A case of caecal "PECOMA": An uncommon entity

Area of Science:

• Oncology
• Gastroenterology
• Pathology

Background:

• Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms defined by the World Health Organization (WHO).
• PEComas exhibit distinctive perivascular epithelioid cell morphology and immunohistochemical features, including positivity for melanocytic and smooth muscle markers.
• Clinical presentation of PEComas is often nonspecific, leading to diagnostic challenges.

Purpose of the Study:

• To report a rare case of PEComa incidentally discovered in the cecum.
• To discuss the diagnostic difficulties and management of PEComas in the gastrointestinal tract.

Main Methods:

• A routine colonoscopy revealed a submucosal cecal tumor in an asymptomatic patient.
• Diagnostic workup showed all other parameters within normal limits.
• Surgical resection (Laparoscopic Ileocecectomy) was performed, followed by histopathological analysis.

Main Results:

• Histopathology confirmed the diagnosis of Pecomatosis (PEComa).
• The tumor was characterized by perivascular epithelioid cell differentiation.
• The patient was asymptomatic, with the tumor detected incidentally.

Conclusions:

• PEComas are rare in the gastrointestinal tract, particularly in the cecum.
• Surgical resection is the primary treatment modality for PEComas.
• Close patient follow-up is crucial due to the potential for recurrence and metastasis.