Massive Desmoplastic Fibroma of the Proximal Tibia: Case Report

Desmoplastic fibroma of bone is a very uncommon, benign but locally aggressive fibrogenic tumor. This report describes the case of a 45-year-old patient with a massive desmoplastic fibroma of the proximal tibia. A two-staged surgical procedure was successfully performed: wide resection and endoprosthetic reconstruction. Surgeons should be aware of the complexity of its treatment in the locally advanced and aggressive cases. A comprehensive review of the literature is also provided.

Keywords
bone neoplasms; case report; fibroma, desmoplastic; orthopedic; tibia

Authorship

Oriol Pujol Address for correspondence Oriol Pujol, MD, Orthopedic Surgery Department, Vall d'Hebron University Hospital, Universitat Autónoma de Barcelona, Pg. Vall d'Hebron 119-129, 08035 Barcelona, Spain (e-mail: oriolp-6@hotmail.com).

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

The first draft of the manuscript

Orthopaedic Oncology Unit, Orthopedic Surgery Department, Vall d'Hebron University Hospital, Barcelona, Spain. Universitat Autónoma de Barcelona. Barcelona, SpainUniversitat Autónoma de BarcelonaSpainBarcelona, SpainOrthopaedic Oncology Unit, Orthopedic Surgery Department, Vall d'Hebron University Hospital, Barcelona, Spain. Universitat Autónoma de Barcelona. Barcelona, Spain

https://orcid.org/0000-0003-4760-2730

Sara Castellanos

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

https://orcid.org/0000-0003-0797-8303

María G. Carrasco

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

https://orcid.org/0000-0003-4323-0685

Alejandro Garzón

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

Anatomopathological Department, Vall d'Hebron University Hospital, Barcelona, Spain. Universitat Autónoma de Barcelona. Barcelona, SpainUniversitat Autónoma de BarcelonaSpainBarcelona, SpainAnatomopathological Department, Vall d'Hebron University Hospital, Barcelona, Spain. Universitat Autónoma de Barcelona. Barcelona, Spain

Cleofe Romagosa

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

https://orcid.org/0000-0002-9478-7320

Roberto Vélez

contributed equally to this work

contributed to the study conception and design

material preparation

data collection and analysis

commented on previous versions of the manuscript

read and approved the final manuscript

https://orcid.org/0000-0001-8917-6050

Address for correspondence Oriol Pujol, MD, Orthopedic Surgery Department, Vall d'Hebron University Hospital, Universitat Autónoma de Barcelona, Pg. Vall d'Hebron 119-129, 08035 Barcelona, Spain (e-mail: oriolp-6@hotmail.com).

Conflict of Interests

The authors have no conflict of interests to declare.

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Figures

Figures (4)

Thumbnail

Fig. 1
Preoperative images of the patient. A) The clinical photograph shows an evident knee deformity, with a hard palpable medial mass measuring 10cm. B and C) Radiographic evaluation shows a massive lytic tumor in the proximal tibia. The lesion presented an aggressive growth with bone destruction, trabeculation and extension into the fibula and the surrounding soft tissue. D, E and F) MRI images demonstrated a 13 × 15 × 13 cm mass (AP × ML × CC) with significant soft tissue involvement and displacement of neurovascular structures (white arrow). Heterogenous contrast enhancement can be observed.

Thumbnail

Fig. 2
Images of the first surgical stage. A) The intraoperative photograph shows the tumour wide margin resection. The neurovascular bundle was identified, dissected and protected. A relevant dead space can be observed. B, C and D) The intraoperative photograph and the postoperative x-ray images show the temporary arthrodesis performed with two intramedullary nails (anterograde tibial and retrograde femoral). Antibiotic loaded cement was used to increase the construct stability, obliterate the dead space and locally deliver antibiotic.

Thumbnail

Fig. 3
Images of the anatomopathological study of the surgical specimen. Negative margins were demonstrated. No necrosis was observed and only two mitoses per 10-HPF were counted. Immunohistochemically, the tumor cells were positive for MDM2 and negative for sm-actin, desmin, beta-catenin, S100, CD34. FISH for MDM2 showed a polysomy, but not amplification. A) Macroscopic image of the sectioned surgical specimen showing the tibia with a white lobulated tumor that extends to soft tissues with hemorrhagic areas. B) Infiltration of the lamellar bone by the neoplasm. Image at 50x of H&E. C) Hypocellular proliferation of spindle cells with bland nuclear features organized in a longitudinal fascicular pattern on a collagenized stroma. Image at 400x of H&E. D) Few areas showing mildly atypical cells arranged in a disordered pattern. Image at 400x of H&E.

Thumbnail

Fig. 4
Final postoperative images. A) Knee extension, B) Knee flexion C) Full plantar flexion D) Dorsal flexion deficit ([C0]40°). E and F) Postoperative full-length and lateral x-ray images show a correct implantation of the endoprosthetic replacement.

Fig. 1 Preoperative images of the patient. A) The clinical photograph shows an evident knee deformity, with a hard palpable medial mass measuring 10cm. B and C) Radiographic evaluation shows a massive lytic tumor in the proximal tibia. The lesion presented an aggressive growth with bone destruction, trabeculation and extension into the fibula and the surrounding soft tissue. D, E and F) MRI images demonstrated a 13 × 15 × 13 cm mass (AP × ML × CC) with significant soft tissue involvement and displacement of neurovascular structures (white arrow). Heterogenous contrast enhancement can be observed.

Fig. 2 Images of the first surgical stage. A) The intraoperative photograph shows the tumour wide margin resection. The neurovascular bundle was identified, dissected and protected. A relevant dead space can be observed. B, C and D) The intraoperative photograph and the postoperative x-ray images show the temporary arthrodesis performed with two intramedullary nails (anterograde tibial and retrograde femoral). Antibiotic loaded cement was used to increase the construct stability, obliterate the dead space and locally deliver antibiotic.

Fig. 3 Images of the anatomopathological study of the surgical specimen. Negative margins were demonstrated. No necrosis was observed and only two mitoses per 10-HPF were counted. Immunohistochemically, the tumor cells were positive for MDM2 and negative for sm-actin, desmin, beta-catenin, S100, CD34. FISH for MDM2 showed a polysomy, but not amplification. A) Macroscopic image of the sectioned surgical specimen showing the tibia with a white lobulated tumor that extends to soft tissues with hemorrhagic areas. B) Infiltration of the lamellar bone by the neoplasm. Image at 50x of H&E. C) Hypocellular proliferation of spindle cells with bland nuclear features organized in a longitudinal fascicular pattern on a collagenized stroma. Image at 400x of H&E. D) Few areas showing mildly atypical cells arranged in a disordered pattern. Image at 400x of H&E.

Fig. 4 Final postoperative images. A) Knee extension, B) Knee flexion C) Full plantar flexion D) Dorsal flexion deficit ([C0]40°). E and F) Postoperative full-length and lateral x-ray images show a correct implantation of the endoprosthetic replacement.

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Massive Desmoplastic Fibroma of the Proximal Tibia: Case Report

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[1] Address for correspondence Oriol Pujol, MD, Orthopedic Surgery Department, Vall d'Hebron University Hospital, Universitat Autónoma de Barcelona, Pg. Vall d'Hebron 119-129, 08035 Barcelona, Spain (e-mail: oriolp-6@hotmail.com).