9758/3 Follicular dendritic cell sarcoma



Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: D47.7
           

/span>ollicular dendritic cell (FDC) sarcoma is a neoplastic proliferation of spindled to ovoid cells showing morphologic and immunophenotypic features of follicular dendritic cells.

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Thyroid gland
ICD-O-3 topography code: C73
ICD10: C96.4
           

Follicular dendritic cell sarcoma is a rare neoplasm composed of spindle to ovoid cells that have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It may be associated with Castelman disease, preferentially affects adults and behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy
3
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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The inflammatory pseudo-tumourlike variant of follicular dendritic cell sarcoma affects predominantly females and is consistently associated with Epstein-Barr virus (EBV), with EBVencoded RNA (EBER) being present in virtually all neoplastic spindle cells
4
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Primary follicular dendritic cell tumours of the thyroid are exceptionally rare and the few reported cases have occured in adults
5
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



. Because of the rarity of thyroid primaries, little is known about the prognosis of this tumour 6
Click to access Pubmed
Galati LT, Barnes EL, Myers EN (1999)
Dendritic cell sarcoma of the thyroid.
Head Neck 21: 273-5



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Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14
ICD10: C96.4
           

Follicular dendritic cell sarcoma is a rare neoplasm composed of spindle to ovoid cells that have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It may be associated with Castelman disease, preferentially affects adults and behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy
7
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.

The inflammatory pseudo-tumourlike variant of follicular dendritic cell sarcoma affects predominantly females and is consistently associated with Epstein-Barr virus (EBV), with EBVencoded RNA (EBER) being present in virtually all neoplastic spindle cells
8
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.



Thymus
ICD-O-3 topography code: C37
ICD10: D47.7
           

Follicular dendritic cell sarcoma is a rare neoplasm composed of spindle to ovoid cells that have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It may be associated with Castelman disease, preferentially affects adults and behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy
9
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.

The inflammatory pseudo-tumourlike variant of follicular dendritic cell sarcoma affects predominantly females and is consistently associated with Epstein-Barr virus (EBV), with EBVencoded RNA (EBER) being present in virtually all neoplastic spindle cells
10
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.



Nasopharynx
ICD-O-3 topography code: C11
ICD10: C96.4
           

Follicular dendritic cell sarcoma is a rare neoplasm composed of spindle to ovoid cells that have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It may be associated with Castelman disease, preferentially affects adults and behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy
11
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.

The inflammatory pseudo-tumourlike variant of follicular dendritic cell sarcoma affects predominantly females and is consistently associated with Epstein-Barr virus (EBV), with EBVencoded RNA (EBER) being present in virtually all neoplastic spindle cells
12
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.