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.
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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- Kanamoto A, Nakanishi Y, Ochiai A, Shimoda T, Yamaguchi H, Tachimori Y, et al. A case of small polypoid esophageal carcinoma with multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and sarcomatous components. Arch Pathol Lab Med. 2000;124:1685–7.PubMedGoogle Scholar
- Yamazaki K. A gastric carcinosarcoma with neuroendocrine cell differentiation and undifferentiated spindle-shaped sarcoma component possibly progressing from the conventional tubular adenocarcinoma; an immunohisto-chemical and ultrastructural study. Virchows Arch. 2003;442:77–81.PubMedGoogle Scholar
- Insabato L, Di Vizio D, Ciancia G, Oettinato G, Tornillo L, Terracciano L. Malignant gastrointestinal leiomyosarcoma and gastrointestinal stromal tumor with prominent osteoclast-like giant cells. Arch Pathol Lab Med. 2004;128:440–3.PubMedGoogle Scholar
- Kyogoku M, Okukubo T, Aoki S. An autopsy case of carcinosarcoma which originated in the stomach. Gann. 1960;51:278–9.Google Scholar
- Melato M, Bucconi S, Grillo BP, Angelucci P, Stefano PD, Natolic C. Carcinosarcoma and separate neuroendocrine malignant tumor of a malignancy promoter, the gastric stump. Anticancer Res. 1993;13:2485–8.PubMedGoogle Scholar
- Nakayama Y, Murayama H, Iwasaki H, Iwanaga S, Kikuchi M, Ikeda S, et al. Gastric carcinosarcoma (sarcomatoid carcinoma) with rhabdomyoblastic and osteroblastic differentiation. Pathl Int. 1997;47:557–63.View ArticleGoogle Scholar
- Machida T, Takahashi M, Takeda T, Nakamura K. A carcinosarcoma of the stomach. Gan No Rinsho. 1981;27:1763–8.Google Scholar
- Stout AP. Tumors of the stomach. In: Ackerman LV, Lattes R, editors. Atlas of tumor pathology, section 6, fascicle 21. Washington DC: Armed Forces Institute of Pathology; 1953. p. 98–9.Google Scholar
- Matsukuma S, Wada R, Hase K, Sakai Y, Ogata S, Kuwabara N. Gastric stump carcinosarcoma with rhabdomyosarcomatous differentiation. Pathol Int. 1997;47:73–7.View ArticlePubMedGoogle Scholar
- Fox KR, Moussa SM, Mitre RJ, Zidar BL, Raves JJ. Clonical and pathology features of primary gastric rhabdomyosarcoma. Cancer. 1990;66:772–8.View ArticlePubMedGoogle Scholar
- Sugai A, Takayama K, Sasaki K, Kato R, Sasau S, Fujisawa H, et al. A carcinosarcoma of the stomach. Gan No Rinsho. 1991;37:777–83.Google Scholar
- Tsuneyama K, Sasaki M, Sabit A, Yokoi K, Arano Y, Imai T, et al. A case of gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation. Pathology. 1999;195:93–7.Google Scholar
- Sato Y, Shimozono T, Kawano S, Toyoda K, Onoe K, Asada Y, et al. Gastric carcinosarcoma, coexistence of adenosquamous carcinoma and rhabdomyosarcoma: a case report. Histopathology. 2001;39:540–7.View ArticleGoogle Scholar
- Sorensen PH, Lynch JC, Qualman SJ, Tirabosco R, Lim JF, Maurer HM, et al. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the child’s oncology group. J Clin Oncol. 2002;20:2672–9.View ArticlePubMedGoogle Scholar
- Lee SB, Kolquist KA, Nichols K, Englert C, Maheswaran S, Ladanyi M, et al. The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. Nat Genet. 1997;17:309–13.View ArticlePubMedGoogle Scholar
