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Table 11.2: Ossifying broma not otherwise specied (NOS).
Ossifying form (most common)
(Fig. 11.3) Cementifying form (Fig. 11.4) Storiform form
• Small irregular osteoid
trabeculae typically rimmed
by osteoblasts (similar to
brous dysplasia)
• Hypercellular stromal
element
• Fbroblastic cells devoid
of any atypical cytologic
features
• Mature lesions: osteoblastic
rimming is minimal, lamellar
irregular trabeculae
• Osseous trabeculae and
cemental structures (ovoid
or droplet in shape)
• Resemble normal
cementicles present in
periodontal ligament
• Ovoid lesions are often
referred to as cementifying
bromas
• Those with both osseous
and cementoid calcications
are labeled as cemento-
ossifying bromas
• Streaming of
the broblastic
stromal elements
in a pinwheel
conguration
similar to
benign brous
histiocytoma
• Mimics
dystrophic bone
153Chapter 11 Pathology of Bony/Cartilaginous Sinonasal Tumors
Fig. 11.3: Ossifying form of ossifying broma (OF): Small irregular osteoid trabeculae
that are typically rimmed by osteoblasts (arrows) and are suspended in mildly cellular stroma.
Juvenile ossifying bromas: Two distinct clinic-pathologic entities are
known:
Trabecular juvenile ossifying broma:
■ Cell-rich brous stroma containing bundles of cellular osteoid and bone
trabeculae without osteoblastic rimming, and aggregates of giant cells.
■ Stromal cells are spindle or polyhedral and produce little collagen and the
brillary osteoid matrix gives the tumor a characteristic loose structure.
■ Cellular, immature osteoid, with plump eosinophilic osteoblastic cells,
forms strands that may be long and slender or plump (paint brush
strokes).

Section 3 Tumor and Tumor-like Conditions154
Fig. 11.4: Cementifying form of ossifying broma (OF): Typical osseous trabeculae in
addition to the cemental structures (arrows).
Fig. 11.5: Juvenile psammomatoid ossifying broma (OF): Multiple small acellular
calcied structures—psammomatoid bodies (blue arrow); suspended in cellular stroma
(green arrow), composed of uniform, stellate, and spindle-shaped cells.
■ Irregular mineralization takes place at the center of the osteoid strands,
and progressive calcication results in anastomosing trabeculae of
immature woven bone.
Juvenile psammomatoid ossifying broma:
■ Multiple small acellular calcied structures, round and uniform and
with concentric lamellar calcication, called ossicles/psammomatoid
bodies—homogenously distributed (Fig. 11.5).
■ Relatively cellular stroma that may have whorled appearance, composed
of uniform, stellate, and spindle-shaped cells.

Fibrous Dysplasia (Fig. 11.6)
■ Fibrous dysplasia is a benign intramedullary bro-osseous lesion
originally described by Lichtenstein in 1938 and by Lichtenstein and Jae
in 1942.
■ Fibrous dysplasia can present in one bone (monostotic) or multiple
bones (polyostotic) and can be associated with other conditions.
■ e lesions of brous dysplasia develop during skeletal formation and
growth and have a variable natural evolution.
■ e etiology has been linked with a mutation in the Gs α gene that occurs
after fertilization in somatic cells and is located at chromosome 20q13.2-
13.3.
■ Gs α mutation was rst identied in patients with McCune-Albright
syndrome, a rare disorder that combines polyostotic brous dysplasia,
skin pigmentation, and one of several endocrinopathies.
■ Biopsy from the lesion is mandatory for conrmation of diagnosis.
Histologic Features
Gross examination
■ A yellowish white tissue with a distinctive gritty feel, imparted by the
small trabeculae of bone.
■ Can be cut with a scalpel and may bleed briskly when cut, as a result of its
concentration of small vessels.
Histologic Features
■ Key histologic feature—delicate trabeculae of immature bone, with
no osteoblastic rimming, enmeshed within a bland brous stroma
155Chapter 11 Pathology of Bony/Cartilaginous Sinonasal Tumors
Fig. 11.6: Fibrous dysplasia: High power view showing irregular bony trabeculae lacking
osteoblastic rimming (arrow) and are suspended in mildly cellular stromal mesenchyme.

