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     Atypical Fibroxanthoma

          Dr Sampurna Roy  MD 

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February  2009

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Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm which was was named by Rosai et al. in 1984.Ectopic hamartomatous thymoma. A distinctive benign lesion of lower neck.Am J Surg Pathol. 1984 Jul;8(7):501-13.

It arises from misplaced branchial pouch derivatives (which normally forms thymic tissue).

Some studies suggest its origin from salivary gland. Pitfalls in the diagnosis of ectopic hamartomatous thymoma.Histopathology. 1996 Dec;29(6):549-55.

According to recent studies it has been suggested that the tumour should be reclassified as a 'branchial anlage mixed tumor 'Ectopic hamartomatous thymoma: a clinicopathologic and immuno histochemical analysis of 21 cases with data supporting reclassification as a branchial anlage mixed tumor.Am J Surg Pathol. 2004 Oct;28(10):1360-70.

Clinical presentation:

The tumour arises in adults, mostly male, as a slowly growing, sub-fascial swelling in the supra-sternal or supra-clavicular region.

The tumour usually does not recur.

Gross:

A well circumscribed swelling measuring 5 cm or less.

Microscopic features:

Consists of variable proportions of :

i) Bland spindle cells with pale cytoplasm & tapering nuclei, arranged in fascicles. 

ii) Island of mature adipose tissue

iii) Epithelial components are arranged in tubules or glands with epithelium that varies from non-keratinizing squamous, cuboidal or glandular elements.

Character of the cells are benign and mitosis is rare.

Immunohistochemistry:

Strong & diffuse positivity for cytokeratin and epithelial membrane antigen (EMA) in the spindle cell areas suggested thymic nature.

According to recent studies immunohistochemical analysis of the tumour revealed a complex immunophenotype with a diverse keratin profile. The plump spindled cells revealed a myoepithelial phenotype, as evidenced by the coexpression of cytokeratins , alpha-smooth muscle actin and CD10.

The tumour is negative for S-100 protein, glial fibrillary acidic protein, and CD34.

Differential diagnosis:

The differential diagnosis includes mixed tumours of skin adnexal or salivary gland origin, synovial sarcoma, glandular malignant peripheral nerve sheath tumor and cystic teratoma.

 Visit: Myxoid Tumours of Soft Tissue ;  Soft Tissue Tumours of Uncertain Differentiation ;   Neural tumours Vascular tumours ; Fibroblastic/Myofibroblastic tumours Myofibroblastic tumours ; Myogenic tumours ;  ChondroOsseous tumours

                

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Abstract:

Ectopic hamartomatous thymoma: a clinicopathological and immunohistochemical study of two cases.Zhonghua Bing Li Xue Za Zhi. 2005 Jul;34(7):397-401.

Ectopic hamartomatous thymoma: a case report and review of the literature.Ann Pathol. 2004 Apr;24(2):176-8.

Ectopic hamartomatous thymoma: a case report showing CD99+ lymphocytes and a low proliferation index.Arch Pathol Lab Med. 2003 Sep;127(9):e378-81.

Ectopic hamartomatous thymoma: a case report with immuno-histochemical and ultrastructural studies. APMIS 2002;110:565–570.

Two cases of ectopic hamartomatous thymoma.Virchows Arch. 2000 Dec;437(6):643-7.

                     

Pathopedia-India.com:

Contents ; Introduction of Pathology ; An outline of Diagnostic Techniques available in Pathology ; Cellular Injury ; Diagram showing Structural Changes in Reversible and Irreversible Cell Injury ; Autolysis; Heterolysis ; Necrosis; Coagulation (Coagulative) necrosis ; Caseative (Caseous) necrosis ; Liquefaction necrosis ; Fat necrosis ; Fibrinoid necrosis ; Apoptosis ; Gangrene ; Hyaline Change ; Atrophy ; Hypertrophy ; Hyperplasia ; Metaplasia ; Aplasia ; Hypoplasia ;Cellular Accumulations ; Accumulation of Glycogen, complex lipids and carbohydrates ; Pigments ; Melanin ; Pigments derived from Hemoproteins; Hemosiderin and Hemosiderosis ; Primary Hemochromatosis ; Hematin; Bilirubin; Lipofuscin; Mineral Dusts ; Silica ; Urate ; Amyloid ; Inflammation ; Inflammatory cells in acute and chronic inflammation ; Acute Inflammation; Types of Acute Inflammation; Chemical Mediators ; Chronic Inflammation; Wound Healing ; Circulatory Anatomy, Physiology and Regulation; Normal Fluid Balance; Edema; Morphology of Edema; Diagram showing Capillary System and Mechanisms of Edema Formation; Hyperemia and Congestion; Hemostasis and Thrombosis; Embolism; Fat Embolism; Air Embolism ; Decompression Sickness ; Amniotic Fluid Embolism ; Diagram showing Sources of Arterial Emboli ; Diagram showing Sources of Venous Emboli ; Infarction ; Diagram showing common sites of Systemic Infarction  from Arterial Emboli; Shock; Pathology of Shock; Diagram showing Complications of Shock; Hemorrhage; Glycogen Storage Diseases(Part I ) ;Glycogen Storage Diseases ( Part II ); Alkaptonuria; Neurofibromatosis; Down Syndromie (Trisomy 21); Edward Syndrome (Trisomy 18); Patau Syndrome (Trisomy 13); Chromosome 22q11 Deletion Syndrome: (Also known as DiGeorge or Velo-Cardio-Facial Syndrome).

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