DOI: https://doi.org/10.21203/rs.3.rs-2072110/v1
Duodenal squamous cell carcinoma is extremely uncommon among gastrointestinal malignancies. The diagnosis and treatment were less understood. We report a case of duodenal squamous cell carcinoma with liver and adrenal metastasis, who was treated with gemcitabine and S-1 and obtained a PFS of 8 months eventually. Additionally, the features and treatment of primary duodenal carcinoma reported so far were reviewed in this paper. Obviously, further case reports such as ours can generate deeper cognition, which is essential for the nature of this entity and establish management guidelines.
As a type of rare tumor, duodenal cancer accounts for 0.35% of gastrointestinal malignancy[1]. Adenocarcinoma is the most common pathological type of primary duodenal cancer, followed by neuroendocrine carcinoma. However, the primary duodenal squamous cell carcinoma is exceptional[2], which has been rarely reported so far. Here, we reported a primary duodenal squamous cell carcinoma with liver and adrenal metastasis who was administrated with gemcitabine and S-1, consequently achieved partial remission and obtained a PFS of 8 months.
A 70-year-old male patient developed abdominal pain for 1 month. A gastroscopy detection found an irregular tumor covered with ulceration, moss, and black scab at the junction of horizontal descending site of duodenum (Fig. 1). The pathological result demonstrated a poor differentiated squamous cell carcinoma (Fig. 2). After a PET-CT scan, it revealed a soft tissue mass located horizontal duodenum wall with liver and left adrenal soft tissue nodules. Multiple lymph nodes in mesenteric and paraaortic region were also detected. The above masses performed high FDG metabolism. Next-generation sequencing revealed mutation of G12V in exon 2 of KRAS gene, R273C in exon 8 of TP53 gene Take these considerations, the patient was diagnosed with primary duodenal squamous cell carcinoma with liver and adrenal metastasis and was administrated with gemcitabine (1600mg D1,D8) and S-1 (50mg bid D1-D14) in September 2019. A CT scan showed tumor partial response in January 2020. Totally, he received 6 cycles of above chemotherapy. Unfortunately, the disease progressed in March 2020 confirmed by an enhanced contrast CT scan. The patients died in May 2020 due to cancer progression with multiple organs dysfunction.
Considering anatomical and physiological characteristics of intestinum tenue, malignant tumors originating in the duodenum has low incidence. Most duodenal squamous cell carcinomas are metastatic tumors from other organs such as the esophageal, cervix or lungs[3]. Although an uncommon cancer type, we diagnosed present patient with primary squamous cell carcinoma based on adequate evidence. Firstly, endoscopic and pathological results confirmed a squamous cell carcinoma. In addition, the PET-CT detection did not found primary malignant tumors in other sites, which excluded the possibility of a metastatic duodenum tumor from distance.
After literature review, we found that primary duodenal squamous cell carcinoma was extremely rare. So far, only 22 cases have been reported. Their median age was 63.78 years-old. 10 of them (45.5%) were male. There were 8 patients (36.4%) diagnosed with ampulla tumor. Six of them were given palliative chemotherapy with a mean overall survival of 11.5 months. The most common chemotherapy agents include gemcitabine and TS-1. Fourteen patients received primary tumor resection. Their mean overall survival was 15.6 months (Table 1).
By summarizing the current and reviewed cases, we provide reference for the treatment of duodenal squamous cell carcinoma. Duodenal squamous cell carcinoma lacks relevant studies in vivo and in vitro to clarify its etiology and mechanism. Due to the small number of cases, the corresponding diagnosis and treatment guidelines have not been formed, which is worthy of further study and discussion so as to improve the clinical diagnosis and treatment level of the disease.
