Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4518_Библиотеки_им_академика_М_И_Перельмана
.pdf
23 Vascular Malformations andTumors
https://t.me/medicina_free
– EtOH: associated with permanent nerve injury
– Bleomycin: associated with pulmonary brosis
– Doxycycline
• Surgical excision
– Often treatment of choice for localized macrocystic lesions
– Usually delayed until 5years of age if possible
– Delayed at least 3months following infection
– Subtotal excision may be necessary to protect vital structures
427
Venous Malformation (VM)
• Collection of dilated vascular channels with normal endothelium
• Associated with receptor tyrosine kinase (Tie2) dysfunction.
Presentation
• Commonly at lips and cheeks
• Can be isolated supercial skin lesions that appear in bluish color
• Can be complex lesions spanning various tissue planes including muscle with
otherwise normal appearing skin
• Increases in size in dependent positions and with Valsalva maneuver
• May be associated with pain if VM develops phleboliths
– May be complicated by a consumptive coagulopathy
– Diagnosed by elevated D-dimers, low brinogen
– Treated with antiplatelet and anti-inammatory ± heparin
Imaging
• Duplex scanning shows slow blood ow
• Bright signal on T2-weighted MRI
• CT scan: calcication representing phleboliths (because low ow)
Treatment
• Mostly observation
• Surgical resection or sclerotherapy reserved for symptomatic lesions or signicant cosmetic deformity
428
https://t.me/medicina_free
B. M. Laitman et al.
Arteriovenous Malformation (AVM)
• Aberrant communication between arteries and veins bypassing capillary bed
• Possible complications
• Heart failure, ulceration, skin necrosis
Presentation
• Commonly presents in 20s and 30s
• Red, warm pulsatile mass with a thrill or a bruit
• Associated with pain, pulsation, or tinnitus
Imaging
• No enhancement on T2-weighted images
• Flow voids on T1- and T2-weighted images
• Angiography shows dilation of veins and early shunting
Clinical Stages: Associated withSeverity
andTreatment Outcome
• Dormancy
• Expansion
• Local destruction
• Heart failure
Treatment
• Observation
• Preoperative embolization and surgical resection reserved for AVM
complications
23 Vascular Malformations andTumors
https://t.me/medicina_free
429
Hemangioma
Epidemiology
• Most common benign vascular tumor of the head and neck
• Mainly affects cutaneous surface of head and neck but mucosal surfaces also
affected
• Must rule out subglottic hemangioma in pediatric patient with stridor and cutaneous hemangioma
– 50% of pediatric subglottic hemangiomas associated with cutaneous
hemangioma
• Most common adult laryngeal hemangioma is in supraglottis
• Most commonly present at birth
• Glucose transporter 1 (GLUT1) positive expression
• More common in females and premature infants
Presentation
• Clinical course
– Rapid proliferation in rst year
– Doubles in size in rst 2months, achieve nal size by ~5months
– Followed by spontaneous involution over several months
50% involute by 5years
70% involute by 7years
• Pediatric subglottic hemangioma presents with failure to thrive or stridor
• Associated with cutaneous hemangiomas in the “beard distribution”
