Lipofibromatosis is an extremely rare benign pediatric soft tissue tumour, characterized by slow and painless growth, with a predilection for distal extremities, a poor margination and a high rate of local recurrence. The classic histology consists of abundant adipose tissue traversed by bundles of spindled fibroblastic cells.
We present the case of a 25-year-old female patient with a retroclavicular growing lipofibromatosis. The expansion caused recurrent paresthesia in the left hand. Magnetic resonance imaging of the affected cervico-thoracal area showed a 4.6 × 4.3 × 6.4 cm encapsulated lesion, reaching up to the supraspinatus muscle. An incisional biopsy was done for diagnostic reasons, leading to the diagnosis of lipofibromatosis. As a consequence, the tumour was resected marginally. During a follow-up period of 48 months, no local recurrence was detected.
In the past, lipofibromatosis was interpreted as infantile or juvenile fibromatosis, fibrous hamartoma or fibrosing lipoblastoma, which may explain the rarity of this entity. To the best of our knowledge, this is the first report of a non-pediatric case of lipofibromatosis. In conclusion, the entity of lipofibromatosis should be kept in mind as a potential differential diagnosis for fibrofatty tumours in children and young adults.
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Friesenbichler, J., Leithner, A., Beham, A. et al. Retroclavicular lipofibromatosis: case report and review of the literature. Eur Orthop Traumatol 1, 115–118 (2010). https://doi.org/10.1007/s12570-010-0025-7
- Magnetic resonance imaging
- Soft tissue neoplasms
- Adipose tissue