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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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12 Head andNeck Pathology
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Fig. 12.38 Sinonasal undifferentiated carcinoma: (a, b) Two examples of high-grade malignancy
with necrosis
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Fig. 12.39 Olfactory neuroblastoma: lobular tumor aggregates separated by brovascular tissue
Sinonasal Undifferentiated Carcinoma (SNUC) (Fig.12.38)
• High-grade cytokeratin positive aggressive malignancy, lacks clear squamous- or
glandular differentiation and overlying surface dysplasia
• Diagnosis of exclusion
• Hypercellular neoplasm with pleomorphic cells, high nuclear-to-cytoplasm ratio,
large nucleoli, elevated mitotic activity
• Vascular/perineural invasion and necrosis common
• IHC:
– Positive: pan-cytokeratin, possible p16 (but HPV negative), subset with IDH2
– Patchy positive: synaptophysin, chromogranin
– Negative: CK5/6 and p40
Olfactory Neuroblastoma/Esthesioneuroblastoma (Fig.12.39)
• Malignant neoplasm of neuronal/neural crest (neuroectodermal) origin, arises
from olfactory epithelium progenitor cells.
• Tumor centered around cribriform plate.
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Fig. 12.40 Sinonasal adenocarcinoma: (a) Intestinal-type adenocarcinoma (ITAC), abundant
mucin intermixed with malignant epithelium with resemblance to colonic epithelium; (b) NonITAC, low-grade, low-grade morphology, back-to-back glandular proliferation
C. J. Fan et al.
• Histologic grade is a helpful tool in prognostication.
• 4-Tier grading system (Hymes etal.), may be contracted into two groups:
– Well differentiated (grade I, II)
Lower grade.
Lobular pattern, surrounded by vascular tissue.
Dark nuclei appear like lymphocytes (“lymphocyte-like” nuclei).
Neurobrillary matrix: pink, cobweb, or cotton-candy appearance.
Homer-Wright pseudo-rosettes: nuclei ring around neurobrillary matrix.
– Least differentiated (grade III, IV)
Higher grade
Increasing mitotic rate and sheet-like, similar to SNUC in appearance
Less differentiated, nuclei ring with scant or absent neurobrillary matrix
Flexner-Wintersteiner rosettes
Sinonasal Adenocarcinoma (Fig.12.40)
• Primary malignant glandular neoplasm that originates from mucoserous glands
or respiratory epithelium
• Salivary gland-type adenocarcinoma
– Tumors resemble salivary gland counterparts (see salivary gland section).
– Most common: adenoid cystic carcinoma >> mucoepidermoid carcinoma, etc.
• Non-salivary gland-type adenocarcinoma
– Intestinal-type adenocarcinoma (ITAC)
Abnormal epithelium is similar to gastrointestinal adenocarcinoma.
Associated occupational exposures: woodworking.
Usually high grade, stage most important.
IHC: CDX2+, SATB2+, usually CK20+, CK7+/−.
Dx requires exclusion of metastasis from gastrointestinal primary.
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Fig. 12.41 Mucosal melanoma: Occasional cells with melanin (arrows) within tumor
proliferation
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– Non-intestinal-type adenocarcinoma (Non-ITAC)
Lacks intestinal-type histology
Generally divided into two groups by grade:
• Low-grade non-ITAC: tubular-papillary back-to-back glandular formation, relatively bland cytomorphology
• High-grade non-ITAC: more diverse morphology, abundant mitoses,
necrosis
Sinonasal Mucosal Melanoma (SMM) (Fig.12.41)
• Neural crest-derived neoplasm with melanocytic differentiation
• Biologically distinct from cutaneous and uveal counterpart
– Mucosa melanoma lack UV signature.
– Prognostic factors such as Clark level of invasion and Breslow tumor thick-
ness are not applied to sinonasal mucosal melanoma.
• Multiple tumor cell appearances possible: spindle, small, pleomorphic, epithelioid, rhabdoid, spectrum possible even within one tumor
• Large nucleoli (“cherry red” or “stop-sign”), elevated nuclear/cytoplasm ratio
• IHC (panel approach essential): SOX10+, S100+ (can be weak or negative–
diagnostic pitfall), HMB 45+, Melan A (melanocytic antigen), Tyrosinase + (produced by melanoma)
Rhabdomyosarcoma (RMS) (Fig.12.42)
• Malignant soft tissue tumor with features of skeletal muscle differentiation
• Most common sinonasal sarcoma
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Fig. 12.42 Rhabdomyosarcoma (ARMS): Dyscohesive cells in groups. Strap cells (arrow) and
