Case Reports in Gastrointestinal Medicine

Case Reports in Gastrointestinal Medicine / 2019 / Article

Case Report | Open Access

Volume 2019 |Article ID 5305023 | https://doi.org/10.1155/2019/5305023

Michael Beattie, Ramy Mansour, Derek Thigpin, Carolyn Haus, "Metastatic Primary Gastric Squamous Cell Carcinoma: An Uncommon Presentation of a Rare Malignancy", Case Reports in Gastrointestinal Medicine, vol. 2019, Article ID 5305023, 4 pages, 2019. https://doi.org/10.1155/2019/5305023

Metastatic Primary Gastric Squamous Cell Carcinoma: An Uncommon Presentation of a Rare Malignancy

Academic Editor: Hideto Kawaratani
Received26 Apr 2019
Accepted09 Aug 2019
Published07 Oct 2019

Abstract

Primary gastric squamous cell carcinoma is a very rare disease. A 53-year-old male with history of hypertension, alcoholism, and nicotine abuse presented to the hospital after a syncopal episode. He complained of bloating abdominal pain, early satiety, and poor appetite. A CT of his abdomen and pelvis revealed a gastric mass with diffuse hepatic metastasis. A gastric mass was seen on upper endoscopy and biopsies revealed gastric squamous cell carcinoma. There was no involvement of the esophagus. This case should add to the limited literature and serve as a reminder that while this is a rare malignancy, it must be considered when evaluating a gastric mass.

1. Introduction

Primary gastric squamous cell carcinoma (PGSCC) accounts for less than 0.2% of all primary gastric carcinomas [1]. The presentation is nonspecific and given its rarity the etiology is not well known. Several strict diagnostic criteria have been established to make the diagnosis [25].

2. Case Report

A 53-year-old white male with hypertension, chronic alcoholism, and tobacco abuse presented to the emergency department for syncope while standing outside. Review of systems was positive for four weeks of abdominal discomfort and poor oral intake due to increased abdominal pressure. He denied any changes in weight, melena, hematochezia, hematemesis, or dysphagia.

Physical examination revealed a soft, moderately distended abdomen with mild epigastric and right upper quadrant tenderness with guarding. Bowel sounds were hypoactive. There was no supraclavicular lymphadenopathy, and the rest of the exam was unremarkable.

Initial work-up found the patient to be hyponatremic at 118 mmol/L (136–144 mmol/L), Aspartate Aminotransferase (AST) of 184 U/L (15–41 U/L), Alkaline Phosphatase 390 U/L (40–129 U/L). Gamma-Glutamyltransferase (GGT) was 1080 U/L (7–50 U/L), total bilirubin 1.7 mg/dL (0.3–1.2 mg/dL) with direct bilirubin of 0.9 mg/dL (0.1–0.5 mg/dL). Alanine Aminotransferase (ALT) was within normal limits at 53 U/L (17–63 U/L). Complete blood count (CBC) revealed a leukocytosis of 15.3 K/cmm (4.0–11.0 K/cmm), Hemoglobin 10.9 g/dL (12–17.3 g/dL), Hematocrit 32.3% (36–53%), and a platelet count of 384 K/cmm (140–440 K/cmm). CT of the abdomen and pelvis with IV contrast revealed a 6.1 cm mass lesion in the lesser curvature of the stomach contiguous with the gastrohepatic ligament, as well as extensive hepatic metastatic lesions, with the largest measuring 9.5 cm (Figure 1(a)). Serum CA 19-9 was 54.2 U/mL (0–35.5 U/ml) and Carcinoembryonic Antigen (CEA) 1.6 ng/mL (0–3.0 ng/mL).

Upper endoscopy revealed a large necrotic mass of approximately 6 cm in the lesser curvature with ulceration in the gastric cardia without extension into the esophagus (Figure 2). Biopsies were taken and pathology revealed sections of gastric mucosa with nests of infiltrative neoplastic cells with spindled, vesicular nuclei, irregular nuclear contours, and variably prominent nucleoli. Brisk mitotic activity was noted with focal necrosis, as well as a small focus of keratinization (Figure 3(a)). Immunohistochemical (IHC) stains showed neoplastic cells positive for pancytokeratin including CK7 and CK20, as well as p63 and p40 (Figure 3(b)). Neuroendocrine markers synaptophysin, chromogranin, and CD56 were negative. Pathological diagnosis was made for invasive poorly differentiated squamous cell carcinoma with rare keratinization (Figure 3(a)).

Initially, gastric primary was thought to be less likely initially due to the evidence of keratinization; however, outpatient PET scan showed a 6.5 cm hyper metabolic ulcerative gastric mass and no additional source for a primary tumor. The liver metastasis was appreciated once again, as well as hypermetabolic metastatic adenopathy in the abdomen (Figure 1(b)). A liver biopsy was offered; however, the patient declined.

