Tag Archives: benign tumor

Desmoplastic fibroma of bone: Case report and review of the literature

by Zachary M. Thomas, DPM, AACFAS,CWSP1,  Matthew J. Sabo, DPM, FACFAS2pdflrg

The Foot and Ankle Online Journal 8 (2): 3

This is a case report on an unusual presentation of desmoplastic fibroma of bone. We feel that this presentation should be documented and reviewed in comparison to the available literature, as this tumor is seldom documented in the foot. We review the differential diagnosis, pertinent imagine findings, and surgical management of this pathology.

Key words: desmoplastic fibroma, benign tumor, bone tumor

ISSN 1941-6806
doi: 10.3827/faoj.2015.0802.0003

Corresponding author: Zachary Thomas, DPM
zachthomasdpm@gmail.com

1 Academy of Podiatry, Pittsburgh, PA
2 Foot and Ankle Wellness Center of Western Pennsylvania, Ford City, PA


Desmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence [1]. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. The tissue is described as being poorly cellular with nuclei that are ovoid or elongated. The incidence of whole-body desmoplastic fibroma is less than 0.1% [1,2]. The most common presenting symptom is pain and/or swelling, although pathologic fracture has been described previously as a common reason for seeking treatment. This tumor has also presented as an incidental finding [2]. The most common differential diagnosis radiographically is fibrosarcoma, however a wide variety of entities can masquerade as a desmoplastic fibroma including fibrous dysplasia, giant cell tumor, unicameral bone cyst, chondromyxoid fibroma, nonossifying fibroma, periosteal desmoids, eosinophilic granuloma, low-grade intraosseous osteosarcoma, adamantinoma, as well as distant metastasis [1-4].

This tumor has been described in patients ranging from 15 months to 75 years of age, and it is most commonly seen in the mandible, femur, pelvis, radius, and tibia [4].

Roentgenograms typically reveal an oval, long axis oriented lytic, expansile lesion with a lucent matrix. The cortex of the involved bone appears thinned and may be violated in more advanced cases [2-4] (Figures 1,2). MRI features include inhomogeneous low signal intensity on T1 weighted images and mixed signal intensity on T2 weighted images. Post-contrast T1 images show irregular, non-enhancing areas incased within an enhanced mass. The areas of low signal intensity on T2 and post-contrast T1 correlate with abundant collagen fibers. The high signal intensity areas on T2 and post-contrast T1 were correlated with fibroblasts and loose collagen fibers [5,6].

Case Report

A 75-year-old male patient presents with the complaint of pain in the first metatarsal region. He states he had been having pain, which has been getting progressively worse for the past several months. On physical exam, there was edema in the right first ray region extending to the proximal foot and into the right leg.  There was exquisite pain to palpation dorsally along the entire course of the first metatarsal.

fig1

Figure 1 Lateral Pre-op x-ray of desmoplastic fibroma.

fig2

Figure 2 AP/MO standard radiographic views pre-operatively.

fig3

Figure 3 Intraoperative view of resected tumor through cortical window.

A visible deformity and limited, painful range of motion of the 1st MTPJ was noted. Clinical examination was consistent with hallux abductovalgus with associated dorsal exostosis. Radiographs displayed an expansile, lytic lesion at the distal diaphysis extending in to the metaphysis/epiphysis. MRI showed a small cortical break in the distal segment of the lesion. Our main concern at the time of discovery was aggressiveness of this lesion. Bone scan and chest x-ray revealed no other lesions in the body. At this point, biopsy was elected to determine the nature and aggressiveness of the lesion.

Intraoperatively, a linear incision was made along the first metatarsal and deepened to the deep fascial layer where a capsular-periosteal incision was made. There was gross deformity of the first metatarsal head as well as deformity extending to the midshaft of the aforementioned bone. The specimen measured 1.5 cm X 1.2 cm X 0.3 cm. The most proximal aspect of the first metatarsal base was the only unaffected portion of the bone. The mass was removed by excising a rectangular window with the tumor housed inside. The tumor was very soft and, upon removal, it was made certain to excise normal margins on all sides of the tumor (Figure 3).  The mass was sent to the pathologist intraoperatively where it was confirmed a benign mass. At this point, the remaining void was curetted to ensure adequate removal of tumor, flushed, and finally packed with a mixture of putty and cancellous bone chips. The dorsal cortical window was replaced and the wound was closed in layers. The pathology report returned with the diagnosis of desmoplastic fibroma. The characteristics of this particular tumor were a bland spindle cell appearance and lack of woven bone formation. The lesion was, however, negative for beta-catenin.

