Dados do Trabalho

Título

Cervical PEComa: Navigating the Challenges of a Rare Cancer Diagnosis, an Updated Review

Introdução

PEComas arise from perivascular epithelioid cells (PECs), which are cells located around small blood vessels in various organs throughout the body. It can occur in many parts of the body, including uterus. They are classified based on their location and characteristics: renal angiomyolipoma (most common, arising in the kidney), pulmonary lymphangioleiomyomatosis (affecting the lungs), malignant PEComa (less common, aggressive form). Therefore, the wide-ranging characteristics of PEComa pose significant challenges for screening and monitoring this tumor, coupled with limited scientific evidence in the literature.

Objetivo

This study delves into the intricate challenges associated with the screening and diagnosis of PEComa, a rare mesenchymal neoplasm.

Casuística

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Método

Due to the rarity of PEComa and the scarcity of literature on this topic, a comprehensive review of eleven relevant articles was conducted. Search terms employed included "PEComa," "Uterine Cervical Neoplasms," and "Rare Neoplasm."

Resultados

The average age at diagnosis was 44 years, with the most common symptoms being irregular vaginal bleeding, abdominal pain, and palpable masses. The absence of a specific tumor marker limits detection in cervical cytology, while preoperative imaging often mimics leiomyomas or polyps on ultrasound and computed tomography (CT) scans. Tumor size, in most malignant cases, ranged from 6 to 12 cm, while benign tumors were smaller than 4 cm. While there are no established protocols for sentinel lymph node evaluation in malignant PEComa, pelvic lymph node assessment may be considered as a diagnostic adjunct. Additionally, cone biopsy in conjunction with extrafascial hysterectomy may be considered for benign tumors smaller than 4 cm.

Conclusões

In essence, the study highlights the pressing need for robust evidence-based diagnostic tools to effectively screen for PEComa. Furthermore, it underscores the urgent requirement for interventions to mitigate the risk of metastasis. The scarcity of research on this rare cancer leaves many questions unanswered, including potential associations with factors contributing to uterine tumor development. In the future, molecular profiling and immunohistochemical staining may provide valuable insights for tumor subtyping and prognosis assessment and future studies should focus on identifying novel diagnostic biomarkers and developing targeted therapies for PEComa.

Palavras Chave

"PERComa"; "Cervical Intraepithelial Neoplasms"; "Rare Neoplasm"