Alveolar Soft Part Sarcoma

Skip to end of metadata
Go to start of metadata
Table of Contents

DEFINITION AND PATHOGENESIS

  • Unknown cell of origin (although possibly striated muscle)

IMPORTANCE

  • Should be considered esp in anterior thigh lesions as bx can result in a large amount of bleeding
  • ?0.5-1% of all soft-tissue sarcomas

CLINICAL FEATURES

  • Usually occurs in adolescents and young adults
  • F>M
  • ? pulsatilve mass/bruit
  • Thigh and buttock most common location in adults
  • Head and neck most common in children and infants (orbit, tongue)
  • Slow growing and painless
  • Cerebral metastases cause sxs
  • Unusual locations

RADIOLOGIC FEATURES

  • X-rays may reveal the rare underlying bony erosion
  • Nuclear medicine studies
    • Bone scan shows soft-tissue uptake
    • FDG PET
      • Heterogeneous intense uptake to variable uptake reported
  • Angiogram can show the rich vascularity
    • Large vessels sometimes seen with marginal fat suggesting AV malformation
  • CT: soft tissue mass with attenuation ? to skeletal muscle
    • Lesion enhances markedly, especially peripherally
  • MRI: intermediate signal intensity on T1-weighted images (90% greater than muscle--due to slowly flowing blood through the extensive vascularity) and very bright intensity on T2; intense uptake of gadolinium
    • Poorly defined margins, serpiginous vessels evident (serpentine flow voids in 67%)
    • Central necrosis in 75%

GROSS PATHOLOGY

  • Extremely vascular lesion, well to poorly circumscribed and friable, occasionally extending to the subcutaneous tissue
  • Yellow-white-gray-red with foci of hemorrhage, extracapsular invasion

HISTOLOGIC FEATURES

  • Large rounded to polygonal tumor cells usually in nests in a pseudoalveolar pattern (from poor central cellular cohesion) separated by vascular spaces
  • Cells have abundant granular cytoplasm (intracytoplasmic crystals) and distinct cell borders
  • Vascular invasion is common
  • ± ? pleomorphism
  • ± ? mitoses
  • Peripherally dilated veins
  • Special stains:
    • + PAS (diastase resistant) rod-shaped crystals (80%)
    • + reticulin outlining nests of cells
  • Immunohistochemistry:
    • S-100 and neuron-specific enolase occasionally +
    • Vimentin, muscle-specific actin, and desmin have been reported by some to be +
    • Cathepsin K +
      • ASPSCR1-TFE3 t(X;17)(p11;q25) carcinomas - for cathepsin K
    • + VEGFR 1, 2, 3 reported
    • + PDGRR- alpha and beta reported
  • Cytology
    • Fragility of cytoplasm
    • Prominence of bare nuclei
    • Granular-appearing background or the presence of cytoplasmic fragments
    • 3-dimensional alveolar structures
  • EM:
    • Rod-shaped crystals (structurally similar to actin filaments) in a lattice pattern
    • Prominent SER and Golgi apparatus
    • Nests of tumor cells surrounded by a basal lamina
  • Cytometry:
    • Usually a diploid peak
  • Cytogenetics
    • t(X;17)(p11;q25) results in ASPSCR1-TFE3 fusion gene*** fusion product functions as aberrant transcription factor
      • nuclear immunoreactivity for TFE3
      • immunoreactivity for monocarboxylate transporter 1 and CD147
    • trisomy 7, monosomy 8, and monosomy 18 also reported
    • Dual-color break-apart FISH assay reported

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Arteriovenous malformation
  • Hemangioma
  • MFH
  • PVNS
  • Metastatic renal cell carcinoma
  • Paraganglioma
  • Granular cell tumor
  • Melanoma
  • Rhabdomyosarcoma

DISEASE COURSE AND TREATMENT

  • Massive hemorrhage can occur with bx or resection
  • Up to 1/3 develop metastases before detection of a primary
  • ?60% 5 yr survival falls to 15% at 20 yrs (metastases reported 33 yrs after inital presentation)
  • poor response to chemotherapy and/or radiation
  • Novel txs
    • Sunitinib
      • Multitargeted receptor tyrosine kinase inhibitor
        • Care must be taken postop with STSG as necrosis has been reported
        • Mediated by PDGFRB, VEGFR2, and RET (all expressed in tumor cells)
  • CELL LINES

