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Perivascular epithelioid cell tumour (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, with few reports of paediatric cases, and there is no standardized treatment for the tumour type. Here, the case of a 17-year-old female, who presented with painless haematochezia for 2 days and was diagnosed with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis after serial examination, is presented. She was treated by surgical resection of the tumour and cytotoxic chemotherapy comprising 900 mg/m2 gemcitabine and 100 mg/m2 docetaxel every 3 weeks for six cycles. Haematochezia did not recur, and complete response was achieved, with progression-free survival at the 24-month follow-up examination. Surgical resection with adjuvant conventional cytotoxic chemotherapy may be considered as an option for treating gastrointestinal PEComa.

Background Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, and few pediatric cases have been reported. However, standardized treatment for the tumor type remains unestablished. Herein, we report a case of a pediatric patient with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. The patient was successfully treated with complete tumor resection and gemcitabine/docetaxel combination chemotherapy without local or distant recurrence after 12 months postsurgery.Case presentation A 17-year-old female adolescent presented with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. She was treated with surgical resection of the tumor and cytotoxic chemotherapy with gemcitabine (900 mg/m2) and docetaxel (100 mg/m2) every 3 weeks. There was no recurrence of hematochezia stool, and complete response was achieved, with a progression-free survival of 12 months.Conclusion Surgical resection with adjuvant conventional cytotoxic chemotherapy can be considered as the first-line treatment for early-stage gastrointestinal PEComa.