At this time, he has completed six rounds of systemic chemotherapy including IV Carboplatin and IV Paclitaxel for Stage IV PGSCC with diffuse liver metastasis and gastric lymphadenopathy. He has developed chemotherapy induced thrombocytopenia with platelets of 22,600 K/cmm, and neuropathy treated with gabapentin. Report of the PET scan after 6 months of therapy revealed an enlarging gastric mass, increased adenopathy, and worsening hepatic metastasis.

3. Discussion

PGSCC is considerably rare, with incidence reported at 0.2% of all gastric carcinomas [16].

Adenocarcinoma accounts for 95% of all gastric malignancies. PGSCC occurs more commonly in middle-aged men [6].

Because many gastric masses, which are initially thought to be PGSCC, ultimately turn out to be of a different cell origin (i.e., gastric adenosquamous cell carcinomas), or from a different location (i.e., extending from the esophagus), criteria developed by Parks et al. exclude any such cases: Originates in the gastric cardia, extends into the esophageal mucosa, or the presence of squamous cell carcinoma from any other site must be ruled out [2, 3]. Prior to the development of Parks et al. criteria, Boswell and Helwig developed histopathologic criteria, where one of the following must be met: (1) Keratinized Cell masses forming keratin pearls, (2) Mosaic cell pattern, (3) Intercellular bridges or (4) Presence of Keratin or Cytokeratin [4].

In respect to these diagnostic criteria, our patient’s findings of gastric mass originating in the lesser curvature without extension into the esophagus (Figure 2), PET imaging ruling out another primary site, and histopathologic findings support the diagnosis of PGSCC.

P63 tumor marker has been shown to be positive in 81% of squamous cell carcinomas [7] (Figure 3(a)). Moreover, P40 positivity has been shown to improve diagnostic yield for squamous cell carcinoma in poorly differentiated carcinomas, as well as differentiate squamous cell carcinoma from adenocarcinoma [810](Figure 3(b)). We provide a sample image of normal appearing gastric mucosa with typical mucus producing glands as a means of comparison to our pathology images of invasive squamous epithelium with high mitotic activity [11] (Figure 4).

The presentation is variable and non-specific. Symptoms include abdominal discomfort, dysphagia, melena, hematochezia, and weight loss. SIADH has not been reported in a PGSCC previously; however, it has been seen in other sites of squamous cell carcinomas [12]. A hyponatremia workup was performed including Urine Osmolality (353 mOsm/kg) and Urine Sodium (<20 mmol/L). These findings along with the patient’s clinical picture suggested a multifactorial etiology including hypovolemia, poor solute intake, as well as excess ADH secretion from gastric tumor.

Given its rarity, the etiology is still not well understood. Several theories exist: gastric mucosal stem cells, ectopic squamous epithelium in the gastric mucosa, squamous differentiation of preexisting adenocarcinoma, origin from endothelium of gastric vessels, and squamous metaplasia of glandular epithelium induced by noxious agents [13]. Given our patient’s significant history of tobacco and alcohol use, he was at a greater risk of developing squamous cell carcinoma [14]. Along with smoking history, white race and male gender did preclude a higher risk of gastric cancer [15].

When disease is local, radical resection is the therapy of choice. The benefit of adjuvant chemotherapy remains uncertain [16]. Due to the extent of diffuse metastases, chemotherapy was initiated in our patient. The prognosis tends to be very poor across all stages in comparison to gastric adenocarcinoma with a median overall survival of 8 months vs. 19 months [1]. This is most likely due to the commonly involved lymphovascular system, propensity for poorly differentiated tumor grade and late disease state at the time of diagnosis—of note, each of these were factors in our case [17].

While PGSCC is a rare disease, it is vital to keep this in mind when evaluating gastric pathology. With surgical resection being the recommended therapeutic approach, prompt identification with attention to the strict diagnostic criteria is essential.

Informed consent was obtained from the patient for the publication of this case report and any accompanying images.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

Authors’ Contributions

Michael Beattie: data acquisition/analysis, interpretation, drafting of the work, agreement for accountability of the work authorship; Ramy Mansour: conception/design, data acquisition/analysis, revision, agreement for accountability of the work; Derek Thigpin: data interpretation, critical revision, final approval, agreement for accountability of the work; Carolyn Haus: data interpretation, critical revision, agreement for accountability of the work.

Acknowledgments

A special thank you to Dr. Jason Zell for his contributions to this case report.

