Tag Archives: curettage

Multi-centric giant cell tumor involving the distal fibula and talus misdiagnosed as tuberculosis: A case report

Jeetendra Bajpai1, Om Singh Meel1, Vipin Khatkar1, Sumit Saini1, Akansha Bajpai2pdflrg

The Foot and Ankle Online Journal 7 (3): 2

Multi-centric giant cell tumor (MCGCT) is a rare benign disease presenting as polyostotic osteolytic lesions in approximately 1% cases of giant tumors. It presents as multiple lytic lesions that span the metaphyseal-epiphyseal region of short bones of hands and feet. Although MCGCT has been reported at various sites, involvement of distal fibula has never been reported earlier. We present a case of MCGCT involving distal fibula and talus. It presented as swelling and pain in the ankle for four months.  Initially on clinical and radiologic basis, it was diagnosed as osteoarticular tuberculosis and treated with Anti-tubercular drugs, without any relief. Diagnosis of MCGCT was made with open biopsy. Complete curettage of tumor from both sites and bone grafting was done. Patient has been symptom free at one-year follow-up. In India, osteoarticular tuberculosis presents as a common diagnostic dilemma. Biopsy and histopathological confirmation is necessary for definitive management.

Key words: Multicentric Giant Cell Tumor (MCGCT), Open biopsy, Curettage, Bone grafting.

ISSN 1941-6806
doi: 10.3827/faoj.2014.0703.0002

Address correspondence to: Jeetendra BAJPAI, M.S orthopaedics, Senior Resident Department of Orthopaedics, e-mail: dr.jbajpaii@gmail.comm

1 Department of Orthopaedics, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India
2 Department of Radiology, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India


Giant cell tumor (GCT) of bone is a benign aggressive tumor with features of frequent local recurrences and potential for metastasis and malignant transformation [1]. GCT is commonly seen in young adults 20-40 years of age with slight female predominance, and constitutes 5% of total primary neoplasms [2]. Giant cell tumor of bone typically presents as a solitary lytic lesion that spans the metaphyseal-epiphyseal region of long bones of adults. In approximately 1% of cases, however, it manifests as multiple synchronous or metachronous lesions in single or multiple bones. Such cases have been designated multicentric giant cell tumors and their pathogenesis is unknown.

Their clinical and radiographic differential diagnosis usually includes a variety of polyostotic skeletal lesions such as Brown tumor, Paget’s disease, osteomyelitis, Non-ossifying fibroma, enchondromatosis, fibrous dysplasia, giant cell reparative granuloma, Langerhans cell histiocytosis, vascular tumor, osteosarcoma, hematopoietic malignant tumor, and metastasis. Although it is not common worldwide but in developing countries like India, osteoarticular tuberculosis is considered as differential diagnosis of lytic bony lesions [3]. In most situations, a definitive diagnosis requires biopsy with histopathologic confirmation.

To our knowledge, around 100 cases of multicentric giant cell tumor have been reported in the literature, and most of these have been small case reports [4-35]. Most of the series and case reports have reported occurrence of MCGCT in the hand and foot.  Though synchronous lesions in the foot have been reported in distal tibia, calcaneus, navicular, talus and even metatarsals, we found no case of MCGCT involving the distal fibula in the literature.

We present one such case of synchronous MCGCT involving the distal fibula and talus on the same extremity for its rarity. This case becomes more so important to report because lesions like this can initially be confused with tuberculosis, especially in Indian scenario.

Case Report

A 32 year old female presented with complaints of gradually increasing painful swelling of the left ankle for four months. Patient was on anti-tubercular drugs for six weeks without any relief. On examination there was diffuse tender swelling of 2cm x 1.5cm on the anteromedial ankle and 1cm x 1.5cm on posterolateral aspect of the left ankle. The temperature over the swelling was not raised. There was no sinus tract. ESR was 40mm/hr and TLC 11,000/cumm.  Radiographs and MRI of the left ankle were showed lytic lesions in the neck and body of talus and distal end of fibula (Figures 1 and 2). Open biopsy was done from the distal end of the fibula. Histopathological examination suggested   presence of giant cells (Figure 3). Curettage of the neck and body of the talus and distal fibula along with bone grafting was performed. The material from distal fibula and talus were marked separately and sent for histopathology which confirmed GCT at the two sites. A below knee splint was applied. Patient was kept non-weight-bearing for 6 weeks. There were no signs of recurrence at one year follow up.

Discussion

Primary multicentric giant cell tumors are rare and should be a diagnosis of exclusion [22,25,28,31,32]. MCGCT is more common in the short bones of hands and feet as compared to solitary GCT which tends to mainly involve the knee region and distal radius.  GCT is capable of extending along fascial planes and directly crossing the joint space to a neighboring bone [5,14]. Controversy regarding pathogenesis of MCGCT exists to date. Mechanisms described include contiguous spread, iatrogenic seeding of tumor cells, benign metastasis, malignant transformation and de-novo multifocal formation [22,25,32].

fig1a fig1b

Figure 1 A and B Left foot radiograph antero-posterior and lateral views showing lytic areas in body of Talus and Distal end Fibula.

fig2a fig2b fig2c

Figure 2 A, B and C MRI left foot showing lytic areas in body of Talus and distal end Fibula.

Synchronous tumors are lesions arising from different locations and are discovered at different times within a short time period or simultaneously [20,28,32,43].  Metachronous tumors are discovered at different times and at different places [26,31,44]. The question of whether multifocal GCT is synchronous, metachronous, or metastatic lesion is difficult to answer [32]. It is difficult to state definite criteria for distinguishing multifocal lesions from metastatic involvement, especially when second lesions are discovered later. MCGCT tends to involve the younger population with mean age reported between 20-24 years.  MCGCT have been reported in a 10 year old as well as a 62 year old [3,25,32]. MCGCT of the foot is rare [20,25,36]. MCGCT has been known to involve the calcaneus, talus, navicular, cuneiform, proximal fibula but involvement of distal fibula has never been previously reported.

fig3a fig3b

Figure 3 A and B H&E slides depicting Giant cells marked with yellow arrows.

