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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4518_Библиотеки_им_академика_М_И_Перельмана
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• Head and neck origins account for 10% of all soft tissue sarcoma
• Primary treatment is surgery, with radiation and chemotherapy as adjuvant
treatment
V. F. Kaul et al.
Epidemiology
• Less than 10% of all sarcomas occur in the head and neck, but has a much worse
prognosis because it is locally invasive and highly metastatic
• It accounts for 1% of all head and neck tumors
• Most head and neck sarcomas arise sporadically with no identiable causative factor
• Sarcomas can be divided into those arising in the soft tissue and those arising in
bone and classied based on their histological cell of origin
• Within head and neck sarcomas, its more commonly soft tissue than boney origin
• Malignant brous histiocytoma (MFH) and liposarcoma are the most common of
all adult sarcomas in soft tissue
• Exposure to radiation can cause late-onset radiation-induced sarcoma of the head
and neck
• There may be an association between sun exposure and cutaneous angiosarcoma
of the head and neck in Caucasians
• The most common site of distant metastatic disease is the lungs
Staging
AJCC soft tissue sarcoma (2018) does not include angiosarcomas, embryonal, and
alveolar rhabdomyosarcoma and Kaposi sarcoma (do not have an AJCC staging system)
Primary tumor—separated by specic tumor locations (trunk and extremity, ret-
roperitoneum, head and neck, and visceral sites)
TX: Primary tumor cannot be assessed.
T0: No evidence of primary tumor.
T1: ≤2cm in greatest dimension
T2: >2 to ≤4cm in greatest dimension,
T3: > 4cm in greatest dimension
T4: invasion of adjoining structures
T4a: orbital invasion, skull base, dural invasion, invasion of central compartment
viscera, involvement of facial skeleton, or invasion of pterygoid muscles
T4b: brain parenchymal invasion, carotid artery encasement, prevertebral muscle
invasion, central nervous system involvement via perineural spread
Grade (differentiation, mitotic count, tumor necrosis).
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GX—grade cannot be assessed.
G1—grade 1
G2—grade 2
G3—grade 3
Regional Lymph Nodes
NX cannot be assessed.
N0—no regional lymph node metastasis.
N1—regional lymph node metastasis.
Distant Metastases
M0—no distant metastases.
M1—distant metastases.
There are no longer any stage grouping in the newest eighth edition
AJCC osteosarcoma/chondrosarcoma of the head and neck
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Primary Tumor
TX Primary tumor cannot be assessed.
T0 No evidence of primary tumor.
T1—≤8cm in greatest dimension.
T2—>8cm in greatest dimension.
T3—Discontinuous tumor in primary site.
Grade (differentiation, mitotic count, tumor necrosis).
GX—grade cannot be assessed.
G1—grade 1
G2—grade 2
G3—grade 3
Regional Lymph Nodes
NX cannot be assessed.
N0—no regional lymph node metastasis.
N1—regional lymph node metastasis.
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Distant Metastases
M0—no distant metastases.
M1—distant metastases.
M1a—lung.
M1b—bone.
Stage Grouping
Stage IA—T1aN0M0 G1,GX; T1bN0M0 G1,GX.
Stage IB—T2N0M0 G1,GX; T3N0M0 G1,GX.
Stage IIA—T1N0M0 G2,G3
Stage IIB—T2N0M0 G2,G3
Stage III—T3N0M0 G2,G3
Stage IVA—Any T N0 M1a Any G.
Stage IVB—Any T N1 M1b Any G.
Malignant Fibrous Histiocytoma
V. F. Kaul et al.
Epidemiology
• Most common soft tissue sarcoma in adults, accounting for 20–30% of soft tissue sarcomas
• Heterogeneous group of sarcomas without a specic line of differentiation
• Exposure to radiation is a very common etiology for malignant brous histiocytoma (MFH), grave prognosis
• 3–10% occur in head and neck
Presentation
• Present as a mass in the scalp, neck, parotid, skull base, or orbit
• May have associated pain, compression
• Skin involvement common
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Histology
• Very difcult, diagnosis of exclusion
• Most lesions of high grade
• Two more common types:
• Undifferentiated high grade
• Pleomorphic sarcoma and myxobrosarcoma
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
Treatment
• Surgery is the mainstay of treatment
• Adjuvant chemotherapy and radiotherapy may improve outcomes but this is not
clear due to lack of data
Rhabdomyosarcoma
Epidemiology
• Fifth most common cancer in the pediatric age group, most common childhood
soft tissue sarcoma
• Worse outcome in adults
• Rare aggressive variant spindle cell rhabdomyosarcoma (RMS) found in adults
has a propensity for the head and neck
Presentation
• May involve orbit (most common), nasopharynx, nasal cavity, paranasal sinuses,
and temporal bone (most common primary temporal bone malignancy in
children)
• High incidence of lymph node and distant metastasis, especially the parameningeal tumors
• Fine-needle aspiration is not helpful in diagnosis
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V. F. Kaul et al.
