VASCULAR TUMORS OF SOFT TISSUE

DR. SAURAV SINGH

WHO CLASSIFICATION Benign vascular tumorsCongenital hemangiomasRICHNICHCapillary hemangioma Variants: Juvenile hemangioma Cherry angioma Tufted angioma

Lobular capillary hemangioma(pyogenic granuloma)

Cavernous hemangiomaVerrucous hemangiomaMicrovenular

hemangiomaHobnail hemangiomaEpithelioid hemangioma Acquired elastotic hemangiomaArteriovenous hemangiomaAngiomatosisSpindle cell hemangioma

Benign vascular tumorsTumor-like Conditions

Papillary endothelial hyperplasia (Masson's tumor)

Reactive angioendotheliomatosis

Glomeruloid hemangioma

Bacillary angiomatosis

Intermediate vascular tumorsLocally Aggressive

Kaposi-like hemangioendotheliomaGiant cell angioblastoma

Rarely MetastasizingKaposi's sarcomaRetiform hemangioendotheliomaPapillary intravascular angioendotheliomaComposite hemangioendothelioma

Malignant vascular tumorsEpithelioid hemangioendothelioma

AngiosarcomaIdiopathic (head and neck)Associated with chronic lymphedema (Stewart-Treves)PostirradiationEpithelioid

Congenital HemangiomasFully formed at birth, non progressive

Types: Rapidly Involuting Congenital Hemangioma (RICH) Non-Involuting Congenital Hemangioma (NICH)

GLUT-1 negative

Sites - extremities or post auricular skin, can occur elsewhere

RICH (Rapidly Involuting Congenital Hemangioma )

NICH (Non-Involuting Congenital Hemangioma )

Capillary hemangiomaTYPES

Juvenile capillary hemangioma Most common type

Complete involution by age 6-7 yrsC/F : purple to reddish macule or nodule centered in skin or subcutaneous tissueM/E : Lobules of capillary sized vessels supplied by feeder vesselVascular space formations varies

MarkersGlucose transporter-1 (GLUT-1) - immunohistochemical marker

CD31 & CD34- endothelial markers & CD 133, expressed in primitive cell - endothelial progenitor cells are involved in pathogenesis.

Myeloid markers- CD14, CD15, CD32 and CD83.

Infantile hemangioendothelioma showing vaguely lobulated architecture

High cellularity and high mitotic activity of juvenile hemangioendothelioma should not lead to overdiagnosis of malignancy

Perineural spread of juvenile hemangioendothelioma

Variants : a) Lobular capillary hemangioma (Pyogenic granuloma)

Common proliferative lesion, related to trauma Site- superficial dermis like lips, gums, hands, fingers, face of

pregnant women (epulis) M/E- Low power - organised pattern seen. lateral edges show lobular

arrangement – group of capillaries proliferate High power –Small capillary epithelium lined spaces seen, also

surrounded by perithelial or pericytic layer of cells

Pyogenic granuloma

b) Tufted angioma:

Acquired in childhood (1-5 yrs), congenital forms existMay persist unchanged or regress completely in few years resembles cellular JCH but discontinuous capillary lobules.

TUFTED ANGIOMA

C) Cherry angioma :suspected involuted LCH

Bright red, soft, raised, dome-shaped lesion of varying in size often present in large numbers.

M/E- Numerous capillaries with narrow lumina Prominent endothelial cells, arranged in lobular fashion in subpapillary region Intercapillary stroma edema and collagen

Cavernous hemangiomaAge and site same as JH but less frequentNo tendency to regressLocally destructive due to pressure symptomsM/E: Pattern of grouped dilated thin walled blood vessels with inconspicuous endothelial lining

Associated Syndromes :Blue-rubber-bleb nevus syndrome – cavernous hemangioma of skin and GITMaffuci syndrome – Cavernous hamangiomas and enchondromas

Cavernous hemangioma of soft tissue of orbit

Cavernous hemangioma

Sinusoidal hemangioma : the vascular spaces are widely dilated

Verrucuous hemangioma

Wart like, dark blue papules or nodules

Predilection for distal lower limbs

Histology - mixture of cavernous and capillary vessel immediately below hyperkeratotic and acantholytic epidermis

Microvenular Hemangioma

Small, reddish lesion in young to middle-aged individuals

Favored sites- arms, trunk, legs

Thin, branching capillaries and small venules widely throughout the dermis

Infiltration of arrector pili muscles by proliferating vascular channels

Hobnail Hemangioma

Usually over trunk or extremities of young or middle-aged adults

Male predominance

Small solitary brown to violaceous papule surrounded by a thin, pale area and a peripheral ecchymotic ring