Section 3 Tumor and Tumor-like Conditions156
of dysplastic spindle-shaped cells without any cellular features of
malignancy.
■ Margins of the lesion to be separated from surrounding bone by a thin
shell of mature lamellar reactive bone.
■ Variable number of immature, non-stress oriented, disconnected
dysplastic trabeculae oating in a sea of immature mesenchymal cells
that have little or no collagen—“alphabet soup.”
■ Characteristic absence of plump osteoblasts rimming the isolated
immature trabeculae.
■ Bland brous stroma of monomorphic spindle-shaped cells without any
cellular features of malignancy.
■ Multiple delicate capillaries are found throughout the lesion.
■ Lobules of cartilage are infrequently seen composed of mature hyaline
cartilage.
Aneurysmal Bone Cyst
■ e morphologic characteristics of aneurysmal bone cyst (ABC) include
the most conspicuous pattern of presence of cavernous spaces separated
by brous tissue walls which contains osteoclastic giant cells, and strands
of bone.
■ Solid ABC has been mentioned as giant cell reparative granuloma in
extragnathic location by some authors.
Histopathology (Fig. 11.7)
■ Microscopically it shows cavernous spaces with brous walls which
contain scattered or small aggregates of osteoclastic giant cells, and
strands of woven or mature bone.
Fig. 11.7: Aneurysmal bone cyst: Histological photomicrograph showing blood-lled
cavernous spaces (asterisk) with brous septae (thin arrows) and a brous osteoblastic solid
component (thick arrow) with giant cell.

■ Spindle broblastic/brohistiocytic cells.
■ Calcifying bromyxoid foci, if present represent one of characteristic
features.
■ Mitosis usually present and may be high, but atypical mitosis is never
seen.
MALIGNANT TUMORS
Chondrosarcoma (Fig. 11.8)
■ Chondrosarcoma of the head and neck region is a rare disease,
representing approximately 0.1% of all head and neck neoplasms and
constituting only 4% of nonepithelial tumors of the nasal cavity, paranasal
sinuses and nasopharynx.
Histological Features
■ Chondrosarcomas show various histological patterns ranging from
benign chondroid tumor to undierentiated neoplasm, which make
them dicult to diagnose pathologically.
■ Histologic examination reveals lobules of hyaline cartilage with variable
degrees of cellularity, myxoid change, and calcication. e chondrocytes
usually have enlarged hyperchromatic nuclei with binucleation. Necrosis
and mitoses are mostly seen in high-grade lesions.
■ Evans et al.5 classied chondrosarcomas into three grades, from grade I
to grade III, according to cellular density, nuclear dierentiation, and the
size of nucleus.
157Chapter 11 Pathology of Bony/Cartilaginous Sinonasal Tumors
Fig. 11.8: Well-differentiated chondrosarcoma: Photomicrograph showing lobulated
appearance of hyaline cartilage and mild increase in cellularity (arrow).

Section 3 Tumor and Tumor-like Conditions158
Immunohistochemical characteristics of common bony/cartilaginous
tumors are listed in Table 11.3.
Table 11.3: Immunohistochemical characteristics of
common bony/cartilaginous tumors.
Tumor Immunohistochemistry
Central giant cell granulomas (CGCG) Immunoreactive to anti-CD68 antibody
Chondrosarcoma S 100
IDH1 (isocitrate dehydrogenase)
Plasmacytoma Immunopositive for CD138, CD38 and vs38c.
Variable immunoreactivity is seen with CD45
and CD79a
Fig. 11.9: Plasmacytoma: Histological (H&E) section showing sheets of plasma cells
(arrows) including few binucleated one in cases of plasmacytoma of nasal bone.
Plasmacytoma (Fig. 11.9)
■ Plasma cell neoplasms include multiple myeloma, medullary (solitary
plasmacytoma of the bone) and the extramedullary plasmacytoma.
■ It accounts for less than 1% of head and neck tumors. In nasal cavity,
however, plasmacytoma represents approximately 4% of tumors.
About 80–90% of extramedullary plasmacytomas involve the mucosaassociated lymphoid tissue (MALT) of the upper airways, and 75% of
these aect the nasal and paranasal regions.
Histopathology
■ ere is neoplastic proliferation of plasma cells frequently in large
nodules or sheets. Binucleated and immature forms are often admixed
among mature plasma cells.

