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193Chapter 14 Malignant Tumors of Sinonasal Cavities: Imaging
A
C
B
Figs. 14.5A to C: Adenoid cystic carcinoma.
Hetero geneous mass lesion (arrows) involving
the right orbit, ethmoidal sinuses and temporal
fossa. (A) Lesion is isointense on T1w image;
(B) hyperintense on T2w image; (C) showing
intense enhancement on postcontrast image.
■ Meckel’s cave and cavernous sinus are the favored sites. It is important to
recognize this complication because it is generally associated with local
recurrence and poor survival.
■ About half of these SN adenoid cystic tumors have distant metastasis to
lungs, brain, bone and lymph nodes.
■ Treatment of choice is wide local excision with or without local irradiation.

Section 3 Tumor and Tumor-like Conditions194
A
C
Figs. 14.6A to D: Adenoid cystic carcinoma with perineural invasion. (A) Mass lesion
arising from right maxillary sinus which is hyperintense on T2w image. (B to D) Contiguous
perineural extension of tumor (arrows) reaching up to the right cavernous sinus which is
showing intense enhancement after contrast injection.
B
D
Sinonasal Neuroendocrine Carcinoma and
Undierentiated Carcinoma
■ Both of these tumors are more undierentiated as well as more aggressive.
■ ese lesions are more common in elderly age group (in 50–60 years).
■ Imaging appearance largely comprises of more aggressive features like
destruction of SN skeleton, invasion of orbit and skull base, nodal disease
and metastasis at presentation (Fig. 14.7).

Fig. 14.7: Undifferentiated sinonasal carcinoma. Heterogeneous mass lesion with
extensive destruction and invasion of ipsilateral orbit, sinuses, maxillary bone and oor of
anterior cranial fossa with intracranial extension. The mass was sampled and histopathology
showed undifferentiated sinonasal carcinoma––T4bN2bM0 Stage 4C.
NEUROECTODERMAL MALIGNANCIES
195Chapter 14 Malignant Tumors of Sinonasal Cavities: Imaging
Melanoma
■ In SN tract, usually the melanoma is of mucosal variety (unlike cutaneous
elsewhere).
■ Rare tumor of SN tract with poor prognosis.
■ Low overall survival with frequent local as well as distant recurrence.
Imaging Features
■ Site: e junction of mucosa and skin at anterior nasal septum, lateral
nasal wall and inferior turbinate.
■ Polypoid in appearance with well-dened margins.
■ Bone remodeling is more common as compared to bone erosion.
■ e peculiar signal on MRI is homogenously hyperintense on T1WI and
isointense to low signal intensity of T2w images (melanin or hemorrhage).
■ Treatment: Wide local excision with or without postoperative radiation
therapy.
■ e average survival time is only 2–3 years.
Ewing’s Sarcoma Family of Tumors
■ Ewing sarcoma is a highly aggressive small round cell tumor.
■ Only small percentage (1–4 %) of all ES occur in the SN tract.
■ Most common in mandible followed by maxilla and calvarium.
■ Ewing sarcoma and primitive neuroectodermal tumor (PNET), both
have common chromosomal translocation.

Section 3 Tumor and Tumor-like Conditions196
Fig. 14.8: Primitive neuroectodermal tumor of maxilla. Relatively homogeneous mass
lesion (arrow) arising from right maxillary sinus with destruction of medial wall and roof and
contiguous extension into right orbit, nasal cavity and ethmoidal sinuses. Homogeneous
mass lesion with bone as epicenter is the typical appearance.
■ On imaging, aggressive destructive soft tissue with typical onion peel
type of periosteal reaction; less often sunburst type of periosteal reaction
can also be seen (Fig. 14.8).
■ Treatment: Local excision with radiotherapy, if local residual lesion is
present, chemotherapy for micrometastases.
■ Signicant improvement in survival rate of these tumors (from 10% to
60–70%) in recent times.
HEMATOLYMPHOID NEOPLASMS
Lymphoma
■ Sinonasal tract is uncommon site for lymphomas.
■ B-cell, T-cell type and natural killer (NK) cell type varieties can aect SN tract.
■ NK cell type occurs in nasal cavity whereas B-cell type occurs in maxillary
sinus.
■ NK cell type occurs as sheet of soft tissue which may mimic granulomatous
infection or Wegener granulomatosis or carcinoma (Figs. 14.9A and B).
■ NK cell type is usually not associated with cervical lymphadenopathy.
■ Hypo- to isointense on T2w and hypointense on T1w images.
■ Bone destruction and erosion without sclerosis.
■ Association with Epstein-Barr virus.
■ Poor prognosis.
B-cell Type Lymphoma
■ Occurs more commonly in maxillary sinus (Figs. 14.10A and B).
■ Causes more of bone remodeling as compared to destruction.

