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Neoplasms of the Nose and Paranasal Sinus University of Texas Medical Branch Steven T. Neoplasms of the Nose and Paranasal Sinus University of Texas Medical Branch Steven T. Wright, M. D. Anna M. Pou, M. D. May 19, 2004 1

Neoplasms of Nose and Paranasal Sinuses Very rare 3% n Delay in diagnosis due Neoplasms of Nose and Paranasal Sinuses Very rare 3% n Delay in diagnosis due to similarity to benign conditions n Nasal cavity n ½ benign n ½ malignant n n Paranasal Sinuses n Malignant 2

Neoplasms of Nose and Paranasal Sinuses Multimodality treatment n Orbital Preservation n Minimally invasive Neoplasms of Nose and Paranasal Sinuses Multimodality treatment n Orbital Preservation n Minimally invasive surgical techniques n 3

Epidemiology Predominately of older males n Exposure: n Wood, nickel-refining processes n Industrial fumes, Epidemiology Predominately of older males n Exposure: n Wood, nickel-refining processes n Industrial fumes, leather tanning n n Cigarette and Alcohol consumption n No significant association has been shown 4

Location n Maxillary sinus n n Ethmoid sinus n n 20% Sphenoid n n Location n Maxillary sinus n n Ethmoid sinus n n 20% Sphenoid n n 70% 3% Frontal n 1% 5

Presentation n Oral symptoms: 25 -35% n n Nasal findings: 50% n n Obstruction, Presentation n Oral symptoms: 25 -35% n n Nasal findings: 50% n n Obstruction, epistaxis, rhinorrhea Ocular findings: 25% n n Pain, trismus, alveolar ridge fullness, erosion Epiphora, diplopia, proptosis Facial signs n Paresthesias, asymmetry 6

Radiography n CT Bony erosion n Limitations with periorbita involvement n n MRI 94 Radiography n CT Bony erosion n Limitations with periorbita involvement n n MRI 94 -98% correlation with surgical findings n Inflammation/retained secretions: low T 1, high T 2 n Hypercellular malignancy: low/intermediate on both n n Enhancement with Gadolinium 7

Benign Lesions Papillomas n Osteomas n Fibrous Dysplasia n Neurogenic tumors n 8 Benign Lesions Papillomas n Osteomas n Fibrous Dysplasia n Neurogenic tumors n 8

Papilloma Vestibular papillomas n Schneiderian papillomas derived from schneiderian mucosa (squamous) n Fungiform: 50%, Papilloma Vestibular papillomas n Schneiderian papillomas derived from schneiderian mucosa (squamous) n Fungiform: 50%, nasal septum n Cylindrical: 3%, lateral wall/sinuses n Inverted: 47%, lateral wall n 9

Inverted Papilloma 4% of sinonasal tumors n Site of Origin: lateral nasal wall n Inverted Papilloma 4% of sinonasal tumors n Site of Origin: lateral nasal wall n Unilateral n Malignant degeneration in 2 -13% (avg 10%) n 10

Inverted Papilloma Resection n Initially via transnasal resection: n n Medial Maxillectomy via lateral Inverted Papilloma Resection n Initially via transnasal resection: n n Medial Maxillectomy via lateral rhinotomy: n n n 50 -80% recurrence Gold Standard 10 -20% Endoscopic medial maxillectomy: n Key concepts: n n n Identify the origin of the papilloma Bony removal of this region Recurrent lesions: n n Via medial maxillectomy vs. Endoscopic resection 22% 11

Osteomas Benign slow growing tumors of mature bone n Location: n n Frontal, ethmoids, Osteomas Benign slow growing tumors of mature bone n Location: n n Frontal, ethmoids, maxillary sinuses When obstructing mucosal flow can lead to mucocele formation n Treatment is local excision n 12

Fibrous dysplasia Dysplastic transformation of normal bone with collagen, fibroblasts, and osteoid material n Fibrous dysplasia Dysplastic transformation of normal bone with collagen, fibroblasts, and osteoid material n Monostotic vs Polyostotic n Surgical excision for obstructing lesions n Malignant transformation to rhabdomyosarcoma has been seen with radiation n 13

Neurogenic tumors 4% are found within the paranasal sinuses n Schwannomas n Neurofibromas n Neurogenic tumors 4% are found within the paranasal sinuses n Schwannomas n Neurofibromas n Treatment via surgical resection n Neurogenic Sarcomas are very aggressive and require surgical excision with post op chemo/XRT for residual disease. n When associated with Von Recklinghausen’s syndrome: more aggressive (30% 5 yr survival). n 14