• Adult supraglottic hemangioma most commonly presents with hoarseness; with
bleeding and respiratory distress less commonly
Classication
• Supercial focal hemangioma
• Supercial segmental hemangioma
• Deep focal hemangioma
• Mixed focal hemangioma
• Mixed segmental hemangioma
430
https://t.me/medicina_free
– Segmental hemangiomas have more associated morbidity than focal
hemangiomas
– Segmental lesions occur in embryologic prominences related to neural
crest cells
– Focal lesions associated with lines of embryonic fusion
• Congenital hemangioma: GLUT-1 negative
• Rapidly involuting congenital hemangioma: involute over the course of the
rst year
– Treatment: observation
• Noninvoluting congenital hemangioma
– Treatment: surgical/laser therapy
B. M. Laitman et al.
Imaging
• Well-circumscribed, high-ow, contrast-enhancing vascular tumor
• MRI: high intensity on T2-weighted images
• Flow voids on T1 and T2
Pathology
• Gross: well circumscribed
• Low power microscopy: submucosal endothelial hyperplasia and presence of
mast cells
• High power microscopy: frequent mitosis
– Two phases
Proliferative phase: increased angiogenesis-stimulating proteins
Involuting phase: mast cells and tissue inhibitor metalloproteinases
Management
• Observation is strongly recommended unless
– Very large disguring ulcerative lesions
– Lesion affecting function
23 Vascular Malformations andTumors
https://t.me/medicina_free
Vision, hearing, deglutition, airway compromise, lumbosacral
– Associated with high-output cardiac failure
• Medication: directed as stopping angiogenesis
– Propranolol: rst-line therapy if impairing function
Must have baseline cardiac evaluation
Severe complications including bradycardia, hypoglycemia, and bronchoconstriction have been reported
2mg/kg/day in three doses
– Intralesional or systemic corticosteroids
– Interferon alpha-2a: associated with neurologic complications in children
<1year of age
• Surgical
– Supercial hemangioma
Treated successfully with pulsed-dye laser (PDL) and cooling device
– Subglottic hemangioma
CO2 laser resection, laryngotracheoplasty, tracheotomy
Wide excision and reconstruction may be indicated in rare cases
431
Juvenile Nasopharyngeal Angiobroma (JNA)
• Histologically benign but locally aggressive vascular tumor
Epidemiology
• Typically in adolescent males, diagnosed between 10 and 25years old (hormonesensitive tumor)
• Most common tumor in the nasopharynx
• Presents with unilateral epistaxis or nasal obstruction
• Less commonly with OME, proptosis, diplopia, recurrent sinusitis
• Originates from superior margin of sphenopalatine foramen, at the level of pterygopalatine fossa
• Both spontaneous resolution and malignant transformation have been reported
but are uncommon
432
https://t.me/medicina_free
B. M. Laitman et al.
Chandler Classication
• Stage I: conned to nasopharynx
• Stage II: extending into nasal cavity and/or sphenoid
• Stage III: extending into one or more of the following: antrum, ethmoid, pterygomaxillary and infratemporal fossae, orbit and/or cheek
• Stage IV: extending into cranial cavity
Pathology
• Gross: Sessile, lobulated, rubbery dark red to tan gray mass
• Histology: Multiple staghorn-shaped vessels of varied sizes in a rich brous connective tissue stroma without smooth muscle or elastic bers around vessels
Imaging
• CT: soft tissue contrast-enhancing mass with enlarged sphenopalatine foramen
• Holman-Miller sign: anterior bowing of posterior maxillary sinus wall
• MRI: multiple ow voids on T1 and T2. Should be ordered to rule out intracranial, infratemporal, or intraorbital extension
Work Up
• Endoscopy with conrmation on CT/MRI
• Biopsy is not indicated unless evidence of rapid growth or atypical pattern of
extension; if undertaken before complete excision, should be done in controlled
OR setting where hemostatic control is possible.
Treatment
• Surgical therapy is gold standard
– Historically approached via lateral rhinotomy, midfacial degloving, infratem-
poral fossa, transpalatal approaches
– Standard approach is now endoscopic transnasal, transmaxillary
– Combined endonasal–external approach in rare circumstances
23 Vascular Malformations andTumors
https://t.me/medicina_free
• Preoperative embolization (~48–72h prior) is commonly performed to decreased
intraoperative bleeding
– Risk of inadvertent cerebral embolization
• Radiation therapy reserved for unresectable tumors or non-operable recurrences
• Hormonal therapy (utamide, estrogen) lacks sufcient evidence
433
Angiosarcoma
Epidemiology
• Aggressive malignant endothelial soft tissue sarcoma most commonly present in
head and neck; scalp subsite most common
• Commonly in elderly white males
• 2% of soft tissue sarcomas, 4% of cutaneous sarcomas
• Overall 5-year survival of ~20–25%
• May arise spontaneously or secondary to external radiation, exogenous toxins, or
immunosuppression
• May be associated with syndromes
– NF-1, Maffucci syndrome, Klippel–Trenaunay syndrome
Presentation
• Raised purplish-red papule, classically multifocal
• Aggressive disease with a 10–20% reported rate of lymph node metastases
• Hematogenous spread typical with lungs most common metastatic site
Pathology
• Pleomorphic, multilayered malignant endothelial cells with aberrant and chaotic
architecture and abnormal vascular channels
• Distinguished from hemangiomas by the presence of “collagen dissection pattern” and formation of papillae