At 14 days post-operatively, the patient’s incision site was well coapted and sutures were removed. At 4 months post-operatively, the patient’s graft site was fully consolidated, and at 10 months post-operatively the patient was walking with no pain. Radiographs did not display any signs of recurrence (Figures 4,5,6).  At 22 months, the patient has not experienced any recurrent symptoms and radiographs have not shown any recurrence.

fig4

Figure 4 Radiographs at 2 weeks post-operatively.

fig5

Figure 5 Radiographs 10 months post-operative showing consolidation.

fig6

Figure 6 Radiographs 10 months post-operative showing consolidation.

Discussion

Desmoplastic fibroma of bone, although considered benign, can be a debilitating condition leading to loss of limb [1-7]. Pathologic elements of this tumor include fibroblasts, collagen fibers, and elongated nuclei. These tumors begin inside the medullary canal and expand outward at which time it is possible to breach the cortex and invade adjacent tissues [3]. There have been isolated reports of associated osseous sarcomas arising in the areas of these tumors [4-8]. Bohm et al found a 50% recurrence rate with 25% of those leading to amputation in the extremities. The treatment of choice when confronted with this clinical and x-ray picture is bone biopsy, wide excision, and bone grafting [3,7].  Recurrent lesions can lead to selective amputation. Considering this bone tumor’s relative infrequency, we hope this case report provides guidance in treatment for the foot and ankle surgeon who may be confronted with this entity.

References

  1. Min H, Kang H, Lee J, Lee G, Ro J. Desmoplastic Fibroma with malignant transformation.Annals of Diagnostic Pathology 2010 Feb;14(1): 50-55. Pubmed
  2. Inwards C, Unni K, Beabout J, Sim F. Desmoplastic Fibroma of Bone.Cancer 1991 Nov;68(9):1978-1983. Pubmed
  3. Beskin J, Haddad R. Desmoplastic Fibroma of the First Metatarsal A Case Report.Clinical Orthopaedics and Related Research 1985 May;195:299-303. Pubmed
  4. Bohm P, Krober S , Greschnoik A, Laniado M, Kaiserling E. Demoplastic Fibroma of the Bone.Cancer 1996 Sep;78(5):1011-1023. Pubmed
  5. Shuto R, Kiyosue H, Yuko H, Miyake H, Kawano K, Mori H. CT and MR imaging of desmoplastic fibroblastoma.European Radiology 2002 Oct;12(10):2474-2476. Pubmed
  6. Evans, H. Demoplastic Fibroblastoma A report of Seven Cases.American Journal of Surgical Pathology 1995 Sep;19(9):1077-1081. Pubmed
  7. Takazawa K, Tsuchiya H, Yamamoto N, Nonomura A, Suzuki M, Taki J, Tomita K. Osteosarcoma arising from desmoplastic fibroma treated 16 years earlier: a case report. J Orthop Sci 2003;8(6):864-8. Pubmed
  8. Kim OH, Kim SJ, Kim JY, Ryu JH, Choo HJ, Lee SJ, Lee IS, Suh KJ.  Desmoplastic fibroma of bone in a toe: radiographic and MRI findings. Korean J Radiol. 2013 Nov-Dec;14(6):963-7. Pubmed

Ganglion Cyst of the Distal Tibia: A case report

by Ali Abadi, DPM1  , Jennifer Berlin, DPM, Warren Mangel, DPM3

The Foot and Ankle Online Journal 2 (11): 2

A 42 year-old female with persistent pain and numbness to the left foot and ankle is presented. Magnetic resonance imaging shows a ganglion cyst communicating to a cystic region within the tibia. The cyst is surgically excised and the tibia is curettaged and packed with cancellous bone chips. The ganglion is confirmed to be communicating with the left distal tibia bone. We present an unusual case of a ganglion cyst arising from bone.