Before a diagnosis of multicentric giant-cell tumor can be made, it is necessary to rule out the presence of hyperparathyroidism, which can produce features of a polyostotic osteolytic lesion that are virtually identical to those of a giant-cell tumor of bone. Fibrosarcoma of bone is usually a solitary lesion, but in certain rare cases multiple independent tumors develop simultaneously throughout the skeleton [38]. Rarely, osteoblastoma may present as a multicentric lesion [40,41].

Conclusion

In India, osteoarticular tuberculosis presents as a common diagnostic dilemma. Biopsy and histopathological confirmation is necessary for definitive management. It has been reported that recurrence of giant-cell tumor of bone of the solitary type may be expected in 30 to 60 percent of cases; the rate depends on the type of treatment, the anatomical location, and the cortical integrity of the involved bone. Recurrence will usually be found within two years of the initial treatment. Cases have been reported with interval between first and last lesion varying from four months to 16 years [22]. However, in one year follow-up, there are no signs of recurrence in our patient.

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Chondroblastoma of the Talus: A case report

by Prasad Soraganvi1emailsm, Ramakanth R2emailsm, Vijay Kumar M3emailsmpdflrg

The Foot and Ankle Online Journal 6 (1): 1

Chondroblastoma is a benign tumor of immature cartilage cells which primarily occurs in the epiphysis of long bones in the second decade of life with slight male preponderance. The diagnosis is obtained from the microscopic picture, showing large collections of chondroblasts surrounded by a matrix of immature fibrous tissue and a few scattered giant cells. Benign chondroblastoma is rarely seen in the bones of the feet. Very few cases of benign chondroblastoma involving the talus have been reported. We report an unusual case of benign chondroblastoma of the talus in a 19 year-old female. Clinical presentation, histological diagnosis and treatment by curettage and bone grafting are described. Also importance of intact cortex and approaching the tumor by making a window in the more involved thin cortex is highlighted. The patient is now asymptomatic and there is no evidence of recurrence at 3 years follow-up.

Key words: Chondroblastoma, bone graft, curettage

Accepted: December, 2012
Published: January, 2013

ISSN 1941-6806
doi: 10.3827/faoj.2013.0601.001


Address correspondence to: Department of orthopaedics, PES Institute of Medical Science and Research, Kuppam, Chittor District– 517425, Andra Pradesh, India

1Assiatant professor, PES institute of medical science and research, Kuppam,AP, India.
2Senior resident, PES institute of medical science and research, Kuppam,AP, India.
3Jr.Consultant, MMHRC,Madurai,India.


Chondroblastoma is a benign tumor of immature cartilage cells.[1] In 1931, Codman classified it as a chondromatous variant of giant cell tumor when he described these lesions in the proximal humerus.[2] A decade later, Jaffe and Lichtenstein renamed it as chondroblastoma and clearly separated it from giant cell tumor.[3] Tumor seems to arise from secondary centers of ossification and the cell of origin arises from the epiphyseal plate or some remnant of it. The lesion is rare, accounting for approximately one percent of all benign bone tumors.[4,5] Treatment has been highly variable but currently usually consists of curettage and packing with bone graft.[2,6]

Its occurrence in small bones is rare. About 12% of all chondroblastoma occur in the bones of the foot. Chondroblastoma in the foot most commonly occurs in subchondral areas of the talus and calcaneal apophysis.[7] In chondroblastoma of the foot and ankle, recurrence is common, and outcomes are generally worse than in other locations in the skeleton.[7] Very few cases of benign chondroblastoma involving the talus have been reported. We report a case of benign chondroblastoma of talus in a 19 year-old female. Clinical presentation, histological diagnosis and treatment by curettage and bone grafting are described along with review of literature.

ChondtalFig1a ChondtalFig1b

Figure 1A and Figure 1B Radiograph of talus showing the lesion involving most of body and the medial cortex is thin compared to lateral cortex in lateral view. (A) Anterior posterior view. (B)

The lesion was approached with a posteromedial incision and by making a cortical window in the medial cortex. The decision was made based on involvement of cortex by the tumor. This has not been described before in the literature. The patient is now asymptomatic and there is no evidence of recurrence at 3 years follow-up.

Case Report

A 19 year-old girl presented with history of pain and swelling in the right ankle since one year. The swelling was insidious in onset and slow in progression associated with mild dull aching pain. Pain increased on walking, and was relieved by rest and analgesics. The patient had no history of trauma or fever. Clinical evaluation revealed that swelling was on the medial aspect of the right ankle and the skin over the swelling was normal. Tenderness was present on deep palpation and there was no local rise of temperature. The swelling was firm-to-hard in consistency and arising from the talus. Range of movement of the ankle and subtalar joint were restricted and painful. There were no distal neurovascular deficits. No appreciable lymphadenopathy was noted. Conventional radiographs showed well-defined, expansile and lucent area within talus involving the body and posterior subchondral area.

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Figure 2A and Figure 2A Terminal restriction of movements post operatively. Both Plantarflexed (A) and dorsiflexed. (B)

The lesion was approximately 3.5cms in size. There was no breach in the cortex. Most of the talus was involved except the lateral cortex head. The medial cortex was thinner than the lateral cortex. Stippled calcification with thin trabeculae was seen within the radiolucent area (Fig. 1). Based on clinical and radiological findings, a diagnosis of benign cystic lesion of the right talus was made and aneurismal bone cyst, chondroblastoma were considered for differential diagnosis.

An open biopsy, curettage and bone grafting was performed. The lesion was approached by a posteromedial incision. The decision of approaching the lesion by a posteromedial incision was made based on the extent of involvement of the cortex. A window was made in the medial cortex of talus. Extensive intralesional curettage was performed. The defect was then filled in by bone graft harvested from the iliac crest and bone substitutes. Microscopic examination of the tissue revealed sheets of cells with oval to elongated nuclei. Scattered osteoclastic giant cells were present. Isolated island of cartilaginous matrix with focal area of linear calcification (chicken wire calcification) were seen and a diagnosis of chondroblastoma was made. Post operatively below knee cast was applied for two months. The patient was started on partial weight bearing walking after 2 months and full weight bearing walking after 3 months.