■ Plasma cells contain abundant rough endoplasmic reticulum which
stains red with methyl green-pyronin (MGP). ere is usually cytoplasmic
accumulation of immunoglobulin, which forms spherical cytoplasmic
inclusions that are either clearly within the cytoplasm (Russell bodies)
or are overlying the nucleus or invaginated into it (Dutcher body). ese
inclusions are positive with Periodic acid-Schi (PAS) reaction and
diastase-resistant. Amyloid deposition may be present, which is stained
by Congo red and under polarized light it has a characteristic “applegreen” birefringence.
Dierential Diagnosis
■ e dierential diagnoses include reactive plasma cell lesions such as
plasma cell granuloma, granulomatous inammation, rhinoscleroma,
and lymphoma.
BIBLIOGRAPHY
1. El-Mofty S. Psammomatoid and trabecular juvenile ossifying broma of the
craniofacial skeleton: two distinct clinicopathologic entities. Oral Surg Oral Med
Oral Pathol Oral Radiol Endod. 2002;93:296-304.
2. Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of
bone. A clinicopathologic analysis with emphasis on histologic grading. Cancer.
1977;40(2):818-31.
3. Eversole R, Su L, ElMofty S. Benign bro-osseous lesions of the craniofacial
complex a review. Head Neck Pathol. 2008;2:177-202.
4. MacDonald-Jankowski DS. Ossifying broma: a systematic review. Dentomaxillofac Radiol. 2009;38:495-513.
5. Soyele OO, Braimah RO, Taiwo AO, et al. Fibrous dysplasia of the maxillofacial
region, a review of 134 cases at Lagos University Teaching Hospital, Lagos,
Nigeria; 2017.
159Chapter 11 Pathology of Bony/Cartilaginous Sinonasal Tumors

12
CHAPTER
Benign Tumors of the Nose and
Paranasal Sinuses: Imaging
Smita Manchanda, Ashu Seith Bhalla
• Introduction
• Benign Epithelial Tumors
– Sinonasal Papillomas
▪ Inverted Papilloma
▪ Fungiform Papilloma
▪ Oncocytic Papilloma
– Salivary Gland Type Adenomas
▪ Pleomorphic Adenoma
• Borderline/Low Malignant Potential
Soft Tissue Tumors
– InammatoryMyobroblastic
Tumor
– Glomangiopericytoma
• Benign Soft Tissue Tumors
– Hemangioma
– Peripheral Nerve Sheath Tumors
▪ Schwannoma
▪ Neurobromas
• Benign Tumors of Bone and
Cartilage
– Giant Cell Lesions
▪ Giant Cell Reparative
Granuloma
▪ Giant Cell Tumor
▪ Aneurysmal Bone Cyst (ABC)
– Osteoma
– Osteoblastoma
• Benign Germ Cell Tumors
– Mature Teratoma
• Miscellaneous Entities
– Juvenile Nasopharyngeal
Agiobroma(JNA)
– Hemophilic Pseudotumor
– Fibro-osseousLesions
– FibrousDysplasia(FD)
– OssifyingFibroma
INTRODUCTION
■ Amongst the head and neck tumors, sinonasal (SN) tumors are relatively
rare.
■ Malignant lesions are encountered more frequently than the benign
entities.
■ Besides sinonasal masses may also originate in the cranium/skull base
(see Chapter 23) or arise from the maxillary alveolus (see Chapter 24).
■ e recent WHO classication of these tumors is detailed in Chapter 10.
is classication divides the tumors into broad categories of epithelial
tumors, soft tissue tumors, tumors of bone and cartilage, germ cell tumors,
hematolymphoid tumors, neuroectodermal tumors and secondary
tumors. ese are further subdivided into benign and malignant in the