Hobnail Hemangioma

Superficial reticular dermis- thin walled, dilated, and irregular vascular spaces

Lined by endothelial cells with scanty cytoplasm and rounded nuclei that protrude into the lumina, resembling hobnails

Extensive red blood cell extravasation, inflammatory aggregates, extensive stromal hemosiderin deposition seen

Acquired Elastotic Hemangioma

Develops in sun-exposed skin of forearms & neck

Middle-aged to elderly patients

Small, red or blue, circumscribed & asymptomatic plaque

Superficial, proliferation of capillaries in background of solar elastosis

Capillary surrounded by a layer of pericytes

Arteriovenous (Venous) Hemangioma

Solitary dark red papule or nodule on face, lips, extremities of adults

Densely aggregated, thick & thin-walled vessels lined by a single layer of endothelial cells in dermis

Walls of vessels consist of fibrous tissue

Most of the blood vessels are veins

Young adultsSkin and subcutisDistal extremities – handM/E : thin walled cavernous vessels lined by bland flattened endothelium admixed with solid areas composed of plump endothelial cellsRecurrence is common (> 50%) with discontinuous growth pattern

Spindle cell hemangioma

Spindle cell hemangioma : Cavernous hemangioma like area

Kaposi sarcoma like area in the same

Chracteristic spongy low power appearance

Lower extremity – thighM/E : Poorly circumscribed, diffusely infiltrating mass in the muscle composed of thick walled veins to cavernous vascular spacesMitotic activity and intraluminal capillary tufting is seenFreely anastomosing vascular channels are absent

Skeletal muscle hemangioma

Skeletal hemangioma

Seen in women, 20-40 yearsSmall dull erythematous plaque in head and neckM/E : -Well circumscribed nodule in the dermis or subcutis less in deep tissue- Vague lobular pattern of clustered small capillary sized vessels around a feeder vessel- Endothelial cells are plump with abundant cytoplasm

with impingement on lumen of vascular channel“tomb stone appearance”

Epithelioid Hemangioma

Epithelioid hemangioendothelioma – The tumor partially fills the lumen of femoral vein

Prominent cytoplasmic vacuolation is seen on high power examination

TUMOR- LIKE CONDITIONS

INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIAo It represents an exuberant organisation and recanalisation of

thrombuso Can occur in de novo (pure) form in extremities and head and

neck region or on a pre-existing vascular disorder (mixed) form in the trunk region

o M/E- Exclusive intravascular nature with characteristic fibrinous or hyaline appearance of the papillary stalks

Residual organising thrombi are often seen.

Intravascular papillary endothelial hyperplasia.

Poorly circumscribed diffuse network of vascular structures within a soft tissueSeen in childhood or adolescencepresents as deep soft tissue swellingTwo patterns :

Mixed vessel type lesion resembling intramuscular angiomaCapillary predominant type lesion with lobular pattern

Angiomatosis

An uncommon deep soft tissue tumor Seen in children and teenagersAssociated with Kasabach Meritt syndrome especially if retroperitoneal mass > 20 cm

M/E : - Lobular architecture, Highly cellularIn Individual lobules there is capillary sized vessels with absence of mitosis or cytologic atypia

- Microscopic features overlap with hemangioma

Intermediate Vascular tumors Kaposiform Hemangioendothelioma

(locally aggressive)

Early lesions – ecchymotic macules or patchesLater lesions – bluish purple papules,nodules, plaques or tumorsRegardless of type, it is Borderline malignancy with slowly progressive but may involve internal organs

M/E : Early lesions – show dermal proliferation of irregular slit like vascular channels with extravasated erythrocytes, hemosiderin and plasma cells.

Kaposi Sarcoma

The vascular channels infiltrate between collagen bundles and surround existing blood vessels (promontary sign) and appendages

Endothelial cells are plump or inconspicuous with no significant cytologic atypia

Later stage – Increased spindle cells between poorly defined slit like vessels

Tumor cells – Intracytoplasmic PAS positive hyaline globules

Retiform hemangioendothelioma(Rarely metstasizing)

Skin of distal extremitiesYoung adultsGross : Reddish purple, slowly growing plaque centered in reticular dermis, < 2-3 cmM/ E : Elongated, arborizing narrow vessels that resemble rete testis .Endothelial cells are monomorphic with low mitotic rate, hyperchromatic nuclei and hobnail morphologySarcoma between vascular channels is prominent aand shows abundant lymphocytic infiltrate

seen in infants and children in skinSlow growing nodule or plaqueM/E : Thin walled vascular spaces that contain intraluminal papillary tufts of endothelial cells within hobnail morphology are present

