9581/3 Alveolar soft part sarcoma


Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A rare malignant neoplasm histologically characterized by the presence of large epithelioid cells with abundant cytoplasm forming nests and pseudoalveolar structures. Groups of the epithelioid cells are separated by thin-walled sinusoidal spaces. In adults the most common sites of involvement are the extremities, in infants and children the head and neck region. It usually presents as a slowly growing mass and it frequently metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, bone, and brain 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

A rare malignant neoplasm histologically characterized by the presence of large epithelioid cells with abundant cytoplasm forming nests and pseudoalveolar structures. Groups of the epithelioid cells are separated by thin-walled sinusoidal spaces. In adults the most common sites of involvement are the extremities, in infants and children the head and neck region. It usually presents as a slowly growing mass and it frequently metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, bone, and brain
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Cervical alveolar soft part sarcoma presents as a polyp or an intramural nodule measuring <5cm in diameter. Patients typically present with abnormal uterine bleeding. Tumours arising in the female genital tract seem to have a better prognosis than their counterparts in other locations
3
Click to access Pubmed
Nielsen GP, Oliva E, Young RH, Rosenberg AE, Dickersin GR, Scully RE (1995)
Alveolar soft-part sarcoma of the female genital tract: a report of nine cases and review of the literature.
Int J Gynecol Pathol 14: 283-92



4
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Alveolar soft part sarcoma is a rare malignant neoplasm histologically characterized by the presence of large epithelioid cells with abundant cytoplasm forming nests and pseudoalveolar structures. Groups of the epithelioid cells are separated by thin-walled sinusoidal spaces. In adults the most common sites of involvement are the extremities, in infants and children the head and neck region. The vulva is a very rare location
5
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



. The tumour usually presents as a slowly growing mass and it frequently metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, bone, and brain 6
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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