Vascular tumors of soft tissue
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Vascular tumors of soft tissue

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  • Most accepted Classification of vascular tumors is the WHO classification which divides vascular tumors into benign , intermediate and malignant tumors.Rapidly Involuting Congenital Hemangioma (RICH) Non-Involuting Congenital Hemangioma (NICH)
  • Hemangiomas are Most common cutaneous and soft tissue tumor of infancy and childhood(7% of benign soft tissue tumor)
  • Gross= Raised, violaceous tumor with large, radiating veins or with overlying telangiectasia and a halo of pallor M/E=Architecture is lobularBands of fibrosis, focal inflammation, dystrophic calcification, hemosiderin depositionLobules - congested capillaries, surrounded by a layer of pericytes
  • Gross= Flatter than RICH, well-circumscribed round to ovalSlightly indurated or raised soft-tissueMass with overlying telangiectasias and a rim of pallor.M/E=Vascular lobules are large Composed of larger, thicker blood vessels
  • M/E= Minimal with cellular lobules and scattered mitotic figures to obvious luminal formation
  • immunohistochemical marker is Glucose transporter-1 (GLUT-1) ..
  • Low power= A thin collagen layer surrounds each lobule Arrangement is disrupted at base focal anastomosing channelsHigh power= Small frequent mitosis and enlarged endothelial nuclei. Superficial region of ulceration and acute inflammation – neutrophils abound superficial lobulesLacks steroid hormone receptors
  • Gross= Solitary, red, rapidly growing papule or nodule, with a subtle collarette of scaleM/E=Polypoid mass of angiomatous tissue protruding of skin. Superficial inflammatory cell reaction - appearance of granulation tissueSurrounded by myxoidstroma, scattered spindle & stellate shaped connective tissue cells Dilated network of blood-filled capillary vessels
  • JCH- juvenile capillary hemangioma
  • Gross= Large, plaque-like, infiltrated, red or purple lesionM/E= Cannonball appearance- vascular tufts of tightly packed capillaries, randomly dispersed in the dermis
  • LCH- lobular capillary hemangioma
  • JH-juvenile hemangioma…………Those occuring in the skin are traditionaly known as port wine nevus or nevus flammeus.
  • shows Large, irregular spaces containing red blood cells and fibrinous material lined by a single layer of thin endothelial cells
  • It is a cavernous hemangioma containing dilated inter connecting thin walled channels with occasional pseudopapillary projections.
  • VerrucuoushemangiomaGross-dark blue papules or nodules seen on legm/e= Congested capillaries and cavernous vascular spaces extend into the deep dermis and subcutaneous tissue Lined by flattened endothelial cells Lobular growth pattern in deeper component
  • Common in young adults,,,sites are--
  • Sites---
  • There isAngiomatoid hyperplasia with eosinophilia
  • Also called as Massons hemangioma or massons lesion. not a true neoplasm but has capacity to simulate microscopically vascular tumor
  • Mature adipose tissue may be intermixed
  • It is Multinodular, poorly circumscribed, infiltrating tumorLethal in 20 % due to assoiatedcoagulopathy
  • 1st slide shows Classickaposi sarcoma2 nd slide shows Endemic africankaposi sarcoma
  • 1st slide shows AIDS associated kaposi sarcoma2nd slide shows Kaposi sarcoma in Immunocompromised patient
  • It is Uncommon neoplasm..sites is skin of distal extremities…seen in young adults.
  • Picture showing elongated, arborizing blood vessels lined by monomorphic endothelial cells with prominent nuclei and scanty cytoplasm
  • Picture showing Markedly dilated, thin-walled vascular channels Lined by endothelial cells with protruding nuclei & very scanty cytoplasmProminent intra- and extravascular lymphocytic cell infiltrate Intravascular papillae with collagenous cores
  • Intravascular bronchioloalveolar tumor seen in All ages, rare in early childhoodLow grade malignant endothelial neoplasm. 10 % multiorgan disease at presentation
  • In 25-30% cases - focal cytokeratin expression – lead to misdiagnosis
  • Tumor cells are arranged in short fascicles, small nests, “indian file” pattern Hyalinized or mucoidstroma can be seen. No obvious vascular channels Intracytoplasmic vacuoles containing erythrocytes can be seen
  • *Rare malignancy of endothelial cellsIntraluminal papillae lined by hyperchromatic cellsVascular channels with a dissecting pattern of infiltration through dermis or surrounding tissue – is of concern
  • Picture shows
  • Results from failure of the lymphatic system to communicate with the venous systemAsso with tuberous sclerosis - TSC2 mutationsChylous pleural effusion and ascites – exclusively seen in females.They can Replace local lymph nodes. show Pericytomatous histology
  • Present in deep soft tissue
  • Cystic lymphangioma has been traditionally called hygroma.
  • Picture showing
  • Amputated upper extrimity in postmastectomylymphangiosarcoma
  • Post-mastectomy lymphangiosarcoma showing an intricate network of neoplastic vessels.
  • Also called glomangioma
  • The distribution of round glomus cells around the open vascular lumen is a key to the diagnosis.