At the 6 month follow post-operatively the patient was ambulating normally without pain or limp. Range of motion of the ankle and subtalar joint were improved, but terminal 10 to 20 degree range of movement was restricted. (Fig. 2A and 2B)

ChondtalFig3

Figure 3 Two year Post operative radiograph showing satisfactory incorporation of bone graft.

At 2 year follow up radiograph showed good incorporation of bone graft and there was no sign of recurrence. (Fig. 3)

Discussion

Chondroblastoma is benign cartilaginous tumor which has typical clinical pathological features. It accounts for one percent of all benign tumors.[8,9,10,11,12] Chondroblastoma has been seen in people of all age groups. Adults in their second decade of life appear to have a higher prevalence of the tumor. A study of 104 cases by Bloem and Mulder revealed an average age of 16 years in tumors affecting long bones and 28 years in short, tubular bones, most notably the talus and calcaneum.[13] Males are affected more than females by approximately 2:1 rate. The tumor is characteristically centred in the epiphysis of long bones.[14] Chondroblastoma is a benign tumor but occasionally the tumor may show a more aggressive pattern, with invasion of the joint spaces, adjacent bones, and very rarely, the metastases.

Typically on a radiograph a chondroblastoma presents with an eccentrically or centrally located osteolytic lesion that involves the epiphysis or other secondary ossification centers.[15] In 20% to 25% of the cases metaphyseal involvement is also seen.[5]

Cortical expansion, with erosion and periosteal reaction may be present occasionally.[15] There may be stippled calcification or there may be no matrix mineralization. The tumor is adjacent to an articular surface or an apophysis. Extension of the tumor to the articular surface had been observed.[7]

Computerized tomography (CT) scans can provide valuable information in helping diagnose and evaluate the extent of a chondroblastoma. The size of the lesion can be better appreciated with CT scans as compared to plain radiographs. CT scan is useful for defining the relationship of the tumor to the joint, the integrity of the underlying bone, and to identify intralesional calcifications. Also, the amount of calcification can be better evaluated.[16]

Magnetic resonance imaging (MRI) can help in the diagnosis and in the differentiation of a chondroblastoma. MRI is especially useful when plain radiographic findings are inconclusive. MRI scans show the very high signal intensity on T2 weighted scans. Bone scan shows avid tracer uptake in the lesion.[7] In MRI scan surrounding bone marrow and soft tissue edema and periosteal reactions may be seen.[16] Nuclear scans have shown that chondroblastoma are more hyperemic than the surrounding bone but are nonspecific for actually diagnosing a chondroblastoma.[16]

The tumor is composed of cellular and matrix rich areas. Tumor cells are round or polygonal cells with an oval or round nucleus and eosinophilic cytoplasm make up the cellular areas. The nuclei are often indented and lobulated. In non-decalcified sections the chondroblasts appear focally delimited by a thin calcification rim, so called chicken wire.[17] Mitosis is always typical and is quite frequent in the cellular areas. Matrix rich areas are composed of different types of matrix like chondroid, osteoid, fibrous and rarely mature hyaline cartilage.[15]

Of all the bone tumors, chondroblastoma represents less than 1% with only 20% of it occurring in the foot. Chondroblastoma of foot is most commonly found in the talus and calcaneum.[10,11,18,16,19] Approximately 4% of all chondroblastoma arise in the talus.[5,14,20] Very few cases of chondroblastoma involving the talus have been reported.

ChondtalTab1

Table 1 Chondroblastoma of the talus, a review of cases.

The clinical data of 12 reported cases and our case are summarized in Table 1. The average age at presentation was 19 years. Three patients presented in the first decade, five patients were between 10 and 20 years and 5 patients were above 20 years. Males are affected more than females by approximately 2:1 rate which is comparable with other large series reported by Ramappa, et al.,.[5]

In a review of 322 cases of chondroblastoma by Fink only 42 involved the foot and the posterior subchondral area of talus is more common site.[7] Invasion of the sinus tarsi and simultaneous calcaneal involvement has also been reported.[9,16]

Out of 13 cases reviewed here, the talar body was involved in 11 patients and the talar neck was involved in two patients (table 1). In the majority of the patients the left side of the talus was involved. Patients often present with pain and swelling around the joint.[5] Chondroblastoma is known to present with atypical features when the foot is involved. Involvement of the talus can present with pain in the ankle joint more commonly. Approximately 20-50% of the patients give a history of trauma.[9] All of the patients reported with chondroblastoma of talus presented with pain and most of them had swelling (table 1). Most of the patients had pain for several months before presentation. The case reported here had pain in ankle one year before presentation.

Localized swelling and a decreased range of motion are common clinical findings, with the majority of patients having tenderness on direct palpation. Rarely pathological fracture is the presenting feature in about 1-13% of patients.[9] Since chondroblastoma is not common in the talus, patients presented early with complaint of pain are misdiagnosed.[21,16] Two patients presented within one month after pain in ankle had normal radiograph initially (table 1). Both of these patients were misdiagnosed at early presentation. Chondroblastoma was diagnosed when a radiograph taken after few years showed the lesion. One case presented at one year with pain in the ankle, radiograph showed no significant changes, but MRI revealed the lesion in talus.[22] Because of this atypical presentation chondroblastoma of the talus may be confused with synovitis, tendinitis or other lytic lesions of bone like aneurismal bone cyst, tuberculosis.