rst four groups. e hematolymphoid tumors, neuro ectodermal tumors
and secondary tumors include only malignant lesions.
■ SN tumors need to be dierentiated from other masses like inammatory/infective lesions, such as polyps and mycetoma.
BENIGN EPITHELIAL TUMORS
Sinonasal Papillomas
■ Papillomas arise from the Schneiderian mucosa of the sinonasal tract
(which is of ectodermal origin).
■ Papillomas are of three types: Inverted, Fungiform or Oncocytic.
■ ese are uncommon lesions comprising about 0.4 to 4.7 percent of
all SN tumors1 Inverted papilloma and fungiform forms comprise the
majority (about 50% each) and the oncocytic form is rare.
Inverted Papilloma (IP)
■ Synonym: Inverting papilloma.
■ Occurs more frequently in middle aged men (40–70 years).
■ IP characteristically originates from the lateral wall of the nasal cavity
near the maxillary ostium and also extends into the maxillary sinus.
Other sites of origin include the other paranasal sinuses.
■ IP can also show malignant transformation (synchronous or metachronous).
■ Numerous staging systems are used, Krouse system being most commonly
followed. Four stages2 are described in it:
• Stage 1: Conned to nasal cavity.
• Stage 2: Ethmoid sinuses, maxillary sinus (medial and superior region
involved).
• Stage 3: All paranasal sinuses but conned to nose/sinuses.
• Stage 4: Not conned to sinuses/malignant.
■ Tumor has high recurrence rates of 4 to 22%.
■ Imaging Findings:
• Variable size from small polypoidal lesions to large expansile masses.
• Larger lesions expand the nasal cavity with thinning and remodeling
of the bony walls.
• CT (Figs. 12.1A to D):
▶ Soft tissue attenuation lesions.
▶ A focal area of hyperostosis at the site of origin is characteristic.
▶ Resection of this involved area is critical to reduce recurrence
rates.
▶ Bony fragments may be seen within the soft tissue mass. e
calcic foci are thought to represent “entrapped bone” and true
tumoral calcication is in fact uncommon.
▶ Calcic foci are seen in up to 40% tumors.
▶ e convoluted cerebriform pattern of enhancement characteristi-
cally described on MRI may occasionally be seen on CT.
3
4
161Chapter 12 Benign Tumors of the Nose and Paranasal Sinuses: Imaging

Section 3 Tumor and Tumor-like Conditions162
A
B
C D
Figs. 12.1A to D: Inverted papilloma (CT).
• Large expansile soft tissue density lesion (on NCCT) left nasal cavity (asterisk in A) and
sclerosisalonglateralnasalwall(blackarrowinA)
• Nasal septum deviated to right (arrow in A)
• Thinningandremodelingofthebonywallswithboneerosion(arrowheadinB)
• Characteristic“convolutedcerebriform”patternofenhancement(blackarrowinC)
• Focalhyperostosis(arrowinD)inanothercaseofIP.
• MRI (Figs. 12.2A to C):
▶ A typical “cerebriform” appearance is seen on the T2 weighted
images and postcontrast T1 weighted images. It is also referred
to as a convoluted pattern and may be diuse or partial.1
• Malignant transformation is suspected in IP, if there is bone erosion/
destruction with extension outside the sinonasal cavity. Presence of
necrosis and the partial form of cerebriform pattern should also alert
to possibility of malignant change.
Fungiform Papilloma
■ Seen at a younger age than IP (20–50 years). Also more frequent in males.
■ Site of origin is the nasal septum.
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