Key words: Ganglion cyst, benign tumor, distal tibia.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License. It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Foot and Ankle Online Journal (www.faoj.org)

Accepted: October, 2009
Published: November, 2009

ISSN 1941-6806
doi: 10.3827/faoj.2009.0211.0002


The term ganglion was first used by Hippocrates to designate a knot of tissue filled with “mucoid flesh”. [1] It is a cystic swelling,1 – 3 cm in diameter, found typically in close proximity of joints and tendon sheath. The most common sites are the dorsal and volar aspect of the wrist, the dorsum of the foot, and around the ankle and the knee. [1] Ganglion cysts are well demarcated and movable within the surrounding tissue and are often loosely attached to a tendon sheath or the capsule of a joint. The dense fibrous capsule encloses the cyst which contains a viscid, jelly-like fluid rich in hyaluronic acid and other acid mucopolasaccharides. [2]

Case Report

A 42 years-old female patient presents with severe pain and numbness to the left foot and ankle. The patient presents to the doctor’s office complaining of intermittent pain and numbness of the left toes. The patient relates to mild pain while ambulating and going up the stairs. There is no history of trauma. Radiographs are negative for stress fracture. Conservative treatment is initially attempted to relive symptoms. This consisted of oral anti inflammatory medication and ankle bracing for stabilization. This was found to be ineffective. The pain is now intermittent and not affecting quality of life.

Magnetic Resonance Image (MRI) of the foot and ankle reveals a ganglion measuring 3cm above ankle joint. This area was asymptomatic on physical examination. The patient opted to monitor its progression. Two years later, the patient now experiences constant numbness of the left foot and toes as well as painful swelling along the posterolateral aspect of the leg. Surgery is discussed and planned for removal of the ganglion cyst.

There is now point tenderness and pain on palpation around the posterolateral aspect of the left ankle. Numbness is affecting the toes. (Fig. 1) Plain radiographs reveal a moderate increase in soft tissue swelling around the lateral malleolus. (Fig. 2) Radiographic studies are negative for stress fracture or other obvious osseous abnormalities.

Figure 1 Left leg with surgical mark.

Figure 2 Lateral radiograph ,left foot and ankle.

Laboratory examination included corpuscular blood count with differential count, white blood cell count, rheumatoid factor, C-reactive protein, erythrocyte sedimentation rate, and serum uric acid are all unremarkable. Electromyography result is negative for sciatica.

Magnetic resonance imaging of the ankle reveals a cystic lesion of 3cm x 1.5 cm in diameter with tibial bone erosion. Sagittal views showing a decreased signal intensity in T1 (Fig. 3A) and a well defined homogeneous bright signal in T2 (Fig. 3B) extending to the posterolateral aspect of the distal tibia. [3]

 

Figures 3A and B Sagittal view MRI (A) and cyst on T2 image showing cyst origin in bone. (B)

Surgical excision was performed. After inflating the thigh tourniquet, the ganglion cyst is excised through a 7 cm posterolateral incision. (Fig. 1) The mass is separated from the surrounding Flexor hallucis longus muscle and tissues. There is a well-defined capsule. The cystic mass is 3cm x 1.5 cm and penetrating the tibial bone. (Figs. 4A – D) The bone defect is evacuated with a bone currette and packed with cancellous bone chips. The tibial bone defect is about 1 cm. deep.(Figs. 5A and B)

 

 

Figures 4A – D Intra-operative photograph showing the ganglion cyst intact. (A)  A close-up of the cyst as it presents. (B)  The ganglion measures 3 x 1.5 centimeters. (C)  Intraoperative photo showing cyst dissection. (D)

 

Figures 5A and B Intra-operative photograph showing tibial erosion. (A)  When evacuated, there is an exposed cavity within the tibia that reveals the cyst origin. (B)

The pathology report is consistent with the diagnosis of ganglion. The cyst measured 3.5 x 3 cm with a wall of 0.1 cm thick that was composed of collagenous connective tissue. It presented along with several islands of mucinous material. No epithelial lining was noted. There was no reported evidence of neoplasm, inflammation or granuloma.