The diagnosis of these lesions, in uncommon sites, is often delayed for months or even years, and are often treated as ankle sprains. Metastasis of benign chondroblastoma is a rare event. Benign pulmonary metastases have been reported with primary tumor involving the talus and the author concluded that all patients need to be evaluated regularly from the onset for possible lung metastasis so that deposits can be detected early for total resection.[18]

Treatment of the primary lesion consists of complete curettage and bone grafting.[15] Recurrences following this treatment are to occur in 10-45%.[9] Extending the zone of the curettage by removing two or three additional millimetres of bone using a mechanical bur, or by using phenol or liquid nitrogen placed in the tumor cavity have been proposed as in a method to reduce the risk of local recurrence.[7] Involvement of articular cartilage treated with osteochondral autograft transfer from the lateral femoral condyle has been reported with good results.[23] Excision of the talus with calcaneotibial arthrodesis has been reported by Jambhekar, et al.,.[18] Out of thirteen cases, eight cases were treated with curettage and bone grafting, two cases only with curettage, two cases with resection and one case with excision of talus. (Table 1)

Curettage and bone grafting has shown good out come when articular surface is not involved. Total talectomy may be contemplated in cases where there is extensive involvement of the talus. The recurrence rate of chondroblastoma is reported to be 10 % to 15 %.[15] Open growth plates have also been considered as a risk factor for recurrence.[4] Springfield., et al. in their review of 70 cases of chondroblastoma, have suggested that recurrence is secondary to less aggressive surgical curettage due to fear of injury of the physis.[20] While in a review of 73 cases of chondroblastoma by Ramapa., et al. treated between 1977 and 1998 , it is concluded that one possible explanation of recurrence of chondroblastoma in their case might be the anatomic location. Also lower recurrence rate is found in patients treated by packing the defect with polymethylmethacrylate instead of bone graft.[5]

Bloem, et al., in their study of 104 chondroblastoma cases have follow up exceeding 3 years, but they failed to see any recurrences after this period.[13] In chondroblastoma of the foot and ankle, recurrence is common, and outcomes are generally worse than in other locations in the skeleton.[7] Recurrent lesions should be treated with repeat curettage. Recurrence and severe destruction of bone integrity may necessitate ankle arthrodesis or en-bloc resection with associated functional loss. Patients with recurrent lesions should have follow-up CT scans of the chest to detect pulmonary nodules and if present nodules should be excised.[7]

In our case, the lesion was large measuring approximately 3.5cms. Most of talus was involved except the lateral cortex and head. The articular surface and all cortices were intact. Hence, we planned for curettage and packing the cavity with bone graft. The lateral cortex was thick as compared to the medial cortex. Hence, we have decided to approach the tumor by the postero-medial approach. Since the lateral cortex was thick approaching laterally and making a window in the lateral cortex would have weakened it.

The window was made in the medial cortex followed by curettage and bone grafting. Bone was harvested from the iliac crest and mixed with bone substitute to fill the cavity. The patient underwent uneventful recovery and asymptomatic at two year follow up, also follow-up radiograph shows satisfactory incorporation of the bone graft with no signs of recurrence. Most of the reported cases of chondroblastoma of talus have been treated by posterolateral approach or anterolateral approach.[8,21,16,19,24] Medial approach was done in one case where the talus and calcaneum were involved simultaneously.[25] In larger lesions, talectomy was done. Our case had a larger lesion (3.5 cm) with a relatively thicker lateral cortex; hence the posteromedial approach was made with a window in medial cortex. It is desirable to approach the lesion without weakening the intact thick cortex. Posteromedial approach and making a window in the medial cortex have not been described in reports on this condition. Khan and Moore each described a surgical approach using a small anterolateral window in the neck of the talus to gain access to the lesion.[24,26]

Yu and Sellars used a lateral incision with direct curettage near the opening of the sinus tarsi, and gained access to the lesion through this approach.[16] Wu concluded that eccentrically based talar chondroblastoma should be treated with talectomy.[27] Sterling described approaching the lesion without entering the cavity of the adjacent joint via the sinus tarsi.[9] Small-sized tumors can effectively be curetted through arthroscopic portals with minimal morbidity.[22] Anderson reported small chondroblastoma of the talus involving articular surface treated with osteochondral autograft transfer.[23] For best surgical results with minimal morbidity, there should be early diagnosis and proper choice of the best surgical procedure. The case reported here utilizes a comprehensive approach which decided on the extent of the lesion involving the talus. We believe that the surgical approach should be decided on the location of the lesion, articular cartilage involvement and also on involvement of the medial or lateral cortex for maximum restoration of function.

Conclusion

Chondroblastoma of the talus is a rare condition and it should be considered in the differential diagnosis in lytic lesion of the talus. A thorough history, physical examination and proper radiographic studies is mandatory. Diagnosis is confirmed by imaging study supplemented with open biopsy. The surgical technique and exposure of the tumor are modified to suit the requirements in each ease. Properly performed extensive curettage and bone grafting is a good option for complete removal of tumor.

