Case Report: Malignant colonic PEComa presenting as fever of unknown origin with spontaneous perforation.

BACKGROUND

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with variable malignant potential, and gastrointestinal involvement is particularly uncommon. Colonic PEComas are exceedingly rare and may present with nonspecific symptoms, complicating early diagnosis.

CASE PRESENTATION: A 58-year-old male presented with prolonged fever, fatigue, and weight loss. Laboratory findings revealed leukocytosis and anemia, while infectious and rheumatologic evaluations were unremarkable. Following rectal bleeding, colonoscopy identified a perforated lesion in the sigmoid colon. Imaging demonstrated extensive tumoral wall thickening, and the patient underwent emergency surgery due to acute abdomen. Histopathological and immunohistochemical analysis confirmed malignant PEComa. The patient received adjuvant chemotherapy and radiotherapy and remained disease-free at 5-year follow-up.

CONCLUSION

This case highlights an unusual presentation of colonic PEComa with fever of unknown origin and spontaneous perforation. Clinicians should consider rare malignancies in patients with unexplained inflammatory syndromes, particularly when accompanied by gastrointestinal findings.