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Primary clear cell sarcoma of the calcaneus

Report of a rare case and review of the literature

Primäres Klarzellsarkom des Kalkaneus

Bericht eines seltenen Falls und Literaturreview

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Abstract

Clear cell sarcoma (CCS) is a rare soft tissue tumor derived from neural crest cells. It accounts for 1% of all soft tissue sarcomas and usually involves the tendons and aponeuroses in the distal ends of the extremities. The foot and the ankle are the most common primary sites. A CCS predominantly occurs in young adults and has rarely been reported in children and older people. On magnetic resonance imaging the tumor has a benign appearance. It appears isointense, hypointense or slightly hyperintense on T1-weighted images. A CCS is typically strongly positive for HMB-45, S‑100, melanA, MITF and vimentin on immunohistochemical investigations and exhibits the t(12;22)(q13;q12) translocation on cytogenetic studies. Metastasis occurs mainly to the regional lymph nodes. A tumor size ≥5 cm is invariably associated with a poor prognosis. The main treatment is wide excision but local recurrence and distant metastases are common. This article presents the case of a 61-year-old man with right ankle tenderness and malaise. Ultrasound-guided biopsy and pathological examination were performed and the pathological diagnosis was a CCS. The patient underwent a below-knee amputation and had no local recurrence or metastasis at the 6‑month follow-up.

Zusammenfassung

Das Klarzellensarkom (CCS) ist ein seltener Weichgewebstumor, der sich aus Neuralleistenzellen entwickelt. Es macht 1 % aller Weichgewebssarkome aus, und in der Regel sind die Sehnen und Aponeurosen am distalen Ende der Extremitäten beteiligt. Der Fuß und das Sprunggelenk sind die am häufigsten betroffenen Lokalisationen. Ein CCS kommt überwiegend bei jungen Erwachsenen vor und wurde nur selten bei Kindern oder älteren Menschen berichtet. In der Magnetresonanztomographie (MRT) zeigt der Tumor ein benignes Erscheinungsbild. Er erscheint isointens, hypointens oder leicht hyperintens in der T1-gewichteten Sequenz. In den immunhistologischen Untersuchungen reagiert das CCS typischerweise stark positiv auf HMB-45, S‑100, melanA, MITF und Vimentin und exhibiert die t(12;22)(q13;q12)-Translokation in zytogenetischen Studien. Metastasen kommen hauptsächlich in den regionalen Lymphknoten vor. Eine Tumorgröße von ≥5 cm ist unweigerlich mit einer schlechten Prognose vergesellschaftet. Die Hauptbehandlung ist eine weite Exzision, aber Lokalrezidive und Fernmetastasen treten häufig auf. Dieser Beitrag stellt den Fall eines 61-jährigen Patienten mit Schmerzen am rechten Sprunggelenk und Unwohlsein vor. Eine ultraschallgesteuerte Biopsie sowie eine pathologische Untersuchung wurden durchgeführt; es wurde die pathologische Diagnose eines CCS gestellt. Der Patient unterzog sich einer Amputation unterhalb des Knies und hatte kein Lokalrezidiv oder Metastasen beim 6‑Monats-follow-up.

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Abbreviations

ATF1:

Activating transcription factor 1

BPA-Fr:

P-borono-L-phenylalanine-fructose complex

CCS:

Clear cell sarcoma

CK:

Cytokeratin

CREB1:

cAMP responsive element binding protein 1

EMA:

Epithelial membrane antigen

EMPNST:

Epithelioid malignant peripheral nerve sheath tumor

EWSR1:

Ewing sarcoma breakpoint region 1

HGF/c-MET:

Hepatocyte growth factor/Cellular-met

HMB:

Human melanoma black

ILP:

Isolated limb perfusion

INI-1:

Integrase interactor 1

KIT:

CD117

LCA:

Leukocyte common antigen

MITF:

Melanogenesis associated transcription factor

MRI:

Magnetic resonance imaging

SyS:

Synovial sarcoma

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Acknowledgements

The present study was supported by the Health and Family Planning Commission of Jilin Province (grant number 2015Z031) and the Science and Technology Department of Jilin Province (grant number 20190304039YY).

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Correspondence to Wei Feng.

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X. Zhe, S. Haiqiang, Z. Yao and F. Wei declare that they have no competing interests.

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For images or other information within the manuscript which identify patients, informed consent was obtained from them and/or their legal guardians.

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Xu, Z., Suo, H., Zhang, Y. et al. Primary clear cell sarcoma of the calcaneus. Orthopäde 48, 232–238 (2019). https://doi.org/10.1007/s00132-018-03683-5

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