References

1. Rosai J. Ackerman’s Surgical Pathology. 7th Edition, St Louis, CV Mosby, Chapter 24, 1989; Vol 2.
2. Codman EA. The classic:  Epiphyseal chondromatous giant cell tumors of the upper end of the humerus. Surg Gynecol Obstet.1931;52:543-48. Clin Orthop Relat Res 2006 450: 12-6. [PubMed]
3. Jaffe HL, Lichtenstein LL. Benign Chondroblastoma of bone: A reinterpretation of the so-called calcifying or chondromatous giant cell tumor. Am J Pathol 1942 18: 969-691. [PubMed]
4. Campanacci M. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. 2nd edition. New York, Springer; 1999. [Website]
5.  Ramappa AJ, Lee FY, Tang P, Carlson JR, Gebhardt MC, Mankin HJ: Chondroblastoma of bone. JBJS 2000, 82A: 1140-1145. [PubMed]
6. Simon MA Springfield, D. S: Surgery for Bone and Soft Tissue Tumors. Philidelphia, Lippincott-Raven; 1998: 190-191.
7. Fink BR, Temple HT, Chiricosta FM, Mizel MS, Murphey MD:  Chondroblastoma of the foot.  Foot Ankle Int 1997 18: 236-242.[PubMed]
8. Zhang K, Geo Y, Dai H, Zhang S, Li G,  Yu B. Chondroblastoma of the talus: A case report and literature review. Foot Ankle Surg 2012 51: 262-265. [PubMed]
9. Sterling G, Wilson A. Chondroblastoma of the talus: a case report. J Foot Ankle Surg 2002 41: 178-182. [PubMed]
10 Khalifa YE. Chondroblastoma of bone: Management and results of surgical treatment in ten patients. Pan Arab J Orth Trauma 2004 8: 221-229. [Website]
11. Basu N, Roy A, Chatterjee S, Mallik MG, Sengupta S, Basu A, Das A.  Chondroblastoma of talus – A case report. J Indian Assoc Pediatr Surg 2001 6: 58-60. [Website]
12. Dahlin DC, Ivins JC: Benign Chondroblastoma. A study of 125 cases. Cancer 1972 30: 401-413.
[PubMed]
13.Bloem JL, Mulder JD. Chondroblastoma: A clinical and radiological study of 104 cases. Skeletal Radiol 1985 14: 1-9.
[PubMed]
14. Kurt AM, Unni KK, Sim FH, McLeod RA: Chondroblastoma of bone. Hum Pathol 1989 20: 965-976. [PubMed]
15. Sepah YJ, Umer M, Minhas K, Hafeez K : Chondroblastoma of the cuboid with an associated aneurismal bone cyst: a case report. J Med Case Rep. 2007 1:135. [PubMed]
16 Yu GV, Sellers CS. Chondroblastoma of the talus. J Foot Ankle Surg 1996 35: 72-77.  [PubMed]
17.  Monda L, Wick MR.  S-100 protein immunostaining in the differential diagnosis of chondroblastoma.  Hum Pathol 1985 16: 287-293. [PubMed]
18. Jambhekar NA, Desai PB, Chitale DA, Patil P, Arya S. Benign metastasizing chondroblastoma. Cancer 1998 82: 675-678.
[PubMed]  
19.  Sankaran B, Duggal K., Wani GM. Chondroblastoma of talus – A case report. Indian journal of orthopaedics 1979 13 :81-83.
20.  Springfield DS, Capanna R, Gherlizoni F, Picci P, Campanacci M. Chondroblastoma: A review of seventy cases. JBJS 67A: 748-755. [PubMed]
21.  Ochsner PE, von Hochstetter AR, Hilfiker B. Chondroblastoma of the talus: Natural development over 9.5 years. Arch Orthop Trauma Surg 1988 107: 122-125. [PubMed]
22. Marcelo PP, Albert AMM, Daniel TA.  Benign bone tumors subperiosteal on the talar neck resected arthroscopically: case reports. Einstein 2010 8: 354-357.  [Website]
23. Anderson AF, Ramsey JR. Chondroblastoma of the talus treated with osteochondral autograft transfer from the lateral femoral condyle. Foot Ankle Int 2003 24: 283-287. [PubMed]
24  Moore TM, Roe JB, Harvey JP. Chondroblastoma of the talus. A case report. JBJS 1977 59A: 830-831. [PubMed]
25.  Ohno T, Kadoya H, Park P, Yamanashi M, Wakayama K, Ihtsubo K, Tateishi A, Kijima M. Case report 382. Benign chondroblastoma of talus invading calcaneus. Skeletal Radiol 1986 15: 478-483. [PubMed]
26. Khan FA. Benign chondroblastoma of the talus. JR CoIlege Surg Edin 1988 33: 222-224. [PubMed]
27.  Wu, KK Chondroblastoma of the foot. J Foot Surg 1989 28: 72-77. [PubMed]


© The Foot and Ankle Online Journal, 2013

A Rare Case of Aneurysmal Bone Cyst of the Calcaneum

by Prasad Soraganvi, Karan Kukreja, Ramakanth R.

The Foot and Ankle Online Journal 4 (4): 1

Aneurysmal bone cyst (ABC) is a benign solitary lesion of unknown aetiology. ABC’s mainly occur in the long bones but only rarely in the bones of the feet. For example, frequency of occurrence in the foot is only 3% compared to other bones of the body. Very few cases of ABC involving the calcaneum have been reported. We report an unusual case of ABC of calcaneum in a 55 year-old male. Clinical presentation, histological diagnosis and treatment by curettage and bone grafting are described. The patient is now asymptomatic and there is no evidence of recurrence at 2 years follow-up.

Key words: ABC, calcaneum, curettage, bone grafting.

Accepted: March, 2011
Published: April, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0404.0001


The aneurysmal bone cyst (ABC) is an expansile cystic lesion that most often affects individuals during their second decade of life and may occur in any bone in the body. [1-5] Although benign, the ABC can be locally aggressive and can cause extensive weakening of the bony structure and impinge on the surrounding tissues.

Jaffe and Lichtenstein first described ABC in 1942. [6] As defined by the World Health Organization, the ABC is a benign tumor like lesion described as “an expanding osteolytic lesion consisting of blood-filled spaces of variable size separated by connective tissue septa containing trabeculae or osteoid tissue and osteoclast giant cells.” [4]

ABCs both erode and cause ‘expansion’ of underlying cancellous and cortical bone. [7] Around the lesion there is always a shell formed by periosteal new bone and, although this may be only millimeters thick, it prevents direct extension into the soft tissues. [8] The expansile nature of the lesions can cause pain, swelling, deformity, disruption of growth plates, neurologic symptoms (depending on its location), and pathologic fracture. [1-3]

ABC’s in the foot are uncommon. ABC’s present about 1% of all primary bone tumors collectively. [9] Its frequency of occurrence in foot is only about 3% compared to other bones of body. [10] Occurrence within the calcaneum are rare, and generally present as chronic heel pain and swelling, but may rarely present as pathologic fracture. [11]

A plethora of cystic lesions can occur in the calcaneum, which makes definitive diagnosis difficult based on imaging only. The differential diagnosis includes simple bone cyst, ABC (primary or secondary), chondroblastoma, giant cell tumor (GCT), osteosarcoma, ossifying hematoma or pseudotumor of hemophilia. This mandates histopathological diagnosis prior to the definitive management.

