Chapter 11: Tumor Flashcards

Question

1/3 of the Pyogenic Granuloma deveolp after what?

Answer 1

trauma reaching a size of **1 to 2 cm within a few weeks** The proliferating capillaries are often accompanied by **extensive edema and an acute and chronic inflammatory infiltrate,**strikingly similar to exuberant**granulation tissue**.

Answer 2

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

Answer 3

Lymphangiomas are the **benign lymphatic analogues of blood vessel hemangiomas**

Answer 4

. These are **composed of small lymphatic channels** predominantly occurring in the **head, neck, and axillary subcutaneous tissues.** They are **slightly elevated or sometimes pedunculated lesions up to 1 to 2 cm in diameter**.

Answer 5

Histologically, lymphangiomas exhibit networks of endothelium-lined spaces that can be distinguished from capillary channels **only by the absence of erythrocytes.**

Answer 6

These lesions are **typically found in the neck or axilla of childre**n, and **rarely occur in the retroperitoneum;**

Answer 7

cavernous lymphangiomas of the neck are common in **Turner syndrome**

Answer 8

Cavernous lymphangiomas can occasionally be enormous (up to 15 cm in diameter) and may fill the axilla or produce gross deformities about the neck. Tumors are **composed of massively dilated lymphatic** spaces lined by endothelial cells and separated by intervening connective tissue stroma containing lymphoid aggregates. The tumor margins are not discrete and the lesions are not encapsulated, making definitive resection difficult.

Answer 9

Glomus tumors are benign, exquisitely painful tumors arising from modified smooth muscle cells of the glomus body, a specialized arteriovenous structure involved in thermoregulation. Although they can resemble cavernous hemangiomas, glomus tumors constitute a distinct entity by virtue of their constituent cells. They are most commonly found in the distal portion of the digits, especially under the fingernails. Excision is curative.

Answer 10

distinct entity by virtue of their constituent cells. They are **most commonly found in the distal portion of the digits**, especially **under the fingernails.** Excision is curative

Answer 11

* round, * slightly elevated, * red-blue, * firm nodules (usually \<1 cm in diameter) that initially resemble a minute focus of hemorrhage.

Answer 12

**aggregates, nests, and masses of specialized glomus cells** intimately associated with branching vascular channels, all within a connective tissue stroma. Individual tumor cells are small, uniform, and round or cuboidal, with scant cytoplasm and ultrastructural features akin to smooth muscle cells

Answer 13

Vascular ectasias are common lesions characterized by **local dilation of preexisting vessels;** they are **not true neoplasms.** Telangiectasia is a term used for a congenital anomaly or acquired exaggeration of preformed vessels—usually in the skin or mucous membranes —composed of capillaries, venules, and arterioles that creates a discrete red lesion

Answer 14

Telangiectasia is a term used for a **congenital anomaly or acquired exaggeration of preformed vessels**—usually in the**skin or mucous membranes** —composed of capillaries, venules, and arterioles that creates a discrete red lesion

Answer 15

This lesion is the **ordinary “birthmark” and is the most common form of ectasia**; it is * *characteristically a flat lesion on the head or neck,** ranging in color from **light pink to deep purple. ** Histologically, there is only vascular dilation; most ultimately regress.

Answer 16

**special form of nevus flammeus**; these lesions **tend to grow with a child, thicken the skin surface, and demonstrate no tendency to fade**.

Answer 17

**Such nevus flammeus in a trigeminal nerve distribution**are occasionally associated with the Sturge-Weber syndrome (also called **encephalotrigeminal angiomatosis).** Sturge-Weber syndrome is a rare congenital disorder **associated with venous angiomatous masses** in the **cortical leptomeninges and ipsilateral facial port wine nev**i;**mental retardation,** seizures, hemiplegia, and skull radioopacities also occur Thus, a large facial vascular malformation in a child with mental deficiency may indicate the presence of more extensive vascular malformations.

Answer 18

This non-neoplastic vascular lesion g**rossly resembles a spider**; there is a **radial, often pulsatile array of dilated subcutaneous arterie**s or arterioles (resembling legs) about a central core (resembling a body) that blanches when pressure is applied to its center. It is c**ommonly seen on the face, neck,**or**upper chest and is most frequently associated with hyper-estrogenic states such as pregnancy or patients with cirrhosis;**how elevated estrogen levels contribute to “spider” formation is not known.