Malignant lesions n n n Squamous cell carcinoma Adenoid cystic carcinoma Mucoepidermoid carcinoma Adenocarcinoma Malignant lesions n n n Squamous cell carcinoma Adenoid cystic carcinoma Mucoepidermoid carcinoma Adenocarcinoma Hemangiopericytoma Melanoma Olfactory neuroblastoma Osteogenic sarcoma, fibrosarcoma, chondrosarcoma, rhabdomyosarcoma Lymphoma Metastatic tumors Sinonasal undifferentiated carcinoma 15

Squamous cell carcinoma Most common tumor (80%) n Location: n Maxillary sinus (70%) n Squamous cell carcinoma Most common tumor (80%) n Location: n Maxillary sinus (70%) n Nasal cavity (20%) n 90% have local invasion by presentation n Lymphatic drainage: n First echelon: retropharyngeal nodes n Second echelon: subdigastric nodes n 16

Treatment 88% present in advanced stages (T 3/T 4) n Surgical resection with postoperative Treatment 88% present in advanced stages (T 3/T 4) n Surgical resection with postoperative radiation n n Complex 3 -D anatomy makes margins difficult 17

Adenoid Cystic Carcinoma 3 rd most common site is the nose/paranasal sinuses n Perineural Adenoid Cystic Carcinoma 3 rd most common site is the nose/paranasal sinuses n Perineural spread n n Anterograde and retrograde Despite aggressive surgical resection and radiotherapy, most grow insidiously. n Neck metastasis is rare and usually a sign of local failure n Postoperative XRT is very important n 18

Mucoepidermoid Carcinoma Extremely rare n Widespread local invasion makes resection difficult, therefore radiation is Mucoepidermoid Carcinoma Extremely rare n Widespread local invasion makes resection difficult, therefore radiation is often indicated n 19

Adenocarcinoma 2 nd most common malignant tumor in the maxillary and ethmoid sinuses n Adenocarcinoma 2 nd most common malignant tumor in the maxillary and ethmoid sinuses n Present most often in the superior portions n n Strong association with occupational exposures High grade: solid growth pattern with poorly defined margins. 30% present with metastasis n Low grade: uniform and glandular with less incidence of perineural invasion/metastasis. n 20

Hemangiopericytoma n n n Pericytes of Zimmerman Present as rubbery, pale/gray, well circumscribed lesions Hemangiopericytoma n n n Pericytes of Zimmerman Present as rubbery, pale/gray, well circumscribed lesions resembling nasal polyps Treatment is surgical resection with postoperative XRT for positive margins 21

Melanoma 0. 5 - 1. 5% of melanoma originates from the nasal cavity and Melanoma 0. 5 - 1. 5% of melanoma originates from the nasal cavity and paranasal sinus. n Anterior Septum: most common site n Treatment is wide local excision with/without postoperative radiation therapy n END not recommended n AFIP: Poor prognosis n 5 yr: 11% n 20 yr: 0. 5% n 22

Olfactory Neuroblastoma Esthesioneuroblastoma Originate from stem cells of neural crest origin that differentiate into Olfactory Neuroblastoma Esthesioneuroblastoma Originate from stem cells of neural crest origin that differentiate into olfactory sensory cells. n Kadish Classification n A: confined to nasal cavity n B: involving the paranasal cavity n C: extending beyond these limits n 23

Olfactory Neuroblastoma Esthesioneuroblastoma n UCLA Staging system T 1: Tumor involving nasal cavity and/or Olfactory Neuroblastoma Esthesioneuroblastoma n UCLA Staging system T 1: Tumor involving nasal cavity and/or paranasal sinus, excluding the sphenoid and superior most ethmoids n T 2: Tumor involving the nasal cavity and/or paranasal sinus including sphenoid/cribriform plate n T 3: Tumor extending into the orbit or anterior cranial fossa n T 4: Tumor involving the brain n 24

Olfactory Neuroblastoma Esthesioneuroblastoma Aggressive behavior n Local failure: 50 -75% n Metastatic disease develops Olfactory Neuroblastoma Esthesioneuroblastoma Aggressive behavior n Local failure: 50 -75% n Metastatic disease develops in 20 -30% n Treatment: n n En bloc surgical resection with postoperative XRT 25