We report a rare case of ABC involving calcaneum of 55 year-old male confirmed by histopathology report and we performed curettage and bone grafting of cyst.

Case Report

A 55 year-old male, manual laborer by occupation and known diabetic on treatment presented with a chief complaint of swelling in right heel during the last two years. An increase in swelling was associated with pain in heel from the last one year. He had difficulty in walking because of pain. For the last two months, he was unable to work due to pain. He did give history of blunt trauma prior to the onset of symptoms.

Clinical evaluation revealed swelling over the lateral aspect of the heel and the skin over the swelling was stretched. Tenderness was present on palpation but there was no local rise of temperature. The swelling was bony hard in consistency and arising from calcaneum. There were no distal neurovascular deficits or any significant lymphadenopathy.

Radiographic examination of his ankle revealed an eccentric, expansile, multiloculated lytic lesion of the calcaneum with thin trabeculae traversing the cystic cavity. (Fig. 1) There was no breach in the cortex. Based on clinical and radiological findings, a diagnosis of benign cystic lesion of right calcaneum was made.

Figure 1 Pre-operative radiographs, antero posterior and lateral views showing eccentric expansile lytic lesion with thin shell of cortex and trabaculae traversing the cyst.

Open biopsy of the cyst was made to confirm the diagnosis. The cyst grossly consisted of cavities filled with brown altered blood. Histopathological report revealed large blood filled cavities lined by fibrous septa, with occasional osteoclastic giant cells. (Fig. 2A and 2B)

Figures 2A and 2B Histologic slides reveal large blood filled cavities lined by fibrous septa (A), with occasional osteoclastic giant cells, haemosiderin laden macrophages with a thin rim of bone. (B)

Hence the diagnosis of ABC involving the right calcaneum was made. The patient was scheduled for cyst curettage and bone grafting. By curvilinear incision over the lateral aspect of heel, the calcaneum was exposed. A large cortical window was made and the entire cyst curettage was done. Then the cavity was washed with saline and packed with cortico-cancellous bone graft harvested from both iliac crests in addition to synthetic bone substitute. The patient was advised non-weight bearing walking on the affected limb for eight weeks. Later mobilised with partial weight bearing walking for a further four weeks and then followed by full weight bearing on affected limb.

At six months of follow up, the patient was pain free and had returned to his regular activities. At two years follow-up, the patient is clinically asymptomatic. There is no evidence of recurrence. (Figs. 3A, 3B and 3C)

Figure 3: Follow-up radiographs showing incorporation of graft material at 1 month (A), 6 months (B), and at 2 years follow-up(C) showing consolidation of graft and no recurrence.

Discussion

ABC is an entity on its own having unique clinical, radiological and diagnostic behavior. [7] The true etiology of ABCs is unknown. Most investigators believe that ABCs are the result of a vascular malformation within the bone; however, the ultimate cause of the malformation is a topic of controversy. [12]

The concept of an ABC as a secondary phenomenon occurring in a pre-existing lesion is based on the fact that in approximately one-third of the cases a pre-existing lesion can be identified, the most common of which is giant-cell tumor. [13] ABCs are common around the knee joint of the young [11] and have an equal incidence in both genders. About 50-70% of ABCs occur in the second decade of life, with 70-86% occurring in patients younger than 20 years, which makes this case even more unusual. [10]

On histology, the ABC is characterized by blood filled cavities lined by fibrous septa. The stroma contains proliferative fibroblasts, spindle cells, areas of osteoid formation, and an uneven distribution of multinucleated giant cells. The tissue within the septations includes cavernous channels that do not contain a muscular or elastic layer in their walls. Areas of new and reactive bone formation can also be found in the ABC. Mitotic figures are common to ABCs, but no atypical figures should be evident. [10]

Bone cysts of the calcaneum are rare lesions. These may include a wide spectrum of non-neoplastic cysts, benign or malignant neoplastic lesions ranging from simple bone cyst, ABC (primary or secondary), chondroblastoma, giant cell tumor (GCT), and an osteosarcoma (especially telangiectatic). [11]

Clinically, calcaneal cysts are often symptomatic and present with heel pain, although some of these lesions may remain asymptomatic and are detected as incidental findings. Even though there are many typical radiograph, computed tomographic (CT) scan, and magnetic resonance imaging (MRI) findings to confirm a diagnosis of ABC, an open biopsy must be performed because of the high frequency of accompanying tumors. [11] When a biopsy is performed, the sample should ideally include material from the entire lesion; a limited biopsy could easily cause a coexisting lesion to be missed, leaving the patient with a morbid prognosis.

There are various methods of treatment based on the site and size of the lesion, which include curettage, which may be supplemented with various adjuvant therapies such as bone grafting, use of liquid nitrogen, phenol instillation and Poly (methyl methacrylate) (PMMA) cement.

Other modalities such as wide excision or arterial embolisation may be considered. Although relatively rare, there is no reason to assume that ABCs of the feet will respond to treatment or recur any differently from ABCs that occur elsewhere in the body. Surgical curettage is sufficient to treat most ABCs of the feet, including the calcaneum. [14]

Despite a favorable outcome of ABCs with an overall cure rate of 90-95%, [15] one of the most common problems encountered during management is frequent recurrence. The incidence of recurrence has been noted to vary between 59% in cases treated with intralesional excision [16] and 0% in cases with resection. Recurrence usually happens within the first year after surgery, and almost all episodes occur within 2 years. [17] Therefore, a patient of ABC needs to be observed for at least this period of time to exclude any recurrence. It is beneficial to detect recurrence early when the lesion is still small and easier to treat.

To conclude, ABC of the calcaneum is an extremely uncommon entity. Proper diagnosis entails correlating the clinical presentation, anatomical location, radiological profile, and histopathological appearance. This is imperative not only to exclude other more common histological mimics, but also for choosing the appropriate therapeutic regimen and prognosticating the disease outcome.

