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Review
. 2015 Aug 1;8(8):9655-61.
eCollection 2015.

Recurrent retroperitoneal extra-GIST with rhabdomyosarcomatous and chondrosarcomatous differentiations: a rare case and literature review

Pengcheng Zhu  1 , Yan Fei  2 , Yue'e Wang  1 , Qilin Ao  1 , Guoping Wang  1
Affiliations
Review

Recurrent retroperitoneal extra-GIST with rhabdomyosarcomatous and chondrosarcomatous differentiations: a rare case and literature review

Pengcheng Zhu et al. Int J Clin Exp Pathol. .

Abstract

Selective tyrosine kinase inhibitor (TKI) targeting KIT and PDGFRA is the frontline therapy for metastatic and unresectable GIST patients. Some initially responsive patients experience tumor progress because of secondary drug resistance, and some cases can develop heterogeneous differentiation. Here we report a rare case of recurrent retroperitoneal extra-GIST with rhabdomyosarcomatous and chondrosarcomatous differentiation with TKI therapy after surgical tumorectomy. Histology, immunohistochemistry, and mutational analysis were performed on primary and recurrent samples. The current case represents the first report of a recurrent retroperitoneal extra-GIST harboring mixed morphologic phenotypes of rhabdomyosarcoma and chondrosarsoma after TKI treatment. The dual differentiation can represent diagnostic pitfall.

Keywords: Extragastrointestinal stromal tumor; chondrosarcoma; heterogeneous differentiation; rhabdomyosarcom; tyrosine kinase inhibitor.

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Figures

Figure 1
Figure 1
Primary tumor specimen of retroperitoneal extra-GIST shows spindle-shaped cells of a high dense cellularity, hyperchromasia, and nuclear atypia, and a high mitotic activity (A. ×100; B. ×400).
Figure 3
Figure 3
Recurrent tumor showing classic morphology of extra-GIST (A. ×100) and heterogeneous rhabdomyosarcomatous and chondrosarcomatous differentiation (B. ×100; C, D. ×200).
Figure 2
Figure 2
Primary tumor of retroperitoneal extra-GIST was confirmed by immunopositivity for CD117and DOG1 with high Ki-67 labeling index, but immunonegativity for CD34.
Figure 4
Figure 4
Recurrent tumor with dual transdifferentiation was confirmed by immunopositivity for CD117 in classic GIST area, desmin, myogenin in rhabdomyosarcomatous area and S-100 in chondrosarcomatous area.
See this image and copyright information in PMC

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