Histology
• Four subtypes:
– Embryonal (70%) early age presentation, better prognosis
– Alveolar (20%), poor prognosis
– Botryoid and pleomorphic (10%)
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Treatment
• Multimodal approach consisting of multi-agent chemotherapy, surgery, and radiation therapy
• Nonsurgical approach if unresectable due to critical structure involvement
• Proton-beam therapy is used particularly for parameningeal, paraspinal sites
• 5-year survival 64%
• Orbital subsite is favorable compared to parameningeal sites
Angiosarcoma
Epidemiology
• Typically occurs in elderly male
• Arise in the dermal layers of the scalp and facial skin and spread in a radial fashion with multifocal pattern
• Raised purplish-red papule, classically multifocal
• Difcult to achieve clear surgical margins, high risk of local recurrence
• May arise spontaneously or secondary to external radiation, exogenous toxins, or
immunosuppression
• 5-year survival rates of 10–30% for high-grade angiosarcoma
Presentation
• Aggressive disease with a 10–20% reported rate of lymph node metastases
• Typically spread hematogenously with lungs most common metastatic site
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Histology
• 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
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Treatment
• Combination of wide surgical excision and neck dissection with postoperative
radiation±chemotherapy
• Chemotherapy and radiation for metastatic or unresectable disease
• Targeted vascular therapy (bevacizumab) with monoclonal antibodies is
promising
Liposarcoma
Epidemiology
• Only 2–4% of liposarcomas arise in the head and neck
• The neck is the most common location
• Different from other sarcomas in their propensity to metastasize to bone
Presentation
• Deep soft tissue expanding mass
• Rarely painful until large or compressive
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V. F. Kaul et al.
Histology
• Lipoblasts often present; these are cells with an abundant clear multi-vacuolated
cytoplasm and an eccentric darkly staining nucleus that is indented by the
vacuoles
• Round-cell or pleomorphic tumors associated with poor prognosis
• Myxoid liposarcomas have a unique molecular signature characterized by the
presence of the t(12; 16)(q13; p12)
• Late-onset soft tissue or late-onset bone metastases
• High radiosensitivity
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Treatment
• Radical surgical excision with negative margins and adjuvant radiotherapy
Synovial Sarcoma
Epidemiology
• Aggressive slowly growing tumor of young adults—only accounting for 5–10%
of soft tissue sarcomas
• Very rare in head and neck subsites
• Synovial sarcomas are tumors of pluripotent mesenchymal cells that do not arise
in the synovium but microscopically bear resemblance to normal synovium
(name is a misnomer)
• High-grade sarcoma with poor survival
Presentation
• Hypopharynx (most common in head and neck), orbit, larynx, oropharynx
• Dysphagia, pain, and hoarseness may occur
• May present as a painless mass in the neck or the upper aerodigestive tract
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Histology
• Uniform spindle cells with high nuclear-to-cytoplasmic ratio
• Two types of SS:
• Monophasic which consists of spindle cells
• Biphasic includes epithelial cells with spindle cells
• Chromosomal alterations between X and 18
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Treatment
• Treatment is surgical resection
• Adjuvant radiation and chemotherapy may be helpful but remain unproven due
to small number of cases in the head and neck
Dermatobrosarcoma Protuberans (DFSP)
Epidemiology
• Low-grade tumor with a predilection for local recurrence
• Can undergo brosarcomatous transformation after a span of many years
• Predominantly adult presentation
• More common among African Americans compared to Caucasians
Presentation
• Commonly cutaneous presentation seen in the scalp, neck
• Distant metastasis rare but may develop in up to 5% of patients
• Cutaneous pink to red-bluish painless trophic and/or sclerotic plaque-like mass
that develops into lumpy nodular protuberant tumor.
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V. F. Kaul et al.
Histology
• Unclear origin may be broblastic, neuroectodermal, and histiocytic or from pluripotential progenitor cells
• Characterized by the arrangement of spindle-shaped tumor cells in a “cartwheel” pattern
• Honeycomb pattern of inltration into the subcutaneous fat may project up to
3cm peripherally
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
Treatment
• Surgical resection with wide margins although this can be challenging due to
invasion of local tissue planes (villous pattern of extension)
• Adjuvant radiotherapy provides local control of up to 85%
• Imatinib is offered in patients with positive platelet-derived growth factor receptor (PDGF-r) or unresectable local or metastatic disease
• Excellent 5-year survival despite tendency to recur
Ewing Sarcoma
Epidemiology
• Rare, aggressive neoplasm of the bone and, less commonly, soft tissue
• Third most common bone tumor after osteosarcoma and chondrosarcoma
• Majority have translocation t(11;22)(q22;q12) involving the EWS gene and
FLI genes
Presentation
• Tend to be smaller with a lower metastatic rate compared to other regions
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Histology
• Small blue round-cell tumors
• Higher LDH is associated with overall lower survival rate and 5-year diseasefree survival rate
Imaging
• CT and MRI to evaluate bone and soft tissue involvement, respectively
• PET/CT useful to rule out metastatic disease
Treatment
• Wide-local excision is the mainstay of treatment; negative margins are critical
for improved outcomes
• Postoperative chemotherapy and radiotherapy
• Pre-surgical chemotherapy treatment can shrink the tumor size
• If surgery is not an option, radiation can be used as primary management
• Proton therapy is showing promising local control for nonsurgical tumors
• Targeted therapy like monoclonal antibodies with chemotherapy are being trialed
Osteosarcoma
Epidemiology
• Most common malignancy of bone
• Maxilla (alveolar ridge) and mandible (posterior body) are the predominant sites
• Highly malignant tumor with equal sex distribution but occurs in the third and
fourth decades (extremity sarcomas occur earlier)
• May be radiation induced
• Genetic link with retinoblastoma 13q14 chromosomal deletion
• Distinct from Ewing’s sarcoma, an aggressive sarcoma of bone treated with chemotherapy and surgery, considered a systemic disease
Presentation
• Rapidly enlarging mass lesion of facial bones, usually in the fourth decade of life
• May present with paresis or neuropathy, pain depending on location
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