REFERENCES


Agarwal S, Gupta R, Iyer VK, Mathur SR, Ray R: Cytopatholgical diagnosis of alveolar soft part sarcoma, a rare soft tissue neoplasm. Cytopathology 2010 Aug 22. PubMed

Anderson ME, Hornicek FJ, Gebhardt MC, Raskin KA, Mankin HJ: Alveolar soft pasrt sarcoma: a rare and enigmatic entity. Clin Orthop Relat Res 438:144-148,2005.
Auerbach HE, Brooks JJ: Alveolar soft part sarcoma: a clinicopathologic and immunohistochemical study. Cancer 60:66-73,1987.
Bindal RK, Sawaya RE, Leavens ME, : Sarcoma metastatic to the brain: results of surgical treatment. Neurosurgery 35:185-190,1994.
Cardinalli IA, Selig MK, Dickersin GR: Alveolar soft-part sarcoma with unusual mitochondrial findings: a case report. Ultrastruct Pathol 22:321- ,1998.
Carstens HB: Membrane-bound cytoplasmic crystals, similar to those in alveolar soft part sarcoma, in a human muscle spindle. Ultrastruct Pathol 14:423- ,1990.
Casanova M, Ferrari A, Bisogno G, Cecchetto G, Basso E, De Bernardi B, Indolfi P, Fossati Bellani A, Carli M: Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Italian Cooperative Group Ann Oncol 11:1445-1449,2000.

Chen HJ, Chen HS, Chang YL, Liu CS, Hsiao CH: Pathology quiz case 1: alveolar soft-part sarcoma (ASPS) of the anterior tonsillar pillar. Arch Otolaryngol Head Neck Surg 2010 May;136(5):522, 534-5.
Christopherson WM, Foote FW Jr, Stewart FW: Alveolar soft-part sarcoma: structurally characteristic tumors of uncertain histogenesis. Cancer 5:100-111,1952.
Conde N, Cruz O, Albert A, Mora J: Antiangiogenic treatment as a pre-operative management of alveolar soft-part sarcoma. Pediatr Blood Cancer 2011 Dec 1;57(6):1071-3.

Coira BM, Sachdev R, Moscovic E: Skeletal muscle markers in alveolar soft part sarcoma. Am J Clin Pathol 94:799- ,1991.
Craver RD, Heinrich SD, Correa H, : Trisomy 8 in alveolar soft part sarcoma. Cancer Genet Cytogenet 81:94- ,1995.
Cullinane C, Thorner PS, Greenberg ML, : Molecular genetic, cytogenetic and immunohistochemical characterization of alveolar soft part sarcoma: implication for cell origin. Cancer 70:2444- ,1992.
Daly BD, Cheung H, Gaines PA, Bradley MJ, Metreweli C: Imaging of alveolar soft part sarcoma. Clin Radiol 46:253-6,1992.
Enzinger FM, Weiss SW: Malignant soft tissue tumors of uncertain type, In Enzinger FM, Weiss SW (eds): Soft Tissue Tumors, Mosby-Year Book, St.Louis, 3rd ed, 1995, pp 1039-93.
Evans HL: Alveolar spft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer 55:912-917,1985.

Ghose A, Tariq Z, Veltri S: Treatment of multidrug resistant advanced alveolar soft part sarcoma with sunitinib. Am J Ther 2010 Jul 10.

Hanzer M, Nebl A, Spendel S, Pilhatsch A, Urban C, Benesch M: Necrosis of a skin autograft after short-term treatment with sunitinib in a 14-year-old girl with metastatic alveolar soft part sarcoma of the thigh. Klin Paddiatr 2010 May;222(3):184-6.

Hasegawa K, Ichikawa R, Ishii R, Oe S, Kato R, Kobayashi Y, Kuroda M, Udagawa Y: A case of primary alveolar soft part sarcoma of the uterine cervix and a review of the literature. Int J Clin Oncol 2011 Apr 26.

Hayes D Jr, Kesler MV, Skinner SC, Attili AK: A 19-year-old woman with progressive dyspnea and multiple pulmonary nodules. Chest 2011 Jul;140(1):253-7.

