and extensive local invasion of bone or facial sinuses may occur after many years of neglect. These tumors are usually treated with complete local excision, although immunomodulatory therapies that direct the innate immune response against the tumor are currently being tested as well.

Front 54

definition of melanocytic nevus?

Back 54

Strictly speaking, the term nevus denotes any congenital lesion of the skin. Melanocytic nevus, however, refers to any benign congenital or acquired neoplasm of melanocytes.

Front 55

which mutation is common for melanocytic nevi?

Back 55

The majority of benign nevi have been shown to harbor an activating mutation in BRAF (a protein downstream from RAS in the extracellular receptor kinase pathway) or less commonly in RAS itself.

Front 56

what causes hyperkeratosis in psoriasis?

Back 56

T cells homing to the skin secrete cytokines promoting keratinocyte hyperproliferation.