Summary of Microscopic Features Summary of Microscopic Features
• Granulomatous inflammation
• Marked infiltration of lymphocytes and Marked infiltration of lymphocytes and plasma cells
L h id i
• Lymphpoid aggregation
• Large amount of xanthomatized histiocytes g y (foamy cytoplasm)
H li i ti d fib i
• Hyalinization and fibrosis
Differential Diagnosis Differential Diagnosis
•
Granulomatous lesions‐ Xanthogranuloma
xanthomatized histiocyte, lymphoid follicles
‐ Tuberculosis Leprosy
‐ Tuberculosis, Leprosy
‐ Sarcoidosis
•
Histiocytic lesion – Langerhan cell histiocytosis•
Benign lymphoepithelial lesions‐ Sjogren syndrome, Mikulicz’s diseases epi‐myoepithelial islands
epi‐myoepithelial islands
Histopathological Report Histopathological Report
• Xanthogranuloma
Right parotid gland Incision Right parotid gland, Incision
No. KMUOP‐09‐1597
Gross Findings Gross Findings
• Size
R’t parotid gland
‐ 7.0x5.0x3.0 cm R’t SMD gland
‐
R’t buccal tumor
‐
• Consistency: firm
• Cut surface: a well‐
circumscribed, solid mass
• Color: whitish
Right Submandibular Gland And Right Submandibular Gland And
Lymph Nodes y p
Right Parotid Gland
Right Buccal Tumor
Special Stains Special Stains
• Histochemical stain
PAS – mucin, polysaccharide PAS mucin, polysaccharide
• Immunohistochemical stains CD68 – histiocyte
CD1a – Langerhan cell CD1a Langerhan cell
S‐100 – Langerhan cell, myoepithelial cell T‐cell – T lymphocyte
B cell – B lymphocyte
B‐cell – B lymphocyte
PAS( )
PAS(‐)
S 100 (+)
S‐100 (+)
CD1a( )
CD1a(‐)
CD68(+)
CD68(+)
T cell (+) And B cell (+)
T‐cell (+) And B‐cell (+)
Results Of Special stains Results Of Special stains
• PAS (‐) – mucin (X), polysaccharide(X) lipid (?)
lipid (?)
• S‐100 (+) but CD1a (‐) – Langerhan cell (X)
• CD68 (+) – Histiocyte (O)
• T‐cell (+) and B‐cell (+) with even distribution T‐cell (+) and B‐cell (+) with even distribution
‐ admixture
Histopathological Features of NXG i Lit t
in Literatures
NXG Our Case
Necrobiosis +, 100% +
Granulomatous inflammation +, 83% +
Lymphoid infiltration +, 100% +
Xanthomatized histiocytes + 100% +
Xanthomatized histiocytes +, 100% + Touton and foreign body
giant cells
+, 83% +
giant cells
Cholesterol clefts +, 33% ‐
Fib i l i
Fibrosis, slcerosis ‐ +
Sinus histiocytosis in LN ‐ +
S‐100 ‐ +
Wood et al, Arch Dermatol. 2009;145(3):279-284; Fernandez-Herrera et al, Semin Cutan Med Surtg. 2007;26:108-113
Wood et al, Arch Dermatol. 2009;145(3):279-284( )
http://www.dermpath.de/histolog.htm
Histopathological Report Histopathological Report
• Necrobiotic xanthogranuloma Right parotid gland, Excision Right parotid gland, Excision
Right submandibular gland, Excision Right buccal space, Excision
No. KMUOP‐09‐1899
Left Parotid Gland
Size: 4.5x3.8x2.9cm
Size: 4.5x3.8x2.9cm
Histopathological Report Histopathological Report
• Necrobiotic xanthogranuloma Left parotid gland Excision
Left parotid gland, Excision
No. KMUOP‐09‐2439
Slides Consultation Slides Consultation
陳授業
•
Dr. 陳授業 , Temple University, Philadelphia‐ Chronic sclerosing sialadenitis
•
Dr. 鄭懿興 and Dr. Wright, Baylor University, Texas‐ Rosai‐Dorfman disease
‐ Rosai‐Dorfman disease
‐ Chronic Sclerosing sialadenitis
•
Dr. Solt and Dr. Reza , Northwestern University, Illinois‐ Rosai‐Dorfman disease
Summary Summary
•
The nature of lesion‐ Inflammatory /reactive, not neoplastic
•
Diagnoses1 Rosai‐Dorfman Disease 1. Rosai‐Dorfman Disease
(Sinus Histiocytosis with Massive Lymphadenitis, SHML)
2. Chronic sclerosing sialadenitis