Hanzer M, Nebl A, Spendel S, Pilhatsch A, Urban C, Benesch M: Necrosis of a skin autograft after short-term treatment with sunitinib in a 14-year-old girl with metastatic alveolar soft part sarcoma of the thigh. Klin Padiatr. 2010 May;222(3):184-6.
Hermann G, Abdelwahab IF, Klein MJ, Kenan S, Lewis MM: Alveolar soft-part sarcoma. Skeletal Radiol :386-9,1993.
Iwamoto Y, Morimoto N, Chuman H, Shinohara N, Sugioka Y: The role of MR imaging in the diagnosis of alveolar soft parts sarcoma: a report of 10 cases. Skeletal Radiol 24:267-70,1995.

Jia Y, Wu D, Shang C, Yu J, Zhang KR: Alveolar soft part sarcoma occurring on the abdominal wall of a 2-year-old child. J Pediatr Hematol Oncol 2011 Mar;33(2):e80-2.

Kang WD, Heo SH, Choi YD, Choi HS, Kim SM: Alveolar soft part sarcoma of the uterine cervix in a woman presenting with postmenopausal bleeding: a case report and literature review. Eur J Gynaecol Oncol 2011;32(3):359-61.
Kapila K, Chopra P, Verma K: Fine needle aspiration cytology of alveolar soft-part sarcoma. Acta Cytol 29:559- ,1985.

Kenney S, Vistica DT, Stockwin LH, Burkett S, Hollingshead MG, Borgel SD, Butcher DO, Schrump DS, Shoemaker RH: ASPS-1, a novel cell line manifesting key features of alveolar soft part sarcoma. J Pediatr Hematol Oncol 2011 Jul;33(5):360-8.

Kos FT, Uncu D, Oksuzoglu B, Aksoy S, Ozdemir N, Odabas H, Isik M, Civelek B, Zengin N: Long term survival in a metastatic alveolar soft part sarcoma at the time of diagnosis: a case with 17 years of follow-up. J BUON 2011 Apr-Jun;16(2):376-7.

Kumar M, Patne S, Vishwanath A, Hasan Z: Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy. Indian J Cancer 2010 Apr-Jun;47(2):234-5.

Lee GW, Kim TH, Min HJ, Kim HJ, Jung WT, Lee OJ, Ko GH: Unusual gastrointestinal metastases from an alveolar soft part sarcoma. Dig Endosc 2010 Apr;22(2):137-9.
Lieberman PH, Brennan MF, Kimmel M, : Alveolar soft-part sarcoma: a clinicopathologic study of half a century. Cancer 63:1-13,1989.
Lorigan JG, O'Keeffe FN, Evans HL, Wallace S: The radiologic manifestation of alveolar soft-part sarcoma. AJR 153:335-9,1989.

Martignoni G, Gobbo S, Camparo P, Brunelli M, Munari E, Segala D, Pea M, Bonetti F, Illei PB, Netto GJ, Ladanyi M, Chilosi M, Argani P: Differential expression of cathepsin K in neoplasms harboring TFE3 gene fusions. Mod Pathol 2011 May 20.

Montgomery JR, Conrad GR, Sinha P, Absher K: FDG PET of alveolar soft part sarcoma. Clin Nucl Med 2010 Oct;35(10):827-9. PubMed

Mu YL, Liu M, Shi M, Zhao XB, Yin FB, Tang CS, Yelian FD: Clinical analysis of alveolar soft-tissue sarcoma of the uterine cervix: a case report. Chin Med J (Engl) 2010 Jun;123(12):1612-4.

Noussios C, Chouridis P, Petropoulos I, Karagiannidis K, Kontzoglou G: Alveolar soft part sarcoma of the tongue in a 3-year-old boy: a case report. J Med Case Reports 2010 May 8;4:130.

Pang LM, Roebuck DJ, Griffith JF, Kumta SM, Metreweli C: Alveolar soft-part sarcoma: a rare soft-tissue malignancy with distinctive clinical and radiological features. Pediatr Radiol 31:196-9,2001.

Pennacchioli E, Fiore M, Collini P, Radaelli S, Dileo P, Stacchiotti S, Casali PG, Gronchi A: Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol 2010 Dec; 17(12):3229-33.
Radin DR, Ralls PW, Boswell WD, Lundell C, Halls JM: Alveolar soft-part sarcoma: CT findings. J Comput Assist Tomogr 8:344-5,1984.
Reichardt P, Lindner T, Pink D, Thuss-Patience PC, Kretzschmar A, Dorken B: Chemotherapy in alveolar soft part sarcomas: What do we know? Eur J Cancer 39:1511-1516,2003.

