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Intra-abdominal Synovial Sarcoma: Excentric manifestation of a rare entity

Resumo

Case presentation: Patient N.L., 1 year and 2 months old, is admitted to a ward from a secondary hospital on June 6, 2022, with a history of intermittent vaginal bleeding for about 15 days, heavy flow, associated with prostration, and inappetence. The patient was previously healthy, without continuous medication, surgeries, or previous hospitalizations. Upper Abdominal Magnetic Resonance on June 8 revealed an expansive formation in the pelvis, posterior to the bladder and uterus, measuring 6.5 x 4.9 x 8.0 cm, suggesting the possibility of a neoplastic lesion type tumor of germ cells. Exploratory laparotomy on June 10 revealed a ruptured, inoperable tumor mass in the pelvis; an intraoperative frozen section was performed, which was compatible with botryoid sarcoma. An emergency chemotherapy regimen with irinotecan and vincristine was started in the ICU. On June 26, immunohistochemical examination of pelvic lesion characterized positive PAX8 markers B2/3 MRQ-50, strong positive TLE-1 B2/3 1F5 markers, and focal positive CKAE1/AE3 B2/3 AE1/AE3 markers, with negative rhabdomyosarcoma markers, establishing a diagnosis of synovial sarcoma. Given the result, the chemotherapy regimen was changed to a protocol with doxorubicin and ifosfamide, taking into account the poor clinical response to the previous regimen and the aggressiveness of the synovial sarcoma.

Case discussion: Synovial sarcoma is a rare subtype of neoplasm that accounts for about 5-10% of soft tissue sarcomas, more common in men aged 15-35 years and affecting joint extremities, mainly in the lower limbs. In the case presented, the neoplastic presentation was atypical, manifesting as abnormal vaginal bleeding in an infant patient. The location of the lesion was also uncommon, with only 5-10% of synovial sarcomas affecting head and neck anatomy, mediastinum, abdominal wall, esophagus, or retroperitoneum, with a very unique pelvic installation. The anatomopathological study is difficult, and the diagnosis is based on immunohistochemical analysis, which made it possible to optimize the treatment of the patient on screen.

Final Comments: Intra abdominal synovial sarcoma of the pelvis is a rare neoplastic entity in children, with challenging differential diagnoses, especially when presented with atypical symptoms and location. An immunohistochemical examination is essential for therapeutic evaluation, and can significantly change the patient's treatment plan.

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