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Year 2020

Volume: 7 , Issue: 1

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Janaki, Kavitha A, and Sindhura: Extra skeletal myxoid chondrosarcoma: A rare case report


Case Report

Clinical features

82 year old male patient complains of pain and swelling in right elbow and since 31/2 months. Swelling was progressive in size. On examination swelling of size 3.5x3cm was located in right elbow, tenderness, inflammation and mild restriction of movement noted. Right elbow X-ray was taken which was reported as normal. Lesion was excised and sent for histopathological examination.

Pathology

Gross

Received grey white cystic soft tissue measuring 3x2.5x1.5 cm. Cut surface shows multiple cystic spaces filled with grey white gelatinous material.

Microscopy

H/E Section showed a neoplasm composed of multiple lobules showing extensive myxoid area with tumor cells arranged in sheets and trabecular pattern having round to oval nuclei showing moderate pleomorphism and eosinophilic cytoplasm. Increase in mitosis noted. It was reported as extra skeletal myxoid chondrosarcoma.

To confirm following panels of immunohistochemistry was employed which includes S100, Vimentin, NSE, Synaptophysin, CK and EMA. Of which vimentin showed positivity. All other markers were negative.

Figure 1

Section shows a cystic space with tumor cells and myxoid areas (LP)

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Figure 2

Tumor cells arranged in sheets with round to oval nuclei showing moderate pleomorphism and eosinophilic cytoplasm

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/1ee7df70-de7d-411c-b250-f9dea14db1c9/image/55ca6f6e-1a77-4a84-afa0-f346a33b596e-u2.png

Figure 3

Section shows tumor cells with extensive myxoid changes

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Figure 4

Section shows strong positivity with vimentin

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Discussion

EMC is an uncommon soft tissue sarcoma with chondroblastic origin but positivity of neuroendocrine or neural markers suggest likely neuroendocrine or neural differentiation in some Extraskeletal myxoid chondrosarcoma.3,4 Regardless of the name there is no substantial indication of cartilaginous differentiation.

It mostly occurs in adults, about 54 years. Males are affected more than female.5 It is more common in extremities.3 Occasionally occur in foot, trunk, head and neck and paraspinal, and region.6

Clinical features vary depending on their locations, EMC tumors present with nerve compression symptoms.7 Swelling, decreased mobility, pain tenderness.6

Radiologically shows an soft tissue mass with clear border and even density. The mass will have an lobulated appearance, and in T1WI it is hypoechoeic to muscle, but on T2WI hyperechoeic, and it is separated into several lobules by numerous septa which appears hypoechoeic.7

Grossly it is an Large mass with psuedocapsule, C/S- shows an well-defined multiple nodules with gelatinous areas separated by fibrous septa.

Microscopy shows a multinodular architecture with malignant chondroblast like cells arranged in clusters or cords in a myxoid matrix. There is no definite cartilaginous differentiation.

The neoplastic cells have moderate eosinophilic, finely granular to vacuolated cytoplasm and uniform round to oval nuclei and inconspicuous nucleoli. Mitotisis usually low.

Cellular variant – shows epithelioid cells with high mitosis and geographic necrosis along with that conventional EMC noted.

It is essential to differentiate EMC from other myxoid tumors and extraskeletal mesenchymal chondrosarcoma Immunohistochemistry shows reliable positivity for vimentin and shows positivity for SIOO, CK, EMA focally. Few cases shows positivity for NSE, Synaptophysin.5

Translocation t (9;22)(q22;q12) consistently has been identified in EMC.8,9

Conclusion

EMC shows increased incidence of tumor recurrence and distant spread in spite of a prolonged clinical course.10 The only therapeutic option for EMC is early wide local resection with or without radiation for localized disease.11 MRI alone cannot distinguish EMC from common extraskeletal chondrosarcoma. Therefore, diagnosing EMC with both CT and MRI scan is suggested.2

Source of funding

None.

Conflict of interest

None.

References

1 

Kawaguchi Satoshi Takuro Wada Satoshi Nagoya Extraskeletal Myxoid Chondrosarcoma A Multi-Institutional Study of 42 Cases in JapanCancer2003975

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Ling Zhang Ruoning Wang Rong Xu Genggeng Qin Lei Yang Extraskeletal Myxoid Chondrosarcoma: A Comparative Study of Imaging and PathologyBioMed Res Int201896842689684268

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S W Weiss Ultrastructure of the so-called chordoid sarcoma. Evidence supporting cartilaginous differentiationCancer197637300306

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S Okamoto M Hisaoka T Ishida Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 18 casesHum Pathol20013211161124

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J M Meis-Kindblom P Bergh B Gunterberg L Kindblom Extraskeletal Myxoid ChondrosarcomaAm J Surg Pathol1999236636650

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D M Christopher K Fletcher Fredrik Krishnan Unni Fredrik Mertens Pathology and genetics World health organization classification of tumors of soft tissue and boneLyon2002

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G W Zhang A J Wang G H Zhang S N Zhao J L Qu Extraskeletal myxoid chondrosarcoma: A report of two casesOncol Letters20147412891291

8 

G Stenman H Andersson N Mandahl J M Meis-Kindblom L G Kindblom Translocation t(9;22)(q22;q12) is a primary cytogenetic abnormality in extraskeletal myxoid chondrosarcomaInt J Cancer199562398402

9 

N P Agaram Y S Zhang S Sung C R Singer - Antonescu Extraskeletal myxoid chondrosarcoma with non-EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphologyHuman Pathol201445510841091

10 

G Saleh H L Evans J Y Ro A G Ayala Extraskeletal myxoid chondrosarcoma. A clinicopathologic study of ten patients with long-term follow-upCancer19927028272830

11 

S Kawaguchi T Wada S Nagoya Extraskeletal myxoid chondrosarcoma: a multi-institutional study of 42 cases in JapanCancer20039712851292



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