Postoperatively, the patient is pain-free and shows normal strength and sensation in the left foot and ankle. No recurrence has been noted up until the writing of this report.

Discussion

The ganglion cyst is the most common soft tissue mass found in the foot and ankle. [4] It is also a common occurrence in hand or wrist. A ganglion is a well-circumscribed, soft, and fluid-filled cystic mass that is frequently freely movable in the subcutaneous tissues and most often is found on the dorsum of the foot. [4] The exact etiology is unknown; however, traumatic, degenerative or inflammatory process in adjacent joints has been suggested as possible etiological factors. [5]

MRI is the modality of choice in the assessment of soft tissue tumors. On MRI, ganglion cysts demonstrate low intensity on T1 and high signal intensity on T2 weighted images.

Although 75% of all biopsy proved soft tissue masses of the foot and ankle are benign, [6,7] ganglion cysts should be differentiated from other tumors such as lipoma, hemangioma, infection and malignant tumors, such as malignant fibrous histiocytoma. [8]

References

1. Carp L, Stout AP: A study of ganglion with special reference to treatment. Surgery, Gynecology and Obstetrics 47: 460 – 468, 1928.
2. Soren A: Pathogenesis and treatment of ganglion. Clin Orthop Relat Res 48: 173 – 179, 1966.
3. Crim JR, Cracchiolo A, Hall R: Imaging of the foot and ankle. Imaging of the Foot and Ankle. 244. London: Martin Dunitz Ltd, 1996.
4. Hattrup SJ, Amadio PC, Sim FH, Lombardi RM: Metastatic tumors of the foot and ankle. Foot Ankle 8: 243-247, 1988.
5. Steiner E, Steinbachh LS, Schnarkowski P: Ganglia and cyst around joints. Radiol Clin North Am 34: 395 – 425, 1996.
6. Llauger J, Palmer J, Monill JM, Franquet T, Bague S, Roson N: MR imaging of benign soft tissue masses of the foot and ankle. Radiographics 1481 – 1498, 1998.
7. Pontius J, Good J, Maxian S: Ganglions of the foot and ankle: A retrospective analysis of 63 procedures J Am Podiatr Assoc 89: 163 – 168, 1999.
8. Banks AS, Downey MS, Martin DE, Miller SJ, McGlamry ED: McGlamry’s Comprehensive Textbook of Foot and Ankle Surgery. Lippincott Williams and Wilkins; 3rd Revised edition, 1354 – 1366, 2001.


Address Correspondence to: Dr ALI Abadi, West Jersey Virtua Hospital, New Jersey. 101 Carnie Blvd., Voorhees, NJ 08043
E-mail : aa78@georgetown.edu

PGY-1, Virtua Hospital, 101 Carnie Blvd., Voorhees, NJ 08043.
Attending, Virtua Hospital, 101 Carnie Blvd., Voorhees, NJ 08043.
Chief of Podiatry Surgery, Virtua Hospital, 101 Carnie Blvd., Voorhees, NJ 08043.

© The Foot and Ankle Online Journal, 2009

Intraosseous Ganglion of the Ankle: A Case Report

by Vasu Pai MS, D (Orth), National board (Orth), FICMR, FRACS, MCh (Orth) 1, Vishal Pai, M.B., Chb2

The Foot & Ankle Journal 1 (3): 2

The case of a 52 year-old man with a persistent ganglion cyst of the dorsolateral aspect of the ankle is presented. At excision, the ganglion communicated to a cystic region within the bone of the lateral malleolus. The patient was treated with curettage of the lateral malleolus and cancellous bone grafting.

Key words: Intraosseous ganglion, lateral malleous, ganglion, benign tumor

This is an Open Access article distributed under the terms of the Creative Commons Attribution License.  It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Foot & Ankle Journal (www.faoj.org)

Published online: March 1, 2008

ISSN: 1941-6806/08/0103-0002
doi: 10.3827/faoj.2008.0103.0002

Ganglia are most commonly located around the wrist as a soft tissue swelling with a pedicle. This can be traced to the scapholunate or radioscaphoid joints or surrounding tendon sheath. Intraosseous ganglion are rarely reported and occur mainly as carpal bone cysts. [1,2,3,4] A ganglion cyst of the lateral malleolus is a rare occurrence with only one report in the literature. [5] We report a case of an intraosseous ganglion of the lateral malleolus with soft tissue swelling. There was communication between an intraosseous ganglion and the overlying the soft tissue cyst.