Rose AM, Kabiru J, Rose GE: Alveolar soft-part sarcoma of the orbit. Afr J Paediatr Surg 2011 Jan-Apr; 8(1):82-4.

Schultheis B, Kummer G, Tannapfel A, Strumberg D: Successful sequential antiangiogenic therapy for alveolar soft part sarcoma - a case report. Int J Clin Pharmacol Ther. 2010 Jul;48(7):468-9.

Silas OA, Adoga AA, Manasseh AN, Echejoh GO, Vhriterhire RA, Mandong BM: Persistent alveolar soft-part sarcoma with liver metastasis: a case report. J Med Case Reports 2010 Jul 30;4:233.

Stacchiotti S, Negri T, Zaffaroni N, Palassini E, Morosi C, Brich S, Conca E, Bozzi F, Cassinelli G, Gronchi A, Casali PG, Pilotti S: Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 2011 Jul;22(7):1682-90. Pubmed

Strecker T, Schmid A, Agaimy A, Hugo C, Weyand M, Zielezinski T: Giant metastatic alveolar soft part sarcoma in the left ventricle: appearance in echocardiography, magnetic resonance imaging, and hitopathology. Clin Cardiol 2011 Mar;34(3):E6-8.
Suh JS, Cho J, Lee SH, Shin KH, Yang WI, Lee JH, Cho JH, Suh KJ, Lee YJ, Ryu KN: Alveolar soft part sarcomas: MR and angiographic findings. Skletal Radiol 29:680-9,2000.
Temple HT, Scully SP, O'Keefe RJ, Rosenthal DI, Mankin HJ: Clinical presentation of alveolar soft-part sarcoma. Clin Orthop 300:213-8,1994.

Tsuji K, Ishikawa Y, Imamura T: Technique for differentiating alveolar soft part sarcoma from other tumors in paraffin-embedded tissue: comparison of immunohistochemitry for TFE3 and CD147 and of reverse transcription polymerase chain reaction for ASPSCR1-TFE3 fusion transcript. Hum Pathol 2011 Aug 9.
Van Echten J, van den Berg E, van Baarlen J, : An important role for chromosome 17, band q25, in the histogenesis of alveolar soft part sarcoma. Cancer Genet Cytogenet 82:57- ,1995.
Van Ruth s, van Coevorden F, Peterse JL, Kroon BR: Alveolar soft part sarcoma: a report of 15 cases. Eur J Cancer 38:1324-1328,2002.
Wand NP, Bacchi CE, Jiang JJ, :Does alveolar soft-part sarcoma exhibit skeletal muscle differentiation? An immunocytochemical and biochemical study of myogenic regulatory protein expression. Mod Pathol 9:496-1996.
Wang CH, Lee N, Lee LS: Successful treatment for solitary brain metastasis from alveolar soft part sarcoma. J Neurooncol 25:161- ,1995.

Williams A, Bartie G, Sumathi VP, Meis JM, Mangham DC, Grimer RJ, Kindblom LG: Detection of ASPL/TFE3 fusion transcripts and the TFE3 antigen in formalin-fixed, paraffin-embedded tissue in a series of 18 cases of alveolar soft part sarcoma: useful diagnostic tools in cases with unusual histological features. Virchows Arch 2011 Mar;458(3):291-300.

Xin FY, Rana N, Ming Z, Lang YB: Alveolar soft part sarcoma of the retro peritoneum. J Cancer Res Ther. 2010 Jan-Mar;6(1):117-9.

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. PubMed

Unknown macro: {famousquote}
Unknown macro: {rate}
Labels
  • None
Cite this page
Unknown macro: {div}

. *

Unknown macro: {page-info}

*. _

Unknown macro: {builder-spacetitle}

. _In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created

Unknown macro: {page-info}

. Last modified

Unknown macro: {page-info}

ver.

Unknown macro: {page-info}

. Retrieved

Unknown macro: {report-info}

, from

Unknown macro: {page-info}

.

Unknown macro: {builder-hide}
Page contributions
Unknown macro: {div}

The following individuals have contributed to this page:

Unknown macro: {contributors-summary}