Vascular and Lymphatic Tissue Tumors: Types and Characteristics

Posted on Dec 15, 2024 in Biology

Benign Vascular Tissue Tumors

  • Hemangioma
  • Hereditary Hemorrhagic Telangiectasia
  • Encephalotrigeminal Angiomatosis
  • Lymphangioma

Hemangioma

  • Hamartoma (versus true neoplasm)
  • Vascular in origin
  • Congenital hemangioma (versus congenital vascular malformation)
  • Endothelial cell proliferation
  • Also known as strawberry nevus

Clinical Features

  • Appears weeks after birth
  • Male < Female (2:1)
  • Common in head and neck region
  • Flat or raised lesion of mucosa (nodular)
  • Deep red or bluish red
  • Blanches when compressed
  • Common intraoral sites: lips, tongue, buccal mucosa, palate

Congenital Hemangioma

  • Benign congenital neoplasms of proliferating endothelial cells
  • Increase in number of capillaries
  • Appears weeks after birth
  • Rapid growth
  • Spontaneous involution
  • No bruit
  • Rarely affects bone
  • Resectable
  • Surgical bleeding – controllable
  • Often circumscribed
  • Recurrence uncommon

Congenital Vascular Malformation

  • Lesions resulting from abnormal vessel morphogenesis
  • Dilation of blood vessels
  • Present at birth
  • Progressive enlargement
  • Persistent
  • With bruit or thrill
  • Frequently affects bone
  • Difficult to resect
  • Surgical bleeding potential
  • Poorly circumscribed
  • Recurrence common

Histopathologic Features

  • Many small capillaries lined by a single layer of endothelial cells supported by connective tissue stroma of varying density
  • Classification:
    • Capillary type
    • Cavernous type

Differential Diagnosis

  • Pyogenic granuloma

Central Hemangioma

Clinical Features

  • Bone destructive lesion
  • Lesions occurring inside the bone or jaws
  • More common in the vertebrae and skull
  • Rare in jaws (mandible, especially in the mandibular canal)
  • Congenital anomaly
  • May cause early eruption of teeth

Radiographic Features

  • Multilocular or unilocular
  • Honeycomb or soap bubble appearance
  • Root resorption, enlargement of mandibular canal, mental foramen

Differential Diagnosis

  • Osteomyelitis
  • Ameloblastoma

Treatment

  • None
  • Surgery
  • Radiation therapy
  • Sclerosing agents
  • Cryotherapy

Hereditary Hemorrhagic Telangiectasia

  • Rendu-Osler-Weber Disease
  • Congenital, hereditary
  • Vascular malformation, delicate vessels prone to bleeding

Clinical Features

  • Characterized by numerous telangiectatic/angiomatous areas on skin and oral mucosa
  • Skin lesions: red macules or papules, slightly elevated
  • Face and neck
  • Lips, gingiva, buccal mucosa, palate, tongue
  • Symptoms: bleeding and epistaxis

Histopathologic Features

  • Primary intrinsic defects of endothelial cells permitting detachment
  • Defect in perivascular supportive tissue bed

Differential Diagnosis

  • Scleroderma
  • CREST syndrome
  • Lupus erythematosus

Treatment

  • None
  • Careful surgical approach
  • Laser therapy
  • Cautery

Encephalotrigeminal Angiomatosis

  • Sturge-Weber disease
  • Vascular malformation
  • Combination of venous angioma of leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face

Clinical Features

  • Port-wine stains/nevus flammeus (96%)
  • Intracranial convolutional calcifications
  • Glaucoma and exophthalmos
  • Oral: angiomatous lesions in the gingiva and buccal mucosa
  • Neurologic: mental retardation, convulsive disorders, and spastic hemiplegia

Differential Diagnosis

  • Angioosteohypertrophy syndrome

Treatment

  • None
  • Anticonvulsants (Dilantin)
  • Symptomatic treatment
  • Laser treatment

Lymphangioma

  • Congenital lesions
  • Like hemangioma but with lymph fluid
  • May involute

Clinical Features

  • First two decades of life
  • Painless, nodular, vesicle-like swellings when superficial
  • Color: lighter than surrounding tissue to red-blue
  • Palpation: crepitus
  • Common in oral cavity: tongue

Histopathologic Features

  • Endothelium-lined lymphatic channels diffusely distributed in submucosa
  • Non-encapsulated but non-invasive
  • Lymphatic channels directly adjacent to epithelium without any intervening connective tissue, no capsule

