Pathological Processes and Neoplasm Characteristics

Edema

Edema is the abnormal accumulation of fluid in the interstitium or body cavities due to an imbalance in fluid homeostasis.

Types of Edema

Types include pitting edema, caused by protein deficiency, increased hydrostatic pressure, increased capillary permeability, or fluid retention; and non-pitting edema, caused by lymphatic obstruction or hypothyroidism (myxedema).

Edema Pathogenesis

Pathogenesis involves decreased plasma oncotic pressure, increased capillary hydrostatic pressure, lymphatic obstruction, increased interstitial oncotic pressure, increased capillary permeability, and sodium and water retention.

Fluid Types in Edema

Fluid types are classified as transudate, which is low in protein and non-inflammatory, or exudate, which is high in protein and associated with inflammation.

Pulmonary Edema

Pulmonary edema results from heart failure or capillary injury, leading to fluid accumulation in alveoli, presenting as heavy, frothy lungs with hyaline membranes.

Cerebral Edema

Cerebral edema is life-threatening and includes vasogenic, cytotoxic, and interstitial types. It lacks lymphatic drainage and is regulated by the blood-brain barrier.

Clinical Relevance of Edema

Clinical relevance depends on the underlying cause, location, and severity. Edema can be localized or generalized.

Hemorrhages

Hemorrhage is the escape of blood from blood vessels, either externally or internally into tissues or body cavities.

Types of Hemorrhage

Types include external hemorrhage and internal hemorrhage into serous cavities (e.g., hemothorax, hemoperitoneum), hollow viscera, or tissues.

Hemorrhage Terminology

  • Hematoma: Blood in tissue causing swelling.
  • Petechiae: Pinpoint hemorrhages.
  • Purpura: Small hemorrhages less than 1 cm.
  • Ecchymosis: Large hemorrhages in skin or mucosa.
  • Diapedesis: Microscopic red blood cell leakage due to congestion.

Etiology of Hemorrhage

Etiology involves trauma to vessel walls (e.g., labor injury), spontaneous rupture (e.g., aneurysm), inflammatory vessel damage (e.g., ulcers, vasculitis), neoplastic invasion (e.g., carcinoma), vascular diseases (e.g., atherosclerosis), and increased intravascular pressure (e.g., hypertensive retinal hemorrhage).

Clinical Effects of Hemorrhage

Clinical effects depend on the amount, speed, and site of blood loss, with rapid or large losses causing hypovolemic shock or death, especially if vital organs are involved.

Thrombosis

Thrombi Types by Location

  • Arterial thrombi are white, mural, and may cause infarction or gangrene.

  • Venous thrombi are red, occlusive, and may cause thromboembolism or edema.

  • Cardiac thrombi form in atria or valves and may embolize.

  • Capillary thrombi are tiny, seen in DIC or inflammation.

Thrombus Morphology

  • Gross: arterial (white, firm), venous (red, gelatinous), laminated (lines of Zahn).

  • Microscopy: alternating layers of platelets/fibrin and red blood cells in laminated thrombi.

Fate of Thrombi

  • Resolution via fibrinolysis (e.g., by plasmin, urokinase).

  • Organization into fibrous tissue and vessel wall integration.

  • Recanalization may restore blood flow through new channels.

  • Propagation enlarges thrombus, possibly occluding vessels.

  • Thromboembolism occurs if a thrombus fragment detaches and travels in circulation, causing distant obstruction.

Infarction

Infarction is the process of ischemic tissue necrosis, most commonly due to sudden arterial occlusion.

Etiology of Infarction

Etiology includes arterial thrombosis or embolism (most common), less commonly venous obstruction (stagnant hypoxia), or severe narrowing (e.g., atherosclerosis).

Infarction Types

Types are classified by:

  • Color: pale (anemic) or red (hemorrhagic)

  • Age: recent (fresh) or old (healed)

  • Infection: bland (non-infected) or septic (infected)

Infarction Pathogenesis

Pathogenesis involves early edema and hemorrhage, reversible injury (cloudy swelling), followed by coagulative necrosis, inflammation, and later granulation tissue and fibrosis.

