Selma ŞENGİZ, Kutsal YÖRÜKOĞLU, Aydanur KARGI, Hasan HAVITÇIOĞLU

Alveolar soft part sarkom: Olgu sunumu

Yumuşak doku tümörleri içinde yer alan alveolar soft part sarkom (ASPS) oldukça nadir görülen bir neoplazmdır. Olgumuz seyrek görülmesi nedeniyle histokimyasal ve immunohistokimyasal özellikleri ile sunulmuştur. Tibia distal 1/3 yumuşak dokuda şişlik ve ağrı yakınması bulunan 32 yaşındaki erkek hastada yapılan görüntülemelerde yaklaşık 4,5 cm çapında, komşu kemik korteksini erode eden, kontur düzensizliği olan, belirgin vaskülarizasyon gösteren radyolüsen lezyon saptannuştır. Rezeke edilen kitlenin mikroskopik incelemesinde poligonal ya da yuvarlak tümör hücrelerinin alveolar patern ya da kümeler oluşturduğu görülmüştür. Sinüzoidal vasküler kanallarla ayrılmış alveolar alanlarda santral dejenerasyon ve kohezyon kaybı gözlenmiştir. Tümör hücre sitoplazmasmda diastaz-rezistan PAS pozitif yapılar izlen¬miştir. İmmunohistokimyasal yöntemde hücrelerde vimentin, akün ve S-100 ile olumlu boyanma izlenirken keratin, desmin, faktör 8, HMB45, EMA, GFAP ve myoglobin ile boyanma izlenmemiştir. Histogenezisi hala tartışma konusu olan ve nadir görülen yumuşak doku tümörlerinden ASPS tanısı klinik, histomorfolojik ve immunhistokimyasal bulguların birlikte değerlendirilmesi ile mümkündür.

Anahtar Kelimeler:

İmmünohistokimya, Sarcom, alveolar yumuşak kısım

Alveolar soft part sarcoma: Case report

Alveolar soft part sarcoma, taking part in soft tissue sarcomas, is a rare tumour. Because of the rare cases reported in the literature, our case is presented by histochemical and immunohistochemical findings. A 32 years old male complained of pain and swelling in the distal tibia. A mass was determinated radiologically, which was approximatelly 4.5 cm in diameter, eroding the adjacent cortex of bone with disordered margins and prominent vascularization. The tumour cells were polygonal or rounded and constituted alveolar pattern or nests. Central degeneration and cohesion loss were seen. PAS-positive, diastase-resistant structures were noted in the cytoplasm of tumour cells. Immunohistochemically; tumour cells were stained with antibodies against vimentin, actin and S-100, but not with cytokeratin, desmin, factor VIII, HMB45, EMA, GFAP and myoglobin. Diagnosis of ASPS, which is rare tumour with controversial histogenesis is possible with clinical, histopathological and immunohistochemical findings.

Keywords:

Immunohistochemistry, Sarcoma, Alveolar Soft Part,

PDF

___

1.Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft tissue tumors. 4rd ed. St Louis, Mosby, 2001;1509-1521.
2.Portera CA, Ho V, Patel SR, Hunt KK. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001; 91:585- 591.
3.Herbert E, Auerbach DO, Brooks JJ. Alveolar soft part sarcoma: A clinicopathologic and immunohistochemi-cal study. Cancer 1987; 60:66-73.
4.Casanova M, Ferrari A, Bisogno G, Cecchetto G. Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Coo-perative Group. Ann Oncol 2000; 11 1445-1449.
5.Mettinen M, Ekfors T. Alveolar soft part sarcoma: Immunohistochemical evidence for muscle cell differentiation. Am J Clin Pathol 1990; 93:32-38.
6.Radig K, Buhtz P, Roessner A. Alveolar soft part sarcoma of the uterine corpus. Report of two cases and review of the literature. Pathol Res Pract 1998; 194:59-63.
7.Pollard SG, Marks PV, Temple LN et al. Breast sarcoma. A clinicopathologic review of 25 cases. Cancer 1990; 66:941-944.
8.Carstens PHB. Retroperitoneal sarcoma with features suggestive of alveolar soft part sarcoma. Ultrastruct Pathol 1991; 15:509-513.
9.Hirose T, Kudo E, Hasegawa T et al. Cytoskeletal properties of alveolar soft part sarcoma. Hum Pathol 1990; 21:204-211.
10.Suh JS, Cho J, Lee SH et al. Alveolar soft part sarcoma: MR and angiographic findings. Skeletal Radiol 2000; 29:680- 689.
11.Pang LM, Roebuck DJ, Griffith JM et al. Alveolar softpart sarcoma: a rare soft-tissue malignancy with dis-tinctive clinical and radiological features. Pediatr Radiol 2001; 31:196-199.
12.Hunter BC, Frelito A, Devaney KO, Rinaldo A. Clinicopathological consultation alveolar soft part sarcoma of the head and neck region. Ann Otol Rhinol Laryngol 1998; 107:810-814.
13.Foschini MP, Eusebi V. Alveolar soft part sarcoma: A new type of rhabdomyosarcoma? Semin Diagn Pathol 1994; 11:58-68.
14.Cessna MH, Zhou H, Perkins SL et al. Are myogenin and myoD1 expression specific for rhabdomyosar-coma? A study of 150 cases with emphasis on spindle cell mimics. Am J Surg Pathol 2001; 25:1150-1157.
15.Nakano H. Alveolar soft part sarcoma: histogenesis. Anticancer Res 1998; 18:4207-4211.
16.Nakano H. Tateishi A, Imamura T et al. RT-PCR suggests human skeletal muscle origin of alveolar soft part sarcoma. Oncology 2000; 58:319-323.
17.Gomez JA, Amin MB, Ro JY et al. Immunohistochemical profile of myogenin and MyoD1 does not support skeletal muscle lineage in alveolar soft part sarcoma. Arch Pathol Lab Med 1999; 123:503-507.
18.Ordonez NG. Alveolar soft part sarcoma: a review and update. Adv Anat Pathol 1999; 6:125-139.
19.Ordonez NG, Mackay B. Alveolar soft part sarcoma: a review of the pathology and histogenesis. Ultrastruct Pathol 1998; 22:275-292.
20.Cullinane C, Thorner PS, Greenberg ML et al. Molecular genetic, cytogenetic and immunohistochemical characterization of alveolar soft part sarcoma. Cancer 1992; 70:2444-2450.
21.Sciot R, Dal Cin P, De Vos R et al. Alveolar soft part sarcoma: evidence for its myogenic origin and for the involvement of 17q25. Histopathology 1993; 23:439-444.
22.Joyama S, Ueda T, Shimizu K et al. Chromosome rearrangement at 17q25 and Xp11.2 in alveolar soft part sarcoma: a case report and review of the literature. Cancer 1999; 86:1246-1250.
23.Kurtz JE, Andres E, Rohr S et al. Pancreatic metastasis of alveolar soft part sarcoma: a case report and review of the literature. Ann Oncol 2001; 12:865-867.
24.Ladayni M, Antonescu CR, Drobnjak M et al. The precrystalline cytoplasmic granules of alveolar soft part sarcoma contain monocarboxylate transporter 1 and CD147. Am J Pathol 2002; 160:1215-1221.
25.Umar H, Pabuçcuoğlu U, Erdinç E, Bacakoğlu F. Alveolar soft part sarcoma (histokimyasal ve elektron mikroskopik çalışma). Turkish J Pathol 1992; 8:61-62.
26.Wang NP, Bacchi CE, Jiang JJ et al. Does alveolar soft part sarcoma exhibit skeletal muscle differentiation? An immunohistochemical and biochemical study of myo-genic regulatory protein expression. Mod Pathol 1996; 9:496-506.
27.Lillehei KO, Kleinschmidt-Demasters B, Mitchell DH et al. Alveolar soft part sarcoma: an unusually long interval between presentation and brain metastasis. Hum Pathol 1993; 24:1030-1034.
28.Flieder DB, Moran CA, Suster S. Primary alveolar soft part sarcoma of the mediastinum: a clinicopathological and immunohistochemical study of two cases. Histo-pathology 1997; 31:469-473.