Vascular tumors of soft tissue Vascular tumors of soft tissue Presentation Transcript

  • DR. SAURAV SINGH
  • Congenital hemangiomas RICH NICH Capillary hemangioma Variants: Juvenile hemangioma Cherry angioma Tufted angioma Lobular capillary hemangioma(pyogenic granuloma) Cavernous hemangioma Verrucous hemangioma Microvenular hemangioma Hobnail hemangioma Epithelioid hemangioma Acquired elastotic hemangioma Arteriovenous hemangioma Angiomatosis Spindle cell hemangioma
  • Tumor-like Conditions Papillary endothelial hyperplasia (Masson's tumor) Reactive angioendotheliomatosis Glomeruloid hemangioma Bacillary angiomatosis
  • Locally Aggressive Kaposi-like hemangioendothelioma Giant cell angioblastoma Rarely Metastasizing Kaposi's sarcoma Retiform hemangioendothelioma Papillary intravascular angioendothelioma Composite hemangioendothelioma
  • Epithelioid hemangioendothelioma Angiosarcoma Idiopathic (head and neck) Associated with chronic lymphedema (Stewart-Treves) Postirradiation Epithelioid
  • Fully 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
  • TYPES Juvenile capillary hemangioma Most common type Complete involution by age 6-7 yrs C/F : purple to reddish macule or nodule centered in skin or subcutaneous tissue M/E : Lobules of capillary sized vessels supplied by feeder vessel Vascular space formations varies
  • Glucose 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/ELow 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
  • b) Tufted angioma: Acquired in childhood (1-5 yrs), congenital forms exist May persist unchanged or regress completely in few years resembles cellular JCH but discontinuous capillary lobules.
  • C) Cherry angioma : suspected involuted LCH Bright red, soft, raised, dome-shaped lesion of varying in size often present in large numbers. M/ENumerous capillaries with narrow lumina Prominent endothelial cells, arranged in lobular fashion in subpapillary region Intercapillary stroma edema and collagen
  • Age and site same as JH but less frequent No tendency to regress Locally destructive due to pressure symptoms M/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 GIT Maffuci syndrome – Cavernous hamangiomas and enchondromas
  • Cavernous hemangioma of soft tissue of orbit
  • Sinusoidal hemangioma : the vascular spaces are widely dilated
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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 adults Skin and subcutis Distal extremities – hand M/E : thin walled cavernous vessels lined by bland flattened endothelium admixed with solid areas composed of plump endothelial cells Recurrence is common (> 50%) with discontinuous growth pattern
  • Spindle cell hemangioma : Cavernous hemangioma like area
  • Kaposi sarcoma like area in the same
  • Chracteristic spongy low power appearance
  • Lower extremity – thigh M/E : Poorly circumscribed, diffusely infiltrating mass in the muscle composed of thick walled veins to cavernous vascular spaces Mitotic activity and intraluminal capillary tufting is seen Freely anastomosing vascular channels are absent
  • Skeletal hemangioma
  • Seen in women, 20-40 years Small dull erythematous plaque in head and neck M/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 hemangioendothelioma – The tumor partially fills the lumen of femoral
  • Prominent cytoplasmic vacuolation is seen on high power examination
  • INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA o It represents an exuberant organisation and recanalisation of thrombus o 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 tissue Seen in childhood or adolescence presents as deep soft tissue swelling Two patterns : Mixed vessel type lesion resembling intramuscular angioma Capillary predominant type lesion with lobular pattern
  • An uncommon deep soft tissue tumor Seen in children and teenagers Associated with Kasabach Meritt syndrome especially if retroperitoneal mass > 20 cm M/E : - Lobular architecture, Highly cellular In Individual lobules there is capillary sized vessels with absence of mitosis or cytologic atypia - Microscopic features overlap with hemangioma
  • Early lesions – ecchymotic macules or patches Later lesions – bluish purple papules,nodules, plaques or tumors Regardless 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.
  • 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
  • Skin of distal extremities Young adults Gross : Reddish purple, slowly growing plaque centered in reticular dermis, < 2-3 cm M/ E : Elongated, arborizing narrow vessels that resemble rete testis . Endothelial cells are monomorphic with low mitotic rate, hyperchromatic nuclei and hobnail morphology Sarcoma between vascular channels is prominent aand shows abundant lymphocytic infiltrate
  • seen in infants and children in skin Slow growing nodule or plaque M/E : Thin walled vascular spaces that contain intraluminal papillary tufts of endothelial cells within hobnail morphology are present
  • 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 componentsRetiform, Epithelioid, Spindle cell, Angiosarcoma, Lymphangioma, Arteriovenous malformation
  • Primary sites: Superficial and deep soft tissue, viscera (liver), bone Gross : 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 features The cells are low grade with a low mitotic rate Endothelial differentiation is evident by formation of intracytoplasmic lumina(signet ring like features)
  • Distinct vascular channels are not prominent Neoplastic 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 angiosarcoma Angiosarcoma of breast Radiation induced angiosarcoma Angiosarcoma of deep soft tissue Gross : Poorly circumscribed hemorrhagic mass from 1-2 cm to > 10 cm M/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
  • 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
  • Variant Epithelioid Angiosarcoma seen in deep soft tissue Malignant 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
  • 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 Lymphedema SITE- upper extremities Presents 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)
  • HistologyThere 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 glomus Classically located in subungual region but may also be found in skin, soft tissue, nerves, stomach, nasal cavity and trachea Tend to be multiple and of infiltrative nature in children Presents as varicosities of lower extremities Superficial lesions are well circumscribed
  • 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 desmin Microscopic types of glomus tumor:  solid  Angiomatous  Myxoid
  • Glomus tumor
  • Immunoreactivity for smooth muscle in glomus tumor
  • Sternberg’s Diagnostic Surgical Pathology – 5th edition Rosai and Ackerman’s Surgical Pathology – 10th edition Robbins and Cotron Pathologic Basis of Disease – 8th edition internet