Lung Infarct

Usually from embolism; wedge-shaped, hemorrhagic, often in lower lobes; microscopically shows coagulative necrosis, hemorrhage, hemosiderin, and fibrosis.

Kidney Infarct

Due to arterial occlusion; pale, wedge-shaped with base under capsule; microscopically shows ghost tubules, inflammatory border, and fibrotic healing.

Spleen Infarct

From splenic artery occlusion; pale, wedge-shaped with coagulative necrosis and inflammatory infiltration.

Liver Infarct

Rare due to dual supply; infarcts of Zahn (non-ischemic) show red-blue areas from reduced portal flow, chronic atrophy, and sinusoidal dilation.

Specific Granulomatous Inflammation

Chronic granulomatous inflammation is a type of chronic inflammation where the body attempts to wall off a persistent stimulus using granulomas.

Etiology of Granulomatous Inflammation

  • Infectious causes: tuberculosis, leprosy, syphilis, cat-scratch disease, actinomycosis

  • Immune-mediated diseases: sarcoidosis, Crohn’s disease, vasculitides

  • Foreign bodies: talc, beryllium, sutures

Mechanism of Granuloma Formation

Mechanism involves antigen-presenting cells activating CD4+ T cells → Th1 cells release IFN-γ → macrophage activation → formation of epithelioid cells and giant cells → granuloma.

Tuberculosis

Tuberculosis is caused by Mycobacterium tuberculosis.

  • Primary TB: Ghon complex = peripheral lung lesion + lymphatics + hilar nodes

  • Secondary TB: apical lung involvement; forms fibrocaseous lesions or cavities

  • Miliary TB: widespread small granulomas in multiple organs

  • Microscopically: granulomas with central caseation, epithelioid cells, Langhans giant cells, and fibrosis.

Syphilis

Syphilis is caused by Treponema pallidum.

  • Primary Syphilis: chancre with plasma cell-rich inflammation and endarteritis

  • Tertiary Syphilis: gumma with central necrosis, surrounded by lymphocytes, plasma cells, and giant cells

Leprosy

Leprosy is caused by Mycobacterium leprae.

  • Lepromatous Leprosy: diffuse skin lesions, foamy macrophages (lepra cells), high bacillary load

  • Tuberculoid Leprosy: granulomas with Langhans cells, nerve involvement, few bacilli

  • Other forms: pure neural, histoid, and reactional types (Type I and II reactions)

Sarcoidosis

Sarcoidosis is an immune-mediated granulomatous disease.

  • Non-caseating granulomas with epithelioid and giant cells

  • Asteroid bodies, Schaumann bodies, and birefringent crystals may be present

  • Lymphocyte-poor (“naked”) granulomas often seen

Actinomycosis

Actinomycosis is a bacterial infection by Actinomyces israelii.

  • Forms abscesses with sulfur granules (bacterial colonies with club-shaped ends)

  • Common forms: cervicofacial, thoracic, abdominal, pelvic

Cat-Scratch Disease

Cat-scratch disease is caused by Bartonella henselae.

  • Granulomatous lymphadenitis with neutrophilic abscesses and palisading histiocytes

  • Organisms visible with silver stains

Rheumatic Fever

Rheumatic fever (proliferative stage) occurs post-streptococcal infection.

  • Aschoff bodies: central fibrinoid necrosis, surrounding Anitschkow cells (caterpillar nuclei), lymphocytes, and plasma cells

  • Mainly affects heart valves (mitral > aortic) and myocardium

Immunodeficiency Diseases

Immunodeficiency diseases are a failure or deficiency of the immune system, leading to recurrent infections.

Types of Immunodeficiency

  • Primary Immunodeficiency: genetic/developmental (e.g., SCID, DiGeorge)

  • Secondary Immunodeficiency: acquired (e.g., HIV/AIDS)

Acquired Immunodeficiency Syndrome (AIDS)

AIDS (Acquired Immunodeficiency Syndrome) is caused by HIV (RNA retrovirus).

AIDS Epidemiology

Global pandemic; highest in Sub-Saharan Africa; ~35 million infected, 1.5 million deaths/year.

HIV Structure

Spherical, 100–140 nm; has core proteins (p24, p18), 2 RNA strands, reverse transcriptase; envelope with gp120 and gp41.

