Clinicopathological features of rectal adenocarcinoma with enteroblastic differentiation / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotype, and genetic alterations of rectal adenocarcinoma with enteroblastic differentiation. Methods: Four cases of rectal adenocarcinoma with enteroblastic differentiation were collected at the Affiliated Hospital of Qingdao University, Qingdao, China (three cases) and Yantai Yeda Hospital of Shandong Province, China (one case) from January to December 2022. Their clinical features were summarized. Hematoxylin and eosin stain and immunohistochemical stain were performed, while next-generation sequencing was performed to reveal the genetic alterations of these cases. Results: All four patients were male with a median age of 65.5 years. The clinical manifestations were changes of stool characteristics, bloody stools and weight loss. All cases showed mixed morphology composed of conventional adenocarcinoma and adenocarcinoma with enteroblastic differentiation. Most of the tumors consisted of glands with tubular and cribriform features. In one case, almost all tumor cells were arranged in papillary structures. The tumor cells with enteroblastic differentiation were columnar, with relatively distinct cell boundaries and characteristic abundant clear cytoplasm, forming fetal gut-like glands. Immunohistochemically, the tumor cells were positive for SALL4 (4/4), Glypican-3 (3/4) and AFP (1/4, focally positive), while p53 stain showed mutated type in 2 cases. The next-generation sequencing revealed that 2 cases had TP53 gene mutation and 1 case had KRAS gene mutation. Conclusions: Rectal adenocarcinoma with enteroblastic differentiation is rare. It shows embryonal differentiation in morphology and immunohistochemistry, and should be distinguished from conventional colorectal adenocarcinoma.

Primary endodermal sinus tumor in the posterior cranial fossa: clinical analysis of 7 cases / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>To clarify the clinical features, therapeutic method and outcomes of the primary endodermal sinus tumors (ESTs) in the posterior cranial fossa.</p><p><b>METHODS</b>The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed. And a 4-year-old boy diagnosed with EST in our hospital was reported. The clinical manifestations, therapy, pathologic features, and prognosis of these cases were analyzed.</p><p><b>RESULTS</b>Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review, including six cases searched from the PubMed and one case from our hospital. Six patients were boy and one patient's gender was not available from the report. Ages ranged from 1 to 5 years (mean 3.14 years). The mean tumor size in our cohort was 4.4 cm. Six cases came from East Asia. Schiller-Duval bodies were found in all seven neoplasms. All tumors were positive for alpha-fetoprotein. The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy. The mean follow-up time was 24.4 months (range 5-52 months). Six tumors were totally removed, and four of them recurred. Three cases died including one whose tumor was partially removed.</p><p><b>CONCLUSIONS</b>The serum alpha-fetoprotein level is well correlated with the severity of the tumor. A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa. The prognosis of extragonadal intracranial EST is poor.</p>