| Sex | Age | Initial symptoms | Pathological results | Location | Treatment options | Outcomes | Report Date | Country | |
|---|---|---|---|---|---|---|---|---|---|
| Terada T [4] | M | 75 | Vomiting, weakness | SCC | D2 | Chemotherapy and radiation | death 17months after presentation | 2009 | Japan |
| Terada T [4] | F | 58 | Abdominal pain | SCC | D2 | Chemotherapy and radiation | death from metastasis 21months later | 2009 | Japan |
| Terada T [4] | M | 54 | Abdominal pain | SCC | D2 | Surgery | unknown | 2009 | Japan |
| Friedman E [5] | M | 61 | Abdominal pain, weight loss | SCC | D3 | Surgery | alive 24months after treatment, rest of clinical course | 1986 | California |
| von Delius S [6] | F | 75 | Upper GI bleeding | SCC | D1 | unknown | unknown | 2006 | Germany |
| Pahl K S [7] | M | 65 | Epigastric pain, weakness | SCC | D3 | Surgery | death 60 months later from prostatic carcinoma | 2012 | American |
| Arita K [8] | M | 63 | Obstructive jaundice | ASC | D2 | Chemotherapy(gemcitabine 1000mg/m2 ) | death 3 months after presentation | 2013 | Japan |
| Fujita T [9] | M | 89 | refractory diabetes mellitus | SCC | D2 | Chemotherapy(TS-1 80mg qd D1-D7) | relapse 19 months after chemotherapy | 2014 | Japan |
| Bolanaki H [10] | M | 68 | Jaundice, fatigue | SCC | AmV | Surgery | death from metastasis 5 months later | 2014 | Greece |
| Graur F [11] | F | 47 | weight loss and melena | SCC | D1 | Surgery | Alive 6 months after presentation | 2014 | Romania |
| Battal M [3] | M | 39 | Epigastric pain, weakness, vomiting | SCC | D3 | Surgery | Alive 10 months after presentation | 2015 | Turkey |
| Hammami M B [1] | F | 64 | nausea, vomiting, and abdominal pain | ASC | D3 | Symptomatic treatment | death | 2017 | American |
| Mccabe P [12] | F | 68 | Bloating and epigastric discomfort with intermittent black stools | SCC | D3 | Surgery | Not mentioned | 2021 | California |
| Ueno N [13] | M | 47 | Fatigue, jaundice | ASC | AmV | Surgery | death 10months after presentation | 2002 | Japan |
| Yang S J[14] | M | 82 | Jaundice | ASC | AmV | Ampullectomy | death 14months after presentation | 2013 | Taiwan, China |
| Yang S J[14] | M | 68 | RUQ pain, jaundice | ASC | AmV | Surgery | death 7months after presentation | 2013 | Taiwan, China |
| Yang S J[14] | F | 34 | RUQ pain, jaundice | ASC | AmV | Surgery | death 10months after presentation | 2013 | Taiwan, China |
| Yang S J[14] | M | 77 | RUQ pain, jaundice | ASC | AmV | Surgery | death 6months after presentation | 2013 | Taiwan, China |
| Kshirsagar A Y [15] | M | 58 | Abdominal pain, jaundice, vomiting, anorexia | ASC | AmV | Surgery | not mentioned | 2014 | India |
| Hoshimoto S [16] | F | 81 | Asymptomatic elevation of liver enzymes | ASC | AmV | Surgery | alive 20months | 2015 | Japan |
| Diffaa A [17] | F | 60 | Epigastric pain, melena, weight loss | SCC | D3 | Palliative chemotherapy | death 1months after presentation | 2012 | Morocco |
| M | 70 | abdominal pain | SCC | D3 | Chemotherapy(gemcitabine 1600mg D1, D8 + S-1 50mg bid D1-D14) | Alive 9 months after presentation | current | China |
Ethical Approval
The case report is conducted in accordance with the Declaration of Helsinki (2008). Ethics approval has been obtained from the ethic committee of Affiliated Hospital of Nanjing University of Chinese Medicine (Number 2018NL-067-09). Written informed consent has been obtained from patient and family members.
Competing interests
There are no conflicts of interest.
Authors' contributions
Hui Yi-fan and Zheng Xia conceived the structure of article. Hui Yi-fan searched literature. Hui Yi-fan and Zheng Xia wrote the paper. Hui Yi-fan, Zheng Xia and Lu Wei reviewed and edited the manuscript.
Funding
This work was supported by the National Natural Science Foundation of China.
Availability of data and material
Supplementary material is available in the online version of this article.