In a case of calcaneal cystic lesion, ABC should be considered as one of the differential diagnosis. Hence histological diagnosis is essential. Curettage and bone grafting is a valuable option.

References

1. Clayer M. Injectable form of calcium sulphate as treatment of aneurysmal bone cysts. ANZ J Surg 2008 78(5): 366-370.
2. Segall L, Cohen-Kerem R, Ngan B Y, Forte V. Aneurysmal bone cysts of the head and neck in pediatric patients: A case series. Int J Pediatr Otorhinolaryngol 21 2008: epub ahead of print.
3. Burch S, Hu S, Berven S. Aneurysmal bone cysts of the spine. Neurosurg Clin N Am 2008 19(1): 41-47.
4. Brastianos P, Gokaslan Z, McCarthy E F. Aneurysmal bone cysts of the sacrum: a report of ten cases and review of the literature. Iowa Orthop J 2009 29: 74-78.
5. Sun Z J, Zhao Y F, Yang R L, Zwahlen R A. Aneurysmal Bone Cysts of the Jaws: Analysis of 17 Cases. J Oral Maxillofac Surg Jan 26 2010 (Medline).
6. Jaffe H L, Lichtenstein L. Solitary unicameral bone cyst with emphasis on the roentgen picture, the pathologic appearance and the pathogenesis. Arch Surg 1942 44: 1004-1025.
7. Campanacci M, Capanna R, Picci P. Unicameral and aneurysmal bone cysts. Clin Orthop 1986 204: 25-36.
8. Enneking WF. Aneurysmal bone cyst. In: Musculoskeletal tumor surgery. New York: Churchill Livingstone, 1983; 1513-29.
9. Duke Orthopaedics: Wheeless’ Textbook of Orthopaedics, Aneurysmal Bone Cyst, Online article, Jan 2007.
10. Anand MK, Wang EA. Aneurysmal Bone Cyst. eMedicine, Jan 2007.
11. Unni KK, Inwards YC. Conditions that normally simulate primary neoplasms of the bone. In: Unni K K, Inwards Y C, editors. Dahlin’s Bone Tumors. 6th edition. Philadelphia: Lippincott Williams and Wilkins; 2010. p. 305-80.
12. Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg 2007 127(2): 105-114
13. Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR 1995 164: 573-580.
14. Chowdhry M, Chandrasekar CR, Mohammed R, Grimer RJ. Curettage of aneurysmal bone cysts of the feet. Foot Ankle Int. 2010 31(2): 131-135.
15. Marcove RC, Sheth DS, Takemoto S, Healey JH. The treatment of aneurysmal bone cyst. Clin Orthop Relat Res 1995 311: 157-163.
16. Schreuder HW, Veth RP, Pruszczynski M, Lemmens JA, Koops HS, Molenaar WM. Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting. JBJS 1997 79B (1): 20-25.
17. Rastogi S, Varshney M K, Trikha V, Khan SA, Choudhury, Safaya BR. Treatment of aneurysmal bone cysts with percutaneous sclerotherapy using polidocanol. A review of 72 cases with long-term follow-up. JBJS 2006; 88B (9): 1212-1216.


Address correspondence to: Dr. Prasad Soraganvi, Dept of Orthopaedics and Traumatology, Meenakshi Mission Hospital and Research Centre, Lake Area, Melur Road, Madurai- 625107, India.

1 Consultant, Dept of Orthopaedics and Traumatology, MMHRC, Madurai.
2 Consultant, Dept of Orthopaedics and Traumatology, MMHRC, Madurai.
3 Senior Resident, Dept of Orthopaedics, DMH, Madurai.

© The Foot and Ankle Online Journal, 2011

Chondromyxoid Fibroma of the Foot: An Uncommon Presentation

by Anil Thomas Oommen, MS (Ortho), DNB (Ortho)1 , Vrisha Madhuri, MS (Ortho), MCh (Ortho)2 , Noel Malcolm Walter, MD3

The Foot & Ankle Journal 2 (1): 2

We report a chondromyxoid fibroma of the proximal phalanx of the left third toe in a 35 year old woman, a rare site for this tumor. The radiological appearance of a trabeculated lytic lesion with sclerotic margins and soft tissue extension raised the possibility of other entities such as intraosseous epidermoid cyst, aneurysmal bone cyst, chondroblastoma, osteoid osteoma and chondrosarcoma as well as infections like pyogenic, tuberculous and leprous dactylitis. However the typical microscopic picture of lobules of cartilage separated by fibrocellular tissue and scattered osteoclasts confirmed the diagnosis. The tumor was successfully treated by curettage and bone grafting using a dorsal and plantar 2 incision technique and has no recurrence at 4 year follow up. In this article we present our experience with this case and discuss the differential diagnosis of a solitary lytic lesion in the phalanges of the toes.

Key words: Foot, chondromyxoid fibroma, cartilaginous tumor, lytic lesion toe, curettage

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)

Accepted: December, 2008
Published: January, 2009

ISSN 1941-6806
doi: 10.3827/faoj.2009.0201.0002

A 35 year old housewife presented with a swelling on the dorsal aspect of the right third toe which had been present for 10 years with gradual increase in size. On examination an ovoid swelling measuring 1.5 x 1.5cm and extending from the metatarso-phalangeal joint up to the middle of the toe was noted. There was fullness on the plantar aspect of the third toe which extended to the web space on either side with the two adjacent toes being pushed aside as a result. Radiological examination revealed a trabeculated lytic lesion with sclerotic margins involving half the length of the proximal phalanx of the third toe. ( Fig. 1 )

cmffig1

Figure 1  Eccentric expansile well defined lytic lesion showing intra lesional trabeculae and  thinned medial cortex of the proximal phalanx of the third toe. Soft tissue swelling around the base of 3rd toe and widening of the webspaces is noted.

A needle biopsy was reported as benign cartilaginous tumor. Through a combined dorsomedial and plantar approach the soft tissue component was excised. The tumor was thoroughly curetted after making a window over the dorsomedial aspect of the proximal phalanx. The plantar approach was added to ensure complete clearance of soft tissue extension which was predominantly on the plantar aspect. The cavity was then filled with cancellous bone graft harvested from the ipsilateral proximal tibia.

