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Background. Klippel – Trenone syndrome (KTS) is a rare genetic disease that occurs with a frequency of 1 per 100,000 people and is characterized by a typical triad of symptoms: malformation of the capillary network (like a “wine stain”), hypertrophy of soft tissues and bones, and venous malformations of the extremities. Adenocarcinoma is a malignant formation of the glandular epithelium. This disease is extremely rare in children (about 1 case per 1 million people under the age of 20 years annually) due to its large regenerative potential and the absence of comorbid conditions that accumulate with age. In the article, we presented a case demonstrating a combination of these rare and severe diseases in a child. Case Report . We present a case report of the manifestation of adenocarcinoma of the transverse colon with the development of an acute intestinal obstruction clinic in a child with KTS. Conclusion . This case report demonstrates the combination of KTS and moderately malignant transverse colon adenocarcinoma in a child. This case is unique and valuable for clinical practice, emphasizing the need for thorough examination of patients with KTS due to the risk of cancer due to mutations in the PIK3CA oncogene. The combination of KTS with aggressive carcinoma leads to the development of complex comorbid pathology. The mutual aggravation of these diseases critically limits the possibilities of therapeutic correction and, as a result, dramatically reduces the expected survival rate of such patients.
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Gabueva et al. (2026) studied this question.
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