3. Other histiocytic or granulomatous lesions eg: Langerhan cell histiocytosis
•
Neither of these diagnoses fit very wellNeither of these diagnoses fit very well.Discussion Discussion
• Introduction of necrobiotic
xanthogranuloma(NXG), Rosai‐Dorfman g ( ) disease(RDD), and chronic sclerosing
sialadentitis(CSS) sialadentitis(CSS)
• Comparison between our case and RDD, CSS
Necrobiotic Xanthogranuloma Necrobiotic Xanthogranuloma
•
A rare chronic granulomatous lesion, about 100 cases in literatures, class II histiocytic disorder•
Multiple involvement of skin, most common site – periorbital area (80‐90%), may involveextracutaneous sites, parotid gland: 1 case
•
Skin lesion – multiple indurate yellow‐red plaques S es o u t p e du ate ye o ed p aques or nodules, slowly growing, telangiectasia,ulceration, scarring, g
•
Associated with lymphoproliferative disorderParaproteinemia (80%) multiple myeloma (10%)
‐ Paraproteinemia (80%), multiple myeloma (10%),
Wood et al., Arch Dermatol. 2009;145(3):279-284; Fernandez-Herrera et al., Semin Cutan Med Surtg. 2007;26:108-113; Zainal et al., J Laryngol & Otol. 2009
Wood et al, Arch Dermatol. 2009;145(3):279-284
Rosai Dorfman Disease Rosai‐Dorfman Disease
•
An uncommon benign systemic histioproliferative disorder•
Multiple, bilateral enlarged lymph nodes, most common – cervical, extranodal sites (25‐43%) – head and neck, major salivary gland: 22 cases•
Age – 80% < 20 y/oge 80% 0 y/o•
May be accompanied by fever, malaise, weight loss, leukocytosis and hyperglobulinemialeukocytosis and hyperglobulinemia
•
Proliferative histiocytes surrounded by lymphocytes and plasma cells S 100(+) emperipolesisand plasma cells, S‐100(+), emperipolesis
Panikar et al.,Diag Cytopathol. 2005;33:187-190; Huang et al., Ann Acad Med Singapore 1998;27:589-93
Guven et al., Dentomaxillofacial Radiology 2007;36:428-433;
http://www.pathconsultddx.com/pathCon/
Chronic Sclerosing Sialadenitis Chronic Sclerosing Sialadenitis
•
An uncommon chronic inflammantory disorder in salivary gland, esp. submandibular gland ÆKuttner’s tumor
•
Systemic – sclerosing pancreatitis, colangitis,retroperitoneal fibrosis Æ IgG4‐related sclerosing disease, multifocal fibrosis
Localized – salivary gland Æ CSS
•
Serum immunoglobulin elevated ANA elevatedSerum immunoglobulin elevated, ANA elevated•
Lymphoplasmacytic infiltration, lymphoid follicles, eosinophilia sclerosis fibrosiseosinophilia, sclerosis, fibrosis
Kitagawa et al, Am J Surg Pathol. 2005;29:783-791
Laboratory Studies Laboratory Studies
NXG RDD CSS Our Case
NXG RDD CSS Our Case
Serum Ig > 80%
Gamma‐
pathy
Mono‐
clonal
Polyclonal Mono‐
clonal
IgG IgGκ 60%
IgGλ 26%
IgG4 IgA 14%
ESR ANA
NXG RDD CSS Our Case
Necrobiosis + ‐ ‐ +
Granulomatous inflammation
+ ‐ ‐ +
Lymphoid infiltration + + + +
Xanthomatized + + +
Xanthomatized histiocytes
+ + ‐ +
Multinucleated giant + + ‐ +
Multinucleated giant cells
+ Touton
+ ‐ +
Cholesterol clefts + ‐ ‐ ‐
Cholesterol clefts + ‐ ‐ ‐
Fibrosis, slcerosis ‐ ‐ + +
Si hi ti t i
Sinus histiocytosis ‐ + ‐ +
Emperipolesis ‐ + ‐ ‐
S‐100 ‐ + ‐ +
Eosinophilia ‐ ‐ + ‐
Diagnosis of Our Cases g Necrobiotic
X th l
Xanthogranuloma
Conclusion Conclusion
因為 的稀有性 對診斷與治療形成很大的挑戰
• 因為NXG 的稀有性, 對診斷與治療形成很大的挑戰 診斷 – 有一部份非典型的病理特徵
治療 沒有正式或已形成共識的療法可參考 治療 – 沒有正式或已形成共識的療法可參考 手術 – facial nerve trauma
• 追蹤照護
Xerostomia – rampant caries, 加強口腔衛生照護
Lesions in left submandibular gland and posterior neck – 不影 響外觀, 是否考慮以藥物治療
Associated malignancy – lifelong follow‐up