434
https://t.me/medicina_free
B. M. Laitman et al.
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Staging (AJCC Eighth Edition Soft Tissue Sarcoma ofHead
andNeck)
• TX: primary tumor cannot be assessed
• T1: ≤2cm
• T2: >2cm to ≤4cm
• T3: >4cm
• T4a: Invasion of orbit, skull base/dura, central compartment viscera, facial skeleton, or pterygoid musculature
• T4b: Invasion of the brain parenchyma, carotid artery encasement, prevertebral
muscle invasion, CNS involvement via perineural spread
• N0—no regional LN metastases
• N1—regional LN metastases
• M0—no distant metastases
• M1—distant metastases
Treatment
• Combination of wide surgical excision and neck dissection with postoperative
radiation ± chemotherapy
• Induction chemotherapy can be considered in metastatic or unresectable disease
Hemangiopericytoma aka Glomangiopericytoma
Epidemiology
• Rare perivascular tumor most common in extremities
• Presents in the head and neck in 15% of cases
• Originates from the pericapillary pericytic cells
• <1% of all sarcomas are hemangiopericytoma
23 Vascular Malformations andTumors
https://t.me/medicina_free
435
Presentation
• Normally hemangiopericytoma has an indolent growth pattern but may be locally
aggressive and has distant metastases
• Uncommonly involve lymph nodes
• Metastatic disease most commonly to the lungs via hematogenous
dissemination
• May present with oncogenic osteomalacia
• Muscle and bone pain
• Electrolyte abnormalities including hypophosphatemia
Pathology
• Spindle or ovoid tumor cells in compacted nests or sheets between vascular
channels with “pericytes of Zimmerman”
• Single layer of attened endothelial cells
• Immunohistochemistry stains for vimentin but not desmin or factor VIII
Treatment
• Even in the setting of complete excision, local recurrence is as high as 20%, at an
average of 46-month follow-up
• Complete surgical excision is the mainstay of therapy
• The role of radiotherapy remains to be claried and is applied in the setting of
positive margins or unresectable disease
Kaposi Sarcoma (KS)
Epidemiology
• Spindle cell tumor derived from endothelial cell lineage, not a true sarcoma
• Four types
• AIDS-related
– Most common malignancy in HIV-positive patients
– Human herpes virus 8 (HHV-8) association, infection predates KS by
5–10years
436
https://t.me/medicina_free
• Immunocompromised (non-AIDS-related)
– KS 100-fold more likely in solid transplant recipients, with an incidence
of 1/200
– Average time to develop KS post-transplant is 15–30months
• Classic
– Elderly men of Mediterranean or Eastern European origin
• Endemic (Africa)
– African men and women who are HIV seronegative
B. M. Laitman et al.
Presentation
• Mucocutaneous lesions of skin, oral mucosa, and viscera
• Cutaneous lesions can be nodular, papular, or plaques
• Typically of violaceous color, but may be pink or gray
Pathology
• HHV-8 genomic sequences present in almost 100% of KS
• Spindle cell proliferation with numerous and narrow vascular spaces and extravasated red blood cells
Investigation
• Must rule out underlying HIV, CD4 counts
• Punch biopsy to conrm diagnosis
Treatment
• HAART therapy for HIV-positive
• Radiation therapy for larger lesion
• Surgical excisions for small lesions
• Intralesional therapy with vinca alkaloids
Соседние файлы в папке Библиотека им академика М.И. Перельмана