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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 123-125

Chondrosarcoma of the distal phalanx of the right great toe: Report of a rare malignancy and review of literature


Department of Pathology, Medical College, Kolkata-73, West Bengal, India

Date of Web Publication19-Apr-2012

Correspondence Address:
Santosh Kumar Mondal
"Teenkanya Complex," R.N. Guha Road, Dumdum, Kolkata - 28, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.95191

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 > Abstract 

Foot is an uncommon site for chondrosarcoma and involvement of phalanges is extremely rare. Here, a case of low-grade chondrosarcoma of the distal phalanx of the right great toe in a 37-year-old male patient is being reported. The patient complained of gradual swelling of the right great toe and pain for last 2 years. X-ray showed a lytic lesion destroying the distal phalanx with soft tissue extension. Amputation of the great toe was done and specimen was sent for histopathological examination. Microscopical examination revealed grade 1 chodrosarcoma infiltrating the surrounding soft tissue and muscle. The patient is on 1-year follow-up which is uneventful.

Keywords: Chonrosarcoma, feet, phalanx


How to cite this article:
Mondal SK. Chondrosarcoma of the distal phalanx of the right great toe: Report of a rare malignancy and review of literature. J Can Res Ther 2012;8:123-5

How to cite this URL:
Mondal SK. Chondrosarcoma of the distal phalanx of the right great toe: Report of a rare malignancy and review of literature. J Can Res Ther [serial online] 2012 [cited 2019 Sep 15];8:123-5. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/123/95191

Primary chondrosarcoma (CHS) is the third most common primary malignancy of bone after myeloma/ plasmacytoma and osteosarcoma, and accounts for 20% of malignant bone tumors in a large series. [1] The most common skeletal sites are pelvic bones, followed by proximal humerus, distal femur, and ribs. The small bones of hands and feet are rarely affected (<1% of all CHS). [2]

Benign chondroid neoplasms like enchondroma and osteochondromas are common bone tumors affecting hands and feet. As many as 54% of the bone tumors of hands are cartilaginous, < 2% of which are malignant. When a tumor occurs in feet, involvement of toes and especially distal phalanx is extremely rare. [3] Not much is known about the biologic behavior of phalangeal CHS, although a favorable prognosis is suggested. Phalangeal CHS behaves like a locally aggressive lesion in which, in contrast to CHS located elsewhere, metastasis is uncommon.

A 37-year-old male patient presented with pain in right great toe with gradual local swelling for 2 years. Pain was of dull boring type and remained throughout the day. Physical examination revealed a swollen right great toe with a drumstick appearance. The swelling was firm to hard in consistency and tender on palpation. The swelling had irregular and ill-defined margins. Movements were restricted at the interphalangeal joint but normal at the intertarsophalangeal joint. No inguinal lymphadenopathy was detected.

X-ray (antero-posterior and lateral view) revealed a bony lytic lesion involving the distal phalanx of the right great toe. Cortical destruction was present along with soft tissue extension. Periosteal reaction was absent [Figure 1]. All routine hematological, biochemical investigations and chest x-ray were within normal limits. The surgeon took the decision of excision of the great toe at the metatarso-phalangeal joint after discussion with the patient. The specimen was sent for histopathologic examination. On gross examination, a glistening tumor measuring 3.5×3 cm was found having marked myxoid changes [Figure 2]. However, overlying skin was unremarkable. Microscopical examination revealed a chondroid neoplasm with prominent chondroid stroma in a lobulated pattern. Myxoid changes were present. The tumor cells had mild nuclear pleomorphism and atypia with occasional binucleated cells. No mitosis or necrosis was found. The tumor involved the surrounding soft tissue and skeletal muscle [Figure 3] and [Figure 4].
Figure 1: X-ray showing a bony lytic lesion involving the distal phalanx of right great toe. Cortical destruction along with soft tissue extension was present

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Figure 2: Gross photograph of the right great toe showing a well circumscribed glistening tumor with myxoid changes

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Figure 3: Photomicrograph showing a chondroid neoplasm with prominent chondroid stroma in a lobulated pattern (arrow). Myxoid changes were also present. The tumor involved the surrounding soft tissue and skeletal muscle. [H&E stain, × 100]

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Figure 4: Photomicrograph showing tumor cells with mild nuclear pleomorphism and occasional binucleated cells (arrow). No nuclear atypia, mitosis or necrosis was found. [H&E stain, × 400]

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The patient was put in foot orthosis during the postoperative period and he recovered well. There was no local or distant recurrence afterward and 1-year follow-up period was uneventful.

