RETROPERITONEAL SOLITARY FIBROUS TUMOR - CASE REPORT AND LITERATURE REVIEW

Case Report: Male, 73 years old, complaining of constipation and dysuria for 10 months, associated with discomfort in the hypogastrium and loss of 3% of body weight. In the physical exam, he presented a massive, hardened mass fixed in the lower abdominal wall. In the rectal touch, it could be detected a bulg in the left anterior and lateral walls, extrinsic to the rectum, starting 3 cm from the anal margin with 6cm of extension. Magnetic resonance imaging showed a well-vascularized lesion of 17 cm, which was anteriorly pushing the rectosigmoid and bladder, in addition to several oval masses with signal intensity and heterogeneous enhancement. The patient underwent a percutaneous radio-intervention biopsy with pathology and immunohistochemistry showing low-grade spindle cell proliferation with hemangiopericytic pattern and CD 34 +, CD 99, BCL 2+, concluding as Solitary Fibrous Tumor (SFT). Due to the extension of the lesion, in which the suggested surgery would be a total pelvic exanteration, it was chosen to initiate neoadjuvant radiotherapy, with similar dose for sarcoma. However, the tumor did not regress, although the patient remains asymptomatic. At this time, he refuses surgical treatment, remaining in outpatient follow-up. Discussion: SFT is a mesenchymal spindle cell neoplasm, first recorded in 1931. It has no gender preference, nor does it have environmental risk factors and affects adults more often between sixty and seventy years of age. The most common location is intrathoracic, followed by intra-abdominal, and the rare retroperitoneal, with less than 100 related cases. It has an average size between 7 and 10 cm and can vary from 1 to 40 cm, producing symptoms due to mass in other organs. The diagnosis is historical with presence of spindle cells and prominent vascularization, aided by immunohistochemistry (CD 34 expressed in 79% of cases). Most SFT are benign, only 10% to 20% of tumors have malignant behavior, which have high recurrence (63%). Treatment and follow-up are still not well defined, following, in most cases, the guidelines for soft part sarcomas. Final considerations:The scientific relevance of this report lies in the rarity of the case presented, especially in the malignancy and infrequent location. And, consequently, the lack of publications and guidelines related to the treatment and follow-up of the exposed theme, contributing to portray the lack of response of neoadjuvant radiotherapy.

 

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Artikel online veröffentlicht:
23. Oktober 2019