Microscopic evaluation of the curetted tissue revealed a tumor composed of lobules of cartilage separated by fibrocellular tissue. (Fig. 2) Osteoclast-like cells were scattered along the interface between these tissues.(Fig. 3) There was no histological evidence of malignancy. At 4 years follow up there is no recurrence. (Figs. 4,5 )

cmffig2

Figure 2 Lobules of cartilage separated by fibrocellular tissue. (HPE H&E x 100)

cmffig3

Figure 3  Osteoclast like cells along the edge of chndromyxoid areas. (HPE H&E x 200)

cmffig4

Figure 4  Radiograph 4 years post op AP shows sclerosis and healed lesion.

cmffig5

Figure 5  Oblique radiograph showing healed lesion 4 years post op.

Discussion

Chondromyxoid fibroma (CMF) is a rare neoplasm constituting less than 1% of all bone tumors. [1,2] It is derived from skeletal connective tissue cells which demonstrate the capacity to produce chondro-myxoid matrix in a distinctive histological pattern. The peak incidence is in the second and third decades of life. The most common bones affected are those of the lower extremities and in only 5% of cases are the toes involved. [2]

CMF in the long bones is usually metaphyseal and eccentrically located with well defined sclerotic margins on radiological evaluation. Lesions in the small bones are osteolytic with scalloped margins, an appearance which overlaps with Non-Ossifying Fibroma. Calcification within the lesion is visible in most cases. [3,4] There is attenuation, expansion or erosion of the overlying cortex. It usually occurs in the metaphyseal side of the growth plate which is situated proximally in the phalanges.

Intra-medullary tumours and tumour-like lesions of the toe phalanges are rare. Among benign lesions enchondroma is probably the most common and may be clinically and radiologically indistinguishable from CMF. [5]

The diagnosis can usually be made by biopsy because CMF is mostly myxoid and often has osteoclastic giant cells whereas enchondroma is generally more obviously cartilaginous and lacks giant cells. However a small biopsy may not have enough tissue for this distinction and in such instances the only diagnosis possible may be “benign cartilaginous tumour” as in this case. Other benign tumours or tumour-like lesions which may rarely occur in the toes include aneurysmal bone cyst, the closely related giant cell reparative granuloma, and true giant cell tumour all of which are histologically quite different from CMF. [5]

Chondroblastoma can be partially or largely similar to CMF microscopically but is virtually unknown in the toes. [2] Among malignant bone tumours chondrosarcoma can be histologically difficult to distinguish from CMF. For making this differentiation the x-ray is critical as the radiological appearance of chondrosarcoma is aggressive, unlike that of CMF. The toes are also an extremely uncommon site for chondrosarcoma.

Other than benign cartilage lesions, a few other tumors of the terminal digits should be considered in the differential diagnosis. A radiological diagnosis of intraosseous epidermoid cyst can be made in the presence of perilesional sclerosis with painless swelling. [5] A painful lesion with perifocal reactive sclerosis is suggestive of an osteoid osteoma and is easily distinguishable radiologically. [5]

Poorly defined lytic lesions can be associated with several pseudotumorous conditions such as osteomyelitis caused by Staphylococcus seen in diabetic patients. [5] Tuberculous and leprous dactylitis are other condition which are clinically seen as phalangeal lesions. [6] Radiologically the tuberculous lesion is central, lytic, cystic and expansive, and soft tissue extension may be seen. [5] These are easily distinguished histologically.

As our report shows, a needle biopsy is of great value in suggesting the diagnosis and can easily be carried out as an outpatient procedure. Conservative surgical treatment, such as curettage and bone grafting, appears to be ideal for CMF.

In conclusion, a wide range of entities, including CMF, needs to be considered when confronted with a lytic lesion in the toe phalanx. Radiological features may help in distinguishing these conditions. Histopathology and microbiology provide a definitive diagnosis. A thorough surgical clearance is required to avoid recurrence in CMF.

References

1. Fletcher CDM, Unni KK, Mertens F (eds). Pathology and Genetics of Tumours of Soft Tissue and Bone, WHO Classification of Tumours, IARC Press, Lyon: 243 – 245, 2002.
2. Wu CT, Inwards CY, O’Laughlin S, Rock MG, Beabout JW, Unni KK. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol 29 (5): 438 – 446, 1998.
3. Schajowicz F , Gallardo H .Chondromyxoid fibroma (fibromyxoid chondroma) of bone. A clinico-pathological study of thirty-two cases.J Bone Joint Surg. 53B (2): 198-216,1971.
4. Sharma H.,Jane M J, Reid R. Chondromyxoid fibroma of the foot and ankle: 40 years’ Scottish bone tumour registry experience. Int Orthop. 30(3): 205 – 209, 2006.
5. Wang BY, Eisler J, Springfield D, Klein MJ. Intraosseous Epidermoid Inclusion Cyst in a Great Toe. A Case Report and Review of the Literature. Arch Pathol Lab Med. 27 (7): 298 – 300, 2003.
6. Olivieri .I.,Scarano E,Padula A,Giasi V,Priolo F. Dactylitis,a term for different digit diseases. Scand J Rheumatol. 35(5): 333 – 340, 2006.


Address correspondence to:Dr Anil Thomas Oommen MS(Ortho)Assistant Profressor,
Dept. of Orthopaedics, Unit 2
Christian Medical College Ida Scudder Road,Vellore 632 004.
 

1 Assistant Profressor, Department of Orthopaedics, Unit 2
Christian Medical College Ida Scudder Road,Vellore 632 004.
2 Profressor, and Head , Department of Orthopaedics, Unit 2
Christian Medical College Ida Scudder Road,Vellore 632 004.
3 Profressor, Department of Pathology,
Christian Medical College Ida Scudder Road,Vellore 632 004.

© The Foot & Ankle Journal, 2009