Introduction: The plexiform fibrohistiocytic tumor is rare, typical for children and adolescents, affects more often the female sex and is most often localized in the upper limb—the region of the wrist joint and the hand. The tumor originates from the subcutaneous adipose tissue, but it is possible to involve the dermis and skeletal muscle. It is characterized by slow growth and sizes between 0.5 and 8 cm, with minimal or no cellular atypism. Three histological varieties have been described: fibrohistiocytic, fibroblastic, and mixed type, which occur with almost equal frequency.
Aim: The aim of this article is to present an extremely rare pathohistologically large tumor atypical for the patient’s gender, location and age, and address the surgical challenges of its removal.
Patients and Methods: The presented patient is a 54-year-old man with a giant tumor formation involving the anteromedial part of the proximal lower leg, with evolution of over 25 years, and an accompanying disease—cerebral palsy.
Result: After en bloc excision of the tumor formation, part of the surgical wound healed secondary. No recurrences were recorded.
Conclusion: The pathohistological nature of the tumor is a prerequisite for a complete cure, but the removal of such giant soft tissue formations from the proximal and medial side of the lower leg is a real surgical challenge. The exposed large area of the tibia, with no soft tissue and periosteum, is difficult to cover by local skin plastic surgery, and at the same time it is extremely unsuitable for free plastic procedure.
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