Answer 19

It is commonly seen on the **face, neck, or upper chest and is most frequently associated with hyper-estrogenic** states such as pregnancy or patients with cirrhosis; how **elevated estrogen levels contribute to “spider” formation is not known.**

Answer 20

* autosomal dominant disorder the telangiectasias are malformations composed of dilated capillaries and veins. * Present from birth, they are widely distributed over the skin and oral mucous membranes, as well as in the respiratory, gastrointestinal, and urinary tracts. * Occasionally, these lesions rupture, causing serious epistaxis (nosebleeds), GI bleeding, or hematuria.

Answer 21

* vascular proliferation resulting from an opportunistic infection in immunocompromised individuals; * lesions can involve skin, bone, brain, and other organs. * **First described in patients with acquired immunodeficiency syndrome (AIDS)**

Answer 22

infection with **gram-negative bacilli of the *Bartonella family.***

Answer 23

* **Bartonella henselae**, the organism responsible for cat-scratch disease (the domestic cat is the principal reservoir), and * **B. quintana,** the cause of “trench fever” in World War I (the organism is transmitted by human body lice)

Answer 24

* Skin lesions are red papules and nodules, or rounded subcutaneous masses; * histologically, there is capillary proliferation with prominent epithelioid endothelial cells exhibiting nuclear atypia and mitoses ( Fig. 11-31 ). * Lesions contain neutrophils, nuclear dust, and the causal bacteria. Though difficult to cultivate in the laboratory, the **bacteria can be unequivocally demonstrated using molecular methods**such as**polymerase chain reaction** with species-specific primers. The vascular proliferation results from **induction of host HIF-1α by the bacteria; HIF-1α in turn drives VEGF production**. [86]

Answer 25

The infections (and lesions) are cleared by macrolide antibiotics (including erythromycin

Answer 26

FIGURE 11-31 Bacillary angiomatosis. A, Photograph of a cutaneous lesion . B, Histologic appearance with acute neutrophilic inflammation and vascular (capillary) proliferation. (Inset) Demonstration by modified silver (Warthin-Starry) stain of clusters of tangled bacilli (black).

Answer 27

* Chronic KS (also called classic or European KS) * Lymphadenopathic KS (also called African or endemic KS) * Transplant-associated KS * AIDS-associated (epidemic) KS

