Gastric carcinosarcoma with rhabdomyosarcomatous differentiation: a case report and review
© The Author(s). 2016
Received: 3 March 2016
Accepted: 21 May 2016
Published: 2 June 2016
We report an unusual case of gastric carcinosarcoma with rhabdomyosarcomatous and neudoendocrinal differentiation in a 71-year-old Japanese female. Gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation is a rare tumor. The tumor developed in the body of the stomach and was exophytic in appearance. By histochemical analysis, the tumor was shown a part of positive for desmin and myoglobin and a part of positive for synaphtophysin and vimentin.
We conclude that, though rare, gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation thus is reviewed in the English literatures.
KeywordsGastric carcinosarcoma Rhabdomyosarcomatous Neuroendocrine
Carcinosarcomas are rare, malignant, biphasic tumors. In the upper gastrointestinal tract, they are most frequently observed in the esophagus, while localization in the stomach has been less frequently reported [1–3]. We present a case of a 71-year-old female having gastric carcinosarcoma with rhabdomyosarcomatous and neudoendocrinal differentiation. Rhabdomyosarcomatous differentiation of the stomach is a rare neoplasm with only twelve cases previously reported [4–13].
We reviewed the scientific literature pertaining to gastric rhabdomyosarcoma and identified several distinctive clinical features of this type of tumor.
Immunohistochemical staining was performed using the ordinary avidine-biotin-peroxidase complex, and reaction products were visualized by benzidine reaction. The sections were slightly counterstained by hematoxylin. Immunohistochemically, the tumor cells were positive for AE1/AE3, synaptophysin, desmin, and myogenin (Fig. 2b) but negative for CAM5.2, chromogranin A, S-100 protein, c-kit, and DOG1. PAX3/7-FKHR or EWS-WT1 fusion gene transcripts were not detected by RT-PCR [14, 15].
In the Japanese and English scientific literature, about 60 cases of gastric carcinosarcoma have been reported. The most commonly reported carcinomatous component of this tumor is tubular or papillary carcinoma. The sarcomatous components have still not been adequately characterized in most of the reported cases. Distinct sarcomatous components showing heterologous differentiation such as leiomyosarcoma [4, 5, 16–19], rhabdomyosarcoma [4–13, 20], osteosarcoma [5, 9, 21], and chondrosarcoma [5, 22–24] have been reported. In our patient, not only rhabdomyoblastic differentiation but also neuroendocrine features were observed. This association of rhabdomyosarcomatous and neuroendocrinal differentiation has been described in only five cases of carcinosarcoma, affecting the pancreas , larynx , ileum , nanorectal junction , and stomach . This case is the second report on gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation.
There is no indication that the gastric rhabdomyomatous component of gastric carcinosarcoma in our case or in other case reports represented a from some other site; indeed, metastases to the stomach by rhabdomyosarcomas are uncommon. De la Monte et al. reported that no instances of metastasis to the stomach in a review of 17 autopsies of 22 patients who died of embryonal and alveolar rhabdomyosarcoma at Johns Hopkins Hospital between 1929 and 1983 .
Reported cases of gastric carcinosarcoma with rhabdomyosarcomatous differentiation
Depth of invasion
Other specified histology
Stout (1953) 
Stout (1953) 
Kyogoku (1960) 
Dead, 36 mon
Matsukuma (1977) 
15 × 11
Dead, 5 mon (Liver meta)
Machida (1981) 
7.0 × 6.0
Fox (1990) 
15 × 10
Dead, 26 mon
Sugai (1991) 
9.0 × 7.0
Spindle cell sarcoma
Dead, 5 mon
Melato (1993) 
7.0 × 5.5
20 × 18
7.0 × 6.5
Alive 10 mon
Sato Y (2001) 
8.0 × 7.0
Alive 11 mon
Surg Case report 2016 (In press)
2.0 × 1.5
Alive, 36 mon
The histogenesis of gastric carcinosarcoma remains controversial. Some authors have reported a bioclonal origin, which supports the collision tumor theory [16, 31]. Others have proposed that these tumors are monoclonal and that the sarcomatous elements originate from a common stem cell that has the ability to undergo both epithelial and mesenchymal differentiation [7, 32]. In our patient, there were occasional transitions between carcinomatous and sarcomatous components, and the immunohistochemical detection of stem in sarcomatous cells may suggest the sarcomatous differentiation of adenocarcinoma. During transdifferentiation, the occurrence of stem cells with multi-differentiation ability is capable to explain the variety of cell types observed in the present tumor.
Our experience with the present case emphasizes that gastric carcinosarcoma with rhabdomyosarcomatous differentiation exhibits aggressive behavior, the tumor, however, is extremely rare.
This report described a very rare case of gastric carcinosarcoma with rhabdomyosarcomatous lesions. This case has survived without tumor recurrence, though most cases of gastric carcinosarcoma with rhabdomyosarcomatous were poor prognosis.
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