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An alveolar soft part sarcoma case mimicking malign melanoma and renal cell carcinoma

Year 2011, Volume: 38 Issue: 2, 239 - 243, 01.06.2011
https://doi.org/10.5798/diclemedj.0921.2011.02.0023

Abstract

Alveolar soft part sarcoma is a rare, distinctive sarcoma most frequently encountered in patients 15-35 years of age, and typically located in the deep soft tissues of the lower extremities in adults. If the tumor affects infants and children, it is often located in the head and neck region. This uncommon neoplasm presents as a slowly growing, painless mass. Because of relative lack of symptoms, it could be easily overlooked and frequent late metastases might be seen at the time of diagnosis after a prolonged clinical course. Herein, we reported an alveolar soft part sarcoma case occurred in a 64 year old male patient with the complaint of a slowly growing mass on his right glutea. The operation specimen was a poorly circumscribed soft tissue and cut section was gray-white with areas of hemorrhage and ne­crosis. On light microscopic investigation, the histological picture reminiscent of alveolar soft part sarcoma with cel­lular tumoral lesion in an organoid patern, and malignant melanoma like morphology somewhere in the tumor were observed. Immunohistochemical staining revealed posi­tivity with vimentin, S-100, EMA, Pankeratin and CD68, but negativity with HMB 45, Melan A, CD 10, sinaptofisin and chromogranin A in the tumor cells. Although immuno­histochemical findings were not specific, the histopatho­logical picture was appropriate for the diagnosis. In conclusion, despite the relatively slow growth, the ulti­mate prognosis is poor so it is very important to recognize this malign lesion with an unexpected clinical presentation and distinct immunohistomorphological features exist.

References

  • Christopherson WM, Foote FWJ, Stewart FW. Alveolar soft- part sarcoma: structurally characteristic tumors of uncertain histogenesis. Cancer 1952; 5(1):100-11.
  • Ackerman LV, Phelps CR. Malignant granular cell myoblas- toma of the gluteal region. Surgery 1946;20(4):511-9.
  • Masson P. Tumeurs humaines: histologie, diagnostics et tech- niques, 2nd edn. Paris: Libraire Maloine, 1956.
  • Weiss SW, Goldblum JR. Enzinger & Weiss’s Soft Tissue Tumors, 5th edn. China: Mosby Elsevier, 2008.
  • Folpe AL, Deyrup AT. Alveolar soft-part sarcoma: A review and update. J Clin Pathol 2006;59(11):1127-32.
  • Roma AA, Yang B, Senior ME, et al. TFE3 immunoreactiv- ity in Alveolar soft part sarcoma of the uterine cervix: case report. Int J Gynecol Pathol 2005;24(2):131-5.
  • Fanburg-Smith JC, Miettinen M, Folpe AL, et al. Lingual al- veolar soft part sarcoma; 14 cases: novel clinical and mor- phological observations. Histopathology 2004; 45(5):526- 37.
  • Portera CA Jr, Ho V, Patel SR, et al. Alveolar soft part sarco- ma: clinical course and paterns of metastasis in 70 patients treated at a single institution. Cancer 2001; 91(3):585-91.
  • Evans HL. Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer 1985; 55(4):912-7.
  • Shipkey FH, Lieberman PH, Foote FW Jr, et al. Ultrastruc- ture of alveolar soft part sarcoma. Cancer 1964;17(3):821- 30.
  • Weiss SW. Alveolar soft part sarcoma: Are we at the end or just the beginning of our quest? Am J Pathol 2002; 160(4):1197-9.
  • Rosai J, Dias P, Parham DM, Shapiro DN, Houghton P: myoD1 protein expression in alveolar soft part sarcoma as confirmatory evidence of its skeletal muscle differentiation: Am J Surg Pathol 1991; 15(10):974-81.
  • Wang NP, Bacchi, Ce, Jiang JJ, McNutt MA, Gown AM. Does alveolar soft part sarcoma exhibit skeletal muscle differentiation? An immunohistochemical and biochemi- cal study of myogenic regulatory protein expression. Mod Pathol 1996; 9(5):496-506.
  • Lieberman PH, Brennan MF, Kimmel M, et al. Alveolar soft-part sarcoma. A clinico-pathologic study of half a cen- tury. Cancer 1989;63(1):1-13.

Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu

Year 2011, Volume: 38 Issue: 2, 239 - 243, 01.06.2011
https://doi.org/10.5798/diclemedj.0921.2011.02.0023

Abstract

Alveolar soft part sarkom (ASPS) ilk olarak Christopher­son ve arkadaşları tarafından bildirilen, nadir ve farklı bir sar-komdur. ASPSlar, erişkinlerde görüldüğünde tipik olarak alt ekstremitelerin derin yumuşak dokularında yer­leşim gös-terirler. Bu nadir neoplazm yavaş büyüyen bir kitle ve tanı esnasında sık rastlanan geç metastazlar ile prezante olur. Tümör histolojik olarak psödoalveolar pa­tern varlığı ile karakterlidir. Yoğun fibröz trabeküller tümö­rü büyük, poligonal tümör hücrelerinden oluşan kompakt gruplara böler ve bu gruplar tekrar ince duvarlı vasküler kanallar ile keskin sınırlı daha küçük adalara bölünürler. Organoid adalar, belirgin sellüler kohezyon kaybı ve san­tral dejenerasyon ile karak-terlidir. Tümör hücreleri, epi­teloid görünüme neden olan belirgin hücre sınırlarına ve bol eozinofilik veya şeffaf sitop-lazmaya sahiptir. Diğer bir belirleyici özellik ise romboid-poligonal kristallerdir. Bizim vakamız, sağ kalçasında yavaş büyüyen kitle şikayeti ile başvuran 64 yaşında erkek hasta idi. Spesmen düzensiz sınırlı bir yumuşak doku örneği olup, kesit yüzü gri-beyaz renkte ve kanama, nekroz alanları içermekte idi. Histolojik tablo ASPS için oldukça karakte-ristik olup, ışık mikros­kopide belirgin hücresel sınırlara sahip, büyük, yuvarlak-poligonal tümör hücrelerinden oluşan, kompakt gruplara ayrılmış, sellüler lezyon izlendi. Nükleuslar veziküler gö­rünümde olup, küçük, belirsiz, eozinofilik nükleol içermek­te idi. Sitoplazmalar bol miktarlarda ve yer yer vakuole, yer yer eozinofilik görünümde idi. İmmünohistokimyasal bulgular çok spesifik olmamasına karşın, histolojik görü­nüm oldukça karakteristik idi. Sonuç olarak, ASPS yavaş büyüyen bir lezyon olmasına karşın, kötü prognozla karakterlidir. Bu nedenle, beklen­me-yen klinik prezentasyon ve farklı immünohistomorfoloji sergileyebilen bu malign lezyonu tanımak çok önemlidir.