Answer 28

Kaposi Sarcoma

Answer 29

``` Chronic KS (also called **classic or European KS)** was first described by Kaposi in 1872; it **characteristically occurs in older men** of **Eastern European (especially Ashkenazi Jews) or Mediterranean descent and is uncommon in the United States**. ``` While chronic KS can be associated with an underlying second malignancy or altered immunity, it is **not associated with human immunodeficiency virus (HIV).**

Answer 30

Chronic KS presents with **multiple red to purple skin plaques or nodules,** usually in the **distal lower extremities;** these **slowly increase in size** and **number and spread more proximally**. Although locally persistent, the tumors are typically asymptomatic and remain localized to the skin and subcutaneous tissu

Answer 31

* also called African or endemic KS) * has the **same general** **geographic distribution as Burkitt lymphoma and is particularly prevalent among South** **African Bantu children** * it is also **not associated with HIV** *

Answer 32

* .Skin lesions are sparse, and * patients present **instead with lymphadenopathy due to KS involvement**; * the tumor occasionally involves the viscera and is extremely aggressive. In combination with AIDSassociated KS (see below), KS is now the most common tumor in central Africa (50% of * all tumors in men in some countries)

Answer 33

occurs in the setting of **solid-organ transplantation** with **its attendant long-term immunosuppression**. It tends to be **aggressive (even fatal**) with **nodal, mucosal, and visceral involvement;** **cutaneous lesions may be absent.** Lesions oc**casionally regress when immunosuppressive therapy is attenuated, but at the risk of organ rejection.**

Answer 34

was **originally found in a third of AIDS patients,** particularly **male homosexuals** ( Chapter 6 ). However, with current regimens of antiretroviral therapy, KS incidence is now less than 1% (although it is **still the most prevalent malignancy** in AIDS patients in the United States). AIDS-associated KS can involve lymph nodes or viscera and disseminates widely early in the course of the disease. Most patients eventually die of opportunistic infections rather than from KS.

Answer 35

human herpesvirus-8 (HHV-8) or KSassociated herpesvirus (KSHV) Indeed, regardless of the clinical subtype (described above), 95% of KS lesions have subsequently been shown to be KSHV-infected. [88] Like Epstein-Barr virus, **KSHV is a member of the γ-herpesvirus subfamily;**it is**transmitted sexually** and by poorly understood nonsexual routes—perhaps **including saliva.**

Answer 36

**KSHV is accepted as a necessary requirement for KS** development, but **tumor progression also requires a cofactor;** *HIV clearly can provide this*, but the identity of the cofactor in non-HIVassociated KS is controversial. KSHV induces a lytic as well as a latent infection in endothelial cells, both of which are probably important in KS pathogenesis. Cytokines derived from HIVinfected T cells, or inflammatory cells recruited in response to the lytic infection, create a **local proliferative milieu**; a virally encoded G protein also induces local VEGF production. In latently infected cells, KSHV proteins disrupt normal cellular proliferation controls and prevent apoptosis by viral production of p53 inhibitors and a viral homologue of cyclin D. Thus, **latently infected cells have a growth advantage**; the local environment also favors cellular proliferation. The increasing recognition of the various viral gene products has nevertheless opened a number of new avenues for therapeutic interventions against affected intracellular kinase pathways and downstream targets. In its early stages, only a few cells are infected; with time virtually all spindle cells of late-stage lesions carry KSHV; these spindle cells express both endothelial cell and smooth muscle cell markers.

Answer 37

* patch, * plaque, and * nodule.

Answer 38

Patches are **red to purple macules** typically **confined to the distal lower extremities** ( Fig. 11-32A ). Histology shows only **dilated irregular endothelial cell**–**lined vascular spaces with interspersed lymphocytes, plasma cells, and macrophages (sometimes containing hemosiderin)**. The lesions can be difficult to distinguish from granulation tissue.

Answer 39

With time, lesions spread proximally and become larger, violaceous, ***raised plaques*** (see Fig. 11-32A ) **composed of dermal accumulations of dilated, jagged vascular channels lined and surrounded by plump spindle cells.** Scattered between the vascular channels are extravasated red cells, **hemosiderin-laden macrophages, and other mononuclear inflammatory cells.**

Answer 40

Eventually, **lesions become nodular and more distinctly neoplastic**. These lesions are composed of **sheets of plump, proliferating spindle cell**s, mostly in the **dermis or subcutaneous tissues**( Fig. 11-32B ),**encompassing small vessels and slitlike spaces containing red cells.** More marked hemorrhage, hemosiderin pigment, and mononuclear inflammation is present; mitotic figures are common, as are round, pink, cytoplasmic globules of uncertain nature. The nodular stage often heralds nodal and visceral involvement, particularly in the African and AIDS-associated variants.

Answer 41

FIGURE 11-32 Kaposi sarcoma. A, Gross photograph, illustrating coalescent red-purple macules and plaques of the skin. B, Histologic appearance of nodular form, demonstrating sheets of plump, proliferating spindle cells.

Answer 42

