Sevil SAYHAN, Osman ZEKİOĞLU, Yıldız ERHAN

Overin primer rabdomyosarkomu: Olgu sunumu

Rabdomyosarkomlar genetikle çocukluk döneminde, baş boyun bölgesi ve ekstremitelerde görülen yumuşak doku tümörleridir. Primer over yerleşimli rabdomyosarkomlar ise çok nadir olup malign mikst müllerian tümörlerin bir komponenti şeklinde izlenmektedirler. Karında kitle ve ağrı yakınmaları ile başvuran 26 yaşındaki olguya yapılan abdominal ultrasonografi ve bilgisayarlı tomografi sonrası her iki overde tümöral oluşumlar saptanmıştır. Cerrahi olarak çıkarılan tümörün mikroskobik incelemesinde, alveolar boşlukları döşeyen oval çekirdekli eozinofilik sitoplazmalı küçük hücreler izlenmiştir. Immünohistokimyasal olarak tümör hücreleri desmin ve düz kas aktini ile boyanmıştır. Evre III olarak değerlendirilen olgu, operasyondan 5 ay sonra yaşamını yitirmiştir. Histopatolojik ve immünohistokimyasal bulgular eşliğinde överin primer rabdomyosarkomu olarak değerlendirilen olgu nadir görülmesi nedeniyle sunulmuştur.

Anahtar Kelimeler:

Rabdomiyosarkom, Erişkin, Tanı teknikleri, kadın doğum ve kadın hastalıkları, Neoplazmlar, bağ dokusu ve yumuşak doku, Ölümle sonuçlanma

Primary rhabdomyosarcoma of the ovary: A case report

Rhabdomyosarcomas are soft tissue tumors mostly seen in childhood on head and neck region and limbs. Primary ovarian rhabdomyosarcomas are extremely rare, and are presented as a component of malignant mixed mullerian tumors. 26-year-old female presented with abdominal mass and pain. The abdominal ultrasonography and computed tomography showed bilateral ovarian tumors. Microscopic examination revealed small tumor cells with oval nucleus and eosinophilic cytoplasm composing an alveolar pattern. Tumor cells were stained positively with smooth muscle actin and desmin. The case was evaluated as stage III and died five months after the surgery. By the association of the histopathologic and immunohistochemical findings this case was diagnosed as primary rhabdomyosarcoma of ovary. This case is presented because it is unusual and rare tumor of the ovary.

Keywords:

Rhabdomyosarcoma, Adult, Diagnostic Techniques, Obstetrical and Gynecological, Neoplasms, Connective and Soft Tissue, Fatal Outcome,

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1. Weis SW, Goldblum JR. Enzinger's and Weiss's Soft Tissue Tumors. Fourth Edition. St. Louis: Mosby, 2001; 785-835.
2. Nielsen GP, Oliva E, Young RH, et al. Primary ovarian rhabdomyosarcoma. A report of 13 cases. Int J Gynecol Pathol 1998; 17:113-119.
3. Payan H. Rhabdomyosarcoma of the ovary. Obstet Gynecol 1965; 26:393-395.
4. Nunez C, Abboud SL, Lemon NC, Kemp JA. Ovarian rhabdomyosarcoma presenting as leukemia. Case report. Cancer 1983; 52:297-300.
5. Asmar L, Gehan EA, Newton WA, et al. Agreement among and within groups of pathologist in the classification of rhabdomyosarcoma and related childhood sarcomas. Cancer 1994; 74:2579-2588.
6. Dodd S, Malone M, Mcculloch W. Rhabdomyosarcoma in children. A histological and immunohistochemical study of 59 cases. J Pathol 1989; 158:13-18.
7. Prat J, Young RH, Scully RE. Ovarian Sertoli Leydig Celi tumors with heterologous elements. Cartilage and skeletal muscle. A clinicopathologic analysis of twelve cases. Cancer 1982; 50:2465-2475.
8. De Brito Pa, Silverberg SG, Orenstein JM. Carcinosarcoma (malignant mixed mullerian (mezodermal) tumor) of the female genital tract. İmmunohistochemical and ultrastructural analysis of 28 cases. Hum Pathol 1993; 24:132-142.
9. Kawai M, Kano T, Furuhashi Y, et al. Immature teratoma of the ovary. Gynecol Oncol 1991; 40:133-137.
10. Eichhorn JH, Young RH, Scully RE. Primary ovarian small celi carcinoma of pulmonary type. A clinicopathologic, immunohistochemical and flow cytometric anaiysis of 11 cases. Am J Surg Pathol 1992; 16:926-938.
11. Ruymann FB, Newton WA, Ragap AH, Donaldson MH, Foulkes M. Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. A report of intergroup rhabdomyosarcoma study. Cancer 1984; 53:368-373.
12. Shimada H, Newton WA, Soule EH, Beltangady MS, Maurer HM. Pathology of fetal rhabdomyosarcoma. A report from intergroup rhabdomyosarcoma study (IRS-I and İRS- III) Cancer 1987; 59:459-465.
13. Kleinman GM, Young RH, Scully RE. Primary neuroectodermal tumors of the ovary. A report of 25 cases. Am J Surg Pathol 1993; 17:764-778.
14. Monterroso V, Jaffe ES, Merino MJ, Medeiros LJ. Malignant lymphoma involving the ovary. A clinicopathologic analysis of 39 cases. Am J Surg Pathol 1993; 17:154-170.
15. Young RH, Scully RE. Alveolar rhabdomyosarcoma metastatic to the ovary. A report of two cases and a discussion of the differential diagnosis of small celi malignant tumors of the ovary. Cancer 1989; 64:899-904.
16. Tsokos M. The role of immunohistochemistry in the diagnosis of rhabdomyosarcoma. Arch Pathol Lab Med 1986; 110:776-778.
17. Dias P, Parham DM, Shapiro DN, et al. Myogenic regulatory protein (Myo D1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. Am J Pathol 1990; 137:1283-1291.