Bilgehan TOSUN, Tuncay BARAN, Hakan SARMAN

Elde Alveolar Rabdomyosarkom: Yumuşak Doku Sarkomlarinda Oldukça Nadir Bir Olgu

Ekstremitelerin yumuşak doku sarkomları çok nadir ve oldukça kötü bir prognoza sahiptir.Rabdomyosarkomlar yumuşak doku sarkomu olup, erişkin yaşta nadir olarak görülürler, dahasıklıkla çocukluk ve ergenlik dönemi tümörleridir. Ekstremite tutulumu rabdomyosarkomlariçin alışılmadık bölgeler olup ve genellikle kötü sonuçlarla ilişkilidir. Sağ kalım açısından majorfaktör tümörün cerrahi olarak tamamen çıkarılmasıdır.Vakamız 22 yaşında erkek bir hastanın sol elinde çıkan primer alveolar rabdomyosarkomununinkomplet tümör eksizyonu sonrasında 5. ray ampütasyon ile tedavisini anlatılmaktadır. Hastanın ameliyat sonrası 4 yıllık takiplerinde tümör dokusu saptanmamıştır

Anahtar Kelimeler:

El, hipotenar, tumor, sarkom, rabdomyosarkom, amputasyon

ALVEOLAR RHABDOMYOSARCOMA OF THE HAND: A VERY RARE CASE OF SOFT TISSUE SARCOMA

Soft tissue sarcomas of extremities are very rare and generally have a bad prognosis.Rhabdomyosarcomas are soft tissue sarcomas that, while extremely rare in adults, are one ofthe most common neoplasms in children and adolescents. Extremities are unusual sites of originfor rhabdomyosarcomas and usually associated with unfavorable outcome. Complete surgicalremoval of the tumor represents a major factor in terms of survival.Our study presents the case of primary alveolar rhabdomyosarcoma of the left hand in a 22 yearsold male who was treated by fifth ray amputation after an incomplete tumor excision. The patient is still tumor-free after 4 years following operation

Keywords:

Hand, hypothenar, tumor, sarcoma, rhabdomyosarcoma, amputation,

PDF

___

Rosen PP. Rosen’s Breast Pathology, 2nd edition. Philadelphia: Lippincott Williams & Wilkins, 2001. p:823.

Mahajan A. The contemporary role of the use of radiation therapy in the management of sarcoma. Surg Oncol Clin N Am 2000; 9(3): 503-524.

Pappo AS, Shapiro DN, Crist WM. Rhabdomyosarcoma. Biology and treatment. Pediatr Clin N Am 1997; 44(4): 953- 972.

Wexler LH. Rhabdomyosarcoma and the undifferentiated sarcomas. In Pizzo PA, Poplack DC, eds. Principles and Practice of Pediatric Oncology 5th ed., Philadelphia: Lippincott Williams & Wilkins; 2006. p:971–1032.

Tan GC, Shiran MS, Hayati AR, et al. Alveolar Rhabdomyosarcoma of the left hand with bilateral breast metasteses in an adolescent female. J. Chin. Med. Assoc. 2008; 71(12):639-642.

Siegel H.J, Connor G.S, Lee D, et al. Synchronous bifocal alveolar rhabdomyosarcoma. Journal of Bone and Surgery. 2006; 88(7);955-958.

Gross E, Rao BN, Pappo AS, et al. Soft Tissue Sarcoma of the hand in Children: Clinical Outcome and Managment. Journal of Pediatric Surgery.1997; 32(5):698-702.

Casanova M, Cristina M, Favini F, et al. Rhabdomyosarcoma of the extremities: a focus on tumors arising in the hand and foot. Pediatric Hematology and Oncology. 2009; 26(5):321- 331.

Miller DV, Coffin CM, Zhou H. Rhabdomyosarcoma Arising in the Hand or Foot: A Clinicopathologic Analysis. Pediatric and Developmental Pathology. 2004; 7(4):361-369.

Murray PM. Soft tissue sarcoma of the upper extremity. Hand Clin. 2004; 20(3):325-333.