The course of KS **varies widely** and is **significantly affected by the clinical setting.** Most primary KSHV infections are **asymptomatic.** Classic KS is—at least initially—largely restricted to the surface of the body, and surgical resection is usually adequate for an excellent prognosis.

Answer 43

* **Radiation can be used for multiple lesions** in a restricted area, and chemotherapy yields satisfactory results for more disseminated disease. * Lymphadenopathic KS can also be * *treated** **with chemotherapy or radiation therapy** with good results. * In i **mmunosuppression-associated** **KS**, **withdrawal of immunosuppression** (perhaps with adjunct chemotherapy or radiation therapy) is often effective. * For **AIDS-associated KS, antiretroviral therapy for HIV** is usually helpful, **with or without therapy targeted to the KS lesions.** * **IFN-α and angiogenesis inhibitors** are variably effective, while newer strategies aimed at specific intracellular kinase pathways or the downstream mammalian target of rapamycin are showing promise

Answer 44

Hemangioendothelioma **denotes a wide spectrum of vascular neoplasms** with **clinical behaviors intermediate between benign**,**well-differentiated hemangiomas and highly malignant angiosarcomas**, described below. Epithelioid hemangioendothelioma is an example; it is a vascular tumor of adults occurring around medium-sized and large veins . The tumor cells are plump and often cuboidal (resembling epithelial cells); well-defined vascular channels are inconspicuous. Clinical behavior is variable; most are cured by excision, but up to 40% recur, 20% to 30% eventually metastasize, and perhaps 15% of patients die of the tumors.

Answer 45

Angiosarcomas are **malignant endothelial neoplasms (** Fig. 11-33 ) with **histology varying from well-differentiated tumors that resemble hemangiomas (hemangiosarcoma) to anaplastic lesions** d**ifficult to distinguish from carcinomas or melanomas.**

Answer 46

* FIGURE 11-33 Angiosarcoma. * A, Gross photograph of angiosarcoma of the heart (right ventricle). * B, Photomicrograph of moderately well-differentiated angiosarcoma with densen clumps of irregular, moderate anaplastic cells and distinct vascular lumens. * C, Immunohistochemical staining for the endothelial cell marker CD31, demonstrating the endothelial nature of the tumor cells.

Answer 47

**Older adults are more commonly affected,**with**equal gender predilections;**they occur at**any site but most often involve skin, soft tissue, breast, and liver.**

Answer 48

Hepatic angiosarcomas are associated with carcinogen exposures, **including:** * **arsenic (arsenical** **pesticides),** * **Thorotrast (a radioactive contrast age**nt formerly used for radiologic imaging), and * **polyvinyl chloride (a widely used plastic**). All of these agents have long latent periods between initial exposure and eventual tumor development. The increased frequency of angiosarcomas among polyvinyl chloride workers is one of the well-documented instances of human chemical carcinogenesis.

Answer 49

True Angiosarcomas can also arise in the setting of lymphedema, classically in the i**psilateral upper extremity several years after radical mastectomy**(i.e., with lymph node resection) for breast cancer; the tumor presumably arises from lymphatic vessels (lymphangiosarcoma). Angiosarcomas have also been induced by radiation and are rarely associated with foreign material introduced into the body either iatrogenically or accidentally.

Answer 50

Cutaneous angiosarcomas can begin as deceptively small, sharply demarcated, asymptomatic, often multiple red nodules; most eventually become large, fleshy masses of red-tan to gray-white tissue ( Fig. 11-33A ). The margins blend imperceptibly with surrounding structures. Central areas of necrosis and hemorrhage are frequent.

Answer 51

Cutaneous angiosarcomas can begin as deceptively small, sharply demarcated, asymptomatic, often multiple red nodules; most eventually become large, fleshy masses of red-tan to gray-white tissue ( Fig. 11-33A ). The margins blend imperceptibly with surrounding structures. Central areas of necrosis and hemorrhage are frequent.

Answer 52

Microscopically, **all degrees of differentiation** can be seen, **from plump, anaplastic** but recognizable endothelial cells producing vascular channels ( Fig. 11-33B ) to wildly undifferentiated tumors with a solid spindle cell appearance and without definite blood vessels.

Answer 53

**CD31 or von Willebrand factor**

Answer 54

angiosarcomas are **locally invasive** and **can metastasize readily**. Angiosarcomas are aggressive tumors with **current 5-year survival rates approaching 30%.**

Answer 55

Hemangiopericytomas are **rare tumor**s derived from **pericytes**—myofibroblast-like cells that are normally arranged around capillaries and venules.

Answer 56

Hemangiopericytomas can **occur as slowly** **enlarging, painless masses at any anatomic site**, but are **most common on the lower extremities (especially the thigh) and in the retroperitoneum.**

Answer 57

but are most common on the **lower extremities (especially the thigh) and in the retroperitoneum**.

Answer 58

They consist of **numerous branching capillary** channels and **gaping sinusoidal spaces** enclosed within nests of spindle-shaped to round cells. Special stains confirm that these cells are outside the endothelial cell basement membrane and are therefore pericytes. The t**umors may recur after excision, and roughly half will metastasize, usually hematogenously to lungs, bone, or liver.**