Sarcomas n Osteogenic Sarcoma Most common primary malignancy of bone. n Mandible > Maxilla Sarcomas n Osteogenic Sarcoma Most common primary malignancy of bone. n Mandible > Maxilla n Sunray radiographic appearance n Fibrosarcoma n Chondrosarcoma n 26

Rhabdomyosarcoma Most common paranasal sinus malignancy in children n Non-orbital, parameningeal n Triple therapy Rhabdomyosarcoma Most common paranasal sinus malignancy in children n Non-orbital, parameningeal n Triple therapy is often necessary n Aggressive chemo/XRT has improved survival from 51% to 81% in patients with cranial nerve deficits/skull/intracranial involvement. n Adults, Surgical resection with postoperative XRT for positive margins. n 27

Lymphoma Non-Hodgkins type n Treatment is by radiation, with or without chemotherapy n Survival Lymphoma Non-Hodgkins type n Treatment is by radiation, with or without chemotherapy n Survival drops to 10% for recurrent lesions n 28

Sinonasal Undifferentiated Carcinoma Aggressive locally destructive lesion n Dependent on pathological differentiation from melanoma, Sinonasal Undifferentiated Carcinoma Aggressive locally destructive lesion n Dependent on pathological differentiation from melanoma, lymphoma, and olfactory neuroblastoma n Preoperative chemotherapy and radiation may offer improved survival n 29

Metastatic Tumors Renal cell carcinoma is the most common n Palliative treatment only n Metastatic Tumors Renal cell carcinoma is the most common n Palliative treatment only n 30

Staging of Maxillary Sinus Tumors 31 Staging of Maxillary Sinus Tumors 31

Staging of Maxillary Sinus Tumors n n T 1: limited to antral mucosa without Staging of Maxillary Sinus Tumors n n T 1: limited to antral mucosa without bony erosion T 2: erosion or destruction of the infrastructure, including the hard palate and/or middle meatus T 3: Tumor invades: skin of cheek, posterior wall of sinus, inferior or medial wall of orbit, anterior ethmoid sinus T 4: tumor invades orbital contents and/or: cribriform plate, post ethmoids or sphenoid, nasopharynx, soft palate, pterygopalatine or infratemporal fossa or base of skull 32

Surgery n Unresectable tumors: Superior extension: frontal lobes n Lateral extension: cavernous sinus n Surgery n Unresectable tumors: Superior extension: frontal lobes n Lateral extension: cavernous sinus n Posterior extension: prevertebral fascia n Bilateral optic nerve involvement n 33

Surgery n Surgical approaches: n n n n Endoscopic Lateral rhinotomy Transoral/transpalatal Midfacial degloving Surgery n Surgical approaches: n n n n Endoscopic Lateral rhinotomy Transoral/transpalatal Midfacial degloving Weber-Fergusson Combined craniofacial approach Extent of resection n Medial maxillectomy Inferior maxillectomy Total maxillectomy 34

Tracheostomy 130 maxillectomies only 7. 7% required tracheostomy n Of those not receiving tracheostomy Tracheostomy 130 maxillectomies only 7. 7% required tracheostomy n Of those not receiving tracheostomy during surgery, only 0. 9% experienced postoperative airway complications n Tracheostomy is unnecessary except in certain circumstances (bulky packing/flaps, mandibulectomy) n 35

Treatment of the Orbit Before 1970’s orbital exenteration was included in the radical resection Treatment of the Orbit Before 1970’s orbital exenteration was included in the radical resection n Preoperative radiation reduced tumor load and allowed for orbital preservation with clear surgical margins n Currently, the debate is centered on what “degree” of orbital invasion is allowed. n 36

Current indications for orbital exenteration Involvement of the orbital apex n Involvement of the Current indications for orbital exenteration Involvement of the orbital apex n Involvement of the extraocular muscles n Involvement of the bulbar conjunctiva or sclera n Lid involvement beyond a reasonable hope for reconstruction n Non-resectable full thickness invasion through the periorbita into the retrobulbar fat n 37

Conclusions Neoplasms of the nose and paranasal sinus are very rare and require a Conclusions Neoplasms of the nose and paranasal sinus are very rare and require a high index of suspicion for diagnosis n Most lesions present in advanced states and require multimodality therapy n 38

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