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Table 11.2: Ossifying broma not otherwise specied (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 calcications
are labeled as cemento-
ossifying bromas
• Streaming of
the broblastic
stromal elements in a pinwheel
conguration
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
calcied 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 calcication results in anastomosing trabeculae of immature woven bone.
Juvenile psammomatoid ossifying broma:
Multiple small acellular calcied structures, round and uniform and with concentric lamellar calcication, 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 Jae 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 identied 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 conrmation 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 undierentiated neoplasm, which make them dicult to diagnose pathologically.
Histologic examination reveals lobules of hyaline cartilage with variable degrees of cellularity, myxoid change, and calcication. e chondrocytes usually have enlarged hyperchromatic nuclei with binucleation. Necrosis and mitoses are mostly seen in high-grade lesions.
Evans et al.5 classied chondrosarcomas into three grades, from grade I to grade III, according to cellular density, nuclear dierentiation, 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 aect 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.
Dierential Diagnosis
e dierential diagnoses include reactive plasma cell lesions such as plasma cell granuloma, granulomatous inammation, 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
InammatoryMyobroblastic
Tumor
– Glomangiopericytoma
• Benign Soft Tissue Tumors
– Hemangioma – Peripheral Nerve Sheath Tumors
▪ Schwannoma ▪ Neurobromas
• Benign Tumors of Bone and Cartilage
– Giant Cell Lesions
▪ Giant Cell Reparative
Granuloma ▪ Giant Cell Tumor ▪ Aneurysmal Bone Cyst (ABC)
– OsteomaOsteoblastoma
• Benign Germ Cell Tumors
– Mature Teratoma
• Miscellaneous Entities
– Juvenile Nasopharyngeal
Agiobroma(JNA)
– Hemophilic Pseudotumor – Fibro-osseousLesionsFibrousDysplasia(FD)OssifyingFibroma
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 classication of these tumors is detailed in Chapter 10. is classication 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 dierentiated from other masses like inam­matory/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: Conned to nasal cavity.
• Stage 2: Ethmoid sinuses, maxillary sinus (medial and superior region
involved).
• Stage 3: All paranasal sinuses but conned to nose/sinuses.
• Stage 4: Not conned 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
calcic foci are thought to represent “entrapped bone” and true
tumoral calcication is in fact uncommon. ▶ Calcic 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
sclerosisalonglateralnasalwall(blackarrowinA)
• Nasal septum deviated to right (arrow in A)
Thinningandremodelingofthebonywallswithboneerosion(arrowheadinB)
Characteristic“convolutedcerebriform”patternofenhancement(blackarrowinC)
Focalhyperostosis(arrowinD)inanothercaseofIP.
• 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 diuse 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.