Papillary intralymphatic angioendothelioma (Dabska tumor)

Composite Hemangioendothelioma

Mixture of benign, intermediate or malignant histologic patterns

Long standing red-blue nodules or plaques on hands & feet in adults

Poorly circumscribed, with infiltrative growth pattern

Different components- Retiform, Epithelioid, Spindle cell,

Angiosarcoma, Lymphangioma, Arteriovenous malformation

Primary sites: Superficial and deep soft tissue, viscera (liver), boneGross : poorly circumscribed, multilobular infiltrative mass

upto 10 cm or as multiple masses. M/E : seen as Cords, short strands, solid nests or individual cells that have rounded to slightly spindled featuresThe cells are low grade with a low mitotic rateEndothelial differentiation is evident by formation of intracytoplasmic lumina(signet ring like features)

Malignant Vascular tumorsEpithelioid Hemangioendothelioma

Distinct vascular channels are not prominentNeoplastic cells are embedded into chondroid like hyalinized stroma

Atypical morphological features : Increased mitotic rate increased nuclear pleomorphism more spindled cytology, necrosis

IHC : tumor cells express – CD13, CD34, Ulex Europaeus antigen, variably express Factor XIII related antigen

It is divided into several groups:Cutaneous angiosarcomaAngiosarcoma of breastRadiation induced angiosarcomaAngiosarcoma of deep soft tissue

Gross : Poorly circumscribed hemorrhagic mass from 1-2 cm to > 10 cmM/E : Varies from hemangioma like features but with scattered, enlarged, atypical endothelial cells with occasional mitotic figures and infiltrating growth pattern to that of high grade spindle cell sarcoma

Angiosarcoma

Gross : Hemorrhagic appearance of angiosarcoma of lip

Angiosarcoma of mediastinal mass : Anastomosing vascular channels

vascular Channels are seem to be ,lined by highly atypical endothelial cells

VariantEpithelioid Angiosarcoma

seen in deep soft tissueMalignant epithelioid cells with abundant eosinophilic or amhophilic cytoplasm, large vesicular nuclei and prominent nucleoli

Epithelioid angiosarcoma arising form region of seminal vesicle

Ulex europaeus lectin 1 reactivity in angiosarcoma

LYMPHANGIOMA

Cavernous or cystic vascular lesions composed of dilated lymphatic channels

Seen in Head and neck region in young children M/E : Thin walled dilated lymphatic vessels of varying sizes lined by

flattened endothelium, beneath which are lymphoid aggregates

Can be Mediastinal or retroperitoneal

Lung tumor – multiple, stellate lesions with slips of smooth muscle around spaces filled with proteinaceous lymph fluid

TUMORS OF LYMPH VESSELS

Cavernous lymphangioma

Large cystic hygroma in an infant

Lymphangioma of soft tissue showing dilated spaces lined by flattened endothelium. A scattering of lymphocytes is present in stroma

Microscopic appearance of lymphangioma

Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of Primary or Secondary LymphedemaSITE- upper extremitiesPresents as a bluish or purplish skin discoloration or tender skin nodule. Often multiple.It progresses to an ulcer with crusting, and finally extensive necrosis.Postmastectomy Lymphangiosarcoma (Stewart-Treves Syndrome)

LYMPHANGIOSARCOMA

Histology- There is an admixture of spindle cells and vascular spaces

deformed by pleiomorphic endothelial cells.

Lymphangiosarcoma cells express positive endothelial markers -CD34, vimentin, keratine, VIII factor antigen

It metastasises rapidly to the lungs, chest walls, liver and or bone and the reoccurrence rate is high

GLOMUS TUMOR

Originates in the neuromyoarterial glomusClassically located in subungual region but may also be found in skin, soft tissue, nerves, stomach, nasal cavity and tracheaTend to be multiple and of infiltrative nature in childrenPresents as varicosities of lower extremitiesSuperficial lesions are well circumscribed

TUMORS OF PERIVASCULAR TISSUE

M/E- consist of blood vessels lined by normal endothelial cells and surrounded by a solid proliferation of round or cuboidal “epithelioid” cells with round nuclei and acidophilic cytoplasm IHC- positive for myosin, vimentin, actin, and basal lamina components but not for desminMicroscopic types of glomus tumor:

solid Angiomatous Myxoid

Glomus tumor

Immunoreactivity for smooth muscle in glomus tumor

Sternberg’s Diagnostic Surgical Pathology – 5th editionRosai and Ackerman’s Surgical Pathology – 10th editionRobbins and Cotron Pathologic Basis of Disease – 8th editioninternet

REFERENCES