Skeletal Radiology

1 Skeletal Radiol (1992) 2] :388-391

Case report 728 J.M. Savy, M.D. 1, J.-D. Laredo, M.D. z, A. Prier, M.D. 1, M. Jagueux, M.D. 3, A. Apoil, M.D. 4, and G. Kaplan, M.D. 1 Services I de Rhumatologie, 2 de Radiologie, 3 d'Anatomo-pathologie, and 4 d'Orthop6die, H6pital Saint-Antoine, Paris, France

Imaging studies Fig. 1. A AP and B lateral films of the sacrum obtained in 1989 show a geographic osteolytie lesion of $1 (arrow) involving part of the first sacral foramen (curved arrow), with anterior cortical expansion (open arrow) Fig. 2. AP view of the sacrum obtained in 1980 demonstrates a similar appearance of the osteolytic lesion of $1, well delineated by a peripheral sclerotic rim (arrow) Fig. 3. Contrast-enhanced CT scan with bone (A) and soft tissue (B) windows demonstrates the lesion extending to involve $1 and the spinal canal. The right $1 nerve root is obliterated, while the left $1 nerve root is preserved (arrow). Some rare calcifications are present in the posterior part of the mass (arrowhead). A large central area of low density (10 HU) probably corresponds to necrotic tissue (B, ar-

row)

Address reprint requests to: Jean-Denis Laredo, M.D., Service de Radiologie, H6pital Saint-Antoine, 184rue du Fbg Saint-Antoine, Paris 75012, France 9 1992 International Skeletal Society

J.M. Savy et al. : Case report 728

389 Fig. 4. A Sagittal Tl-weighted MR scan (SE 600/30) shows a targe polycyclic lesion of intermediate signal intensity within the spinal canal and $1. The lower pole of the lesion is at the level of the SI-$2 disc (arrow) B Sagittal proton-density MR scan (1843/30) shows the mass to be inhomogeneous, exhibiting mostly intermediate to high signal intensity with zones of relatively low signal intensity at its anterior and inferior parts (arrow). C Sagittal T2-weighted MR scan (1843/100) demonstrates areas of low signal intensity on TI- and proton-density images changing to very high signal intensity on T2-weighted images, suggesting partial necrosis (arrow). D Post-gadolinium coronal MR scan shows extent of the lesion, sparing the left $1 nerve root (arrow') and partial areas of probable necrosis (open arrow). The $1 nerve root is no longer visible

Clinical information A 69-year-old w o m a n presented with a 25-year history o f recurrent sciatica in the right leg. Previous painful episodes remitted in a few days or weeks with bed rest and nonsteroidal antii n f l a m m a t o r y drugs. Since 1986, the frequency and duration of the sciatica had increased, and in M a y 1989, the patient was referred to hospital because of intense pain. Physical examination was unremarkable except for the absence o f a right ankle jerk. Radiographs revealed a purely lytic lesion o f the right part of $1, crossing the midline and extending into the ipsilateral posterior arch of L5 (Fig. 1). The lesion was well marginated with a thin cortical shell o f sclerotic bone. No in-

trinsic calcification in the lesion was seen. On a previous radiograph obtained in 1980, the lesion was already present and exhibited exactly the same appearance but was overlooked (Fig. 2). Increased uptake limited to the lesion was found on a 99Tc bone scan. L a b o r a t o r y findings and complete radiological evaluation o f the skeleton were normal. CT scan demonstrated a lyric lesion within the right part of $1, extending into the spinal canal up to L5. No calcification was present within the lesion (Fig. 3). M R I of the lumbosacral spine was performed with a 1.5-T unit, using a spin echo sequence with T1- and

T2-weighted images (Fig. 4A, B). The t u m o r had polylobar margins and involved the whole right segment of $1. The $1 right root was no longer visible. The left $1 nerve root was displaced by the lesion. On its upper pole, the lesion extended to the L 4 L5 disc space. N o presacral soft-tissue mass was present. On the T2weighted sequence, a low intense signal area located within the anterior and inferior part of the mass was defined as a necrotic area. On post-gadolinium Tl-weighted images, the signal intensity of the other parts of the lesion became m o r e intense. The results o f two trephine biopsies being inconclusive, a surgical biopsy was carried out in June 1989.

