Metastasis and death may occur late (>10 years), even if clearly excised with no local recurrence
5 year survival 60% but 20 year survival 15%
Pediatric cases may have better survival
Grading / Staging / Report
According to the guidelines of the ADASP, alveolar soft part sarcoma is not considered gradable although it often metastasizes
French Federation of Cancer Centers System grading scheme for adult sarcomas
Tumor differentiation score = 3 for alveolar soft part sarcoma
Mitotic index
Score 1 0-9 mitoses per 10 hpf (0.1744 sq mm)
Score 2 10-19 mitoses per 10 hpf
Score 3 >19 mitoses per 10 hpf
Tumor cell necrosis
Score 0 No necrosis on any slide (one slide per 2 cm tumor diameter)
Score 1 <50% of tumor is necrotic on slides examined
Score 2 >50% of tumor is necrotic on slides examined
Final Grade (add the three scores above)
Grade 1 Sum of scores = 2 or 3
Grade 2 Sum of scores = 4 or 5
Grade 3 Sum of scores = 6 or more
Use TNM Staging
The surgical pathology report should contain or address the following:
Location
Type of resection or biopsy
Histologic diagnosis
Managerial category IV (Systemic disease assumed to be present at the outset)
Extent of tumor cell necrosis
Size
Depth (dermis, subcutis, below fascia, body cavity)
Margins
Involved
Not involved
If under 2 cm give all such distances and sites
If over 2 cm give minimum distance and site
Results of supplementary studies if performed
Relationship to other specimens from the same patient
Lists
Pending
Bibliography
Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 4th edition, 2001
Argani P, Antonescu CR, Illei PB, Lui MY, Timmons CF, Newbury R, Reuter VE, Garvin AJ, Perez-Atayde AR, Fletcher JA, Beckwith JB, Bridge JA, Ladanyi M. Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am J Pathol 2001 Jul;159(1):179-92.
Casanova M, Ferrari A, Bisogno G, Cecchetto G, Basso E, De Bernardi B, Indolfi P, Fossati Bellani F, Carli M. Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group. Ann Oncol 2000 Nov;11(11):1445-9.
Cessna MH, Zhou H, Perkins SL, Tripp SR, Layfield L, Daines C, Coffin CM. Are myogenin and myoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics. Am J Surg Pathol 2001 Sep;25(9):1150-7.
Chetty R. Alveolar soft part sarcoma with psammoma bodies. Histopathology 1990 Aug;17(2):188
d'Amore ES, Ninfo V. Tumors of the soft tissues composed of large eosinophilic cells. Semin Diagn Pathol 1999 May;16(2):178-89.
Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers EL. Lingual alveolar soft part sarcoma; 14 cases: novel clinical and morphological observations. Histopathology. 2004 Nov;45(5):526-37.
Flieder DB, Moran CA, Suster S. Primary alveolar soft-part sarcoma of the mediastinum: a clinicopathological and immunohistochemical study of two cases. Histopathology 1997 Nov;31(5):469-73.
Gomez JA, Amin MB, Ro JY, Linden MD, Lee MW, Zarbo RJ. Immunohistochemical profile of myogenin and MyoD1 does not support skeletal muscle lineage in alveolar soft part sarcoma. Arch Pathol Lab Med 1999 Jun;123(6):503-7.
Hess, Cohen….Costa, AI 1997, 5:94 Chromogranin 1/2 positive? check ref
Hoon V, Thung SN, Kaneko M, Unger PD. HMB-45 reactivity in renal angiomyolipoma and lymphangioleiomyomatosis. Arch Pathol Lab Med 1994 Jul;118(7):732-4.
Jong R, Kandel R, Fornasier V, Bell R, Bedard Y. Alveolar soft part sarcoma: review of nine cases including two cases with unusual histology. Histopathology 1998 Jan;32(1):63-8.
Ladanyi M, Lui MY, Antonescu CR, Krause-Boehm A, Meindl A, Argani P, Healey JH, Ueda T, Yoshikawa H, Meloni-Ehrig A, Sorensen PH, Mertens F, Mandahl N, van den Berghe H, Sciot R, Cin PD, Bridge J. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 2001 Jan 4;20(1):48-57.
Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P, Flehinger BY. Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer 1989 Jan 1;63(1):1-13.
Miettinen M, Ekfors T. Alveolar soft part sarcoma. Immunohistochemical evidence for muscle cell differentiation. Am J Clin Pathol 1990 Jan;93(1):32-8.
Ordonez NG. Alveolar soft part sarcoma: a review and update. Adv Anat Pathol 1999 May;6(3):125-39.
Pappo AS, Parham DM, Cain A, Luo X, Bowman LC, Furman WL, Rao BN, Pratt CB. Alveolar soft part sarcoma in children and adolescents: clinical features and outcome of 11 patients. Med Pediatr Oncol 1996 Feb;26(2):81-4.
Portera CA Jr, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, Yasko AW, Benjamin RS, Pollock RE, Pisters PW. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001 Feb 1;91(3):585-91.
Tornoczky T, Kalman E, Sapi Z, Orosz Z, Pajor L. Cytogenetic abnormalities of alveolar soft-part sarcomas using interphase fluorescent in situ hybridization: trisomy for chromosome 7 and monosomy for chromosomes 8 and 18 seem to be characteristic of the tumor. Virchows Arch 2001 Feb;438(2):173-80.
Wang NP, Bacchi CE, Jiang JJ, McNutt MA, Gown AM. Does alveolar soft-part sarcoma exhibit skeletal muscle differentiation? An immunocytochemical and biochemical study of myogenic regulatory protein expression. Mod Pathol 1996 May;9(5):496-506.
Argani P, Lal P, Hutchinson B, Lui MY, Reuter VE, Ladanyi M. Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am J Surg Pathol. 2003 Jun;27(6):750-61.
Zhong M, De Angelo P, Osborne L, Keane-Tarchichi M, Goldfischer M, Edelmann L, Yang Y, Linehan WM, Merino MJ, Aisner S, Hameed M. Dual-color, break-apart FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of Xp11 translocation renal cell carcinoma and alveolar soft part sarcoma. Am J Surg Pathol. 2010 Jun;34(6):757-66.
Dickson BC, Brooks JS, Pasha TL, Zhang PJ. TFE3 Expression in Tumors of the Microphthalmia-Associated Transcription Factor (MiTF) Family. Int J Surg Pathol. 2010 Feb 16. [Epub ahead of print]
