Tinoco TL, Aguirre TJ, Domínguez GLG, Domínguez CLG

Language: Spanish
References: 11
Page: 230-234
PDF size: 252.70 Kb.

ABSTRACT

Background: The low-grade fibromyxoid sarcoma is a rare tumor, described by Evans in 1987 as having a seemingly benign clinical course and histology, but local and distant aggressive behavior; it corresponds to 0.7% of all soft tissue tumors. Case report: Forty-nine-year-old female athlete, with a three-month history of pain in the middle left buttock during the race; exploration: 5 x 4 cm, firm mass in the gluteus medius. She underwent resection, with a diagnosis of low histologic grade fibromyxoid sarcoma with positivity only to vimentin. She was subjected to radiotherapy and 10 cycles of chemotherapy. The thoracic tomography was normal at 12 months; at 17 months, PET/CT showed multiple metastatic disease. At present (two years after the diagnosis), she is receiving palliative management and performing moderate exercise. Conclusions: Low-grade fibromyxoid sarcoma is a rare tumor and may mimic other soft tissue tumors; recurrences and metastases are described in up to 68% and 40% of cases, respectively.

REFERENCES

1. Evans HL. Low-grade fibromyxoid sarcoma. A report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clin Pathol. 1987; 88: 615-619.

2. Folpe AL, Lane KL, Paull G, Weiss SW. Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol. 2000; 24: 1353-1360.

3. Folpe A, Van den Berg E, Molenaar WM. Low-grade fibromyxoid tumor sarcoma. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. In: Fletcher CD, Unni KK, Mertens F, editor. Low grade fibromyxoid sarcoma. Lyon AND IARC Press; 2002. pp. 104-105.

4. Wu X, Petrovic V, Torode IP, Chow CW. Low-grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance. Pathology. 2009; 41: 155-160.

5. Rose B, Tamvakopoulos GS, Dulay K, Pollock R, Skinner J, Briggs T et al. The clinical significance of the FUS-CREB3L2 translocation in low-grade fibromyxoid sarcoma. J Orthop Surg Res. 2011; 6: 15-17.

6. Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD. [Internet] Available in: http://seer.cancer.gov/csr/1975_2011/

7. Scoggins CR, Pisters PW. Diagnosis and management of soft tissue sarcomas. Adv Surg. 2008; 42: 219-228.

8. Miyake M, Tteishi U, Maeda T, Arai Y, Seki K, Hasegawa T et al. CT and MRI features of low-grade fibromyxoid sarcoma in the shoulder of a pediatric patient. Radiat Med. 2006; 24: 511-514.

9. Citores PM, Tinoco CC, Arenal-VJ, Fernández CB, Torres NM, Zamora MT. Sarcoma fibromixoide de bajo grado: a propósito de 3 casos y revisión de la bibliografía. Cir Cir. 2013; 81: 333-339.

10. Wardelmann E, Chemnitz JM, Wendtner CM. Targeted therapy of soft tissue sarcomas. Onkologie. 2012; 35 (Suppl 1): 21-27.

11. Ludwig JA. Personalized therapy of sarcomas: integration of biomarkers for improved diagnosis, prognosis, and therapy selection. Curr Oncol Rep. 2008; 10: 329-337.