A B
Figs. 14.9A and B: Natural killer T-cell lymphoma. Mass lesion seen in the right nasal
cavity with destruction of nasal turbinates (arrows in A and B). Obstruction to the osteomeatal
complex causing opacication of right maxillary antrum.
197Chapter 14 Malignant Tumors of Sinonasal Cavities: Imaging
A B
Figs. 14.10A and B: Diffuse large B-cell lymphoma. Homogeneous enhancing mass
lesion (arrows in A and B) in the right side maxillary sinus with extensive bone destruction.
No necrosis or calcication seen in the lesion which is typical for lymphoma.
■ Intermediate signal on T2w images (rather than hypointensity as seen
with NK cell type).
■ Moderate enhancement.
■ Burkitt’s lymphoma is a type of B-cell lymphoma involving facial skeleton
apart from other parts of lymphatic system.
■ Role of imaging in SN lymphomas is to recognize the primary site of
disease, to localize the site for biopsy and if radiotherapy is planned, then
to map the lesion in its entirety.
Plasma Cell Neoplasm
■ May manifest as part of multiple myeloma (MM) or truly solitary
plasmacytoma.
■ On imaging, multiple punched out lytic bone lesions will be seen in MM,
lytic bone lesion with associated soft tissue in plasmacytoma or only soft
tissue, if it is extramedullary plasmacytoma.

Section 3 Tumor and Tumor-like Conditions198
A B
Figs. 14.11A and B: Granulocytic sarcoma of orbit. Homogeneous intensely enhancing
mass lesion arising from the right orbit with contiguous extension into right maxillary
sinus (arrow in A). Clinical background of leukemia and homogeneous mass lesion with
enhancement are the clues to diagnosis.
■ e soft tissue component usually enhances homogeneously.
■ Moderate enhancement with intermediate signal on T2w images.
Granulocytic Sarcoma or Chloroma
■ ese are soft tissue lesions which develop as a spectrum of
myeloproliferative disorders.
■ Word “chloroma” indicate green color (on gross specimen).
■ Seen in around 3% patients of acute leukemias.
■ Can involve any part of head and neck, also in SN tract.
■ On imaging, enhancing relatively homogeneous masses with inltrative
margins (Figs. 14.11A and B).
■ On MRI, intermediate signal on both T1 and T2w images.
3
TUMORS OF SOFT TISSUE, BONE AND CARTILAGE
Rhabdomyosarcoma
■ In pediatric population, RMS is the most common SN malignancy.
■ It has a quite aggressive behavior.
■ Early metastases to nodes and lungs are common.
■ ese are usually of quite advanced stage at presentation with extensive
involvement of multiple compartments in sinuses.
■ Botryoid rhabdomyosarcoma is a subtype of RMS which has a polypoid
appearance on clinical examination. It has the most favorable outcome
amongst all subtypes of RMS.
Imaging Findings
■ Large soft tissue mass with heterogeneous enhancement.
■ Bone destruction is common with invasion into the adjacent compartments.

■ On MRI, these lesions are isointense to hyperintense on T1w images and
heterogeneously hyperintense on T2w images.
Osteosarcoma
■ In SN tract, the onset of osteosarcoma is slightly late (one decade later
than long bones).
■ Osteosarcoma can be primary or secondary to other preexisting
conditions like Paget’s disease, previous radiation therapy, etc.
Imaging Findings
■ Most common site is maxilla.
■ Aggressive periosteal reaction is commonly seen along with large soft
tissue mass. Presence of osteoid matrix on radiograph or computed
tomography (CT) is typical.
■ On MRI, the tumor is predominantly T2 hyperintense. Hypointense
signal can also be seen due to new bone formation.
Chondrosarcoma
■ Primary chondrosarcoma of SN tract are uncommon.
■ Secondary chondrosarcoma occur after radiation therapy, or in
association with Maucci syndrome or Ollier disease.
199Chapter 14 Malignant Tumors of Sinonasal Cavities: Imaging
Imaging Features
■ Alveolar process of maxilla and nasal septum are most common sites.
■ Large soft tissue mass lesion with stippled calcication seen on CT (Figs.
14.12A and B).
■ Because of high content of water in chondroid matrix, chondrosarcomas
are hyperintense signal on T2w images.
■ Heterogeneous appearance on postcontrast images due to enhancement
of the brovascular core and nonenhancement of chondroid matrix.
METASTASIS
■ Metastasis to SN tract is quite rare.
■ Most common site is maxillary sinus.
■ Renal cell carcinoma, breast, lung, thyroid and prostate are the common
primaries.
■ Lesions may be either multiple or single.
■ Imaging characteristics are nonspecic; however, if the primary is thyroid
malignancy or renal cell carcinoma, it may present with large enhancing
soft tissue.
MISCELLANEOUS
In addition to these above mentioned neoplasms of SN tract, there are other
neoplasms which can extend into the SN area because of direct anatomical
contiguity, e.g. malignant lesions of retromolar trigone area, skin carcinomas