390

Diagnosis: Desmoplastic fibroma

J.M. Savy et al. : Case report 728

Pathological study

Differential diagnoses included chordoma, solitary plasmacytoma, low grade fibrosarcoma, leiomyosarcoma, chondrosarcoma, neurogenic tumors such as neurofibroma, ependymoma, and schwannoma. A white firm tissue was present under the lamina of $1. The soft tissues were intact. The tumor consisted of storiform bundles of fibroblasts, with a multinodular architecture (Fig. 5). Some cells had the appearance of myofibroblasts; however, electron microscopy was not performed. No polymorphism or mitotic activity was noted. The cells were separated by bands of collagen fibers, some of which were hyalinized. The tumor was well vascularized with some perivascular inflammatory infiltrates. No hemosiderin macrophages were present. Bone tissue exhibited normal trabecular structure but was infiltrated in some areas by tumor. The pathological diagnosis was desmoplastic fibroma with an aggressive pattern.

Discussion Desmoplastic fibroma (DF) is a rare benign neoplasm of bone, described by Jaffe in 1958 [4, 8, 9]. It may be considered as the osseous counterpart of the soft-tissue desmoid tumor. In 1985, Gebhardt et al. documented 8 personally observed cases and 77 cases in the literature [5]. Since this report, only a few additional cases have been published [1, 6, 101. D F occurs preferentially in the 2nd and 3rd decades of life, with almost 75% of cases arising before the age of 30 years [2, 5, 12, 16]. The extremes are 20 months and 75 years old, our patient being among the elderly group [3]. No sexual predilection exists. Clinical manifestations include pain and swelling of variable duration, sometimes related to a history of trauma. Pathological fracture and incidental finding of the lesion on a radiograph performed for an unrelated cause are other situations which may lead to the diagnosis. Our patient presented with nerve root compression, and it was not possible

Fig. 5. Photomicrograph shows elongated, slender, spindle-shaped cells separated by varying amounts of collagen (H & E, x 120)

to determine whether her first episode of sciatica, 25 years previously was related to the presence of the neoplasm. D F can be located in any bone, but limb bones, especially the femur and tibia, mandible, and ilium are the most common sites. In tubular bones, the lesion is usually located at the central aspect of the metaphysis. However, any part of a bone may be involved. Vertebrae are rarely affected. Only 4 cases of vertebral D F are reported in the literature [13, 14] and only I case in the sacrum

[31.

Radiographically, D F is characterized by a lytic defect which often exhibits a multicystic honeycomb appearance. It is usually well-delineated by a dense rim of sclerotic (reactive) bone. In long bones, endosteal erosions are frequent. On occasion, D F exhibits an aggressive appearance with a large degree of bone destruction, cortical erosion, and soft-tissue involvement [3, 5]. As lymphomas and fibrous tumors, desmoplastic fibroma is one of the few lesions that show characteristically low signal intensity on both

J.M. Savy et al. : Case report 728 T1 and T2-weighted M R images [7]. In our case, the tumor exhibited low to intermediate signal intensity on Tl-weighted sequence and mostly discrete high signal intensity on T2weighted sequence. Pathologically, the gross features of the tumor are those of a grayishwhite, firm or rubbery mass. At the cut surface, small cysts may be present, but no calcification. Histologically, D F is characterized by a monomorphic proliferation of small, thin, spindle fibroblasts with wavy, elongated nuclei, but without atypical cells and/or mitoses. The fibroblasts are sparse throughout the lesion and are interspersed by collagen fibers. This feature distinguishes D F from other fibroblastic tumors. Ultrastructural studies have revealed three cellular types in fibroblastic tumors: fibroblasts, myofibroblasts, and mesenchymal cells. Typical fibroblasts were the predominant type of cells in our case. Additionally, some cells could simulate myofibroblasts particularly under the optical microscope, but myofibroblasts were not formally identified, since electron microscopy was not utilized. No giant cells were present in the biopsy specimen. However, giant cells are not always found at pathological examination of desmoid fibromas [4, 11]. The presence of scattered inflammatory infiltrates is a rare finding [5, 6], first noted by Gebhardt et al. [5]. A moderate infiltrate o f lymphocytes and plasma cells was present in our case. For treatment, most authors recommend a wide resection to eradicate all of the tumor [5, 12, 13, 15]. Metastases have never been noted but local recurrence is not uncommon. In instances of vertebral lesions, complete extirpation is usually impossible. Partial excision plus arthrodesis were performed in the three published cases [13, 14]. In two cases the clinical and radiological outcome was satisfactory on long-term followup. However, the third case was corn-