Differential Diagnosis

  • Hemangioma
  • Mucocele

Treatment

  • Surgical excision
  • Common recurrence

Malignant Vascular Tissue Tumors

  • Hemangioendothelioma
  • Hemangiopericytoma
  • Kaposi’s Sarcoma
  • Ewing’s Sarcoma

Hemangioendothelioma

Clinical Features

  • Most commonly skin and subcutaneous lesions
  • Not common intraorally; lips, palate, gingiva, tongue
  • Any age
  • Male < Female (2x)
  • Chief complaint: localized swelling, pain
  • Similar to hemangioma
  • Lesion may be ulcerated, tendency to bleed, may involve bone
  • Infiltrative lesion, not encapsulated, rapid spread

Histopathologic Features

  • Masses of endothelial cells arranged in columns
  • Anastomosing vascular channels
  • Tumor cells within reticulin sheath
  • Cells: pleomorphic, large, polyhedral, with faint outline, round nucleus with multiple minute nuclei

Treatment

  • Surgery
  • Radiation

Prognosis

  • Poor
  • 2-year survival rate (50%)
  • Common metastasis (regional lymph nodes or distant organs via bloodstream)

Hemangiopericytoma

Clinical Features

  • Rare neoplasm derived from the pericyte
  • Uncommon, wide anatomic distribution
  • No sex predilection
  • Circumscribed, firm, nodular, red lesions
  • Fast growth
  • No distinguishing signs except for pain, bleeding, swelling, rapid spread

Histopathologic Features

  • Great variation
  • Proliferation of well-differentiated, oval to spindle-shaped mesenchymal cells separated by small, slit-like vascular channels

Treatment

  • Wide surgical excision
  • Common metastasis
  • High recurrence rate (52%), highly unpredictable

Kaposi’s Sarcoma

  • Angioreticuloendothelioma
  • Multiple Idiopathic Hemorrhagic Sarcoma
  • Unusual and uncommon
  • Etiology: unknown
  • Cancer and AIDS
  • Etiologic factors: genetic predisposition, viral infection, geographic regions, immune dysregulation
  • Generally regarded as a neoplasm

Clinical Features

  • Any age, common at 5th-7th decades
  • Male > Female (85-90%)
  • Multiple skin lesions: reddish, brownish, red nodules, blue lesions, etc.
  • Varying sizes
  • May be flat to nodular, exophytic lesions
  • 1/3 of AIDS patients (half – oral lesions; palate)
  • Most common sites: palate, gingiva, tongue

Patterns

  • Classic
  • African type
  • Immunodeficient type
Classic
  • Rare
  • Older men
  • Skin lesions: lower extremities
  • Oral lesions: rare
  • Affects other organs
  • Indolent course
  • Prognosis: fair
African
  • Endemic
  • Children, adults
  • Skin lesions: upper and lower extremities
  • Oral lesions: rare
  • Affects other organs
  • Prolonged course
  • Prognosis: fair
Immunodeficient
  • Relatively common
  • Adults
  • Skin lesions: any side
  • Oral lesions: common
  • Frequently affects other organs
  • Rapid and aggressive
  • Prognosis: poor

Histopathologic Features

  • Extremely variable
  • Endothelial cell proliferation
  • Inflammatory cell infiltration
  • Numerous small capillary type vessels with or without blood (hemangioma)
  • Early lesions: spindle cells and ill-defined vascular channels
  • Late lesions: resemble pyogenic granuloma, red blood cells, hemosiderin, atypical vascular channels, inflammatory cell infiltrates
  • Macrophages, factor XIIIa-positive dendrocytes, lymphocytes, and mast cells

Differential Diagnosis

  • Hemangioma
  • Erythroplakia
  • Melanoma
  • Pyogenic granuloma

Prognosis

  • Good except if associated with AIDS
  • Chronic and slow progressive nature
  • Many lesions will regress over time

Ewing’s Sarcoma

  • Endothelial myeloma, round cell sarcoma
  • Uncommon malignant neoplasm
  • Primary destructive lesion of long bones, relatively rare in jaws
  • Not inherited, 11, 22 translocation “fusion gene”

Clinical Features

  • Male > Female
  • Children and young adults, 5-30 years old
  • Signs/symptoms: swelling, pain, loose teeth, paresthesia, exophthalmos, ptosis, epistaxis, ulceration, shifted teeth, trismus, sinusitis
  • Caucasians > Blacks and Asians
  • Jaw involvement: lip paresthesia & facial neuralgia
  • Most common in pelvis > long bones, femur, ribs, tibia, fibula

