Unusually aggressive type 1 gastric carcinoid

Abstract

Gastric carcinoids are rare tumors of the stomach. Gastric carcinoid type 1 is associated with chronic atrophic gastritis, and because of a low metastatic potential, is the most benign type. Death from metastatic disease has been reported in only three patients in a review including 724 cases. The present report refers to a 60-year-old man who was affected by type 2 diabetes mellitus and pernicious anemia and died from metastatic gastric carcinoid type 1. In 1998, a well-differentiated 1.2 cm gastric neuroendocrine tumor, immunoreactive for chromogranin A, with a Ki-67 index less than 2% and with infiltration to the submucosal layer was diagnosed and enucleated. In 2002, a new well-differentiated gastric endocrine tumor 6 mm in size with a Ki-67 of approximately 2% was detected, and endoscopic ultrasound confirmed it to be limited to the submucosal layer. The patient refused antrectomy and started long-acting somatostatin analog (lanreotide) in 2005 when the Ki-67 index was 7%, but he stopped the treatment after 4 months. In 2007, despite previous endoscopic stability, endoscopic ultrasound showed an infiltrating gastric lesion of 7 cm. At surgery, the disease appeared to be extended to the liver and to the peritoneum (well-differentiated endocrine carcinoma, Ki-67 40%) with both hepatic and massive peritoneal metastases. A regimen of somatostatin analog was soon restarted; however, the disease continued to spread, and the patient died 6 months later. Overall, despite their generally benign course, type 1 gastric carcinoids may have malignant potential, a finding that should be considered when planning the medical workup of these patients.

Prognostic Evaluations Tailored to Specific Gastric Neuroendocrine Neoplasms: Analysis Of 200 Cases with Extended Follow-Up

“…A second type I G3 NET (case 2), sent to us for histopathologic consultation and later published in detail by Spampatti et al [34], was found during follow-up in a 70-year-old male with an initial diagnosis of multifocal G1 type I NET disease, whose larger (1.2 cm), lympho-invasive nodule, with deep invasion of the submucosa, was successfully resected surgically (wedge resection). Several subsequent endoscopies showed recurrence of intramucosal NETs (carcinoidosis) of 0.2–0.6 cm in size and a Ki67 index of 2% (Fig.…”

“…), occasionally intervening over an otherwise indolent type I NET disease, might account for its very rare progression to more ominous, metastatic disease, as it occurred in the 2 G3 NETs (out of 123 cases in the present series, 1.6%). Interestingly, only 0.4% of 724 type I NET cases from the literature considered by Spampatti et al [34] were found to cause tumor-related death. The occurrence of G3 NETs in the stomach is now documented by our findings ([14] and the present study) and those of Lee et al [29].…”

A Case of Metastatic Gastric Neuroendocrine Tumor: Therapeutic Considerations

“…Moreover, there is the opinion that dysplastic cells are less dependent on gastrin stimulation and do not regress in response to reduced circulating gastrin levels. Indeed, a few metastatic cases with a bad prognosis have been reported in type I or II gastric NETs [11, 12]. …”

Predictive Value of Gastrin Levels for the Diagnosis of Gastric Enterochromaffin-Like Cell Hyperplasia in Patients with Hashimoto's Thyroiditis

“…However, recent prospective data have shown that gastric NEN constitute the most common gastrointestinal NEN, whereas a subset may develop more aggressive disease [10]. Although some clinical (tumor size) and histopathological (invasion and proliferation index) data can be used to identify patients at risk for an aggressive course, the background of such tumor development is ECLH [17].…”

“…Although the great majority of gastric type 1 and 2 NENs follow an indolent course, a subset may become aggressive and develop distant metastases. This has recently been emphasized in a study which showed that gastric type 1 NEN metastatic potential appears to be related to a tumor size of ≥1cm, an elevated Ki-67 labelling index and high serum gastrin levels [10]. …”