Dados do Trabalho

Título

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

Introdução

The present report aimed to highlight a case with a modified evolution due to the immunohistochemical examination of a rare tumor, resulting in a change of treatment and clinical control of the patient. Intrabdominal synovial sarcoma manifested as abnormal uterine bleeding in infants is quite rare, with few reports in the literature, and should be considered as differential diagnosis specially in those with poor therapeutic response.

Objetivo

To report a case of a rare abdominal neoplasm with uterine manifestations in a pediatric patient.

Casuística

Pediatric patient admitted to pediatric oncology bed.

Método

Review of electronic medical records, laboratory and imaging tests; narrative review of international literature.

Resultados

Case presentation: Patient N.L., 1 year and 2 months old, is admitted to a ward from a secondary hospital on June, 2022, with a history of intermittent vaginal bleeding for about 5 days, heavy flow, associated with prostration, and inappetence. The patient was previously healthy, without continuous medication, surgeries, or previous hospitaizations. Upper Abdominal Magnetic Resonance on June 8 revelaed an expansive formation in the pelvis, posterior to the badder and uterus, measuring 6.5 x 4.9 x 8.0 cm, suggesting the possibiity of a neoplastic lesion type tumor of germ cells. Exporatory 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 pevic lesion characterized positive PAX8 markers B2/MRQ50, strong positive TLE-1 B2/3 1F5 markers, and focal positive CKAE/AEB2/AE/AE markers, with negative rhabdomyosarcoma markers, establishing a diagnosis of synovial sarcoma. Given the resut, the chemotherapy regimen was changed to a protocol with doxorubicin and ifosfamide, taking into account the poor clinica response to the previous regimen and the aggressiveness of the synovial sarcoma.

Synovial sarcoma is a rare subtype of neoplasm that accounts for about 50% of soft tissue sarcomas, more common in men aged 55 years and affecting joint extremities, mainy in the lower limbs. In the case presented, the neoplastic presentation was atypical manifesting as abnormal vagina bleeding in an infant patient. The location of the lesion was also uncommon, with ony 50% of synovial sarcomas affecting head and neck anatomy, mediastinum, abdominal wall, esophagus, or retroperitoneum, with a very unique pevic instalation. The anatomopathological study is difficut, and the diagnosis is based on immunohistochemical analysis, which made it possibe to optimize the treatment of the patient on screen

Conclusões

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

Palavras-chave

Synovial sarcoma. Botryoid sarcoma. Abnormal uterine bleeding. Pediatrics. Rare tumours.

Fotos e tabelas

 

 

Conflitos de interesse

No conflicts of interest.

Área

Tumores raros