Primary sarcomatoid carcinoma of the small intestine: very rare and aggressive tumour

Andrawes, Peter; Shariff, Masood A.; Chang, Qing; Grinberg, Roman

doi:10.1136/bcr-2016-217895

Cited by 8 publications

(24 citation statements)

References 18 publications

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“…In our review of the literature, 9 cases of primary SISC have been reported in the last decade [6][7][8][9][10][11][12][13][14], and these 10 cases (including the present case) are shown in Table 1. Abdominal pain was noted in 7 cases [6-9, 13, 14], and 3 cases, including the present case, were complicated by peritonitis due to tumor perforation [6,9].…”

Section: Discussionmentioning

confidence: 97%

Primary Small Intestinal Sarcomatoid Carcinoma: Report of a Rare Case and Literature Review

et al. 2021

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Sarcomatoid carcinoma (SC) is a rare malignant tumor with properties of both epithelial and mesenchymal carcinomas. SC has been reported in various organs, but the number of reports for each type is small. Small intestinal tumors make up about 3–6% of gastrointestinal malignancies. Discovering them in the early stage is rare and difficult, with anemia and/or abdominal pain as the major symptoms of small intestinal tumors. Primary small intestinal SC (SISC) is rare among small intestinal tumors, and currently very few cases have been reported in the literature. Previous studies have reported that neither chemotherapy nor radiotherapy improves the overall survival rate of patients with SISC, and the prognosis is extremely poor. Currently, surgical resection remains the only optimal therapeutic approach for SISC. Here, we present the case of a 90-year-old woman who had acute peritonitis due to perforation of a small intestinal tumor. She underwent emergency exploratory laparotomy and partial resection of the small intestine, including the tumor. The tumor was pathologically identified as a primary SISC with mesenteric lymph node metastasis. Subsequently, she had recurrence in the intra-abdominal area and lymph node metastasis anterior to the inferior vena cava and died 15 months after surgery without any additional treatment.

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Section: Discussionmentioning

confidence: 97%

Primary Small Intestinal Sarcomatoid Carcinoma: Report of a Rare Case and Literature Review

et al. 2021

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“…Primary small intestinal carcinoma is rare with an incidence of 0.5-0.8 per 100,000 population per year [1,2]. Sarcomatoid carcinoma (SCA) of the small intestine is an extremely rare, aggressive variant of small intestinal malignancy accompanying a poor prognosis [1][2][3]. The tumor primarily affects middle-aged and elderly patients with a mean age of 57 years at the time of presentation [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…Sarcomatoid carcinoma (SCA) of the small intestine is an extremely rare, aggressive variant of small intestinal malignancy accompanying a poor prognosis [1][2][3]. The tumor primarily affects middle-aged and elderly patients with a mean age of 57 years at the time of presentation [3,4]. The cancer cells of this subtype display a mimicry of mesenchymal spindled cells.…”

Section: Introductionmentioning

confidence: 99%

Synchronous Jejunal Sarcomatoid Carcinoma and Incidentally Associated Localized Peritoneal Malignant Mesothelioma

Tachibana¹,

Nozawa²,

Kamimura³

et al. 2022

Cureus

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Sarcomatoid carcinoma (SCA) of the small bowel is a rare aggressive variant of small intestinal cancer accompanying a poor prognosis. The tumor primarily affects middle-aged and elderly patients. We report herein a 67-year-old Japanese male who manifested anemia. He had a history of asbestos exposure 30 years earlier. An abdominal computed tomography (CT) scan showed a 6.5-cm aneurysmal, dilated mass of the small intestine. Capsule endoscopy revealed a large circumferential hemorrhagic ulcerative lesion in the jejunum. Biopsy indicated sarcomatoid carcinoma, and partial resection of the small bowel and adjacent transverse colon and omentum was performed. In addition to the T3N0M0 jejunal giant sarcomatoid carcinoma (SCA), a 3-mm small localized peritoneal (omental) malignant mesothelioma (LMM) was also incidentally included. Synchronous presentation of small intestinal and mesothelial malignancies is extremely rare, and the avoidance of incorrect clinical staging is critically important. Surgical resection is still considered the best first-line therapy, because of a poor response to chemotherapy and radiotherapy. Dual-color fluorescent in situ hybridization (FISH) for p16/CDKN2A and chromosome 9 indicated homologous deletion of p16/CDKN2A in SCA and a normal pattern in LMM. Methylthioadenosine phosphorylase (MTAP) was negative in SCA but positive in LMM. Both tumors consistently expressed BRCA1-associated protein 1 (BAP1). Tumor necrosis factor receptor-associated factor 7 (TRAF7) was suppressed, and neural cell adhesion molecule L1 precursor (NCAML1/L1CAM) was agitated in both tumors. Diffuse and strong expression of programmed death-ligand 1 (PD-L1) and the association of tumorinfiltrating lymphocytes in SCA may indicate a potential for PD-L1-targeted immunotherapy for treating this type of aggressive cancer. PD-L1 was focally expressed in LMM. The postoperative course was uneventful for two years.

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“…1 Sarcomatoid carcinoma of the small bowel is even more uncommon, with fewer than 35 cases reported to date and an incidence rate of 0.5-0.8 per 100,000 population per year. 2,3 This disease usually manifests in the sixth decade of life and carries a poor prognosis. 1,2 Clinically, the patient may present with abdominal pain, anaemia, gastrointestinal bleeding, intestinal obstruction, vomiting and weight loss 3,4 as seen in the case presented.…”

mentioning

confidence: 99%

“…2,3 This disease usually manifests in the sixth decade of life and carries a poor prognosis. 1,2 Clinically, the patient may present with abdominal pain, anaemia, gastrointestinal bleeding, intestinal obstruction, vomiting and weight loss 3,4 as seen in the case presented. Histological identification of mixed carcinomatous and sarcomatous components with a panel of immunohistochemical stains are required to arrive at the diagnosis.…”

mentioning

confidence: 99%

Sarcomatoid Carcinoma of Small Intestine: A Case

Rosli

Shukor

2021

Chonnam Med J

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Primary sarcomatoid carcinoma of the small intestine: very rare and aggressive tumour

Cited by 8 publications

References 18 publications

Primary Small Intestinal Sarcomatoid Carcinoma: Report of a Rare Case and Literature Review

Primary Small Intestinal Sarcomatoid Carcinoma: Report of a Rare Case and Literature Review

Synchronous Jejunal Sarcomatoid Carcinoma and Incidentally Associated Localized Peritoneal Malignant Mesothelioma

Sarcomatoid Carcinoma of Small Intestine: A Case

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