Effectiveness of Anti-PD-1 Antibody Monotherapy for the Primary Malignant Melanoma of the Esophagus： A Case Report

Primary malignant melanoma of the esophagus（PMME）is extraordinarily rare with a high degree of malignancy and poor prognosis, and a standard therapy remains to be established. The anti-PD-1 antibody nivolumab is a promising agent for various cancers. To our knowledge, this is the first case report of PMME where a complete response was achieved using nivolumab. We report an 80-year-old woman who was diagnosed with PMME with bone metastasis and lymph node metastases. Although dacarbazine combined chemotherapy was performed and continued for six cycles, the primary tumor progressed and liver metastases appeared. The patient then received nivolumab monotherapy. After three cycles, nivolumab monotherapy for PMME resulted in a complete response as shown by positron emission tomography, computed tomography, and esophagogastroduodenoscopy. In our case, nivolumab exerted a curative effect on PMME, thus suggesting that nivolumab can be effective in the treatment of this rare disease

Esophageal granular cell tumor successfully resected by endoscopic submucosal dissection

Granular cell tumors of the esophagus are rare neoplasms and their diagnosis is mainly based on histopathologic examination of endoscopic biopsies. With the development of endoscopic techniques, there has been a marked increase in local treatment modalities for early esophageal neoplasms. In this case report, we describe the removal of a granular cell tumor by the endoscopic submucosal dissection technique, and briefly discuss the literature on clinicopathologic aspects and management of granular cell tumors

Selection of Postoperative Systemic Chemotherapy for Advanced Gastric Cancer Patients with Hepatic Metastasis Using the Histoculture Drug Response Assay

The usefulness of histoculture drug response assay (HDRA) before the start of antitumor chemotherapy was explored to predict the antitumor response of chemotherapy for progressive gastric cancer patients with hepatic metastasis. Cancer tissue was collected from 6 advanced gastric cancer patients with hepatic metastasis, to assess whether the tumor is sensitive to chemotherapeutic agents of 5-fluorouracil (5-FU), mitomycin C (MMC), docetaxel hydrate (TXT), and cisplatin (CDDP) by the HDRA method. The size of hepatic tumor was measured by CT scan 3 and 6 months after the start of the chemotherapy and examined the relationship between the selected chemotherapy and antitumor response. Of 6 patients investigated in the present study, 3 patients received monotherapy with TS-1[○!R], which was found to be effective in 2 patients (66.7%). In the patient who received MMC monotherapy, the HDRA showed the cancer tissue to be less sensitive to any of antitumor agents tested, resultantly indicating no antitumor response. In the remaining 2 patients with combination therapy with TS-1[○!R] and MMC, the selected chemotherapeutic agents showed anti-tumor effect. The HDRA is useful to predict the antitumor response of postoperative adjunctive chemotherapy for advanced gastric cancer patients with hepatic metastasis, and plays an important role in selection of antitumor agents

Waardenburg Syndrome with Isolated Deficiency of Myenteric Ganglion Cells at the Sigmoid Colon and Rectum

Waardenburg syndrome (WS) has the characteristic clinical features caused by the embryologic abnormality of neural crest cells. WS patients sometimes suffer from functional intestinal obstruction. When it is Hirschsprung disease (HD), the WS is diagnosed as type 4 WS. We report a case of WS which did not have myenteric ganglion cells in the sigmoid colon and rectum. Whether to diagnosis this case as type 1 or 4 WS is controversial. Moreover, this is the third report which has peristalsis failure caused by abnormal myenteric plexus. In all three cases, the eosinophils had aggregated in the myenteric layer of the transition zone. During embryonic life, enteric ganglion cells migrate to the myenteric layer from the proximal to the distal side sequentially and, subsequently, to the submucosal layer through the circular muscle. Therefore, we hypothesize that myenteric ganglion cells that had already migrated were eliminated by an eosinophil-mediated mechanism in these three cases. We believe this report may be helpful to elucidate the pathogenesis of some types of HD