References

  • Christopherson WM, Foote FWJ, Stewart FW. Alveolar soft- part sarcoma: structurally characteristic tumors of uncertain histogenesis. Cancer 1952; 5(1):100-11.
  • Ackerman LV, Phelps CR. Malignant granular cell myoblas- toma of the gluteal region. Surgery 1946;20(4):511-9.
  • Masson P. Tumeurs humaines: histologie, diagnostics et tech- niques, 2nd edn. Paris: Libraire Maloine, 1956.
  • Weiss SW, Goldblum JR. Enzinger & Weiss’s Soft Tissue Tumors, 5th edn. China: Mosby Elsevier, 2008.
  • Folpe AL, Deyrup AT. Alveolar soft-part sarcoma: A review and update. J Clin Pathol 2006;59(11):1127-32.
  • Roma AA, Yang B, Senior ME, et al. TFE3 immunoreactiv- ity in Alveolar soft part sarcoma of the uterine cervix: case report. Int J Gynecol Pathol 2005;24(2):131-5.
  • Fanburg-Smith JC, Miettinen M, Folpe AL, et al. Lingual al- veolar soft part sarcoma; 14 cases: novel clinical and mor- phological observations. Histopathology 2004; 45(5):526- 37.
  • Portera CA Jr, Ho V, Patel SR, et al. Alveolar soft part sarco- ma: clinical course and paterns of metastasis in 70 patients treated at a single institution. Cancer 2001; 91(3):585-91.
  • Evans HL. Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer 1985; 55(4):912-7.
  • Shipkey FH, Lieberman PH, Foote FW Jr, et al. Ultrastruc- ture of alveolar soft part sarcoma. Cancer 1964;17(3):821- 30.
  • Weiss SW. Alveolar soft part sarcoma: Are we at the end or just the beginning of our quest? Am J Pathol 2002; 160(4):1197-9.
  • Rosai J, Dias P, Parham DM, Shapiro DN, Houghton P: myoD1 protein expression in alveolar soft part sarcoma as confirmatory evidence of its skeletal muscle differentiation: Am J Surg Pathol 1991; 15(10):974-81.
  • Wang NP, Bacchi, Ce, Jiang JJ, McNutt MA, Gown AM. Does alveolar soft part sarcoma exhibit skeletal muscle differentiation? An immunohistochemical and biochemi- cal study of myogenic regulatory protein expression. Mod Pathol 1996; 9(5):496-506.
  • Lieberman PH, Brennan MF, Kimmel M, et al. Alveolar soft-part sarcoma. A clinico-pathologic study of half a cen- tury. Cancer 1989;63(1):1-13.
There are 14 citations in total.

Details

Primary Language Turkish
Journal Section Case Reports
Authors

Ayşenur Keleş This is me

Uğur Fırat This is me

Hüseyin Büyükbayram This is me

Publication Date June 1, 2011
Submission Date March 2, 2015
Published in Issue Year 2011 Volume: 38 Issue: 2

Cite

APA Keleş, A., Fırat, U., & Büyükbayram, H. (2011). Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu. Dicle Tıp Dergisi, 38(2), 239-243. https://doi.org/10.5798/diclemedj.0921.2011.02.0023
AMA Keleş A, Fırat U, Büyükbayram H. Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu. diclemedj. June 2011;38(2):239-243. doi:10.5798/diclemedj.0921.2011.02.0023
Chicago Keleş, Ayşenur, Uğur Fırat, and Hüseyin Büyükbayram. “Malign Melanomu Ve Renal hücreli Karsinomu Taklit Eden Alveolar Soft Part Sarkom Olgusu”. Dicle Tıp Dergisi 38, no. 2 (June 2011): 239-43. https://doi.org/10.5798/diclemedj.0921.2011.02.0023.
EndNote Keleş A, Fırat U, Büyükbayram H (June 1, 2011) Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu. Dicle Tıp Dergisi 38 2 239–243.
IEEE A. Keleş, U. Fırat, and H. Büyükbayram, “Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu”, diclemedj, vol. 38, no. 2, pp. 239–243, 2011, doi: 10.5798/diclemedj.0921.2011.02.0023.
ISNAD Keleş, Ayşenur et al. “Malign Melanomu Ve Renal hücreli Karsinomu Taklit Eden Alveolar Soft Part Sarkom Olgusu”. Dicle Tıp Dergisi 38/2 (June 2011), 239-243. https://doi.org/10.5798/diclemedj.0921.2011.02.0023.
JAMA Keleş A, Fırat U, Büyükbayram H. Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu. diclemedj. 2011;38:239–243.
MLA Keleş, Ayşenur et al. “Malign Melanomu Ve Renal hücreli Karsinomu Taklit Eden Alveolar Soft Part Sarkom Olgusu”. Dicle Tıp Dergisi, vol. 38, no. 2, 2011, pp. 239-43, doi:10.5798/diclemedj.0921.2011.02.0023.
Vancouver Keleş A, Fırat U, Büyükbayram H. Malign melanomu ve renal hücreli karsinomu taklit eden alveolar soft part sarkom olgusu. diclemedj. 2011;38(2):239-43.