391 plicated by rapid local recurrence and irreversible spinal cord damage [13]. As our patient became asymptomatic with medical treatment, surgical resection was deferred because of her age and the absence of an increase in size of the tumor in a 9-year radiographic survey and the risk of neurological sequelae. A m o n g the lytic lesions of the sacrum, c h o r d o m a is the most c o m m o n primary tumor, usually involving o f individuals in the 6th decade of life or older. Radiographically, it is located in the lower sacrum or coccyx close to the midline. The tumor has ill-defined margins and is associated with a large, presacral, soft-tissue mass. Solitary plasmacytoma may present as an expanding lesion, defined by a dense peripheral rim of new bone with a multicystic appearance. Low-grade fibrosarcoma is the most difficult lesion to differentiate from DF. Cortical destruction and periosteal reaction are c o m m o n features in fibrosarcoma. Fibrosarcoma is typically more cellular than DF, with longer, plumper, hyperchromatic cells. Mitotic activity is more pronounced. Because of the location o f the lesion and the presence of nerve root pain, neurogenic tumors may be expected. They present as nonspecific, osteolytic lesions with sclerotic margins. In s u m m a r y , a 69-year-old w o m a n with a 25-year history of recurrent sciatica presented with an expanding lytic lesion o f the right side of the first sacral vertebra. Histological examination proved the lesion to be a DF. D F is usually diagnosed in young patients and is very rarely located in the spine and sacrum. The growth rate o f the tumor was presumed to be very low because it had not progressed from radiological studies performed 9 years previously. The clinical, radiological, and pathological features of D F are described, and the differential diagnoses are discussed. The most difficult problem is

to distinguish D F from a low-grade fibrosarcoma or chordoma. References

1. Bertoni F, Calderoni P, Bacchini P, Campanacci M (1984) Desmoplastic fibroma of bone. A report of 6 cases. J Bone Joint Surg [B] 66 : 265 2. Chan KW, Pun WK, Choi CH (1987) Desmoplastic fibroma of bone. Pathology 19:201 3. Crim JR, Gold RH, Mirra JM, Eckardt JJ, Basset LW (1989) Desmoplastic fibroma of bone. Radiographic analysis. Radiology 172:827 4. Dahlin DC (1973) Bone tumors, 3rd edn. Charles C. Thomas, Springfield 5. Gebhardt M, Campbell CS, Schiller AL, Mankin HJ (1985) Desmoplastic fibroma of bone. J Bone Joint Surg [A] 67 : 732 6. Hadjipavlou A, Lander PIt, Begin LR, Eibel P (1986) Desmoplastic fibroma of a metatarsal. J Bone Joint Surg [A] 68 : 459 7. Hamlin D J, Paige R, Petterson H et al. (1986) Magnetic resonance characteristics of an abdominal desmoid tumor. Comput Radiol 10:11 8. Jaffe HL (1958) Tumors and tumorous conditions of the bones and joints. Lea and Febiger, Philadelphia 9. Jouvin MH, Tomeno B, Carlioz A, Forest M, Abelanet R (1984) Fibrome desmo'ide des os longs. Revue de la litterature et probl~mes diagnostiques. Semin Hop Paris 60:2875 10. Lichtman EA, Klein MJ (1985) Case report. Desmoplastic fibroma of the proximal end of the left femur. Skeletal Radiol 13:160 1l. Mirra JM (1980) Bone tumors: diagnosis and treatment. Lippincott, Philadelphia 12. Nilsonne U, G6thlin G (1969) Desmoplastic fibroma of bone. Acta Orthop Scand 40:205 13. Rabhan WN, Rosal J (t 962) Desmoplastie fibroma. Report of ten cases and review of the literature. J Bone Joint Surg [A] 50:487 14. Scheer GE, Kuhlman RE (1963) Vertebral involvement by desmoplastic fibroma. Report of a case JAMA 185: 669 15. Schultz E, Hermann G, Irwin GA, Shih H (1986) Desmoplastic fibroma of the mandible. Case report 380. Skeletal Radiol 15 : 560 16. Sugiura I (1976) Desmoplastic fibroma. Case report and review of the literature. J Bone Joint Surg [A] 58 : 126

Case report 728: Desmoplastic fibroma.

A 69-year-old woman with a 25-year history of recurrent sciatica presented with an expanding lytic lesion of the right side of the first sacral verteb...
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