HIV Transmission Routes

  • Sexual (homo/heterosexual)

  • Blood (transfusions, IV drug use)

  • Perinatal (mother to child)

  • Occupational and other fluids

Stages of HIV Infection

  • Acute Stage: flu-like illness, high viremia, CD4+ drop, seroconversion in 3–6 weeks

  • Middle (Chronic) Stage: asymptomatic or lymphadenopathy, gradual CD4+ fall, high viral replication

  • Final (AIDS) Stage: CD4+ <200/μL, severe immunosuppression, opportunistic infections, malignancies

Clinical and Pathological Effects of HIV

  • Direct Viral Damage: lymphoid + CNS involvement

  • Opportunistic Infections: bacterial, viral, fungal, parasitic

  • Tumors: Kaposi’s sarcoma, lymphomas

  • Drug Effects: toxicity from HIV treatments

HIV Diagnosis

  1. HIV detection: ELISA, Western blot, p24, RNA PCR

  2. Immune monitoring: CD4+, CD8+ counts, immunoglobulins

  3. Detect opportunistic infections, tumors

Neoplasm

Neoplasm is an abnormal, excessive, uncoordinated, purposeless, and autonomous growth of cells (benign or malignant).

Neoplasm Components

Neoplasms consist of two main components: parenchyma (the neoplastic cells) and stroma (the supportive connective tissue, vessels, and nerves).

Macroscopic Features of Neoplasms

  • Benign Neoplasms: spherical, encapsulated, firm, mobile

  • Malignant Neoplasms: irregular, poorly circumscribed, invasive, often with hemorrhage or necrosis

Microscopic Patterns of Neoplasms

  • Epithelial Tumors: acini, sheets, cords

  • Mesenchymal Tumors: separated by matrix (e.g., chondroma, osteosarcoma)

  • Hematopoietic Tumors: little/no stroma (e.g., leukemia, lymphoma)

Cytomorphology: Differentiation & Anaplasia

  • Differentiation: resemblance to normal tissue

  • Anaplasia: lack of differentiation

  • Benign Neoplasms: well-differentiated

  • Malignant Neoplasms: poorly to undifferentiated, anaplastic

Features of Anaplasia

  1. Structural/tissue: disorganized architecture

  2. Cellular: pleomorphism (size/shape), large nuclei, coarse chromatin

  3. Nuclear changes: hyperchromasia, increased nuclear-cytoplasmic ratio, atypical mitosis

Angiogenesis and Tumor Stroma

  • Tumor stroma: connective tissue + vessels (no lymphatics)

  • Growth via basic fibroblast growth factor (BFGF)

  • Angiogenesis: new vessel formation via VEGF → essential for growth/metastasis

  • Tumors with excess stroma: desmoplastic (hard); soft = medullary

Inflammatory Reaction to Tumors

The inflammatory reaction is the host immune response (acute or chronic) involving lymphocytes, macrophages, and plasma cells attempting to destroy the tumor.

Benign Epithelial Tumors

Benign epithelial tumors are non-invasive, slow-growing, encapsulated, and resemble the tissue of origin.

Types by Epithelium of Origin

1. Papillomas

From surface epithelium (keratinizing/nonkeratinizing squamous, urothelium)

  • Example: Skin Papilloma (Verruca Vulgaris): small, cauliflower-like, hands/feet, caused by HPV

  • Microscopy: finger-like epithelial projections, fibrovascular cores, intact basement membrane

2. Adenomas

From glandular epithelium or mucosa

  • Variants: acinar, tubular, solid, papillary, mucinous, villous, cystic, etc.

Examples of Benign Epithelial Tumors

Fibroadenoma of Breast

  • Small, firm, white-grey, well-circumscribed

  • Intracanalicular: compressed, irregular ducts

  • Pericanalicular: round/oval ducts in proliferating stroma

Serous Papillary Cystadenoma of Ovary

  • Large, >5 cm, serous fluid, papillary structures, psammoma bodies

Ovarian Mucinous Cystadenoma

  • Large, multiloculated, mucin-filled cysts, lined by tall columnar cells with basal nuclei and mucus, no cilia

Malignant Epithelial Tumors

Malignant epithelial tumors, also known as carcinomas, invade and metastasize. They are arranged in nested or back-to-back glandular patterns with a common basement membrane and have a granular appearance.