- Dundas SA, Slater DN, Wagner BE, Mills PA. Gastric adenocarcinoleiomyosarcoma: a light electron microscopic and immunohistochemical study. Histopathology. 1988;13:347–50.View ArticlePubMedGoogle Scholar
- Kawabata Y, Nakai T, Ooba K, Okumura T, Matsuura Y, Miyazaki Y, et al. A case report of carcinosarcoma of the stomach. Nippon Shoukaibyo Gakkai Zasshi. 1993;26:2180–93 (in Japanese with English abstract).View ArticleGoogle Scholar
- Miyauchi K, Takano Y, Ohshima Y, Shimizu M, Watanabe T, Nishi Y, et al. A case of carcinosarcoma of the stomach. Nippon Syokakibyo Gakkai Zasshi. 1994;91:75–9 (in Japanese with English abstract).Google Scholar
- Tanimura H, Furuta M. Carcinosarcomas: current perspectives and an historical review of nosological concepts. Semin Diagn Pathol. 1993;10:118–27.Google Scholar
- Pinto JA, Gonzalez-Alfonso JE, Gonzalez L, Gonzalez L, Stevenson N, et al. Well differentiated gastric adenocarcinoma with rhabdoid areas: a case report with immunohistochemical analysis. Pathol Res Pract. 1997;193:801–8.View ArticlePubMedGoogle Scholar
- Ayral X, Cornud F, Favriel JM, Bocquet L, Debray C. Carcinosarcoma of stomach: report of a case and review of the literature. Gastroentrol Clin Biol. 1980;4:362–7.Google Scholar
- Cho KJ, Myong NH, Choi DW, Jang JJ. Carcinosarcoma of stomach: a case report with light microscopic study. APMIS. 1990;98:991–5.View ArticlePubMedGoogle Scholar
- Minamoto T, Okada Y, Nakanishi I, Sawasaki K. So call gastric carcinosarcoma: a case of chandrosarcomatous dysdifferentiated in the metastatic foci. Gan No Rinsho. 1984;30:1321–6.PubMedGoogle Scholar
- Seigel A, Freund U, Gal R. Carcinosarcoma of the stomach. Histopathology. 1988;13:350–3.View ArticleGoogle Scholar
- Ferreiro J, Lewin K, Herron R, Bhuta S. Malignant islet cell tumor with rhabdomyosarcomatous differentiation. Am J Surg Pathol. 1989;13:422–7.View ArticlePubMedGoogle Scholar
- Doglioni C, Ferlito A, Chimenti C, Viale G, Rosai J. Laryngeal carcinoma showing multidirectional epithelial neuroendocrine and sarcomatous differentiation. ORL J Otorhinolaryngol Relat Spec. 1990;52:316–26.View ArticlePubMedGoogle Scholar
- Dikman SH, Toker C. Enterblastoma complicating regional enteritis. APMIS. 1990;98:991–5.View ArticleGoogle Scholar
- Roncaroli F, Montironi R, Felicotti F, Losi L, Eusebi V. Sarcomatoid carcinoma of the anorectal junction with neuroendocrine and rhabdomyoblastic features. Am J Surg Pathol. 1995;19:217–23.View ArticlePubMedGoogle Scholar
- de la Montre SM, Hutchins GM, Moore GW. Metastatic behavior of rhabdomyosarcoma. Pathol Res Prat. 1986;181:148–52.View ArticleGoogle Scholar
- Enzinger FM, Weiss SW. Soft tissue tumors, vol. 49. 2nd ed. St. Louis: Mosby-year Book; 1988. p. 177–87.Google Scholar
- Bansal M, Kaneko M, Gordon RE. Carcinosarcoma and separate carcinoid tumor of the stomach: a case report with light and electron microscopic studies. Cancer. 1982;50:1876–81.View ArticlePubMedGoogle Scholar
- Tomashino RM. Gastric carcinosarcoma: study of a case and its possible interpretation with reference to a new histogenetic theory. Arch Ital Anat Istol Pathol. 1970;43:364–85.Google Scholar