scattered large pink cells with myoid differentiation
C. J. Fan et al.
• Subtypes: embryonal, alveolar, pleomorphic, spindle cell/sclerosing
• Embryonal RMS (ERMS, ~80%):
• Most common in the head and neck
– Primitive appearing
– Round to spindle cell morphology, hyperchromatic, scant cytoplasm
– Alternating hyper and hypocellular areas, may have rhabdomyoblasts
• Alveolar RMS (ARMS, ~20%):
– Primitive appearing
– More monotonous appearance to round cells, hyperchromatic, scant cytoplasm
– Tumor nests and sheets separated by brous septa
– “Alveolar” appearance comes from dyscohesive tumor cells (loosely arranged,
falling apart) at tumor nest periphery and nests accentuated by bands of
brous stroma
Reminiscent of lung alveoli histology on low power
– Most cases with PAX3/7-FOXO1 fusion, other fusions possible
Biphenotypic Sinonasal Sarcoma (BSNS) (Fig.12.43)
• Formerly low-grade sinonasal sarcoma with neural and myogenic features
• Exclusively in sinonasal tract (nasal cavity, ethmoid most common), adult females
• Monotonous and cellular spindle cell sarcoma with a neural and muscle-type
phenotype
• Inconspicuous nucleoli, low mitotic activity, no necrosis
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Fig. 12.43 Monotonous long sweeping spindle cell sarcoma arranged in fascicles. Entrapped
overlying respiratory epithelium (arrow)
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Extranodal Natural Killer (NK)-T cell Lymphoma, Nasal Type (Fig.12.44)
• Formerly known: lethal midline granuloma/midline malignant reticulosis
• Malignant proliferation of NK or cytotoxic T cells with reactive lymphoid
inltrate
• EBV associated, occurs in extranodal location
• Polymorphous appearance, with cell-size variability indicating malignancy
• Inltrate frequently angiocentric and angioinvasive, associated with massive
necrosis that result in non-diagnostic biopsy
• Overlying reactive pseudoepitheliomatous hyperplasia of squamous mucosa
possible
• IHC: CD3+ (T-cells), CD56+ (NK cells), TIA-1+ (NK cell marker), EBV+
DDx of Small Round Blue Cell Tumors of the Sinonasal Tract
• Melanoma (+HMB, +S 100, Melan A, tyrosinase+)
• SNUC (epithelial markers)
• Olfactory neuroblastoma (Esthesioneuroblastoma)
• Lymphoma (lymphocyte markers, especially leukocyte common antigen)
• Poorly differentiated SCC (NUT carcinoma, SMARCB1-decient)/
adenocarcinoma
• Ewing sarcoma
• Rhabdomyosarcoma (myocyte markers)
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Fig. 12.44 Extranodal NK-T cell lymphoma: Cellular, dense proliferation of neoplastic lymphocytes with adjacent necrosis (light pink material)
C. J. Fan et al.
Infectious/Inammatory
Tuberculosis (Fig.12.45)
• Caseating granulomatous disease, result of acid-fast bacilli (Mycobacterium
tuberculosis)
• Elongated-epithelial histiocytes, palisading appearance
• Central acellular necrotic debris
• AFB: bright red on acid-fast/Ziehl-Neelsen stain
Mucormycosis/Fycomycosis/Zygomycosis (Fig.12.46)
• Caused by Mucor, Rhizopus, Absidia
• Typically affect immunocompromised individuals
• Angioinvasive lesions: invasion of fungal elements with resultant thrombosis of
blood vessels and necrosis of soft tissue
• Fungal organism:
– Branching organisms
– Non-septate fungal hyphae
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Fig. 12.45 Tuberculosis: uniformly pink necrotic area surrounded by histiocytes in this necrotizing granuloma. (inset) Rod shaped, red color of mycobacterium stained with AFB (arrows)
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Fig. 12.46 Mucormycosis: branching, pauciseptate ribbon-like fungal organisms
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C. J. Fan et al.
Otology
Keloid (Fig.12.47)
• Abnormal degradation of broblasts after local injury
• Epidermis often attenuated, absent hair follicles in area
• Haphazardly arranged dermal collagen bundles
• Eosinophilic hyalinized appearance “strips of bubble gum” collagen
Gout (Fig.12.48)
• Pink nodules in external auditory canal with chalky substance.
• Microscopic appearance depends on processing:
– Formalin: monosodium urate crystals (MSU) dissolve but leave pale uffy,
amorphous deposits.
– Without processing: MSU crystals are refractile and needle-shaped under
polarized light.
– Regardless of processing: Crystals cause foreign body giant cell reaction in
tissue (tophi).
Fig. 12.47 Keloid: broad bands of densely eosinophilic keloidal collagen (“bubble gum”)
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Fig. 12.48 Gout: Amorphous uffy deposits surrounded by inammation
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Fig. 12.49 Exostoses: Dense lamellar bone, not pedicled to underlying bone
Exostoses (Fig.12.49)
• Usually bilateral, multiple broad-based bony outgrowths in the medial external
auditory canal (EAC)
• Dense lamellar bone deposition with onion-skin appearance
• No marrow spaces or trabecula
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Fig. 12.50 Osteoma: trabeculae of bone with brovascular spaces attached to underlying bone by
a bone pedicle (not shown)
C. J. Fan et al.
Osteoma (Fig.12.50)
• Benign neoplasm of bony canal
• Solitary, unilateral, pedunculated bony mass at bony-cartilaginous junction
• Viable bone with marrow spaces and trabeculae with osteoblasts
Ceruminous Adenoma (Fig.12.51)
• Benign neoplasm of ceruminous glands within external auditory canal (EAC)
• Located lateral 1/3 or 1/2 of the cartilaginous EAC
• Subtypes include:
– Ceruminous pleomorphic adenoma (clinically exclude direct extension of
parotid mass)
– Ceruminous syringocystadenoma papilliferum
• Unencapsulated proliferation of closely packed glandular and cystic structures
• Irregular distribution of glands, but banal cytologically
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