Case Report

A 52 year-old man was referred for treatment of swelling involving the lateral aspect of his right ankle. Two years before presentation, the swelling appeared to have grown to significant size with associated aching pain. The patient also noticed pain around his ankle on weight bearing.Upon further inquiry, the patient reports a twisting injury to his right ankle in a rugby game that occurred in 1972. The injury was treated by cast immobilization and physiotherapy. The patient then underwent surgical repair in 1975 of which the details were not available. This surgery was successful and he was able to go back to work and also play rugby.Physical examination revealed a 6 cm diameter swelling over the dorsolateral aspect of the right ankle. There was no warmth or tenderness over the swelling. Swelling appeared fixed to the lateral malleolus. There was deep tenderness over the posterior aspect of lateral malleolus. (Fig 1)

 

Figure 1 Soft tissue swelling of the right lateral ankle.

Examination of the ankle revealed a range of motion of 15° dorsiflexion and 30° plantar flexion of the ankle, motion beyond which was painful. There was evidence of joint effusion and tenderness over the joint line on deep palpation. Distal neurovascular status appeared normal.
Blood examination including total lymphocyte count, ESR, CRP, rheumatoid factor and serum uric acid were unremarkable.
Magnetic resonance imaging of the foot showed discrete fluid collections both outside as well as within the lateral malleolus and was consistent with diagnosis of benign swelling of ganglion. (Fig . 2)
 

Figure 2 Coronal T2 and T1 weighted MR image showing a well defined homogeneous bright signal in T2 and decrease signal in T1, extending into the Anterolateral soft tissues.

Plain radiograph revealed moderate osteoarthritis of the ankle with joint space narrowing. There was a subtle multiloculated osteolytic lesion in the lateral malleolus with thin sclerotic margins along the intramedullary portion of the lateral malleolus. The distal fibula had mild remodeled expansion with thin cortical bone. There was a huge soft tissue swelling related to the lateral malleolus. (Fig. 3ab)

Figure 3a Ankle mortise view showing soft tissue swelling lateral to lateral malleolus.  A multiloculated osteolytic lesion is seen in the distal fibula.

Figure 3b Lateral radiograph showing diffuse osteoarthritis of the anterior and posterior ankle mortise.  Soft tissue swelling is also appreciated anteriorly.

The differential diagnosis includes giant cell tumor, gouty arthropathy, ganglion cyst, aneurysmal bone cyst or synovial sarcoma.

Under tourniquet control the ganglion was excised through a lateral incision. There was a 4 cm x 4 cm soft cystic mass which communicated directly with the lateral malleolus. There was a small defect over the posterior aspect of the malleolus. (Fig. 4)

Figure 4 Intra-operative photograph showing dissected superficial swelling from the lateral malleolus.

Discussion

Ganglion cysts are common mucin-containing tumors that affect a wide variety of joints of the body, including those of the foot and ankle. Despite their benign histology, the cysts can be problematic.

Pain and mass effects associated with the lesions can make ambulation and wearing shoes difficult.

In 1989, a series of 83 cases of soft-tissue tumor of the foot and ankle were retrospectively analyzed to determine the relative frequency of the lesions. [15] Seventy-two (87 percent) of the tumors were benign. Ganglion cysts and plantar fibromatoses were the most common. Ganglion have also been described in rarer areas of tendon, nerve and bone [6,7,8]

The etiology of intraosseous ganglia is uncertain. It has been shown by Landels that cysts in osteoarthritis arose from an intrusion of synovial fluid into bone at the joint surface. [9]

Kambolis reviewed 15 cases of ganglionic cystic defects of bone, and suggested that the intraosseous lesion was the result of extension of the overlying soft tissue ganglion into bone. [8] Other theories as to the cause of intraosseous ganglia include degeneration in intramedullary connective tissue and metaplasia of skeletal connective tissue