Phalangeal CHS are rare neoplasms and little is known about the biologic behavior of chondrosarcoma (CHS) of phalangeal bones. Ogose et al. [4] reviewed 163 CHS located in the phalangeal, (meta) carpal, and (meta) tarsal bones of the hands (n=88) and feet (n=75) and suggested that these tumors have the potential to be fatal. CHS of the calcaneus and the talus were more likely to metastasize. [4] Kinoshita et al. reviewed 83 cases of bone and soft tissue tumors of the foot. They found 33 benign tumors, one primary CHS and 2 metastatic bone tumors. [5] For the only malignant bone tumor (CHS of left talus) below-knee amputation was performed but the patient (52-year old man) died because of pulmonary metastasis. [6] Patil et al. studied 12 CHS of the bones of feet, of which 4 tumors affected the tarsal bones and the rest involved the short tubular bones. The mean age of the patients was 52.3 years (range 17--83 years) and men were predominantly affected. [6] A tabulation of previous studies and summary of the literature have been listed in [Table 1].
Table 1: Summary of review of literature on chondrosarcoma of bones of feet and hands

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CHS are graded according to the system of Evans et al. [7] Histologic parameters for grading include cellularity (high/moderate/low), binucleated cells (<1/1--5/>5 per high power field), cellular distribution (regular/irregular), nuclear pleomorphism (low/moderate/high), bone formation (absent/focal/diffuse), differentiation (percentage chondroid, mucoid and myxoid differentiation), calcification (absent/focal/diffuse) and cortical destruction (absent/present/unobservable). [8] In a series of 35 cases of phalangeal CHS, Bovee et al. reported 8 grade 1, 26 grade 2, and 1 grade 3 CHS. Median age of phalangeal CHS was 67 years (21--37 years) with a slight predilection for females (21 vs.14). The median duration of the disease was 18 months (range 1-480 months). [2] But the present case was a male patient of age only 37 years with a disease duration of 2 years (24 months). In this case the greatest diameter of the tumor was 3.5 cm which corroborates the median diameter of 3 cm found by Bovee et al. [2] Though nuclear pleomorphism, binucleation, and cellularity were not high in the present case, but cortical destruction and soft tissue extension were obvious. These findings are important to differentiate grade 1 CHS from enchondroma, because enchondroma of the hands and feet display greater cellularity and mild nuclear atypia than is seen elsewhere. [2],[4]

Phalangeal CHS occurs far more frequently in the hand compared with the foot and majority of the cases are located in the proximal phalanx. [2] In contrast to CHS located elsewhere, phalangeal CHS appears to have female preference. Furthermore, the median age (67 years) was higher than for CHS in general (grade 1:44 years, grade 2:55 years). [2]

Although for all grades and subtypes of nonmetastatic CHS, complete surgical treatment only offers the chance for cure, the most optimal type of surgical management is still debated. Wide, en bloc excision is the preferred surgical treatment of intermediate and high grade CHS. [9] For the low-grade/grade 1 CHS, some prefer extensive intralesional curettage followed by local adjuvant treatment, for example, phenolization or cryosurgery (liquid nitrogen) and filling the cavity with bone graft. [10] Below-knee amputation was also advocated by some authors. In our case excision of great toe at the metatarso-phalangeal joint was done and 1-year follow-up was uneventful.

In conclusion, phalangeal CHS behaves as locally aggressive lesion and rarely metastasize. Curettage with local adjuvant and adequate follow-up can be tried as the first-line management, especially where amputation would lead to significant loss of function like in hands.

 
 > References Top

1.Unni KK. Dahlin's Bone Tumors: General aspects and Data on 11,087 cases. 5 th ed. Philadelphia: Lippincott-Raven; 1996.  Back to cited text no. 1
    
2.Bovee JV, van der Huel RO, Tamminiau AH, Hogendoorn PC. Chondrosarcoma of the phalanx: A locally aggressive lesion with minimal metastatic potential. A report of 35 cases and a review of the literature. Cancer 1999;86:1724-32.  Back to cited text no. 2
    
3.Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP imaging of primary chondrosarcoma: Radiologic-Pathologic correlation. Radiographics 2003;23:1245-78.  Back to cited text no. 3
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4.Ogose A, Unni KK, Swee RG, May GK, Rowland CM, Sim FH. Chondrosarcoma of small bones of the hands and feet. Cancer 1997;80:50-9.  Back to cited text no. 4
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5.Kinoshita G, Matsumoto M, Maruoka T, Shiraki T, Tsunemi K, Futani H, et al. Bone and soft tissue tumors of the foot: Review of 83 cases. J Orthop Surg (Hong Kong) 2002;10:173-8.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Patil S, de Silva MV, Crossan J, Reid R. Chodrosarcomas of the bones of the feet. J Foot Ankle Surg 2003;42:290-5.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of bone. A clinicopathologic analysis with emphasis on histologic grading. Cancer 1977;40:818-31.  Back to cited text no. 7
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8.Gelderblom H, Hogendoorn PC, Dijkstra SD, van Rijswijk CS, Krol AD, Taminiau AH, et al. The clinical approach towards chondrosarcoma. Oncologist 2008;13:320-9.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Leerapun T, Hugate RR, Inwards CY, Scully SP, Sim FH. Surgical management of conventional grade 1 chondrosarcoma of long bones. Clin Orthop Relat Res 2007;463:166-72.  Back to cited text no. 9
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10.Veth R, Schreuder B, van Been H, Pruszczynski M, de Rooy J. Cryosurgery in aggressive, benign and low-grade malignant bone tumors. Lancet Oncol 2005;6:25-34.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]


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