Section 3 Tumor and Tumor-like Conditions200
A
Figs. 14.12A and B: Chondrosarcoma. Large mass lesion (arrows in A and B) with extensive
chondroid type of matrix seen involving the left side maxilla, orbit, masticator space and
nasal cavity. There is also erosion of base of anterior cranial fossa with intracranial extension.
A
Figs. 14.13A and B: Basal cell carcinoma. Soft tissue mass arising from the area of medial
canthus without underlying bone destruction.
B
B
of eye or cheek region (Figs. 14.13A and B) as well as aggressive lesions of orbit
can also invade the SN tract. Once these tumors are large in size; it becomes
dicult to dierentiate the primary site of origin.
REFERENCES
1. Eggesbø HB. Imaging of sinonasal tumours.Cancer Imaging. 2012;12:136-52.
2. Sen S, Chandra A, Mukhopadhyay S, et al. Imaging approach to sinonasal
neoplasms. Neuroimaging Clinics. 2015;25(4):577-93.
3. Guermazi A, Feger C, Rousselot P, et al. Granulocytic sarcoma (chloroma): imaging
ndings in adults and children. AJR Am J Roentgenol. 2002;178(2):319-25.

15
CHAPTER
Malignant Tumors of Nose and
Paranasal Sinuses: Surgical Perspective
Rajeev Kumar, Hitesh Verma
• Introduction
• Clinical Presentation
• Local Examination
• Imaging
• Biopsy
• Classication
• Management
• Staging
– Epithelial Tumors of Sinonasal
Origin
▪ Squamous Cell Carcinoma
Staging System (AJCC 2017)
•
ClassicationofSurgical
Procedures
– Medial Maxillectomy
– Inferior or Infrastructural
Maxillectomy
– Posterior Maxillectomy
– Total Maxillectomy
– Craniofacial Resection
• Other Common Sinonasal Tumors
– Adenocarcinoma
– Adenoid Cystic Carcinoma
– Olfactory Neuroblastoma
INTRODUCTION
■ Malignant sinonasal tumors account 3% of all aerodigestive tract
malignancies.1
■ 5th and 6th decade of life is the most common age of presentation with
male to female ratio as 2:1.1
■ Maxillary sinus (55%) and nasal cavity (35%) is the most common site for
origin of tumors.1
■ e most common histological type of tumor is squamous cell carcinoma1
followed by adenocarcinoma.
■ Smoking, aatoxin, heavy metal like chromium, nickel and polycyclic
hydrocarbons are etiological factors.
■ Occupational exposure to hard and soft wood increases chances of
adenocarcinoma.
1
CLINICAL PRESENTATION
■ e presenting symptom will be depending on the area of involvement.
■ Nasal obstruction and bleeding are the leading symptoms.

Section 3 Tumor and Tumor-like Conditions202
■ Other symptoms are loosening of teeth, facial pain, facial swelling,
proptosis, diplopia, neck swelling, etc.
LOCAL EXAMINATION
■ Detailed endoscopic examination is mandatory for suspected malignancy
patients.
■ Proliferative irregular mass which bleeds on manipulation is the
commonest nding in nasal cavity.
■ Nonaxial proptosis, hard to soft swelling, ulceration of face skin, etc. can
be found on examination.
■ Enlarged level II lymph node is the commonest nding in neck, if tumor
had metastasized.
IMAGING
■ Both contrast-enhanced computed tomography (CECT) and contrastenhanced magnetic resonance imaging (CEMRI) are required for
accurate evaluation and staging purposes (see Chapter 14 for details).
BIOPSY
■ For histological classication, tissue biopsy is mandatory.
CLASSIFICATION
Malignant sinonasal tumors are classied as per World Health Organization
classication (see Chapter 10 for details).
MANAGEMENT
e treatment options for malignant sinonasal tumors are:
■ Surgery
■ Radiotherapy
■ Chemotherapy.
e surgical options are discussed subsequently in this chapter.
Nonsurgical options are discussed in Chapter 16.
STAGING
e decision for treatment depends upon stage of the tumor. American Joint
Committee on Cancer (AJCC) staging system is used for malignant sinonasal
tumors of epithelial tumors.
Epithelial Tumors of Sinonasal Origin
Squamous Cell Carcinoma Staging System (AJCC 2010)
■ For maxillary sinus malignancy [Tumor, node and metastasis (TNM)
staging]2 (Table 15.1).
1
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