References

  1. C. Dong, M. Jiang, Y. Tan et al., “The clinicopathological features and prognostic factors of gastric squamous cell carcinoma,” Medicine, vol. 95, no. 34, p. e4720, 2016. View at: Publisher Site | Google Scholar
  2. R. E. Parks, “Squamous neoplasms of the stomach,” The American Journal of Roentgenology, Radium Therapy, and Nuclear Medicine, vol. 101, no. 2, pp. 447–449, 1967. View at: Publisher Site | Google Scholar
  3. J. A. González-Sánchez, R. Vitón, E. Collantes, and J. A. Rodríguez-Montes, “Primary squamous cell carcinoma of the stomach,” Clinical Medical Insights: Oncology, vol. 11, p. 117955491668607, 2017. View at: Publisher Site | Google Scholar
  4. J. T. Boswell and E. B. Helwig, “Squamous cell carcinoma and adenoacanthoma of the stomach. A clinicopathologic study,” Cancer, vol. 18, no. 2, pp. 181–192, 1965. View at: Publisher Site | Google Scholar
  5. Japanese Gastric Cancer Association, “Japanese classification of gastric carcinoma: 3rd English edition,” Gastric Cancer, vol. 14, no. 2, pp. 101–112, 2011. View at: Publisher Site | Google Scholar
  6. D. Callacondo-Riva, A. Ganoza-Salas, W. Anicama-Lima, A. Quispe-Mauricio, and T. A. Longacre, “Primary squamous cell carcinoma of the stomach with paraneoplastic leukocytosis: a case report and review of literature,” Human Pathology, vol. 40, no. 10, pp. 1494–1498, 2009. View at: Publisher Site | Google Scholar
  7. O. B. Gülçiçek, A. Solmaz, K. Özdoğan et al., “Primary squamous cell carcinoma of the stomach,” Turkish Journal of Surgery, vol. 32, no. 3, pp. 221–223, 2016. View at: Publisher Site | Google Scholar
  8. O. Kaufmann, E. Fietze, J. Mengs, and M. Dietel, “Value of p63 and cytokeratin 5/6 as immunohistochemical markers for the differential diagnosis of poorly differentiated and undifferentiated carcinomas,” American Journal of Clinical Pathology, vol. 116, no. 6, pp. 823–830, 2001. View at: Publisher Site | Google Scholar
  9. A. K. Alomari, E. J. Glusac, and J. M. McNiff, “p40 is a more specific marker than p63 for cutaneous poorly differentiated squamous cell carcinoma,” Journal of Cutaneous Pathology, vol. 41, no. 11, pp. 839–845, 2014. View at: Publisher Site | Google Scholar
  10. J. Ha and M. Yang, “The use of p40 helps to differentiate squamous cell carcinoma from adenocarcinoma,” American Journal of Clinical Pathology, vol. 138, no. suppl 2, p. A150, 2012. View at: Publisher Site | Google Scholar
  11. D. Ortaç, M. Cemek, T. Karaca et al., “In vivo anti-ulcerogenic effect of okra (Abelmoschus esculentus) on ethanol-induced acute gastric mucosal lesions,” Pharmaceutical Biology, vol. 56, no. 1, pp. 165–175, 2017. View at: Publisher Site | Google Scholar
  12. M. Yoo, E. O. Bediako, and O. Akca, “Syndrome of inappropriate antidiuretic hormone (SIADH) secretion caused by squamous cell carcinoma of the nasopharynx: a case report,” Clinical and Experimental Otorhinolaryngology, vol. 1, no. 2, pp. 110–112, 2008. View at: Publisher Site | Google Scholar
  13. W. von Waagner, Z. Wang, and A. I. Picon, “Rare case of a primary squamous cell carcinoma of the stomach presenting as a submucosal mass,” Case Reports in Surgery, vol. 2015, Article ID 482342, 5 pages, 2015. View at: Publisher Site | Google Scholar
  14. T. L. Vaughan, S. Davis, A. Kristal, and D. B. Thomas, “Obesity, alcohol, and tobacco as risk factors for cancers of the esophagus and gastric cardia: adenocarcinoma versus squamous cell carcinoma,” Cancer Epidemiology, Biomarkers & Prevention, vol. 4, no. 2, pp. 85–92, 1995. View at: Google Scholar
  15. P. Karimi, F. Islami, S. Anandasabapathy, N. D. Freedman, and F. Kamangar, “Gastric cancer: descriptive epidemiology, risk factors, screening, and prevention,” Cancer Epidemiology Biomarkers & Prevention, vol. 23, no. 5, pp. 700–713, 2014. View at: Publisher Site | Google Scholar
  16. Y. Chen, H. Zhu, F. Xu et al., “Clinicopathological characteristics, treatment, and prognosis of 21 patients with primary gastric squamous cell carcinoma,” Gastroenterology Research and Practice, vol. 2016, Article ID 3062547, 6 pages, 2016. View at: Publisher Site | Google Scholar
  17. D. C. Bonnheim, O. K. Sarac, and W. Fett, “Primary squamous cell carcinoma of the stomach,” American Journal of Gastroenterology, vol. 80, no. 2, pp. 91–94, 1985. View at: Google Scholar

Copyright © 2019 Michael Beattie et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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