Radiographic Features

  • Mandible > maxilla (posterior)
  • May stimulate new layers of periosteal bone
  • Irregular, diffuse radiolucency, poorly demarcated, never corticated, ragged border

Histopathologic Features

  • Extremely cellular, solid sheets or masses of small blue neural cells with very little stroma
  • Tumor cells: small, round, little cytoplasm, large, round, or ovoid nuclei
  • Increased mitotic figures
  • Intracytoplasmic glycogen
  • Necrosis

Differential Diagnosis

  • Osteomyelitis
  • Eosinophilic granuloma

Treatment

  • Surgery or radiation therapy
  • Chemotherapy

Prognosis

  • Dependent on size and location
  • Metastasis: fast (bones and other organs)

Lymphoma

  • Neoplastic proliferative process of the lymphoietic system involving either the lymphocytes or histiocytes
  • Only <5% of malignant tumors
  • Hodgkin’s lymphoma
  • Non-Hodgkin’s lymphoma
  • Burkitt’s lymphoma
  • Multiple myeloma

Hodgkin’s Lymphoma

Clinical Features

  • Bimodal age incidence
  • Male > Female
  • First sign: firm, rubbery, painless enlargement of one or more cervical nodes
  • May involve extranodal lymphoid tissue
  • Pain in abdomen and back
  • Pain depends on involved area
  • Skin pruritus, cough, dyspnea
  • Fever (Pel-Ebstein)

Oral Lymphoma

  • Unilateral tonsillar enlargement
  • Submucosal swelling with mucosal ulceration with erosion of overlying bone

Histopathologic Features

  • Non-neoplastic cells (plasma cells, lymphocytes, macrophages)
  • Reed-Sternberg cells
  • Lukes-Butler Classification:
    • Lymphocytic predominant
    • Mixed cellularity
    • Lymphocyte depletion
    • Nodular sclerosis

Prognosis

  • Dependent on clinical stage

Treatment

  • Radiation therapy
  • Chemotherapy

Non-Hodgkin’s Lymphoma

Clinical Features

  • More common in oral cavity
  • Not exclusive to extranodal lymphoid
  • Middle age
  • Male > Female
  • Waldeyer’s ring
  • Acute or insidious onset

Oral Lesions

  • Soft tissue: rapidly growing swelling, ulceration, necrosis, foul smell
  • Bone: bone loss, tooth mobility, pain
  • Slow-growing, painless, bluish mass at palate

Histopathologic Features

  • Nodular
  • Diffuse

Differential Diagnosis

  • Salivary gland tumor which is slow-growing, painless, bluish mass at palate

Treatment

  • Radiation therapy
  • Chemotherapy

Burkitt’s Lymphoma

Types

  • Endemic African form
  • Non-endemic American form

Endemic African Form

Clinical Features
  • 2-14 years old
  • Extranodal tissue
  • Rapidly growing tumor of jaw
  • Fastest growing human tumor with the potential doubling time of 24 hours
  • Pain, paresthesia, bone destruction, tooth mobility
  • Visceral organ: kidney, liver, testes

Non-endemic American Form

Clinical Features
  • Older children
  • Lymph nodes and lymphoid tissues
  • Localized jaw involvement
  • Toothache, lip paresthesia

Radiographic Features

  • Moth-eaten, poorly marginated bone destruction
  • Expanded cortex

Differential Diagnosis

  • Osteomyelitis

Histopathologic Features

  • Starry sky effect – macrophages

Multiple Myeloma

Clinical Features

  • 40-70 years old
  • Male > Female
  • Mandible: ramus, angle, molar area
  • Pain
  • Swelling, jaw expansion, numbness, tooth mobility, pathologic fracture

Radiographic Features

  • Numerous sharply punched-out areas in the bone

Laboratory Findings

  • Hypergammaglobulinemia
  • Bence-Jones proteinuria
  • Anemia
  • Amyloidosis

Histopathologic Features

  • Monotonous proliferation of pure plasma cells
  • Plasma cells: eccentrically placed nucleus, cartwheel-looking chromatin

Treatment

  • Chemotherapy
  • Steroid
  • Radiation

Prognosis

  • Poor – due to kidney failure, anemia, infection