1. Adenocarcinoma

From glandular epithelium

  • Example: Bowel Adenocarcinoma

    • Macroscopic Features: polypoid mass in proximal colon (bleeding), napkin-ring lesion in distal colon (obstruction)

    • Histology: irregular glandular lumens, invasion into submucosa/serosa, hyperchromatic nuclei, loss of goblet cells, grading G1–G3 based on differentiation

2. Urothelial Carcinoma

From urothelium (commonly bladder), high recurrence

  • Macroscopic Features: papillary (cauliflower-like), becomes firm, rough, invasive with progression

  • Histology: fibrovascular stalk covered with layers of urothelial cells, graded as low or high grade

Mesenchymal Tumors (Soft Tissue)

General Features of Mesenchymal Tumors

Cells in fascicles, oriented variably; secondary changes: hyalinization, ossification, teratogenesis, hemorrhage, myxoid, cystic.

Fibrohistiocytic Tumors

Fibroblast-like cells, phagocytic traits, CD68+; variants include dermatofibroma, sclerotic fibroma, giant cell fibroblastoma.

Fibromatosis

Fibrous tissue overgrowth, superficial (adults) or deep (juvenile), fascicles of spindle fibroblasts, collagen-rich (fibroma durum) or soft (fibroma mole).

Osteoma

Benign bone tumor, compact or trabecular, usually in frontal sinus, hard, asymptomatic, no Haversian canals, mature lamellar bone.

Chondroma

Benign cartilage tumor, distal bones/joints, firm, oval, semi-translucent, resembles hyaline cartilage with fibrous septa and chondrocytes.

Lipoma

Benign fat tumor, soft, oval, sharply demarcated, composed of mature adipocytes with fibrous septa, may have lipoblasts.

Rhabdomyoma

Benign striated muscle tumor, cardiac or extracardiac, types: adult, fetal, genital.

Leiomyoma

Benign smooth muscle tumor, uterus most common, oval, white, firm, spindle cells in bundles, cigar-shaped nuclei.

Hemangioma

Benign vascular tumor, skin/liver/mucosa, red/blue soft mass, dilated vessels with or without thrombi, types: capillary, cavernous, epithelioid.

Lymphangioma

Lymphatic malformation, thin-walled cysts (cystic hygroma), dermis or subcutis, dilated lymphatics, hemosiderin, endothelial cells with vesicles and junctions.

Malignant Mesenchymal Tumors

General Features of Malignant Mesenchymal Tumors

Not sharply demarcated, soft/fish-flesh appearance, hemorrhage, necrosis, hematogenous spread, grading based on differentiation, mitosis, necrosis, tumor cellularity.

1. Fibrosarcoma

Malignant fibroblast tumor

  • Macroscopic Features: white-tan, fleshy, necrotic, hemorrhagic

  • Histology: herringbone pattern, elongated nuclei, scant cytoplasm, variable collagen, mitosis, no giant cells

2. Chondrosarcoma

Malignant cartilage tumor

  • Macroscopic Features: pearly white/light blue, may calcify, contain cysts

  • Histology: cartilaginous matrix, well to poorly differentiated types, variants: clear cell, myxoid, mesenchymal

3. Osteosarcoma

Malignant bone tumor

  • Common Presentation: males <20 years, metaphyseal long bones (knees)

  • Macroscopic Features: large, destructive, infiltrative, grey-white with cysts/hemorrhage

  • Histology: osteoid matrix formation, pleomorphic osteoblasts, giant osteoclasts, associated with bone resorption

4. Liposarcoma

Malignant tumor from lipoblasts

  • Location: deep tissue (thighs, retroperitoneum)

  • Macroscopic Features: large nodular, grey-yellow, infiltrative

Malignant Mesenchymal Tumors (Continued)

Leiomyosarcoma

Malignant smooth muscle tumor

  • Location: uterus, vessels, retroperitoneum

  • Macroscopic Features: infiltrative, fleshy, hemorrhagic, necrotic

  • Histology: spindle cells, nuclear pleomorphism, mitoses, necrosis

  • Prognosis: poor, metastasizes, chemo- and radioresistant

Rhabdomyosarcoma

Skeletal muscle origin, common in children

  • Common Sites: head, neck, genitourinary tract

  • Types:

    • Embryonal (best prognosis): <5 yrs, head/neck, GU

      • Botryoid: grape-like in mucosa (vagina, bladder)

      • Spindle Cell: fascicular, paratesticular

    • Alveolar: round cells, extremities/trunk, poor prognosis

    • Anaplastic: pleomorphic, large nuclei, mitoses, adults

    • Sclerosing: rare, hyalinized stroma, eosinophilic cytoplasm

Synovial Sarcoma

Rare, near joints/tendon sheaths

  • Macroscopic Features: painless mass

  • Types: fibrous spindle cell + epithelial (biphasic)

  • Genetics: t(x;18)(p11.2;q11.2) translocation

Angiosarcoma

Malignant tumor of endothelial cells

  • Spread: via blood/lymph → metastasis

  • Macroscopic Features: red/dark lesion, poorly defined

  • Histology: spindle/epithelioid cells, red blood cell-filled capillaries, pleomorphism, mitoses, vacuoles

  • Variant: epithelioid can mimic melanoma/HCC

Benign and Malignant Melanocytic Tumors

Pigmented nevi are benign melanocytic proliferations.

Types of Pigmented Nevi

  1. Epidermal Nevus: nests in epidermis

  2. Junctional Nevus: nests at dermo-epidermal junction

  3. Compound Nevus: nests at junction + dermis

  4. Dermal Nevus: nests only in dermis

Malignant Melanoma

Malignant melanoma is a highly malignant tumor from melanocytes.

  • Macroscopic Features: variable color (black, brown, red), irregular borders, may ulcerate

  • Histology: nests/clusters or spindle-shaped cells, pleomorphic, large nuclei, eosinophilic nucleoli, loss of cohesion, +/- melanin (achromatic melanoma)

Melanoma Growth Phases

  1. Radial Phase: lateral spread in epidermis/dermis

  2. Vertical Phase: deep dermal invasion (nodular)

Clinical Types of Melanoma

  1. Superficial Spreading Melanoma: from nevi, horizontal → vertical

  2. Nodular Melanoma: aggressive, cauliflower-like

  3. Lentigo Maligna Melanoma: sun-exposed skin, slow radial growth

  4. Acral Lentiginous Melanoma: palms, soles, nail beds, vertical growth

Clark Levels of Invasion

  • Level 1: epidermis

  • Level 2: papillary dermis

  • Level 3: filling papillary dermis

  • Level 4: reticular dermis

  • Level 5: subcutis

Breslow Thickness

Depth is also measured by Breslow thickness.

Tumors of the CNS and PNS

Astrocytoma

Astrocytomas show narrow or diffuse infiltration. Pilocytic astrocytomas have a better prognosis and are often located in the cerebellum, optic nerve, or thalamus. Cells typically have hair-like processes.

Glioblastoma

Glioblastoma is the most common malignant brain tumor, often presenting as a butterfly lesion. It carries a poor prognosis, with survival typically around 15 months. Histologically, it is characterized by hyperchromatic, pleomorphic, and necrotic cells.

Meningioma

Meningiomas are benign tumors originating from the meninges. They are typically round, encapsulated, and compress the brain. Microscopic features include onion-skin whorls and psammoma bodies.

Schwannoma

Schwannomas are tumors, often affecting Cranial Nerve VIII. They exhibit two main patterns: Antoni A, characterized by palisading nuclei with Verocay bodies, and Antoni B, which shows a loose, myxoid pattern.

Neurofibroma

Neurofibromas are associated with Neurofibromatosis Type 1 (NF1), presenting with café au lait spots from birth and multiple tumors in nerves. Neurofibromatosis Type 2 (NF2) is associated with slow-growing tumors of Cranial Nerve VIII.

Neuroblastoma

Neuroblastoma is a malignant tumor originating from neural crest cells, often found in the adrenal gland, typically in children under 4 years old. Symptoms include abdominal pain, fever, and diarrhea. Histologically, it features “Homer-Wright” rosettes and hyperchromatic cells. It commonly metastasizes to bone and bone marrow.