In a review of 54 cases of ganglion cyst, Rozbruch reported 4 patients with intraosseous ganglia located in the proximal tibia, patella and the first metatarsal head. [10] They also suggested that curettage of the bone gives better results. Isolated tarsal ganglion without soft tissue has also been reported. [11,12,13,14] A ganglion cyst of the lateral malleolus with a soft tissue extension is a rare occurrence, and there was only one report found in a literature review. [5]

Surgical excision is required in the majority of cases to confirm the diagnosis as well as alleviate pain when conservative measures are unsuccessful. The recurrence rate following excision of soft tissue ganglions can range from of 7 to 43 percent. [15] It appears that the recurrence following curettage of an intraosseous ganglion is rare. [16]
Bone lesions with a similar radiographic appearance (giant cell tumors, aneurysmal bone cyst, enchondromas, synovial sarcoma and gouty arthropathy) must be differentiated from intraosseous ganglia. Curettage of the cyst followed by packing of the defect with bone graft is the treatment of choice.

References

1. Tuzuner T, Subasi M, Alper M, Kara H, Orhan Z. : Penetrating type intraosseous ganglion cyst of the lunate bone West Indian Med J. 54(6):384-6, 2005.
2. De Smet L, Van Ransbeeck H.Intraosseous ganglion of the triquetrum. A transpisiformal approach. : Acta Orthop Belg. 66(2):194-6, 2000.
3. Fealy MJ, Lineaweaver W.Intraosseous ganglion cyst of the scaphoid. : Ann Plast Surg. 34(2):215-7, 1995.
4. Ikeda M, Oka Y.: Cystic lesion in carpal bone. Hand Surg. ;5(1):25-32, 2000.
5. Ahn JI, Park JS. Intraosseous ganglion. Report of a case.Int Orthop.;17(3):184-7, 1993.
6. Costa CR, Morrison WB, Carrino JA, Raiken SM. MRI of an intratendinous ganglion cyst of the peroneus brevis tendon. : Clin Orthop Relat Res.;445:254-60, 2006.
7. Adn M, Hamlat A, Morandi X, Guegan Y. Intraneural ganglion cyst of the tibial nerve. Acta Neurochir (Wien). 148(8):885-9; 2006.
8. Kambolis C, Bullough PG, Jaffe HI. Ganglionic cystic defects of bone. J Bone Joint Surg Am. 55(3):496-505, 1973.
9. Landells JW. The bone cysts of osteoarthritis. J Bone Joint Surg 35B,643-6,1953.
10. Rozbruch SR, Chang V, Bohne WH, Deland JT. Ganglion cysts of the lower extremity. Orhopedics 21:141- 148, 1998.
11. Murff R, Ashry HR. Intraosseous ganglia of the foot. J Foot Ankle Surg.;33(4):396-401,1994.
12. Uysal M, Akpinar S, Ozalay M. Arthroscopic debridement and grafting of intraosseous talar ganglion. Arthroscopy.21(10);1269,2005.
13. Wu KK. Intraosseous ganglion cyst of the middle cuneiform bone of the foot. J foot ankle surg 33(6):633-5, 1994.
14.Seymour N Intraosseous ganglia of medial malleolus, JBJS[Br](50),134-7,1968.
15. Kirby EJ, Shereff MJ, Lewis MM.Soft-tissue tumors and tumor-like lesions of the foot. An analysis of eighty-three cases. JBJS (Am). Apr;71(4):621-6, 1989.
16. Pontius J, Good J, Maxian S. Ganglions of the foot and ankle, an retrospective analysis of 63 procedures. J Am Pod Assoc 89:163 168, 1999.


Address correspondence to: Dr. Vasu Pai, Gisborne Hospital, Ormand Road, Gizborne, New Zealand.
E-mail: vasuchitra@gmail.com

1Orthopaedic Specialist, Gisborne, Hospital, Ormand Road, Gisborne, New Zealand.2House Surgeon, Middlemore Hospital, Auckland, New Zealand.

 

© The Foot & Ankle Journal, 2008

 

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